Was this in the plan????

It could be you

2012-01-06T22:58:00.001Z

So how did I come to this point in my life where I made a shift from wage slave to benefit scrounger?

Up until November 2004 I had a full time marketing career, I had degree and a master's under my belt together with professional qualifications and memberships. I had travelled the world with work, managed multi-million pound budgets and had either worked for or manged the accounts of some heavy weight, blue chip companies. The role I was in by November 2004 was supposed to be my "slowing down, being more close to home" job - I was head of Marketing and Student Support for a Further Education College. The plan had been to take some maternity leave then return on a part-time basis, in time for the college's busiest time of the year, Student Enrolment.

I loved working, I loved the sense of identity and purpose it gave me, I loved being part of a team and being appreciated for a job well done. I enjoyed dressing in "corporate" clothes, receiving my pay slip at the end of the month (even though a huge chunk of it always seemed to go on childcare!), putting my education and training into practice but most importantly it was great to leave it all behind at the end of the day and go home (well, when I wasn't travelling). I was not ashamed to admit that going to work was a bit of a cop out, I was not cut out to be a stay at home mum.

I had a stereotypical view of people who claimed benefits - I wanted to show my children that you could have it all, career and family and that an education was worth pursuing as it led to wonderful rewards. I knew nothing of the world of disability, illness, hospices - except for once a year when Comic Relief and Children in Need was on the TV, at which point I would get my credit card out a pledge some money and think nothing more of it.

So November 2004, when I was 29 weeks pregnant with our fourth (and much wanted) child and second daughter, I had no idea how my world was going to change so dramatically and so permanently. Being my fourth child I had a good idea when things did not feel right in pregnancy, I was being monitored for polyhydroamnios and I was now looking like a woman who was full term with twins, I was huge. I wandered into my ante-natal clinic which happened to be on the way home from work and that's when my life changed. I was admitted straight away. given steroids to strengthen the baby's lungs, and had several amnioreduction procedures to try and minimise the risk of pre-term labour. As it was Daisy was delivered by c-section (a normal labour was deemed too risky as all three previous ones had been very long - and very natural!) at 33 weeks, three days before Christmas. I spent Christmas in hospital, Daisy was in intensive care and poor Andy was at home 7,5 & 2 year old children. I remember shuffling into the neonatal unit on Boxing day, clutching my meagre bottle of expressed milk to see the staff gathered around a TV watching coverage of the asian tsunami - and I wondered what had happened to the world, to my world...

Needless to say, despite optimistic plans and strategies I never did return to work and with a heavy heart I handed in my notice so that the college could get on with recruiting a replacement. The world as I knew it had changed; after two months in neonates, Daisy did come home for a few (very fraught) weeks before being rushed up to Great Ormond Street Hospital at four months old for investigations for a possible tumour. She ended up staying there for three months during which time she visited intensive care with respiratory failure, had the first four of many general anaesthetics, was diagnosed with heart problems, a severe visual impairment and failure to thrive. They did not find a tumour and we know know in hindsight the agony she was in and the symptoms she displayed were down to rare nerve disorder, probably completely separate to her overall diagnosis of Costello Syndrome, in her gastro-intestinal system which meant that every drop of milk we were trying to force into her caused her huge pain.

We soldiered on for a few years, we embraced the diagnosis of Costello Syndrome, we even manage to attend the syndrome conference in Portland, Oregon. I thought of ways I could get back to work in between the hospital stays, when we were lulled into a false sense of security as we managed a few months out of hospital and on a reasonably even keel. We struggled financially on one income, but always in the back of my mind I thought this was a temporary situation and I thought that Daisy, like the other children with Costello Syndrome we had met would eventually stabilise enough for me to freelance occasionally or go back to college to train as a teacher. I even started an application for a part time teacher training course..

Again, we had a turning point in our life with Daisy - September 2008, when after a difficult summer as she struggled with pain and weight gain and only a week into starting school (and Theo just starting high school) she became unwell and we had to rush her to A&E for what we thought would be another few days of vomiting, fluids and then she would pick up. But she did not pick up, she got worse and worse and eventually on being transferred to GOS and having upper and lower scopes she was found to have severe and agressive inflammatory bowel disease, we know now probably caused by the severed dysmotility and repeated attempts to feed her, and in November 2008 she started TPN and left hospital nearly 12 months after first being taken to A&E.

With TPN and Daisy's subsequent deterioration our lives, already changed, changed immeasurably. There was not turning back, no hope for recovery, no part time work or college course. During one of our long hospital stays Andy was made redundant and the unthinkable happened - we were both out of work, with four children, one of whom was needing 24 hour care. Tax credits to supplement the carers allowance and Disabilyt Living Allowance were an essential part of our lives.

Andy and I would have driven eachother mad being at home together all day, so now he runs a very successful consultancy business. This allows him some flexibility to fit around Daisy's medical needs, but most of the time I go to hospital appointments, clinics, procedures on my own as he has to work. My full time job is Daisy and the three other children. And it seems to me that with each year my job description keeps expanding and yet I receive minimal remuneration for it - I am now qualified to access a central line (many nurses will not/are not able to do this), I can catheterise a mitrofanoff stoma, change a stoma bag, replace a jejenostomy and gastrostomy button, I am fairly proficient in makaton sign language, I can administer intravenous antibiotics, make judgements on types of pain relief analgesia required. And when I am not doing all those things I am checking stock levels of all the equipment and ancilliaries needed to run our mini hospital room, washing copious amounts of bedding after nights Daisy has spent vomiting or with a leaking stoma bag, cleaning, tidying, lugging heavy wheelchairs into the back of a car followed by lifting a 20kg child and various bags and attachments! I did not anticipate the new languages I would have to learn - carefully choosing my words when I make requests of social services versus healthcare because using a word such as "respite" with the wrong person may just come back with the answer "that's someone elses budget". I have had to become au fait with the world of statements and special education, and not just for Daisy - just incase I become too complacement I have another child with additional needs that can take me away from the day job of just being a mum - Asperger's and everything that comes with that syndrome has taken over our lives also.

I am a different person to the career woman I was 7 years ago, I have been to the edge I guess and seen another world and it really does put things into perspective. I value what I do immensely, this is the most important, rewarding, bone-achingly tiring job I have ever had to do. It's manual labour, intellectually challenging, goalpost moving, unrelenting, 24/7 slog. This is not just about being a mum plus, this is about being a nurse, an advocate, a housekeeper, politician and diplomat......I get thanks and appreciation from my family and friends, I know I am contributing to society, I am an essential component in the social and health care system, without me doing what I do the cost to the taxpayer would be huge as Daisy would need full time, funded specialist medical care in a residential setting.

My reward is to see my children achieve and grow, I truly believe that the reason Daisy continues to live to fight another day is down to the skills and effort Andy and I have put in to make that happen. But does our society value what I do? I am dependent on tax credits and carers allowance, all £55.55 per week of it. Andy works, sometimes I envy him that opportunity to escape into the corporate world and switch off. I would love to put my new skills to use, now I google part time nursing degrees and daydream about becoming a Gastrointestinal Nurse Specialist......

This is why I write this blog, so that hopefully by sharing a bit of our lives people will understand our world and look beyond theirs. We all walk such a fine line, we are all just a faulty gene, a birth defect, an accident or a chance happening away from entering a world like mine.

It's still about one day at a time

2011-12-31T17:29:00.000Z

So, it's New Year's Eve and already the stream of texts and emails are coming through from friends and family over the world, full of good wishes for 2012, hope that things will improve....as you all know, I am always optimisitc, glass half full but also profoundly realistic. Andy and I chatted about our hopes for 2012 earlier today and we both agreed that actually, our philosophy of one day at a time still holds true today, new year's eve, it's just a date, a number, our hope remains for stability, and time...whether it's tomorrow, next week, month or year.....

As it is, Daisy is in hospital. Yesterday she woke up in severe pain and despite the overwhelming cocktail of drugs we have at our disposal at home we could not get ontop of it and more worryingly her breathing was becoming laboured when she did fall asleep. So it was time to visit our local hospital, and such a relief to have some great registrars on duty who made our visit less stressful. Daisy needed intranous morphine and fluid resuscitation and today is tired but better. In fact we have brought her out on home leave in the hope that we can manage her pain at home but will return if it gets worse. Hopefully if she can get through the night without an escalation in her pain management requirements then she can be discharged. It is really worrying how bad her pain became yesterday and how quickly and we just hope that this is not the shape of things to come. We are due to go to Great Ormond Street in a couple of days so maybe they will have an idea why she is continuing to get such bad pain or at least we can tweak her pain management plan to avoid it getting to the point where she needs to be in hospital.

As it is New Year's Eve I have been reflecting on 2011 and what it has thrown at us. It has been the year of surgery and bladder issues for Daisy and facing facts and some pretty tough conversations for us, her parents. Daisy now ends the year with an ileostomy stoma which continues to have high output and episodes where is completely shuts down (goes into pseudo-obstruction), she has a surgically formed jejenostomy through which she receives her medication, her gastrostomy is now permanently on free drainage with a bag attached as her stomach does not empty and her reflux and reverse motility is worsening, she has a mitrofanoff stoma and we are now able to change her catheters daily at home through this, and of course she has her hickman line, a central line which goes into a main vein into her heart through which she receives her nutrition (TPN). She is on permanent antibiotics and anti-fungal medications because of the bugs which are colonised in her body, these treatments keep the bugs dampened down to a level where they do not overwhelm her causing sepsis, but we live with this fear permanently, especially as she becomes resistant to antiobitics as a result of long term usage. And of course there is the cocktail of pain meds she is on - enough to fell an elephant but sometimes only enough to take the edge off the pain for Daisy. From a medical point of view things have deteriorated hugely - her TPN requirements are going up not down and now we are faced with the situation where there is no more surgery or treatment that is going to make things better, just symptom management and our hope for time and stability.

But Daisy's cognitive development is outstanding, she signs constantly and when she is well and confident this is backed up with lots of talking. She is completely in love with her siblings and if you mention any of their names she signs brother or sister and then signs "love". She is also obsessed with telling people if they are boys or girls having worked on this in school which is always amusing when strangers, such as the man who came to read the electricity meters, come to the house and they are greeted with shouts of "boy!" and the associated sign!

I have to keep focussing on the positives - she is still with us, this has been a year when we have said goodbye to others close to our family, she is still fighting, our determination to keep her at home is unwavering. We just cannot dwell on what 2012 will bring, we could not have predicted what 2011 brought our family and I just have to be grateful that we have ended the year as we started it, as a family of six - we have more battlescars, we have increase our medical knowledge, we have come to embrace the rollercoaster of Asperger syndrome, we have ridden the storms, plummetted the depths of our emotions and yet we still seem to be standing. The only thing I ask for next year is time....more time together, time away from hospital, time for me, time to be a couple, time to be a family. But I am aware that we have already been so blessed, with seven years with Daisy, and thinking of this is what reminds me that the cup will always be half full, against all the odds, she has given us seven years of joy and love....I think I will leave it at that and continue to take things one day at a time.....

Thank you to all my wonderful friends who continue to give me such strength and support, so many of you I have never met, some of you have only come into my life since having Daisy, but to know that all over the world there are people who are thinking of us and rooting for us really keeps me going....I feel the love, thank you.

Happy Days!

2011-12-24T09:07:00.000Z

Despite everything going on Daisy is determined to live life to the full - here is a selection of pictures of Daisy having fun on her birthday and in the run up to Christmas. We are off to our hospice ShootingStarChase later today after a quick visit to Hampton Court Ice Skating Rink. Daisy continues to defy the odds and she is currently on intravenous antibiotics as well as her usual cocktail of drugs but nothing is going to stop her having fun this Christmas!

Happy Christmas to all of our friends and supporters! xxx

Daisy was an Angel in the School Nativity

With big brother Theo at the Shooting Star Chase Hospice Christmas Party

Spending her Birthday Money at the Oxford Street Disney Store!

Meeting Santa at the Wetlands Centre in Barnes

Birthday Party with Godparents & friends at Bloomsbury Bowling!

A new phase begins

2011-12-17T01:22:00.003Z

We have entered a new phase of our lives as a family with Daisy, the phase where there is nothing more that can be done, where the only active treatment now is the maintentance of her nutrition and management of her pain, no more tests that will change things, no more operations, there will be no transplanting of diseased organs for new ones (because the increased cancer risk of Costello Syndrome rules out the immunosuppressant therapy a transplant requires), no more long hospital stays, no more invasive procedures unless she is in a life threatening situation. From now on all care will be managed at home unless Daisy's life is at risk.........and far from being depressing, this feels fantastic!

Now we have our daughter back and our family together. Now we have accepted that the doctors have reached the end of the road we can concentrate on what is important - time together as a family and for Daisy, time to be a little girl again.

Daisy has enjoyed the run up to Christmas in school, playing an angel in her nativity and stealing the show when she told the rest of the cast to be quiet because Baby Jesus was sleeping. She has had some fun times at our hospice and we have paid a visit to Santa to tell him what is on her Christmas list.

The price we pay is that our workload has gone through the roof and we still live with the worry of bugs and germs and infections and the responsibility we now have to decide whether or not to manage and treat her at home or to take her into hospital. Years of living with Daisy have given us the instincts which tell us when to act, such as yesterday when we happened to be at Great Ormond Street for the annual Christmas Party. My instinct told me that Daisy's hickman line was not quite right, I asked one of the nurse specialists to check it and sure enough, it would have probably broken, most likely on her birthday or Christmas, which would have meant a hospital stay until it could be repaired. Fortunately there was time to repair it yesterday, and while I hate Daisy having a repaired line as this now means it has a point of weakness, it is better than a broken line.

The other thing we are having to accept is that Daisy will never be free of the bugs that colonise her - she normally has three main ones ; candida, pseudomonas and ecoli and now our battle is to keep these at a level where she does not become septic with them but in doing so we also have to ensure that she does not become resistant to her antibiotics. She is increasingly resistant to an ever increasing range of antibiotics and this worries all the professonals who care for her. She is currently on a month's course of intravenous antibiotics and has been on treatment doses of antifungals for weeks. We will review the situation in a month and see whether she can switch to prophylactic antibiotics or she will need to switch to another drug.

So ontop of TPN, bladder washouts, dressing changes, regular meds, enteral feeds and all the other stuff that comes with Daisy we now do regular intravenous medication. This is what is needed to keep her at home, so this is what we do, with the assistance of occasional stays at the hospice when we regroup and get our breath back. Daisy is so complicated and her care is now so specialised that it just makes more sense that we nurse her at home. We now have nursing support four nights a week, although I still have to do the TPN and IVs but at least they can administer pain meds while I catch up on sleep.

Being at home for an extended period has meant that at long last we have been able to function as a complete family again. Daisy's bedroom is now downstairs and has become the heart of our home, often she needs to just lie in her bed as her stamina is not great, and the children wander in and out, chat to her, read her stories, watch dvds with her.

Andy and I have been able to be proper, full time, hands on parents with all the children, attending meetings at school, doing the school run, being there when they all come home, getting them back on track with their school work. We were bursting with pride when Theo and Jules turned in the most brilliant performances in their end of term plays at Lantern Arts Centre recently and then the following week Theo just went one better by coming second in the regional finals of a Public Speaking Competition, representing his school. (Here's the link to his speech http://www.youtube.com/watch?v=d17tHznWWaU)

Over and over I have stressed that all my children are special, just that Daisy takes a little bit more of our time. I never think of myself as having a disabled child and three able children, they are all able and unique in their own ways. In fact, if we are going for labels then I very rarelly blog about the fact that my eldest son, Theo has a form of high functioning autism called Asperger Syndrome. Sometimes (actually, most of the time), dealing with the challenges of Asperger's is harder than dealing with Daisy's medical needs. Her needs are physically challenging, Theo's are emotionally challenging - for a few weeks after we brought Daisy home following the last surgery he could not settle, he was so anxious she would be taken back into hospital again. Children with Aspergers need order, routine and predictability, something that can be very hard to achieve in our house so often having held it together all week Theo will have an all consuming migraine which will wipe him out for 24 ours. We have also noticed how Jules and Xanthe have been forced to become independent very quickly, couple this with the challenges of the emerging pre-teen hormones in Xanthe and there have been a few shouting matches and groundings over the past month!

But this is now normality for us, and it's great as we are all under one roof and when the children get up in the morning their sister is still in the house and she is at home when they come home. We can't guarantee how long it will last, it really is one day at a time and as I said in a previous post, small goals - we have ticked a few things off the list - Singing Hands came to school to sing to Daisy and her friends, we visited Santa, she went Ice Skating (in her wheelchair), I saw her school play, we went to the GOS Christmas Party (even with a couple of appointments and a hickman line repair tacked around it!) and next week we have a trip to the Disney store followed by bowling with friends to celebrate her birthday.

7 whole years of life with Daisy, the biggest white knuckle ride of our lives, the biggest highs and the biggest lows and I wouldn't change it for the world!

Simple Pleasures

2011-11-22T10:20:00.000Z

The simplest pleasures - I decided to travel to Great Ormond Street by train yesterday as I had been struck with the winter lurgy and couldn't face driving - Daisy loved the train, thedouble decker bus and the taxi, she waved at everyone, rang the bell on the bus and couldn't wait to get to school to tell her teacher about her big adventure. Sadly the tests at the hospital were inconclusive and didn't give us any answers to why Daisy continues to vomit so much bile despite her gastrostomy being on free drainage all the time but this was compensated for by the joy in Daisy's face on the journey there and back. It's a rare occasion that we actually thank Transport for London for being the highlight of the day!
And here are some more recent pics of life with Daisy...

A kiss from mummy before going to school

Not too happy about having to leave school early for a hospital appointment

A bed fit for a princess (and Donald, Minnie, Oso, Peppa...)

Small Goals, Big Celebrations

2011-11-21T21:54:00.001Z

Well we left Great Ormond Street on Monday 17th October, very late at night (anyone who has been long term in hospital knows how long it takes to actually get out, especially with the wait for the dreaded TTO's - prescription meds to take home). The plan had actually been to transfer to our local hospital but Andy and I decided unanimously that as there was nothing that the hospital was doing that we couldn't do at home so we did just that - and no-one objected!

Since getting home the work has started - each time we bring Daisy home after a long stay it's just like those early days bringing home a newborn baby. The family has to adjust to a new person, new routine, this is what it is like for us. Similarly, when you bring your child home from hospital after a long stay you expect things to be better, not worse. Both are true for us, now we have adjusted to having a child with complex bladder issues, with even more pain medication, with even more sources of infection. But the more things change, the more we rise to the challenge and the more determined we are to keep Daisy out of hospital and home with the family.

Which is why for the first time in years we actually managed a complete half term break together as a family, we even managed some days out and family time together. This was all worth the huge military effort that had to go into place to make it happen, but it did happen, and for a fleeting few days we had what thousands of other families in the UK had for a week, a normal(ish) half term holiday.

So now we are midway through the second half of the term and it's taken this long for me to dust down the old blog and write. Not through lack of content, but sometimes it's just hard to keep writing about the slow decline we are going through. Even compared to earlier this year things have deteriorated. Daisy has five different stomas/catheters that need to be dealt with, managed, dressed, washed out, cleaned, changed.... all can be potential infection sources. And infection is what we are battling with - the old nemesis Candida is back with a vengeance - colonising her bladder and two of her stoma sites, it is clearly making her unwell and causing her pain. Having so many bugs in her bladder (she also is colonised with ecoli) means that the mitrofanoff catheter has to be on free drainage permanently to try and stop the continuous overgrowth. She is also on permanent antifungals and antibiotics which are making her feel rough. If the battles with the bugs were not enough we are having continuing problems with Daisy vomiting bile, mainly at night time and particularly after she has her meds. The whole point of her gastrostomy is just to vent out bile, that also has a bag attached to it 24 hours a day, but something is stopping it working properly and Daisy will still vomit, despite our best efforts.

So we now have a child who has at least two drainage bags attached to her day and night and overnight (and often for a good portion of the day) an extension bag attached to her ileostomy bag to drain the watery, high volume output. I find myself up to my neck in various body fluids at all shades of green a yellow and to add insult to injury the tiny (5cm) bit of rectum that was left following the colectomy surgery is still inflammed and bleeds most days. Daisy's pain management regimen has gone through roof - she has a minimum of 24 drugs a day and often more. She goes to school with a bottle of oxycodone now as the pain is starting to break through to the school day. We still do not know what each day will hold.

But we can't give up on Daisy as her will to get on with life and overcome the obstacle after obstacle that is thrown in her way is huge. Sometimes we stand back and ask are we doing the right thing for Daisy but just to look at her face when she is wheeled onto the school bus or has a visit to our hospice says it all. All she wants is to be at home, school or hospice - these are the three places where she can be a little girl, she can feel safe and she can be happy. We are doing everything in our power to keep her out of hospital at the moment, despite positive cultures, resistance to drugs, anaemia, increased pain, line infection risks, the pyschological impact of being a patient versus being a little girl cannot be underestimated. For Daisy to keep fighting and to keep strong she needs to be happy, and if it means that we have to work even harder to keep her at home, so be it.

We continue to be so grateful for support of our palliative care team and our hospice, it is reassuring to know that their aim is that same as ours - to give Daisy a childhood as possible and to keep her as comfortable as possible. We were over the moon to hear that we are going to be able to stay at our hospice for Christmas! This will give us all a chance to enjoy Christmas and to be together without being sleep deprived or worrying about when the next med is due. My mum is going to join us too which means Andy and I will be able to nip out to the pub for some grown up time too.

And of course, just before Christmas, on the 22nd December it's Daisy's 7th Birthday. Seven years old - that is such a big age, I can hardly believe it, after all she has been through she will be celebrating her 7th birthday. Every child's birthday is special but Daisy's is so special - she was born prematurely and spent her first 8 weeks in the neonatal unit, our Christmas that year was spent apart - I had a caesarean section, my first after three normal births, so had to stay in hospital, Daisy was in the intensive care unit and poor Andy had to soldier on at home with Theo, Xanthe and Jules aged 7, 5 & 2 respectively. They all came up to the hospital later on Christmas day with my dinner on a plate under some tin foil. After that Christmas and Daisy's traumatic birth a couple of days before it doesn't matter where we are as long as we are all together, however being at Chase hospice will definitely be the icing on the cake. Daisy's birthday is also a big excuse to party and we will be visiting the Oxford Street Disney store on the morning of her birthday for a bit of a shopping spree before meeting up with her Godparents and some close friends for a party in central London.

To get through this time of continuing uncertainties we set ourselves small goals - Celebrate Daisy's birthday, get to Chase for Christmas, then keep resetting new ones when we reach them, nothing too ambitious, things other people take for granted, but small term goals that can be changed if Daisy's health changes but things we can look forward too. For the next few weeks I will look forward to Christmas, hopefully we will visit Santa at one of his stop off points in London, we will celebrate Daisy's 7th Birthday, we will go to Chase for Christmas, beyond that who knows, we remain in Daisy's hands but brave and indestructable Daisy continues to defy odds, scales huge mountains, challenges perecpetions day in day out so the least we can do is be there to help her do what she wants to do.

Waiting for the school bus wearing a new coat from Grandma and a Disney Santa Hat with her name on in from one of our friends at PostPals!

Watching Toy Story with Big Brother Theo - she adores Theo as he sorts out anything technical, she calls him YoYo!

I wish someone had told me........

2011-10-13T23:16:00.001+01:00

While having a quick tea break in the parent's kitchen today I got talking to the mum from the next door cubicle, recently arrived with a newborn baby. As we chatted I realised that I could have been talking to the person I was nearly 7 years ago when I arrived at this hospital with a tiny baby and a whole load of question marks and worries about how her life would pan out, she too has 3 other children, similar ages to my children when Daisy was born (&, 5 & 2) and as we talked and she shared her thoughts I realised I was telling her all the things I wish someone had told me all those years ago.....

So for the benefit of anyone else embarking on this journey of hospitals, disability and caring here are the things I want you to know -

Allow yourself to grieve - mourn the child you thought you would have, adjust to the child you now have and find a love for that child that you never thought possible

Worry about the future if you must but learn to go with the flow and live for the moment, as time goes on you will stop fighting the things you can't change and only focus on the things you can influence, you will find that you can live in the moment and gain perspective.

Don't be afraid to accept help or ask for help. That was the hardest thing we had to do, we saw it as an admission of failure, that we could not look after our own child, it is not.

Remember that you are your child's mother - when your child has complex medical needs and you spend so much time in hospital you become medicalised and institutionalised and bogged down in numbers, values, rates, ranges, results - monitor these things if you must but remember that your mother's instinct has never failed you yet

Look after yourself, if you fall apart you are no good to anyone. I am determined not to turn into one of those women you see at the front of the hospital dragging on a cigarette, still wearing their pyjamas after lunch time - I dress, do my hair, stick on some lippy ready to face the day. I go out for a walk every day, I drink skinny lattes and read the paper......do whatever is necessary to keep body and soul together through the stress

There is no such thing as the perfect family. Look around you, the families you think are perfect, are not - even perfect Kate Middleton has a black sheep uncle lurking around.

Your other children will get through this. I felt so guilty that I had ruined the other three children's lives, I have not. It's not what we planned for them but we hope that their lives have been enhanced. They are more caring, they are more independent, they have had a varied range of life experiences already. Yes it is really really tough on them having to be parented by rota or having plans cancelled at the last minute by their little sister's major spanners in the works, but they will survive and as Theo's play therapist once said to me " this is just how it is, no-one's life is perfect (see above)". I truly believe they will be better people for it.

You will worry about money then you will discover what is important, you will stop sweating the small stuff and things will start to fall into perspective and with some careful planning and management you will survive the practicalities of this new life

Make lists, write things down, ask questions but don't always expect answers, know when to stop asking and just to live in the moment

Know that you are not indispensible - your child needs to be independent from you more than any other child. Your job as a parent is to prepare your child for the big bad world, when you have a child with additional needs - whether they are medical, physical or a learning disability (or in Daisy's case all three), this job starts on day one. Leave them with a carer, leave the ward occassionally so they know you will come back, teach them independence, help them learn confidence and to be their own person.

You will have a life again, the extremes will be great, the moments of happiness will be concentrated into smaller timescales but you will apppreciate them so much more

You will meet people, go to places, experience things that your old life will never ever have introduced you to.

There will always be someone worse off than you, be there for them, people will be there for you

Savour every single day with your child, you do not know what tomorrow will bring.....

Is respite a right or a privilege?

2011-10-11T22:23:00.002+01:00

Into week five now and this is where things get interesting - not for the doctors or surgeons, they have done their bit, and very successfully. Now we are onto the phase of getting a regimen for Daisy that will keep her reasonably stable and minimise time at hospital. The Mitrofanoff surgery was originally intended to provide an easy route for regular catheterisation, however inserting a catheter into the stoma is proving difficult both as Daisy finds it traumatic and because of all of her previous abdominal surgery it isn't the most straightforward channel to catheterise. It has now been decided that she will have a catheter left in the stoma for a week at a time which we can drain during the day and keep on free drainage overnight, I am going to be taught to change this catheter and manage any problems that may occur with the long term plan being that we will build up changes so that eventually, several months down the line, we can catheterise Daisy intermittently and leave her tube free in between. The theory behind all this is great apart from the fact that again she is open to infection from a permanently placed catheter and also bladder pain is a big issue requiring ever increasing doses of anti-spasmodics. But what everyone agrees is that we will get there, at Daisy's pace and in Daisy's time.

And this is the issue - again Andy and I are taking on responsibility for managing yet another element of Daisy's care that is not common place in the community and we will need to be confident that anyone who is involve in her care understands. Our concern also is the courses of antibiotics she has received over the past few years as she rebounds in and out of hospital with infections and sepsis, antibiotics which she is developing increasing resistance to. Our hope is to stabilise this pattern and reduce the admissions but this needs a period of time when drugs can be tweaked and changes made to protocols so that when she does come home it is for a good period of time. But does all this need to be done in an acute hospital setting?

Our other big concern as Daisy approaches 7 is for her mental health - this is a child who has spent more than half her life in hospital, who has been subjected to countless painful and traumatic procedures, she is making it very clear what she wants and what she doesn't want, and our role as her parents is to make sure that everyone takes her opinions into account too.

What Daisy wants more than anything in the world is to be with her family, all day long she talks (we always think of Daisy talking as she communicates fluently through signing) about her brothers and sister. If an advert comes on the TV that she thinks Jules would like for Christmas she will tell me, if her Ipad doesn't work she calls for Theo, if we are choosing her clothes to wear she tells me what Xanthe likes. Daisy loves being part of the family and wants for us all to be together again,..and that is what Andy and I want more than anything else . A referral has now been made for care at The Children's Trust at Tadworth but I keep being told unofficially over and over that there is no way our PCT will fund a transitional stay at this facility, even though it will provide holistic care for Daisy and support for the family en route home. The possibility of transition via our hospice for an extended period is also not an option, they are so stretched that committing to taking a funded child for a long term child is not possible although there is a glimmer of hope that we may be able to have a short stay for a few days. The only other options are to just bring her home or to transition via our local hospital, which would give the team there a chance to get used to her latest drug/catheter/TPN/Stoma regimens - these latter two options do not really allow us much opportunity to just be parents to Daisy. Our biggest hope, and our request to our local services, when we first knew that Daisy would have this big surgery was that we would be offered the chance to have some time to catch our breath before coming home, away from an acute hospital setting with medical support so that we could remind Daisy and the other children that first and foremost we are their parents and spend time together as a family without having to worry about fully managing all of Daisy's care. Sadly my fear is that we will be so ground down by the relentless strain of never seeing eachother, trying to juggle childcare, trying to be in several places at once that we will throw in the towel and just take Daisy home and try and manage as best we can.

Over the years, as Daisy has become more and more medically complicated and the care involved more specialised is seems like the support we need to get a break from caring is being whittled away. Without support (as I always emphasise - at not cost to the taxpayer!) from our wonderful hospice Shooting Star Chase Andy and I would get no break from the constant care that Daisy needs. We do have a funded package of support provided by Social Services but it does not allow us a break from looking after Daisy (it used to but sadly as Daisy has become more complicated the support package has decreased), it merely props us up in order to care for her. We have PCT funded night nurses now - initially we were allocated 3 nights a week but following their professional feedback on the level of Daisy's care needs this was increased to 4 nights (bearing in mind we have only been able to use them for a few weeks this summer in between hospital stays!. On a night we have a nurse booked she will arrive at 10pm and I will hand over how Daisy is doing, this can take a while so I don't get to go to bed until 10.30pm, so much for having an early night. The nurse is able to administer the pain medication Daisy needs into her tube but if intravenous pain medication is required she has to wake me up, similarly if Daisy is unwell and needs to be transferred to hospital she has to wake me up and then can finish her shift early. I have to get up at 7.30am in order to have a handover on how the night went and prepare to disconnect Daisy from her TPN and get her ready for the day. When the nurse is in the house, we are not allowed to leave. Apparantly this is our respite - sorry, but I thought the opportunity to have a night's sleep every other night was a basic human right? We receive some direct payments to buy in care, but the carers cannot be left unsupervised while Daisy's TPN is running and we cannot be too far away when it is not, occassionally we save up our direct payments money and blitz it on a four hour babysit from one of the hospice nurses - I won't say how much it costs but if you consider that Daisy is classed as high dependency you can imagine the hourly rate.

So I'm thinking about my own fantasy bucket list that the people behind desks who make decisions on who should receive funding should read:-

Things I would like to do:-

Have a weekend away with my husband, without children

I'd like to take all three of the older children to the cinema/theatre with Andy rather than toss a coin on who stays at home

I'd like to go on holiday and not return more knackered than when we left - holidays are wonderful but they also mean that we get no night care at all so not only are we managing the days we are struggling with the nights

I'd love a weekend away with my girlfriends knowing I'm not leaving Andy managing four children and all of Daisy's medical care for the weekend

I'd love to go back to work part time - how can I when Daisy spends so much of her time in hospital and always will?

Reading this through I sound so negative - truly I am not. I am so grateful to have Daisy in our lives, she makes our family complete. I also know parents who have lost their children who would do anything to swap places with me. She is the funniest, feistiest, little girl I know and I will do anything for her, I am so grateful that she is still here and fighting her corner constantly- and that's the hook, at the end of the day, no matter how much crap is thrown at us, we will always do whatever it takes to keep our family together and to give all our children some sort of childhood, regardless of our problems. So we will keep struggling on, battling bureaucracy, educating ourselves on our rights by trawling through endless government white papers and charity publications on the rights of carers while knowing that they count for nothing in the real world. We can say things like; "well if we just give up then they will have to pay for Daisy's care and that will cost them a huge amount more" - but everyone knows we won't give up, we'll keep fighting, and it's a fight for all of us carers of the most medically complex, forgotten children - for an occasional night off, to be parents not carers once in a while, to be a couple, to be a family...

Postcode Lottery

2011-09-23T23:34:00.001+01:00

Two weeks post surgery and this amazing girl continues to defy the odds and exceed expectations. No-one who knows Daisy well would have believed how strong and well she would look two weeks in. She is still on intravenous morphine, paracetamol and antispasmodics, but her ketamine is now going into her jejenal tube along with all her other meds, including the latest additions to her pain cockatil - oxybutinin and diazepam...

She is also nearly back up to her old enteral feeding regimen, although her TPN is still running over 24 hours . She is clearly over the acute, post surgical phase and now reality bites as we work, at Daisy's pace , to get her back to a regimen of nutrition and pain management where she is comfortable and we can manage at home. Our aim is to get her home and keep her there for as long as possible, that was why we agreed to the surgery, in the hope that it would reduce the infections, but while the medics are all very pleased at how well things have gone, Andy and I are more guarded, relieved that she got through the surgery in one piece but biding our time before we say that the surgery achieve the desired outcome.....I won't be able to say that for certain until a good few months down the line when Daisy has been home and we have a semblance of normality in our family life.

And soto thoughts of going home....we had always prepared ourselves for another long stay, preferring to put the hours in now in the hope that this will pay dividends. The next milestone in Daisy's recovery will be to remove the drains from her bladder and start to train it in preparation for catheterisation via her new Mitrofanoff stoma. We don't anticipate this will be a quick process, Daisy has been experiencing a lot of pain from her bladder and we can't imagine that she is going to be too tolerant of a plastic catheter being shoved into a newly formed bladder stoma that she currently associates with pain. However we know that slowly slowly things will get better and we will perfect our catheterisation skills in the same way that we perfected our Hickman Line & TPN skills and Ileostomy skills and all the other medical procedures we have had to learn on the way, and the mitrofanoff and bladder emptying will become another part of our lives.

Standing back from all of this we can start to see how easy it is to become swept up in the world of caring for Daisy's medical needs, we have become competent in so many areas, I wonder is this a good thing? What if something was to happen to us, would someone else know what to do??? One thing I have put in place is a "Daisy Bible" a huge file all about Daisy with her care plans, key contacts, letters, information on her communication needs, even pictures of her enjoying life at home - everything anyone would need to understand how to look after her, the nurses have told me that it is invaluable in helping them get to know her and to deal with her needs without always having to ask me. Increasingly Andy and I have been aware that we need to make sure more and more people know and understand Daisy's care needs so that we get the support we need to parent her and also so that people who look after Daisy realise that she has a say in how she wants things done and what she wants in life. Daisy is nearly 7 years old, we need to make sure that everyone understands that her opinion matters.

We have been very fortunate during this hospital stay in that there was a huge amount of planning that went on beforehand and teams involved in Daisy's care are co-ordinating and communicating better than they ever have been. We actually had a multi-disciplinary meeting yesterday to discuss Daisy and next steps now she has recovered from the acute surgical phase. I am so relieved that the team have clearly got a good understanding of Daisy's holisitic care needs, and specifically that to make her best recovery she needs to be allowed to be a little girl. They have suggested that instead of condemning both Daisy and as a result the whole family to another extended stay where we tweak TPN volumes and drug regimens Daisy goes to a setting which can manage both her medical and social needs better, this sort of setting would be somewhere like our hospice or a rehabilitation centre like the Children's Trust at Tadworth. Both options would be fantastic, they would allow Daisy to have fun while having one to one nursing care and an input from her GOS teams, the downside is that this would involve funding at Primary Care Trust (PCT) level

From our point of view how wonderful would it be for Daisy to be able to be out of hospital and transition to home safely with appropriate medical care so that when she did come home she was stable and we were able to confidently support her medical needs - we know that Daisy would want this, however great this plan sounds it comes at a cost, someone (ie the PCT) would have to pay. If they refuse to allocate the funding for this then Daisy will have to remain in hospital as we just do not have the 24 hour nursing care at home which would enable us to manage her medical needs until she is stable and the price will be Daisy missing out on precious childhood days, her parents not seeing eachother while one stays at the hospital and the other struggles to manage a career to support us and three siblings needs constantly juggled according the to day of the week and which parent can manage to be at home. There is just no consistency in care strategies and budgets in this country and while our community nurse will take Daisy's case to a panel of managers next week to consider together with letters of evidence from her Medical Team at GOSH it will come down to whether they wish to allocate funding to support Daisy's needs, some PCTs will, some wont - it's a postcode lottery and comes down to cash. So Daisy will either strike lucky and receive funding to get out of hospital and into a more holistic environment or the decision will be made that the PCT cannot afford it and, as there is no where else suitable for her to go until she is stable she will remain a guest of Great Ormond Street.

Please keep your fingers crossed that the decision goes our way and Daisy gets to do what she wants for a change.

It's a marathon, not a sprint

2011-09-12T12:05:00.000+01:00

Today is day three post surgery and Daisy is still in a lot of pain. Her morphine and ketamine have been pushed up as last night her epidural came out and this was taking the edge of her pain.

Her bowel has still completely shut down but her mitrofanoff is working well. There is nothing going on that we did not expect or anticipate, from her need to be transfused soon to her low grade fever (as a result of bacteria being release from the colon when it was removed). So while the first big and dramatic milestone, the surgery, is over with no major dramas or suprises, the challenge will now be over the next days and weeks as hopefully Daisy's gastrointestinal system starts to work and we are able to assess the level of chronic pain she has that will need managing from an ongoing basis.

The challenge for me is to turn the gears down and move out of sprint rhythm and onto a marathon pace. It's easy to want to move forward quickly and set yourself up for disappointment, especially after the adrenaline fuelled last few days but the reality, as any parent of a child with complex gastro needs will tell you, it's two steps forward and one step back. While appearing pessimistic to some doctors who don't know Daisy and quite what she is capable of, I prefer to describe myself as realistic. I anticpate that things will go wrong, multiple spanners will be thrown into the works on our journey to get Daisy back home, and if things happen sooner than I anticipated then that's a bonus!

We are on a different ward to our usual Rainforest and under the Surgery and Urology teams, I'm letting them get to know me so that I don't come across as a pushy mother. It's so difficult when your child is so incredibly complex and your main role is to know all about her to reign it in a bit while the professionals do their job! However I (and of course Andy who is a complete hands on dad too!) am first and foremost Daisy's advocate, making sure that she gets what she needs. Daisy's palliative consultant has just been to visit her and it needed her to point out to me that most children don't have a tummy that looks like Daisy so my role has to be to ensure that everyone involved in her care knows what to do - after all she currently has five different bags attached, all with separate labels! Plus a huge scar from her chest to her pubic bone, plus a hickman line and a gastrostomy button and jejenostomy button - I'm so used to all of this I forget that to the unitiated this is all be bit daunting, particularly when you factor in her complex pain management regimen and the fact that she mainly signs to communicate.

Today Andy and I celebrate our 19th wedding anniversary, we really, really did not know what life would have in store for us all those years ago, surviving redundancy, career change, bereavement and the diagnosis of a child with Asperger syndrome would be enough before throwing in the white knuckle ride we embarked on when I was taken into hospital in premature labour in November 2004, but we both believe that we are the living example of what doesn't kill you makes you stronger. Our marriage is stronger today than ever, helped by a healthy sense of humour and stamina to keep going on this ultra marathon that life is throwing at us.

...and onto the next phase

2011-09-10T10:21:00.000+01:00

well, Daisy now officially has only 5cm of large bowel left, a Mitrofanoff channel formed from her appendix for catheterisation and as a bonus a repaired parastomal hernia and a new chapter in her life has begun.

When we met with the surgeons the day before the operation the enormity of what we were consenting for on her behalf hit us, this was huge surgery, with all sorts of risks and possibilities for complications but having spent six hours in theatre yesterday the consensus
among all the team involved is that it could not have gone better. There were so many concerns about abdominal adhesions, viability of her appendix to be used for the Mitrofanoff, bleeding - however everything went according to plan and Daisy is now back on the ward and the long road to recovery can begin.

Again the terrible night she experienced the night before the operation confirmed to us why the surgery needed to go ahead. We have not been able to put any feed into her jejenostomy for many weeks now as she has been refluxing so badly and the night before her operation she vomited constantly and was pouring bile out of her gastrostomy. We hope that this deterioration is not permanent but only time will tell.

In the meantime we are now focused on Daisy's recovery. Not surprisingly her bowel did not like being handled and has shut down, it coUld take a while before it starts to work again. She has an epidural for pain relief plus a constant infusion of double strength ketamine and morphine, we just need to keep her comfortable and let her recover from the anaesthetic , as always our mantra will be " one day at a time"

A Summer, of sorts

2011-09-07T13:38:00.000+01:00

Compared to the past three years, this has been the best summer yet for our family, however as always it was marked by hospital stays and emergency dashes to A&E. In between those times we did manage to fit in some family fun and even had a whole two weeks when we were not in hospital with Daisy, managing instead to have a wonderful family holiday in Devon, thanks to the wonderful charity Torbay Holiday Helper's Network.

But the reality is that no parent should be excited about the fact that they have achieved a two week gap between their child's hospital stays, it is no way to live wondering if the next temperature spike is the one that does not respond to IV paracetamol or is the start of a septic episode. At times it feels like our phone is on speed dial to the London Ambulance Service. Each time Daisy has a hospital admission or a bad night it just confirmed to us that we are right in making the decision in going ahead with the colectomy surgery, this is no way to live, her pain management regimen has been going up and up, and it is not an option to continue like this The other thing that has become evident is the deterioration in Daisy's bladder function making the Mitrofanoff surgery a complete no brainer.

So, after a wonderful summer where we really did manage to fit in quality family time between hospital stays, Daisy will return to Great Ormond Street Hospital tomorrow and will go to theatre on Friday morning for surgery to remove most of her large bowel and to from a Mitrofanoff from her bladder through which we will be able to catheterise her. She will spend some time in intensive care where she will have an epidural for the pain and after that no-one knows, it truly will be one day at a time. We know that by taking away most of the large bowel we are removing a toxic source which is contributing to her infections and pain (her colon is inflammed and leaks bugs into the bloodstream which are increasinly becoming resistant to the antibiotics we use to treat Daisy). We also know that removing a permanent catheter and forming a new channel will hopefully reduce her risk of infection and make her more comfortable. Our biggest wish for this surgery is that it allows her more time at home with us and at her beloved school with her friends. We hope it will help reduce some of her pain but it will at least help her symptom care team better target her pain relief. We hope it will improve her quality of life and give us all more time together. But no-one knows, as always with Daisy we are in completely uncharted territory, and as everyone who knows her says, she writes her own book. We know that her recovery will not be straightforward and we know that this will be another lengthy hospital stay - knowing these things in advance have helped us prepare in some practical ways, but in terms of emotional preparation, there is nothing we can do. Andy likened our lives at the moment as like being in a car knowing it is going to crash but not knowing how bad the crash will be - we a bracing ourselves, and hoping......

So this is it, as every milestone and procedure gets bigger and bigger this is the biggest surgery in Daisy's life, we are in her hands, she has the constitution of an Ox and a lust for life and all we can do is support her in this. Whatever works for you, prayer, positive thoughts, crossing fingers, please do this for Daisy on Friday morning GMT as we hand her over to the best team of surgeons, & anaesthetists you could wish for....

2011-08-07T00:44:00.000+01:00

Happy days in Highgate

Visiting Peppa Pig World

Chilling with big sis Xanthe

2011-08-07T00:03:00.000+01:00

Oh my goodness, a whole month has flown by and I have not updated this blog - big apologies!!! I think I'm going to cut and paste some of my statuses from Daisy's Angels (www.facebook.com/daisysangels) from now on so that those who don't use facebook can still see what is happening in our lives...

It's been a funny few weeks, lots of real progress in terms of plans for Daisy, more emergency trips by ambulance and time at our local hospital and we are even managing time at home - for the first time in years I'm actually getting to spend part of the summer holidays with all the children under the same roof.

The biggest news is that we had our much anticipated meeting with Daisy's surgeon at Great Ormond Street. This was the meeting to discuss whether or not a colectomy was an option for her and whether he was even prepared to carry out the surgery. Andy and I had talked and talked about this prior to the meeting, veering from being 100% sure we wanted to go ahead to deciding that it was not an option as there were no guarantees. In the end meeting with the surgeon reassured us of the safety of performing the surgery, he will not perform a full proctocolectomy as this is just too big for anyone let alone Daisy, instead he will take out most of her large bowel which will hopefully massively reduce the colitis symptoms she experiences every day. It will also mean that she can come off the drugs which supress her immunity and render her at risk of infection and we can treat the colitis more conservatively. This will not be the only surgery Daisy will have at the time, Daisy's Urology surgeon will also perform surgery on her bladder at the same time to help manage the symptoms of it's deteriorating function. Initially the plan was to form a vesicostomy, a stoma, from the bladder into her abdomen to allow urine to drain out into a nappy. After much consultation and discussion however everyone agreed that this was not an option for Daisy, a little girl desparate to get out of nappies. Instead along with the colectomy surgery Daisy will have a Mitrofanoff procedure where the appendix which will be removed as part of the colectomy will be used to form a channel from her bladder to her abdomen allowing us to catheterise her intermittently. Individually these surgeries are big, performed together on a girl who is not in optimum health they are huge. She will need to spend some time post operatively in intensive care and it is likely that we will be looking at another prolonged stay in Great Ormond Street, however our hope, which comes with no guarantees, is that once she has recovered from the surgery, we can reduce some of the drugs she is on which have such awful side effects, her pain may be more manageable and she will be less susceptible to the infections which often lead to emergency trips to the hospital. Making the decision to go ahead with the surgery was the hardest decision we have had to make for Daisy - we don't know if it will make things better or worse but the main thing that swung it for us is that she or we can't keep going on as she is.

The pain she experiences, mainly at night, is getting worse - she now has regular doses of morphine plus ketamine and then when this doesn't work sedating medicine to help her sleep. We are seeing a big deterioration in her health, her hair is falling out, she is getting tireder. But her lust for life is immense - she managed very few days in school this year but every single day was packed to the brim, she won a medal in sports day, had fun times with her friends, learned new skills. She is vocalising more and more, singing away to herself in bed and her hands never stop signing. She has so much she needs to do and this is why we made the decision to go ahead with the surgery - desparate times call for desparate measures so although it doesn't have any guarantees to take the pain away we just hope that it will keep her hospital trips down and improve her quality of life so that she can enjoy the things she loves in life.

In the past month we have called 999 three times, each time Daisy has spiked high temperatures and developed sepsis, mainly from the bugs (ecoli & candida) which are permanent residents in her bladder. I used to drive her to hospital myself when she became ill but she is going downhill so quickly now we have been told not to risk it, so now the whole neighbourhood knows when Daisy has gone back into hospital. But as quickly as she goes downhill, Daisy can bounce back once she has had enough doses of IV antibiotics. So the moment she can come home we have been out and about having fun as a family. We visited Peppa Pig World earlier this week - Daisy was beside herself with excitement, as everyone who knows her knows, she adores Peppa Pig, almost as much as she loves Mickey and Minnie.

We are keeping everything crossed that we can also manage a trip to Devon towards the end of next week, Daisy will be able to meet her new cousin and we will be able to have a lunch to celebrate my mother's 70th birthday (Daisy will be in Great Ormond Street when it is Grandma's birthday so we are having an early celebration). We have been given a date of 9th September for Daisy's surgery, this gives us a few more weeks of family time and also hopefully gives us a chance to be out of hospital in time for Christmas. We are in Daisy's hands as always but have learned flexibility and whatever happens we will adapt to make the best of the situation.

2011-07-05T00:02:00.000+01:00

While staying at the hospice Daisy was delighted to receive a T shirt of her namesake from one of our friends at Post Pals (http://www.postpals.co.uk/)

2011-07-04T23:34:00.000+01:00

Well as always the long gap between posts is because the best laid plans in our world really never go according to plan. I wish I had the luxury of time to do more frequent updates but I squash my blogging in at the end of what is normally an exhausting day....and that is most days at the moment! (for those of you on facebook I have setup a page where you can get daily updates on Daisy, it's at www.facebook.com/daisysangels, and for those who use twitter, I tweet as @stephnimmo)

Of course I should have realised that the "odd temperature spikes" that I referred to at the end of my last post would actually become something more than that - while at Chase, Daisy developed a very high temperature, reaching the level where we have to take her back into hospital. However having just reclaimed her back for ourselves, Andy and I were loathe to take her back to hospital, especially as the one nearest our hospice is not our regular haunt. We dug our heels in as in herself Daisy was well, and once again the wonderful team at our hospice helped us by arranging to have her bloods processed at the nearest hospital and also for IV antibiotics to treat what turned out to be yet another urine infection. We were also able to transfer to Shooting Star hospice which is under the same management as Chase Hospice and is significantly closer to home for us. This meant we were able to manage all of Daisy's IV treatment with the support of the hospice nursing staff rather than struggling on at home.

So we arrived home properly after a lovely 10 day respite break on June 17 and we had a family day out to Wimbledon Village fair the next day - sadly this was rounded off with a call for an ambulance as Daisy's temperature sky rocketted the moment I flushed her hickman line to connect her TPN, no time to spare this could be a line infection! More IV antibiotics, more juggling childcare, but at least we were closer to home. This also meant that although Daisy was back in hospital I did not have to miss my longed for return to Glastonbury Music Festival with Theo and Xanthe. Those few days in Somerset, in the craziness of the festival, ankle deep in mud, camping in the rain, helped me recharge my batteries and find a little time for me. We arrived back home on the Sunday, Daisy had been discharged from hospital and we tried, once again, just to function as a family of six under one roof.

Daisy went back to school for her first full day since February last Monday. She was so pleased to see her teacher Beth and her intervenor (specialist assistant for dual sensory impaired children), Carmen again as well as her school friends. I know everyone at her school, Linden Lodge, was delighted to welcome her back. They have always been so accomodating of Daisy's ever increasing medical needs and frequent hospitalisations and are one of the few constants in our crazy world. Last Monday was a really hot day and by the evening Andy and I put Daisy's lethargy down to being overtired, although in the back of our minds alarm bells were ringing. By 6am the next morning Daisy had spiked yet another temp. It broke my heart to call the ambulance, we could not wait until the other children had gone to school so with only one full day back at home and her siblings eating their breakfast and getting ready to go to school, Daisy went back to hospital again. A week later she is still there.

She has finished her IV antibiotics, this is the fourth course she has been on since staring back on immunosuppressant therapy for her diversion colitis around 6 weeks ago. The colitis has not improved but in herself Daisy has got worse, infection after infection and with the constant antibiotic usage she now has a candida infection in her bladder. This is being treated in hospital as there is a big risk of the candida bugs translocating into her line and once candida gets into a hickman line not only is it a very serious infection it is very difficult to treat and the line has to be removed. She is on her 7th hickman line at the moment and we want to keep hold of this for as long as possible.

At the moment Daisy has a urethral catheter in situ as her supra pubic one (her second) fell out. Next week we will meet with her urology surgeon to talk about a vesicostomy which is a permanent stoma from her bladder, this will mean she will no longer need a catheter but will always be in nappies. We had hoped to avoid this situation as most children with Costello Syndrome are potty trained sooner or later, however we have now realised that this is the least of our issues compared to everything else we are facing.

The biggest issue is whether Daisy remains on immunosuppressant therapy or steroids - in staying on them we are hopefully preventing the diversion colitis from becoming worse although it is likely that she will continue to have flare ups which will require a hospital stay and IV pulsed steroids (which she hates as they make her feel awful) the alternative is the procto-colectomy surgery. This had originally been discounted by the gastro team however now that she is getting infection after infection on the drug therapy her Gastro consultant has asked her surgeon to consider whether he would now be prepared to do this surgery. This would probably be an open procedure as opposed to keyhole as she has had so many surgeries before, it would involved complete removal of her large bowel and rectum and negate the need for drug therapy to treat the diversion colitis. We meet with her surgeon next week to discuss all of this. There are huge risks with both options and the decision we are being faced with is one that no parent should have to make, but at the end of the day Andy and I have to be able to say that we did everything possible for Daisy, and our instinct is that, despite the risks, surgery gives her a fighting chance of some sort of quality of life, even though it means yet another extended stay at GOSH.

So we are now reaching another crossroads in our lives with Daisy and the decisions we make for her are harder and harder. What is even harder is the unpredictability of our lives, difficult enough for any family but try managing our situation where one of the children has Aspergers syndrome and craves predictability, routine and stability. We can offer none of this to Theo at the moment, and at times it is a struggle for him and for us as we try to keep things on a relatively even keel for all the children. It is not only Daisy's life that is being affected but her siblings lives, often Andy and I feel that we are parenting by rota - one is at the hospital while one is at home. We juggle constantly, but we are still determined that our children should have a childhood so we push ourselves to the limits to ensure trips to concerts, the cinema, birthday parties are arranged - the majority of time with only one of us able to participate while the other cares for Daisy. Family trips are difficult to plan as we really do not know what each day will throw at us, so we seize opportunities when we can . We can't go far afield - and by this I mean to visit family, for fear of ending up in a hospital which does not know Daisy. This world is very isolating and immensly stressful and we have tried to make sure we look after ourselves so that we do not buckle under the stress.

So how are we expected to look after ourselves when we do not get any proper respite as a couple or a family from the stresses and strains of our life? The only true respite for us is when we stay at our hospice - there we know nurses will care for Daisy giving us a break, we can stay with her as a family or we can leave her there in order to have time with the other three children. Children's hospices receive no government funding, they are funded solely on voluntary donations, we receive 15 allocated nights a year to stay at our hospice at no cost to the taxpayer but with the benefit that we do not fall apart from the stress. We had also been awarded two funded nights a month at the hospice, paid for by our Primary care trust as no other respite package or facility was in place locally or could meet Daisy's needs. This was how we were able to transition from our long GOS stay to home via the hospice, by using the nights we had not been able to use while in hospital. So you will understand my frustration and anger with the system I am up against when I tell you that we will no longer receive these nights any month Daisy is in hospital (so that's July up the swannee) - apparantly when Daisy is cared for in hospital, this is counted as respite, even though Andy and I do her TPN, administer her drugs, change her stoma bags, replace blocked jejenostomy tubes, act as her translator, play specialist, occupational therapist, physiotherapist - oh and be her parents........no because our daughter has the misfortune to be very ill a lot of the time she costs our Primary Care Trust a huge amount of money, money they have to pay to the Hospital Trust each time she is an inpatient. This is how the NHS internal market works and sadly the sicker and more complex your child the more money they cost and it would appear the less likely you will be to get respite support.

Andy and I are creaking under the strain of looking after Daisy and the other three children, we have been promised a nurse three nights a week, however this may not happen until the end of July and all this gives us is three nights where we don't have to physically get up to Daisy when she cries, we can't leave the house and the nurse will not do TPN (they will be able to administer her pain relief though so I suppose this is some help, if it every materialises). Our funded nights at the hospice have gone because let's face it, will Daisy ever complete a month when she is not in hospital. This means that we are still condemned to parenting by rota, not able to go to the cinema, book theatre tickets, visit our families, make any plans...all those things we used to take for granted, and all because we have done the right thing and have chosen not to give up on our child.

I would love 5 minutes with David Cameron, who made such a deal about defending the rights of carers in one of the televised election debates - I would ask him why are we in a situation where parents of the most complex, fragile children who are doing what is morally right by caring for their child actually have less rights and freedom than prisoners. We chose this path, we knew Daisy would be disabled, we chose to be trained in all her medical care, we will never give up on her but sadly it really feels like by doing the right thing we have done the wrong thing. Perhaps Andy should give up work too and we can both claim benefits, perhaps we should leave Daisy in the hospital long term so that we get a break??? Obviously anyone who knows us knows this is not going to happen...but it does make you wonder why our child is seen as a cost and the more complex she is the higher the cost and therefore the lower the support package....where, Mr Cameron, is the logic in that?

2011-06-07T00:36:00.000+01:00

At long last and 15 weeks after being admitted for a 5 day admission, Daisy is safely tucked up in bed in our beloved Chase hospice. Her arrival here, not suprisingly delayed by several more gigantic spanners in the works.

The temperature spikes I blogged about in my last post were after all caused by a line infection and Daisy was very poorly for two weeks. At one point she was on five different intravenous drugs plus iv pain killers and all her usual meds. The poor nurses looking after her on a one to one basis worked non-stop and went home exhausted. The infection was not shifting and turned out to be a fungal infection which meant her hickman line had to come out. In order to prevent re-infection the best option was not to reinsert a new central line immediately but instead insert a temporary PICC line which is like a very long canula but could be used to give TPN. To do this Daisy needed the Interventional Radiology Service in GOS. A service we are always so grateful to have available to us as they have optimised the potential Daisy has for line insertions however the downside of this very specialised service is that they only operate during weekdays and between the hours of 9 -5 (with a break for lunch of course, this is the public sector!). So Daisy had to wait her turn among all the other top priority children in GOS, it is easy to become deeply frustrated with this situation until you remember this is no ordinary hospital, there are very few paediatric interventional radiology services and the children they are dealing with are the most difficult. For Daisy multiple previous line insertions means that we need the best expertise available to ensure that we do not lose ever decreasing line sites.

Eventually Daisy's line was taken out by the surgeons as she was becoming increasingly unwell and we had to rely on two very precious canulas, inserted by anaesthetists to provide fluids and iv drugs until the interventional radiology slot became available. Within hours she lost one cannula, her little veins are so scarred from previous lines and we held onto her other cannula for a day until that went too and she had to be sedated for a new one to be inserted as every procedure was becoming so traumatic. Eventually, Daisy's name came up for her slot in interventional radiology - following some timely intervention from the on call gastro consultant who questioned the ethics of continuing to keep a child whose only source of nutrition was via a central line waiting for more than 24 hours. Instead of a PICC line she ended up with a new hickman line as the delay had worked in our favour, she had been 72 hours free of plastic in her main veins and hopefully this had put paid to any lingering bugs. At the same time she had to have a new supra pubic catheter inserted, her brand new one, inserted the previous week had failed and come out and the tract where it had been inserted had closed immediately.

The new catheter insertion caused very heavy bleeding and Daisy needed 3 transfusions of FFP or fresh frozen plasma as well as a blood transfusion.
By the beginning of half term things were looking good following what we thought was the last minute drama stopping us from getting to Chase Hospice. I took the older children to my mother's house in Wales as it was the start of yet another half term holiday with Daisy in hospital , and Andy and I made plans for a hospice transfer on the Tuesday with a family reunion on the Wednesday. We even pushed the boat out and stayed in a hotel on what we thought was our last night in hospital........only to be greeting first thing on the Tuesday morning with the news that the brand new hickman line had migrated into her chest cavity and overnight Daisy's neck had swelled up and the plastic surgeons were reviewing the situation...

I'm very glad I did not see Daisy immediately after her line had moved from the jugular vein spilling TPN out under her skin, apparantly it was not a pretty site. When we saw her she was black and blue and very uncomfortable. The good news was the the Plastics team confirmed that there was no lasting tissue damage the bad new was obviously we were going to lose this precious line. So the next day Daisy went back to interventional radiology for her 8th anaesthetic and 7th central line. To say she was fed up is an understatement, she just wanted to get out of the hospital and get away. Ontop of this she was coverered in bruises caused by her poor blood clotting, a side effect of the liver damaged caused by long term TPN use. However she recovered well from this final surgery and made it known to everyone sho came near her that she wanted to go. So after 15 long and traumatic weeks we left Rainforest Ward and transferred to Chase Hospice.

We had asked our consultant at the very beginning of our hospital stay for a second opinion on Daisy's treatment, not because we had any doubts about the treatment she was receiving but as her parents it is vital that we leave no stone unturned in our quest to give Daisy the best posssible quality of life. We were very fortunate that he was able to arrange for a Professor from the US who is a world expert on Paediatric Dysmotility Disorders to come and review Daisy when he was passing through London for a couple of days en route to a conference, Meeting the Professor was both reasurring and daunting - reasurring because he confirmed to us that Daisy is looked after by the best in Europe when it comes to her condition and that everything we have done so far is what he would have done, daunting because her complex condition is so difficult to treat and the reality is that things have not progressed massively in 30 years. He wondered if Daisy's condition was the result of a mitochondrial disorder and therefore, as we have always suspected, a separate condition alongside her Costello Syndrome. He also confirmed that the pain she experiences is very difficult to manage, her neural pathways have been bombarded over the years so that they are very receptive to the pain she feels from her gut making treatment of the pain very very difficult, the reality is she will never be completely pain free. He felt that we now face two choices - to keep her on the immunosuppressant therapy and steroids which keep her colitis and bleeding in check but massively increase her risk of infection, particularly line infections, which can be so damaging to the liver, or remove the need for the these drugs by removing the cause, her large bowel and rectum. However this surgery would be highly risky and probably not possible as a keyhole procedure because of her previous abdominal surgeries. It's a damned if you do, damned if you don't situation however as her parents our instincts are to get Daisy off the drugs which we know from previous experience make her so ill, her recent line infection occurred a matter of weeks after starting the immunosuppressants again, however to do this we have to consent to the biggest operation of her life, with only a 50/50 guarantee of taking away the pain (but at least taking away the immunosuppression). Ultimately it does depend on whether Daisy's surgeon is prepared to take on this risk and her Gastro Consultant has promised to have this conversation with him.

So this is how we left hospital, with the prospect of a return, hopefully in the autumn allowing us a summer at home but early enough to guarantee Christmas at home too. Our family was reunited on Friday evening and thanks to our wonderful Chase Hospice, and despite some odd temperature spikes and hairy moments from Daisy, we have started to relax and live in the moment, knowing that each moment is more and more precious

2011-05-19T21:49:00.000+01:00

I hate rollercoasters, why would anyone pay good money to scare the living daylights out of themselves all in the name of having fun....so why are we still on this non-stop rollercoaster feeling like it's Groundhog Day over and over? Week 13 in the Big Brother House and Daisy Nimmo is still doing time in her cubicle on Rainforest Ward....

Where to begin, this is truly the worst stay ever and probably because of events that have panned out since my last post. Daisy got the catheter eventually - ironically it was a perfect example of when poor communication works in our favour. I was sitting having coffee with a friend when the ward sister called me on my mobile to say that an anaesthetist had arrived and did I know anything about a catheter insertion slot that afternoon? Turns out the Urologists, who had been waiting for a "special type of catheter" for Daisy had booked her an elective surgery slot but completely forgot to tell the Gastro Team, Ward doctors or ourselves. At least the fact that Daisy has a non- functioning gut was another factor in our favour as she was good to go for surgery that afternoon.

And so the catheter was inserted and the cystoscopy showed that her bladder was inflammed as a result of repeated UTIs. Not suprisingly the catheter was not the magic cure all, in the same way as her ileostomy, it has proven to be a necessary evil throwing up more problems. Daisy experienced a lot of bladder spasms, blood and pain from the catheter and has been maxed out on pain medication, however at least we have been able to drain away the excess urine which her bladder was not able to empty and hopefully prevent any more UTIs.

The friend I had been having coffee with when we got the call for the bladder surgery is another mum who has shared a similar path to us, both at our local and at GOS, but living through the rollercoaster ride of cystic fibrosis with her daughter. We live locally to eachother and through our shared experiences and lives have become very close over the years, so I was happy but sad when I had a text from her to say that "my drinking buddy was back" this meant her daughter was being readmitted after only 5 weeks at home but at least I would have a kindred spirit with which to share a bottle of red at the end of a testing day.... As it was I was so glad I was there as later that week, and completely out of the blue, she was told that there was no more that could be done for her daughter, her lung function had deteriorated beyond help and she had only days to live. Sadly she gained her angel wings on Tuesday on the ward at GOS and is now resting peacefully and breathing easily again at our wonderful hospice, Chase, while her family come to terms with life without their little girl....

As you can imagine this has thrown up a multitude of emotions for us, sadness for a friend grieving for her daughter, grief at the loss of a beautiful little girl, fear at the possibility of our own loss.....

And in true Daisy fashion, just as my stress levels were being maxed out on the rollercoaster she throws another spanner into the works but spiking high temperatures and sepsis. For 6 days Daisy has been spiking high temperatures, vomiting and having episodes of rigour and sepsis, she is on 5 different IV antibiotics and an antifungal and not responding. Her blood cultures are negative and the experts in GOS are scratching their heads. She has five different sources of infection - a hickman line, a gastrostomy, a surgical jejenostomy, an ileostomy and a supra pubic catheter so where is the infection coming from? Correct that, actually it's four sources - the supra pubic catheter came out before a viable tract could form and she is booked for another surgical insertion next week.....

The assumption is that the infection is from the line but if we remove the line we limit her options for further line insertions. Today was spent discussing with surgeons and doctors whether or not to remove it...I am constantly being forced into making decsions which could have serious consequences - if we keep the line will the infection overwhelm her, if the line goes will we reduce options for line insertions. What if we are missing something by focusing on the line? What if it is an inflammation of her heart valves? or appendicitis? Daisy does not present in the way text books say she should, all we know is that her inflammatory markers are rising and she is not responding to antibiotics. However during the day she is still smiling and still interested in the TV and this gives me hope.

I have told the doctors that everything must now be focused on getting her into a stable and safe position so that we can transfer for a planned stay at our hospice on 29th May - we need time together as a family and this is our number one priority. This week's events have shown us that we have to seize every opportunity to be together as a family. The doctor's know this, they know that time is precious for us, and this is where I get my strength , by focusing on the most important thing, getting us all back together again.

This post is dedicated to a beautiful little girl, Alica Roa, age 10 years 5 months, who lived life to the full and to her fab mum Catherine, a true friend (& drinking buddy!) - xxxxx <3

2011-04-28T04:17:00.002+01:00

This post has been sitting in edit mode on my laptop for a few weeks now, waiting for me to tweak it and post it, I find myself sitting in a tiny room in the parent's accomodation wing at 3.55am in the morning, unable to sleep yet again and now I find time to finish this post and update.....sorry for the odd timeline but it's just impossible to write during the day with Daisy, I am either spending time with her or talking to the various doctors and professionals who stream into and out of our lives over the week. Weekends are for family visits, and night-time is for mulling over what has happened during the day or swapping shifts with Andy................

A week is a long time in the world I find myself inhabiting, and just one week on from my last post it feels like we have moved forward at nano-speed in all areas of our life. I came home for the evening on 5 April and Andy was staying at Great Ormond Street. At 5.30pm he phoned me to say that one of the Gastro Consultants had come to see him to say that Daisy would not be able to have a slot for the surgery to place a surgical jejenostomy until some time during May. This was going to condemn her to at least another four weeks in her cubicle, attached to intravenous pain relief, with no feed and TPN running for 24 hours a day....clearly this was not acceptable, what had gone so wrong that we found ourselves in a situation where our childs needs were no longer seen as urgent....

Andy came home on the last tube and we fired up the laptop, opened a bottle of wine and began to write:-

Dear Jane Collins (the Chief Executive of Great Ormond Street Hospital) ...We are writing to register our formal complaint that regrettably after a generally positive 6+ year relationship with Great Ormond Street Hospital we feel that our daughter, Daisy’s care and clinical management is substandard and not supporting the objective of improving the quality of her life...

We described, over the following four pages, the impact of not having a surgical jejenostomy was having on her life and our family life. How basic processes in communication and patient advocacy had left us in a situation where our family would continue to sit waiting for things to happen for at least another four weeks....four weeks where Daisy was condemned to life on TPN for 24 hours a day and at constant risk of developing infections.

Within 12 hours our world changed, people we had never met before appeared in Daisy's room and most importantly the surgeon who had performed her ileostomy last year appeared in her room to tell us that she had been put on the emergency list for surgery the next day.... by Thursday evening Daisy had her surgical jejenostomy, technically called a roux en y jejenostomy and although she was not coping too well with her breathing post anaesthetic was back on the ward and we could start to think about life away from hospital again...

We are now 2 weeks post surgery and Andy and I have mulled over why did it take a letter to be the catalyst to action. We are so fortunate to be able to articulate our concerns and to be able to stand back and use our experience as business professionals to look objectively at why processes had failed to support the overall objective of the child first and always. We have never ever criticised Great Ormond Street Hospital and never will - our daughter owes her life to the care and expertise of the many people we come across every time we come to this hospital. However some of the basics of communication and ownership of issues are so clearly missing in some areas leading to situations where children wait for procedures and plans to happen. What was missing in Daisy's case was a clear sense of ownership of the problem and that word which we have used so much recently, advocacy, for her needs. As her parents we trust that people are fighting her corner when she needs things to happen, in many cases this does happen and we know of some brilliant people who are constantly fighting Daisy's corner. But when your child is so complex and teams rotate on a weekly basis who is making sure that things are still happening when they should?

Our letter of complaint has become a catalyst for a root/cause/analysis of this situation - parents of complex children need to be parents, we are advocates too but we trust that the health professionals are then making sure that they are representing our child's needs among all the other priority children. Andy and I did not want our complaint to be just about dealing with our situation, we wanted it to change things for the better, to look at where things have gone wrong and make a difference so that situations like ours don't happen.

We now have over six years of experience of dealing with the machine that is the NHS, over the years our ability to deal with this has grown and evolved. We have learned that we are essentially dealing with a huge, supertanker set in it's ways, but we have also learned that by finding the right people who can help you can slowly find a voice which people will listen too. Whenever people ask me how to manage a situation I always direct them to the Patient Advice and Liaison Service (Pals) - every trust has one. We have a long relationship with the Pals team at GOSH, to the point that we always pop our heads around the door, even to say that things are going well this stay. Often I will go in and ask them to "liaise on our behalf" - sometimes a carefully worded email from Pals on our behalf is all it takes. The Pals team know our situation with Daisy and know us, we have only ever used them when things are becoming difficult and are always clear in knowing what we want as an outcome.

We know we are going to continue to have a long relationship with GOS, many things have changed for the better - the care Daisy receives on Rainforest ward is fantastic, so much better than the early days when we used to come to the hospital, things can improve and we have always taken the view in every aspect of our lives that you can sit and rant about it or you can do something yourself to improve things. Years of diplomacy and tact pay dividends when you become viewed as parents who have views worth listening to...

So now Daisy has her jejenostomy at last and we are working towards increasing her milk feeds and reducing her TPN down from 24 hours so that she has time off. We are also making more and more progress in managing her pain and identifying where it is coming from. For years both Andy and I have been concerned that her pain is not just from her colon but also from her bladder. Eventually the gastro team organised urodynamics testing which has identified another source for her frequent infections and pain - Daisy's bladder does not empty properly, this means that there is always a residue of urine left in it, explaining the reason behind the frequent UTI's and probably also explaining why she experiences so much pain at night as her bladder fills, goes into spasm and because of the UTI's is chronically inflammed. The solution is to help it to empty. The team here would like her to have a vesicostomy, yet another stoma, this time in her bladder so that excess urine could drain into a nappy. Andy and I have not made that mind shift yet for another big bit of surgery or also to condemn Daisy to a life of incontinence when most of the mobile children with Daisy's condition do not spend their lives wearing nappies. The second option is to fit a supra-pubic catheter which would allow us to drain the excess urine from the bladder. This involves another general anaesthetic, it's seen as a medium term solution and in the longer term she would need surgery to provide a more permanent solution to her problems with her bladder.

So at least we have a lot more answers on why Daisy gets so much pain - it is from several sources, the diversion colitis, her dysmotile small bowel and, not suprisingly, a neuropathic bladder. So you would think that given she is now doing so well establishing her milk feed, reducing hours on TPN and we have a good protocol for pain management that surgery to fit the supra-pubic catheter would be prioritised and we could move forward in our plan to transition from GOS to our hospice for respite before going home....

Well in a remarkable moment of deja vu I received a phone call from Andy tonight (it's always when I am at home and he is doing a shift at the hospital!) - at 5.50pm, the Urology Registrar came to visit Daisy to say that it would be likely that it would be a month before they could do the surgery......somehow I think we have been through this all before....however we have some good people who I will speak to tomorrow and once again in my role as Daisy's advocate point out that sitting in a hospital cubicle, at risk of infection (she is just recovering from a bout of shingles) while we wait for a procedure which will enable her to get home and live her life is not acceptable...

And so that is why I am finishing my blog in the early hours of the morning as I run through the conversations I will need to have yet again later today. It's not my daughter's medical problems that cause me sleepless nights, these I can deal with, she is strong and a fighter....it's the fighting and battling I have to do on her behalf to get her the things she needs to live her life, that's what saps my strength and energy.

Better close down the laptop now, big day ahead, fingers crossed the next post will be a positive one!

2011-04-04T00:08:00.000+01:00

This is my favourite song and this video clip is from one of my favourite place - both the song and the place keep me going, the lyrics remind me why I do what I do....

2011-04-03T23:30:00.000+01:00

Today was Mother's Day in the UK. Did any of us think when we became mother's that the journey would take us to where we are now? I never in a million years imagined that I would be sat, as I am now, in a tiny single bedroom in the heart of London, writing about my sick child, lying in a cot in one of the world's best children's hospitals next door....

I also never thought that I would have to find the time to attend meetings to discuss my eldest son and the impact his high funcionting autism is having on our lives and his life...

Do any of us really know what life has in store for us? Out of everyone in my life I though my dad would live for ever, not cruelly snatched away by cancer at 61. I thought I would be still building my marketing career, Andy probably thought he would still be an actor... If anything, life with Daisy has taught us that you have to accept what you have, live for the moment and go with the flow. As I look around I wonder what is the perfect life, the perfect family? It strikes me that no-one has this rose-tinted existence, each person I come across has issues, problems, battles.... so while I enjoyed Mother's day today with one child still in hospital, one very stressed because she is in hospital, one about to hit adolescence and wanting her mum to be around more and one who is growing up far too quickly, I realised that I can crave the ideal family or embrace what I have - and for the record I love every dysfunctional bit of my family, in my eyes - gene mutations, hormones, autism - everything - they are perfect because they are individual and special and reflect Andy and I. Anyone who knows us well know that life was never going to follow a conventional path so I guess the adage "you are only given what you can handle" must hold true for us - although there are moments when I do think, OK, give us a break....

Which, inevitably brings me on to our wonderful youngest child, Daisy. Tomorrow we enter week 7 of our current stay at Great Ormond Street, and where are we? - well since I last updated we have had an multi-disciplinary meeting, and I had the honour of being invited in for the last 30 minutes of it (the first 30 minutes were for health professionals only to discuss my child). The agreement was that colectomy was deemed too risky but a surgical jejenostomy, although it would probably result in a step backward initially, would be OK. It was also agreed that an MRI to rule out malignancy was a priority. After much negotiating by the gastro team the MRI (under general anaesthetic)will happen tomorrow, although we still do not have a date for the surgical jejenostomy...

Of course, true to form, and probably knowing how important this MRI is tomorrow, Daisy has decided to spike a temperature and have problems with fluid balance and fast heart rate...the anaesthetist is going to love her! I just hope it doesn't get cancelled, we need to know where this pain is coming from. She has been on 24 hour TPN for over 5 weeks and a ketamine infusion for the same length of time. This is no way to live. Everything has to be focused on getting her home, but getting her home in a way that Andy and I can manage her. We are hoping when she eventually does get the surgical jejenostomy we can get a good pain management regimen in place and go home via our wonderful hospice at Chase. It is so long, once again, that we have been a family together, at least time at Chase will allow us time to adjust and be together before we go home. In the meantime, I just need to focus on getting through tomorrow, I don't want the MRI to show anything nasty but at the same time we need to know why she is in such pain and why it is so localised and of course ontop of that I just hope she isn't brewing anything nasty which is going to affect whether she will even be anaesthetised..

Hey ho - life goes on, I will take it each day at a time, so in the meantime - Happy Mother's day xxx

2011-03-15T23:13:00.000Z

So much has happened between the last post and now, it's difficult to know where to begin, and to top that I am sleep deprived as no suprises we are still in GOS and Daisy is still in pain at night...

Not long after the last post, Daisy began to lose significant amounts of blood from her bottom, confirming my suspicion that a crisis was looming, I was relieved that she was an inpatient on Rainforest and not waiting at our local hospital for a bed to be available...

The endoscopy had been rescheduled because of Daisy's temperature and infection so eventually she was scoped a week later. Not suprisingly the resuts revealed a significant area of inflammation in her large bowel and explained the heavy blood loss. As a result of the scopes Daisy's pain management requirements went up again and she was stated on an NCA pump (nurse controlled analgesia) of ketamine. That was the beginning of our battle to get ontop of Daisy's pain, night times continue to be worse and worse and her analgesia requirements have been going up and up. Morphine was added to the NCA but in typical Daisy fashion she had a really bad reaction to it, despite tolerating IV morphine before. In fact her reaction was so bad I think that day has gone down as one of our worst - she was screaming in pain, inconsolable and desparately uncomfortable as her bowel stopped working for 10 hours and instead of the usual litre of so of output she lost about 10 mls of green bile all day. The pain team and palliative care team were at a loss as to what was going on and although she improved once the IV morphine was stopped she is still continuing to have prolonged episodes of pain at night requiring increasing doses of iv ketamine as well as a range of other intravenous analgesics. The gastro team have called in the oncology team as Costello Syndrome carries a significantly enhanced risk of malignancy and we need to rule this out as a cause of her acute pain, they just don't feel that the colitis, which is being treated with iv steroids, could be the sole cause of her pain.

So where are we now? Well clearly this stay is now in it's fourth week and the weeks stretch ahead of us, when Daisy first had an infection on arriving I predicted that we would not be home by Easter, now I'm worried if that is a bit optimistic. The priority is for Daisy to have an MRI under general anaesthetic in order to rule out tumours causing her pain. The next step is for a decision to be made about whether or not to remove her colon. We don't believe that the colitis alone is the sole source of her pain but it is the source of discomfort and possibly the reason for her frequent high temperatures and infections. It is inflammed at the moment but the bleeding has decreased following a course of high-dose steroids (pulse steroids), however our arguement is what happens if it flares up again and Daisy is not in hospital or able to access the pain killing analgesia that can only be administered in a high dependency setting like Rainforest ward. However the solution is not as straightforward as removing her colon, in order to eliminate any risk of further inflammation and problems her large bowel and rectum would have to be removed, this is major and radical surgery. We know the colon doesn't work and is not performing any useful function while it is defunctioned however is it worth the massive risk to remove it? All this will be discussed at a meeting we hope is being organised between ourselves, gastro team members together with members of the palliative team and other specialities, where hopefully we can come up with a longer term plan for Daisy. On top of all of this we have asked for a Urology review and Endocrine review. We are concerned that maybe a previously undiagnosed bladder/urological abnormality could be contributing to her problems, this is not unknown in Costello Syndrome. In addition we know that while still within normal limits, Daisy's bone density has decreased dramatically and we need the endocrine to review this to ensure that she is not moving into the territory where she is developing osteopenia, the precursor to osteoporosis.

As always our lives are in the hands of others, all we can do is constantly remind whoever will listen that we do not have the luxury to sit and wait while meetings are planned and procedures booked, we need Daisy home and we need these things to happen for us to be home. If the decision is made to perform surgery then this will delay our getting home even further hence the need to make that decision asap, and I don't even want to go down the road of thinking about what potentially could show up during her MRI, we have faced this worry before and it has always proven unfounded so I just keep reminding myself that all the team want to do is eliminate the possibility of cancer because her risk factors are so much higher than a typical child.

Again we feel disempowered - while the wonderful staff do everything possible to make Daisy comfortable with the extreme pain she is experiencing mainly at night, we wait for meetings to be set up and investigations to be scheduled, knowing that our urgency needs to be weighed against the urgency of all the other complicated children who eventually find themselves in this hospital and we just have to hope (with a little bit of tactical cajoling) that the people responsible for being ontop of these things are doing their jobs.

Tonight Daisy is yet again on IV antibiotics, having spiked a temperature yesterday, again it looks like a UTI, confirming the need to get this investigated properly. Her ketamine is switched up to its night time dose to help manage the pain alongside regular extra boluses of the drug if required, she has received her last dose of IV paracetomol for the day and in a moment her nurse will come in with another one of her IV anti spasmodic meds...

I have phoned home to check that the online grocery order I placed on Monday has arrived and there is enough food to keep everyone happy. The children are asking when I will be home and I think I will sneak home tomorrow night so that I can go along to an important hospital appointment for Theo together with Andy first thing Thursday morning and return to the hospital in the afternoon. It means I'm going to have to leave Daisy but the other children need to see a bit of me for a while. I just hope that by the end of this week we have something more resembling a plan and a firm way forward, already in nearly four weeks Daisy has thrown a few too many spanners in the works so the sooner we move forward the sooner we can think about getting her home and getting on with our lives....

2011-02-22T22:50:00.000Z

Well here we are back in our favourite Bloomsbury residence - Rainforest Ward. This is the planned admission we have been waiting for, the one where maybe we will have even more answers and perhaps a plan for more surgery. This has been planned to be the week when Daisy will have a set of scopes to look at her bowel at both ends and she will also have an manometry to look at the function of the small bowel, this hopefully will help the doctors plan how to manage and treat the high losses she gets from her ileostomy and the pain she experiences at night.......

Over the past few weeks her night time pain has just been getting worse and worse, with our symptom care team we have thrown more and more drugs at her, with the result that I am convinced that the reason she is so happy in the morning is that she is high as a kite. Sadly though the drugs are not working on her pain and all we are doing is managing the symptoms and not the cause, which I am convinced is coming from her defunctioned large bowel. Our hope is that this admission will convince the doctors to proceed with putting in place plans to remove this dysfunctional colon, which biopsies have shown will never work, in the hope that it will help with her pain.    However as always with Daisy the best laid plans go wrong, and no-one, least of all the nurses and doctors on Rainforest Ward were suprised when Daisy spiked a temperature last night and became unwell....this has resulted in the plans for this week of tests and two anaesthetics have had to be put on hold, IV antibiotics have been started and Daisy's pain medication has been ramped up again.

To add to everything, the trans-gastric jejenal tube which delivers meds and some milk feeds into the top part of her bowel was accidentally pulled out during her last hospice visit so we are limited to just TPN for nutrition and whatever water she is able to take orally during the day - we had also hoped to have a new transgastric jej placed under anaesthetic during this admission. It is a relief in some ways, at least she was in hospital, and most importantly in the specialist hospital, when she became unwell. I don't need to convince the medical staff how much pain she can experience at night, they know, but it is always good for them to see it so that plans for management and treatment can be formed. I am convinced this is the crisis that we have been waiting for, the pain meds have just been going up and up and while we just keep throwing more and more drugs at her we are not getting to the root cause. My instinct is that her colon has now flared up and is inflammed again, the discharge from her rear end indicates this and she constantly localises pain in the area of her colon, she is really tender to touch there at the moment.

I am almost hoping that now she is in the decision will be finalised about removing her colon, in my mind I am set for a long stay with some sort of surgical outcome. She could also have a surgically placed jejenostomy at the same time as the colectomy surgery which would save further anaesthetics if it came out again as I could replace this type of tube once I was trained. Obviously the downside to all of this is that we are talking about big surgery again with all the risks of anaesthetic, infection, bleeding, Daisy does not recover well from anaesthetic or surgery but it may give her the chance of being out of pain at night and also we would avoid yet another long summer holiday in hospital, something I want to avoid at all costs. The reality is, however, that none of us can go on with the amount of pain Daisy experiences at night, often she has a bruise on her forehead where she has banged it in frustration against the side of the cot in pain, she is worn out and we are worn out and it is no way for a family to live.

In the meantime we have to wait for blood culture results to see if the infection is in her blood stream and also a plan to reschedule her upper and lower endoscopies, manometry and jej tube replacement and then a conversation about the results and a probably colectomy.. nothing is ever done by halves in Daisy's world....

Of course the icing on the cake is that it is half term - yet another school holiday where the family is torn apart. Andy has taken the older three to France for a few days, at least they are all getting some sea air and I look forward to the wine and cheese they will bring back, my hope is that I will be able to take the older children at least to France for part of the Easter holidays, who knows, maybe Daisy will be well enough to join the whole family?    Our biggest wish this year is to have the summer holidays together, and we will make this happen whatever is thrown in our path as a third year of summertime spent apart is just not an option, the children are growing up too quickly for this and we need to be together.

Which brings me to another battle we have been fighting on behalf of one of our other children. I have discussed blogging this with Theo and he is comfortable letting people know as he wants the world to know there is a reason for what can sometimes appear to be his odd behaviour. Theo has Aspergers Syndrome, this is something we had suspected for a long time but a combination of worries about Daisy, hope that it was just a phase or down to anxiety about our situation and a smattering of middle class denial has meant that Theo has only recently had a confirmed diagnosis. The signs were there since early childhood and I am grateful that many of the things that make Theo the amazing, intelligent, funny person he is are in some ways due to the fact he has Aspergers, he is not defined by the syndrome but it is part of who he is. Unfortunately puberty can really exacerbate some of the negative elements of Aspergers and this has resulted in problems in school and home, especially with anxiety and meltdowns. We are now working with our local authority to get Theo a statement of Special Education Needs in order to get him the support he needs in school (things like laptops, extra time in exams), we also are working with an Occupational Therapist, who just happened to be Daisy's first OT, to address some of Theo's sensory issues which can come with the diagnosis - he cannot cope with bright lights or too much noise and has to have time out breaks when he has sensory overload, this has caused him problems in school before where he has unconciously stood up and walked around the class in order to "get his head together" in the middle of a lesson, or rocked back and fore on his chair. He has been suffering from bad migraines for a while now and much of this is down to being overloaded. Andy and I have had a very steep learning curve as we try to understand Aspergers and how best to help Theo, again we have had to become experts very quickly in order to support and advocate for our son. In many ways it has given us a sense of relief, there is a reason for the meltdowns and our parenting style has had to adjust to parenting a son with Aspergers, breaking down instructions is one example, making sure that we stick to plans and he knows them is another.

Of course sticking to plans is not possible when you have a child like Daisy - once again she is in hospital and the plan has gone pear shaped, this makes Theo anxious and we have to manage his anxieties and stress.  And on top of all of this we have two "typical" children in the middle who need time and love and care - all of this is a full time, full on job!

Again, Andy and I wonder how we cope, I guess we are eternal optimists, we have our unwritten rule whereby if one of us is down the other is not allowed to be, and we desparately try and make time for eachother. On Sunday evening we have a babysitter looking after the older three while Andy comes out to the hospital and we will pop out for a few cheeky glasses of red. We also have another project in the pipeline as we eagerly await the arrival Tulip, our vintage, orange VW camper van (called Tulip as she is originally from Amsterdam). We are hoping to have some fun family days out in Tulip and Andy is also looking forward to taking the boys to some campervan meets and festivals. In fact we are planning in our eternally optimistic way to take Daisy to her first music festival in July. We hope to meet up with old friends at Guilfest, Andy will camp with the boys in the van and as our hospice is not too far away Daisy, Xanthe and I will have a bit more luxury staying at Chase for the weekend!

I have set a mental deadline of Easter for this latest glitch to be sorted, I want the summer months to be about family time and fun - barbeques, day trips, cycling - once again, here's keeping everything crossed...

2011-01-20T23:23:00.000Z

I had a phone call at 11pm last night from a journalist asking if I would be prepared to comment on a radio breakfast show about the current story in the media involving the mother of a child with complex needs. This mother had met with David Cameron before the election where he had promised to support carers, she is now at her wits end trying to care for her disabled child (she is also a mother of four) with minimal respite and had posted on Mumsnet that she was considering putting her child into care. The journalist wanted to know what drives a mother to this point......

At the time of the call I was having a "bad Daisy night" (we class this as one where we have to give maximum doses of all her pain drugs over the night) - she was screaming in pain from her dysfunctional colon, writhing in agony and I was in no state to give any sort of sane comment to the press, however there is so much I want to say that I thought I would blog it...

Much as you can try, you will never know what it is like to parent a child like Daisy. Andy and I are fairly stoic, old fashioned in some ways, in that we try and get on with things. It has always been difficult for us to accept and ask for help. We keep smiling and gritting our teeth and muddle through, changing plans, juggling childcare, dealing with situations, to see us day to day you would not know what we have to do just to be up and out and dealing with the world....

Every single night is broken, sleep does not bring rest anymore, a good night is one where Daisy only wakes about twice, a bad night is one where we are watching the clock waiting until we can draw up the next syringe of ketamine or paracetomol so that we don't overdose her. Sometimes her stoma bag comes off or the night bag which attaches to the stoma bag at night detaches - can you imagine dragging yourself out of bed to clear up nearly half a litre of ileostomy fluid (liquid poo) from a child who has just managed to fall asleep, then change her bedding and settle her down? Sometimes she is itching , we don't know why, and itches to the point that she pulls her hickman line dressing off and we need to re-dress it - using sterile techniques, not easy when your eyes are half closed with sleep....

So we get through the night and Andy has to go to work and appear professional and together. I have to deal with our other three children who all need their mother, either to hunt down a missing oyster card at quarter to seven in the morning, find a missing piece of school uniform, deal with a homework ticket which they did not tell me about the night before and make sure they eat breakfast.. By this time Daisy, who was probably screaming and banging her head against the side of her cot in pain the night before is now all smiles and happy, I have to clean her TPN trolley and prepare everything for disconnection, draw up her meds for the day, disconnect her night bag and empty it and wash it out as I am not allowed to have a new night bag for every day for budget reasons...

I make up her milk to the recipe the dietitian has given, nothing is standard with Daisy so I can't get the pre-made bottles of milk other chidlren get. I curse myself each morning that I didn't make up the milk the night before....I run the milk through her enteral milk pump and give her the morning meds and while the TPN is winding down I throw myself in the shower while shouting to Jules to make sure he has his school bag ready.

I draw up the line locks we use to prevent hickman line infections and then I disconnect her TPN and lock the line. I dress Daisy in her school uniform, she can't do this for herself, put her leg splints on or if I am feeling lazy just her special boots with the retaining straps....she should wear the splints in order to delay the need for more leg surgery but they hurt her and sometimes there is just not enough time to get them on her. All the time I am looking out of the window praying the school bus won't be early so that I can do battle with her hair. It may seem a facile thing to worry about Daisy's hair with all the other things going on, one of the way Daisy's gene mutation and early failure to thrive have affected her is that she has very sparse, coarse, brittle hair, it knots easily and if it is not brushed and oiled daily forms into dreadlocks. So this is the part of the morning where I am not being a nurse, I am being a mum, brushing her hair, putting ribbons into it, anything to make it look tidy so that she is like any other 6 year old school girl, and also avoiding the need for me to cut great matted chunks out of it...

At 8am I emerge from Daisy's room and carry her downstairs. We have to lift and carry Daisy up and down the stairs all the time, always with one of her pumps connected. We have been doing battle with Social Services Occupational Therapy for the past 18 months to get a solution to how to get Daisy up and down stairs, at least we have moved on from the early days of our battle when they sent a man around to fix a bannister to the wall having never even met or assessed our child, needless to say he was sent packing. Today we were told we will hopefully get a stairlift to move her up and downstairs, however we will have to wait a while until new budgets are agreed later this year. I just hope that my back holds out between now and then and that I don't fall downstairs while carrying her......

I put Daisy into her special needs buggy, scribble an update in her home school diary (should have done that the night before too) and then help wheel her onto the school bus. I slurp down a cup of tea (often made but one of my children, they are well trained!) and then Jules and I leave for school.

Very rarely (infact I can only remember 2 occasions) I have gone back to bed for a sleep after the school run, there is always so much to do once the children have gone to school, starting normally with restoring Daisy's room back to a fit state to connect TPN later in the day. The new bag of TPN has to come out of the fridge and the bedding needs to be washed, some days there will be three loads of Daisy's bedding to wash - and I make the bed up in several layers so that I can peel layers off at night to save time!

Every Monday we get a delivery of TPN and ancilliaries, I need to order stoma supplies, enteral feeds and supplies get delivered, repeat prescriptions of meds, . I wish our PCT would just deliver full boxes of enteral syringes to us like friends who live in other areas. Instead we have to contact the homecare nurses when we need new syringes and they will leave a bag in the reception of outpatients if they are not passing our way in the next few days. I am then expected to reuse these syringes several times in order to save money, so more washing out and sterilising to add to my daily list....

The appointments are endless....eye hospital, audiology, orthopaedics, speech and language, local consultant, gastro consultant, cardiology, TPN consultant, ultrasounds, ecgs...... Thank goodness she is now in school and the physio can go and see her. The phone calls never end and the paper work is out of hand...forms for everything. Every year we have to reapply for school transport even though her needs will never change. The Disability Living Allowance form is probably the most soul destroying thing I have to do, and up until last year I was having to fill it in annually even though Daisy's condition will never ever get better....It takes days to fill in the form and it is a depression inducing exeercise to describe on page after page the worst and most negative aspects of your child's life when all you want to do is be positive. But you can never be positive when filling in a DLA form, one positive sentence in error, one hopeful comment and you could have your allowance stopped while they investigate. Like my poor friend whose daughter spends her night on oxygen and is regularly in and of hospital, on a really good day she may be able to manage the walk to school (but not back again), this was interpreted as "can walk to school" and her DLA was stopped, including her entitlement to a mobility vehicle while it was investigated, needless to say at appeal it was reinstated and back dated, but at what cost to the poor family who struggled in the interim.

Some days I just don't want to face all the admin and rubbish at home so I go out for a long walk or meet with friends and just for a few hours I become the person I should be. I'm not Daisy's mum or parent of disabled child trying to negotiate the system, I'm me, trying to have a life.... We have to have such broad shoulders becuase inevitably while our partners are at work we are the ones dealing with the officials and bureaucracy to make sure that our child gets the childhood they deserve. We are not trying to ask for more than we should, just a chance of a level playing field for our children and they need so much more than a typical child. And for me? I've stopped dreaming of going back to work, it's just no longer possible but how nice would it be to go somewhere where I wasn't Daisy's mum and having to use medical speak all the time... I would like an indoor wheelchair for Daisy so that she can participate in family life, so that her siblings don't have to lift her and so that she is not confined to her bed once her TPN is on, the problem is these chairs cost around £2,000 (nothing is cheap when it has the label special needs) and no-one can agree whose budget this should come out of, so while the grown ups are arguing we are left struggling or resorting to using the outside wheelchair inside the home..

I am very lucky in that Daisy has a fantastic key worker who has known us since she first came out of the neonatal unit. She has been our advocate and ally over the years and has fought to get Daisy a package of support, this has taken a long long time and now I'm informed that we have the biggest support package in the borough, and this is what it is - 2 funded nights a month at our hospice ( it went up to 2 nights in November and is only until the end of March when it will go to panel) and direct payments to enable us to buy in care. Bearing in mind we live in the most expensive city in the UK this money does not stretch as far as it would in other areas. However I am able to pay for an after school nanny (plus her tax & insurance) to come to the house from 3pm until 6pm, about twice a month a very good nurse friend comes to the house to babysit so that we can either take the other children out to do things we can't do with Daisy like go to the cinema, or go out ourselves. We are lucky, it is a very good package, but actually it's not respite, when the nanny comes to the house her job is to do the job I can't do because within 30 minutes of Daisy arriving home I have to put her TPN on, so she is talking to the children about their day or cooking their dinner while I am upstairs getting Daisy ready for the evening. She spends around 16hours a day connected to the TPN pump (when she is not on this she is on her enteral pump) so then she is limited to being in her buggy or cot. It is great to have another pair of hands to help even though she cannot do anything medical for Daisy but as Andy is in work and I have no family within 300 miles it is the next best thing.

Evenings are spent running up and downstairs to Daisy, adminstering meds and hoping she will sleep, which of course really doesn't happen...both Andy and I have fallen into a habit of staying up late, mainly because there is no way we can actually have an early night. We live for the nights when Daisy can go to the hospice, it is the only proper respite, I love my daughter desparately and will do anything for her but the only way I can get a break and recharge my batteries is when she is not in the house. We are so lucky to have Chase and to have such a long relationship with them, Daisy has been going there since she was only a few months old so it very much is like a second home. We had to fight and fight for social services to pay for funded, guaranteed nights at Chase Hospice. We had to prove there was no-where and no-one else who could manage her care and needs, Daisy has had her condition all of her life but it took until she was 6 years old for us to get 2 funded nights a month where we get time off from looking after Daisy. And this is where I can understand the mother who was considering putting her child into care, I doubt highly that she would have acutally done it but she probably woke up one day after night after sleepless night and thought, I've had enough of being trodden all over by faceless nameless people in committees and teams and panels who are playing God with me and my child's future. If social services were to look after Daisy full time it would cost them thousands and thousands more pounds than they currently pay to have me stay at home to look after her. However I am never party to any of the conversations or reviews or discussions, I never have the chance to present my child's case or represent her needs, I have to rely on others to do this.

My sense is that things are getting harder for us parents of disabled children, budgets are being stretched, care packages are being scrutinised, time delays are increasing all in the name of spending reviews and cost savings and all while our children are being denied the childhood they deserve. I had hoped that our Prime Minister would understand, he has walked in our shoes, he said so during his election campaign, he specifically singled out the cause of unpaid carers during one of his TV debates....I guess we can just live in hope that he will remember his promises.

2011-01-11T22:29:00.000Z

Sorry if you thought I had disappeared for a while - it really is so difficult to find the time to sit down and put my thoughts down, I find myself relying on quick facebook updates to stave off the inevitable texts which arrive when I have not been in touch for a while...so, where were we - December....

Daisy has continued to have problems with her repaired hickman line, sometimes itching so much she pulls the dressing off, this is not a good thing. A couple of times it has looked very red and infected but cultures have been coming back normal, and bloods are OK apart from Daisy's ongoing problem with chronic anaemia, despite nightly intravenous iron. The hickman line is Daisy's lifeline, without this access she cannot get TPN and would not be able to live so I am worried that things are not right with it - you get to know your child's line after a while and this one does not feel right....Daisy is having a Lineogram at GOS next week to see if there is a tiny tear under the skin which is causing the irritation. It's a difficult one, I hate have having to depend on a line which already has a repair but at the same time a new line would further limit her line access options as we cannot use previously used sites of which there are many, and as we all know Daisy is not good with anaesthetic and line insertions (an interventional radiologist told me once that her veins are very wiggly, imagine that, all those years of medical training for a diagnosis of wiggly veins).

Line worries aside the main thing we continue to be concerned about is Daisy's pain, we had to up her pain medication over Christmas and use buccal ketamine about 5 nights out of seven. We all live with Daisy's pain on a daily basis - in fact during the day she only occassionally experiences pain, night time is another matter and she becomes a different child to the one everyone knows. Our other children rarely have friends over for sleepovers and we only ever invite "understanding" friends and family over to stay because often the household is woken up by her screaming. We continue to wait on a date from GOS for a manomentry and other tests to see if the high output from Daisy's small bowel can be managed, I have my doubts....when we do eventually go back in , Andy and I will ask for a second opionion referral to Birmingham Children's Hospital where they have made a lot of progress with children with intestinal failure. We have been told that transplant is not an option, for various reasons, from the risks posed by her Costello Syndrome to the concerns that the pain may remain despite a new gut, but for Daisy's sake we have to leave no stone unturned. In the remote possibility that Birmingham thinks that transplant may help, Andy and I are not sure that we would even put Daisy through that, but we need to at least have explored all options even if it does confirm once and for all that the only option is life on TPN.

Another option we do want to explore is the possibility of removing Daisy's colon entirely. The biopsies have shown that it will never work properly and is the likely culprit of the pain she experiences, even though it is defunctioned it still gives her grief with daily mucous and bloody discharge from her bottom - again the worry is, what if this does not help, it's a big surgery to put her through but hopefully one with less long term risks. Of course all of these conversations depend on us getting back into GOS for our inpatient stay or at least moving Daisy's outpatient appointment forward. However over the years I have learned not to burn any bridges and to be patient, Daisy's gastro consultant is the best in his field and he has always done the right thing for Daisy so I will try and be patient while we wait for the call....

Of course since my last post we have celebrated Daisy's 6th Birthday, Christmas and New Year - all of which have been significant dates in life with Daisy to date - this year was probably the best yet - no hospital, she was well and there was even snow! Daisy loved the snow, especially sledging on the common opposite our house with her big brothers and sister, however it did put paid to our plans to have a proper birthday party for her as everyone was snowed in. In the end we had an impromtu party with local friends and Daisy's wonderful carer managed to get to the house (probably because she is swedish and not phased by a bit of snow) and did a magic show for the children.

Daisy loved Christmas, especially all her Singing Hands dvds and her ELC Happyland Village. Daisy knows Singing Hands from many months spent in GOS and now appears in one of their DVDs, we bumped into them while visiting Chase just before Christmas and Daisy was beside herself with excitement. Singing Hands are probably the main way Andy and I have learned Makaton, Daisy's signing system so we were pleased they have produced a Christmas DVD so we can sign along to I'm dreaming of a White Christmas,....

The start of this year feels more optimistic than previous years - in a way it shouldn't, Daisy's gastro problems are the worst they have ever been, her TPN requirements keep going up and her stoma output remains at an average of a litre, her pain meds are being increased regularly, but cognitively she is coming on in leaps and bounds, she vocalises more and more, shouting for her brothers to come and put DVDs on, or demanding help with games, she is growing up in so many ways and shows us daily how capable she is. In many ways we now know what is happening with her gut, TPN is forever and the stoma is permanent, we can live with that now. Tomorrow the palliative consultant is coming to visit to adjust the pain meds and add a new one in, hopefully we will get some answers from the gastro consultant soon about colectomy surgery and second opinion referrals, so more than ever before there is a plan and we know what is happening.

My daily battles still continue trying to get support, equipment and respite help - it is these things that wear you down, I know what Daisy and the family needs but sometimes we have to jump through so many hoops to get to that point, and I also think that living where we do doesn't help, there is a postcode lottery element to support for children with disabilities. And beyond these battles we have more mundane issues like the leaking sewer pipes underneath our kitchen floor....at the moment we have no kitchen, all of Christmas was spent with us burning scented candles to hide the smell, hopefully the work will be finished by the end of January and we will have a shiny new kitchen and floor and no more broken sewer pipes...hopefully my sanity will be intact as I fill kettles in the bathroom and washup in the bath....Daisy is oblivious to the chaos of our lives, school is the most important thing in her life, followed by Singing Hands and then her siblings who are at her constant beck and call....she is surounded by love and happiness and that is why, whatever it brings, 2011 will be good for us...

2010-12-01T23:56:00.000Z

Six years ago this week I was admitted to hospital for steroids to strengthen Daisy's lungs in preparation for her early arrival. Up until that point we were a family of five, Theo was 7, Xanthe 5 and Jules 2 and we did normal family things - went on holiday, got babysitters in, did usual Saturday activities, ballet classes, football - all that changed at Daisy's arrival on 22nd December. She was born by caesarean section, my first, (she was supposed to have been a home birth like her brother before her!) and our first Christmas as a family of six was spent with me recovering in hospital, Daisy in intensive care and Andy trying to keep the children's spirits up at home.

Much as our family feels so complete with Daisy, life has never been the same since her birth. In many ways we have changed for the better, we don't sweat the small stuff and have become incredibly adaptable. At the same time however we constantly change plans - trips and holidays get cancelled at the last minute, birthdays are celebrated in hospital rooms, the children miss out on treats and during long hospital stretches the family is torn apart and they rarely get their parents in the same room at the same time...

So the trip to Florida was so important in so many ways - mainly because it was a big opportunity to give the whole family a time out, an opportunity to be a family and most importantly to have some fun!

And, boy did we have fun!!! We started off our adventure at the Gatwick Hilton where we met the other families and Caudwell Staff, Volunteers and Medics joining us on the trip - bless her, Daisy thought that was Disneyland - she was beside herself with excitement.

The flight was not too bad considering Daisy had her TPN running and needed her stoma bag emptying every hour -

We stayed at the Give Kids the World Village. A wonderful place where life limited and terminally ill children can enjoy a week away in the Florida sunshine. Our villa was like something out of a storybook, but for me the best thing was that everything was all on one level, no stairs, no lifting - fantastic.

Since Daisy arrived nothing suprises us anymore, so we should have predicted that we would meet someone we knew while we were away. On our first morning at the Village a family came into breakfast with a little girl who was Daisy's double, there was no doubt she had Costello Syndrome (the hands are a big giveaway), amazingly Cintia Cuperman, Valentina's mum, and I have corresponded online. They were leaving as we arrived but not before we took some pics of the two girls together in their Minnie Mouse ears.

While in Florida we visited Disney's Animal Kingdom, The Magic Kingdom, Hollywood Studios, Universal Studios and Seaworld - the whole experience was truly out of this world. As a family we had never ever done anything remotely like this, the nearest was a couple of trips to Legoland, so we just embraced the magic and suspended reality! Andy and Theo enjoyed the rides, Xanthe enjoyed the shops, Daisy loved meeting all the characters and getting their autographs and Jules just loved everything. There were so many highlights, shows, parades, fireworks, characters, rides, experiences....words just can't describe how fantastic the holiday was. We all laughed so much and had so much fun, we also realised how little time we had spent together as a family unit over the past two years especially and it made us determined to try and make sure we get more time out together.

Not only did we have the whole theme park experience, staying at the Give Kids the World Village was an experience in itself - icecream for breakfast, the wish fairy visiting our villa each day, the playground opposite the villa, the train that Daisy got to drive and the most special thing, Daisy's star being added to the stars placed by thousands of other children in the Castle of Miracles.....a little piece of Daisy will always be in Florida now.

This whole trip was down to the wonderful, wonderful people at the Caudwell Children's Charity. On our journey with Daisy we have met some very special people and the Caudwell team are right there at the top - we know Daisy is a complicated child and without Caudwell there is no way we could have made the trip as a family from a practical, financial or medical aspect, but not only did they bring Daisy out to Florida, 22 families were on the trip, all with highly complex medical needs - nearly half of the children were in power chairs, some had ventilators, Caudwell does not shy away from making sure that the most complex children get to experience the magic of Florida. Sadly one young lad passed away while on the trip bringing home to all of us parents how fine a line we tread and how lucky we were to have made it this far...

It has been a few weeks now since we have been home and I still feel I have Florida sun for energy, I needed it last week when Daisy's hickman line broke and she had an odd allergic reaction to her TPN calling for a Saturday night sprint to A&E and a few days in hospital.

All around the house are reminders of our trip - Micky Mouse toys, photos, souvenirs .....the magic of that experience has got under our skin and changed our family. We have beautiful photos where all four of our children have smiling happy faces, at long last we have photos with all six of us in. Give Kids the World even arranged for Santa to visit while we were there......but the best thing - we got to meet Mickey and Minnie!

I can honestly say my batteries are now back to full charge - the Caudwell Charity gave us the most wonderful gift this year, the chance to be a family again, we are so grateful to them .

2010-11-03T13:40:00.000Z

Well we lasted six whole weeks out of hospital, and I guess all good things come to an end as by week six Daisy was spiking temperatures and clearly unwell so back we went to A&E and we revisited the whole rigmarole of fluid boluses, IV antibiotics and juggling.....I had hoped to take the older 3 children to France for half term while Daisy stayed at Chase hospice, another plan shelved for another time....This admission was for another infection, caused by the bacterial overgrowth in her gut, this time however the bugs have become resistant to one of our usual antibiotics, Gentamicin, inevitable really but now limiting our options further. The other problem was that Daisy's stoma was pouring out fluid and so she was becoming more and more dehydrated. Her TPN has been increased even further and she regularly receives extra IV fluids ontop of this to make up for the losses from her ileostomy which are draining too much sodium and potassium from her. Having hated maths at school I frequently find myself having to calculate fluid balances and top up fluids to replace losses..

Our consultant and Great Ormond Street has decided that Daisy needs to come in for another stay - he feels that Daisy's high stoma output is caused by it being in overdrive, like a driver with their foot on the accelerator, conversely the pain and discomfort she feels from her defunctioned colon is because it does not move at all, like the handbrake is on. He wants to assess if the small bowel is permanently in overdrive in which case there may be a drug based solution to help or whether this pattern varies between too fast and too slow, in which case there is not a lot that can be done. We asked if this stay could be put off until the new year , there is too much happening in our lives between now and Christmas and we want to enjoy family time away from hospital. The main event being our trip to Disneyworld, Florida...

As I type I am surrounded by cases, lists and a milllion and one other "essential" things we need to take. We leave later this afternoon to stay at the Gatwick Hilton where we will meet the other families going on the trip and most importantly, the medical team. I have a spreadsheet of TPN connection and disconnection times taking into account time differences and Daisy's increased fluid needs, we have lists and letters coming out of our ears to help us pass through customs. Tomorrow morning we will meet our TPN delivery driver at the terminal and he will hand over all the TPN we need for the holiday, plus extra bags of fluid, ancilliaries and all the medical stuff we will need just in case....needless to say our personal packing has been kept to a minimum (which is fine because we'll have plenty of space on our return journey for all the souvenirs we anticipate bringing back).

When the chance to go on this holiday was first offered to us, my instinct was to think that there were more deserving cases than ours, sicker children...but looking at our lives I forget how much Daisy and her siblings have gone through not just since she has been born (and for the weeks when I was hospitalised before she even arrived) but particularly in the last two years...she has intestinal failure, is TPN dependent, has a high output stoma regularly requiring extra fluids, because of all the issues and risks associated with her costello syndrome (malignancy, heart problems) she is not a candidate for a transplant of small bowel...this is the end of the road as far as any treatment goes, it's all about management of symptoms. And the older children, two long school holidays in a row spent in hospital, every half term in the past two years, holidays, birthdays...so many times we have had to change plans - they really deserve this fantastic chance to have time together as a family having fun...

watch this space for photos!!!!!

(PS - the house will be occupied in our absence by the way!)

2010-10-05T21:31:00.000+01:00

We have reached a big milestone...I was so busy getting on with life I didn't even notice that this is the longest we have spent out of hospital since 2008, our average prior to this being 21 days between stays. Much of this is down to the fact that Andy and I now can do so much of Daisy's care. Situations that would have ended up in an admission now are managed at home.....how far we have travelled that administering IV fluids, taking bloods, changing ileostomy bags are now part of our daily routine.

This life is so normal for us - each morning I spend an hour in Daisy's room,disconnecting TPN, changing bags, taking bloods all the time checking out of the window to see if the school bus has arrived. Sometimes it arrives early and throws me, sometimes when it is late I get a chance to gulp back a coffee before taking Jules to school. The capacity of the human spirit is amazing, only very occasionally I step back and look at my life. There was a time when we were first out of hospital post-ileostomy when I thought, how can I keep this pace up? But I do, and the days go on and before we know it we create a normality out of this madness.

I never cease to be inspired by the other parents I meet along the way, parents who like us swing between hospital and home and try to make a normality out of this world. This week was sad, two mothers I had come to know over the years lost their children. One had spent years fighting the cancer that can often come with Costello Syndrome, the other saw her child slowly and painfully decline with Huntingdons Disease, but when I think of these mothers I don't think of their struggles, I think of how they embraced every moment they had with their child and how those children packed a lifetime into their short lives. This is what we are trying to do, not mull over the whys and wherefores but just get on with life and pack as much as possible into the time with have with Daisy. So, in November the Nimmo Family will be travelling courtesy of a wonderful charity to Disneyland - we are going to have fun and live life to the full and create some fabulous family memories and not worry about the future, that's for another day......

In loving memory of Micah and Brodie
(and Craig, Bret, Willa, Zachary and all the children who have left this world too soon - RIP)

2010-09-26T23:03:00.000+01:00

Gosh, it has been so long since I have posted, and as always as a family we have adapted and changed to accomodate Daisy's ever changing needs. As I sit here now, Daisy is on a lot more TPN than previously, with her infusions running from 5pm, not long after she is dropped home by the school bus, through to 8am when she is disconnected in time to go onto the bus. She continues to pour around a litre of fluid out of her ileostomy over a 24 hour period and we continue to connect a catheter bag to her ileostomy bag overnight to prevent the output breaking down her skin. We continue to battle with haemoglobin levels and seem to constantly teeter between needing a transfusion and her hb bouncing back up again....life has become even more complicated but after a few weeks at home now we find ourselves adapting to our new routine like it has always been like this!!

Daisy came out of hospital at the begining of September, around 2 months after she was first admitted for surgery - as always her stay was not without complications including a dose of parainfluenza! She also developed a rare side effect of ileostomy surgery, diversion colitis, where the remaining segment of colon which has been defunctioned became inflammed causing her to bleed from her bottom. The treatment for this was twice daily Short Chain Fatty Acid enemas, which were both difficult and distressing to give. The enemas were stopped but unfortunately since coming home Daisy has had a recurrence of this and we are awaiting an outpatients appointment at GOS. It is possible that Daisy may face further surgery next year to remove her colon as it is still causing her a lot of pain and distress and we are now having to give her maximum doses of gabapentin as well as oral ketamine at night to help manage this.

Our saving grace has been the palliative care team, both at GOS and at our hospice Chase - they can't cure Daisy, she is incurable, but they can manage her symptoms and her pain so that the time she has with us is comfortable. We are so lucky to live where we do, there are very few paediatric palliative care teams in the country and we have access to the best. Our joint mission is to maximise Daisy's quality of life and alleviate her pain. Sadly, the pain which we hoped the ileostomy would alleviate is still very much there but with the help of our team we have access to the best expertise possible to make it better.

Daisy started back at school as soon as term started, as always this is the most important thing in her life, so it was important to get her back as soon as possible. Her new one to one support assistant started and has instantly fallen in love with her - who wouldn't! It was nice for me to discover that she knows many of the mums in our area too. Daisy is now in year 1 and is in a group following a very specialist curriculum being pioneered in the school to maximise potential in children with multi-sensory disabilities. She is already more and more vocal and proves to us day in day out how cognitively able she is.

At the beginning of September was a UK meeting for Costello Syndrome run by the UK branch of the charity which supports children with this syndrom. Our good friends, the Deckman Family, who have a teenage son Collin with CS came over for the conference and it was great to catch up with them for a day at our house. Our children have met Collin before so it was a great reunion for them all playing together. We attended one day of the conference with Daisy and met up with other families we had only met online and well as our friends the Stickley and their children including their daughter Rebecca who is 6 months older than Daisy. We spoke with some of the geneticists and researchers who attended the conference and shared Daisy's biopsy results and they confirmed what we had always suspected - Daisy is completely and utterly unique. The problems in her gut have not been seen before in our group of children, they do not know why she has these problems and why she has so much pain. We know believe that without Costello Syndrome it seems likely that Daisy would have had intestinal failure - she has been hit with a double whammy and it serves to make us all the more isolated as we do not belong fully in either world. Daisy shares a gene mutation with the other children with Costello Syndrome but no one else has TPN and ileostomies to contend with. These things Daisy shares with other Gastro families we know, but none of them deal with the other Costello things like the cancer risk, heart problems, growth hormone deficiency, visual impairment and orthopaedic issues like we do.

A week into the new term, Daisy had a respite stay at our wonderful hospice, Chase, in Guildford. As her needs have changed so much I stayed with her for the first 24 hours and Andy also spent a night there, we did have a couple of nights when we were home alone. This really allowed us all to rest and recharge our batteries knowing that Daisy was completely and utterly happy in Chase. She is going back in again for the October half term and we are planning to take the other three children to France, where they are going to practise their french!

Since being back home from hospital I have continued on my mission to declutter the house and get to grips with the paperwork. Many people just do not realise how much paperwork comes with the territory when you have a disabled and medically fragile child - forms for this, applications for that, appointments, letters, info - all of it has to be kept, filled in, dealt with, it's a full time job the cupboard where I have been cramming the paperwork is now officially full so I really have to deal with it! Ontop of that we now have four children in four different schools in four London boroughs all generating their own masses of letters, emails and texts - it's really overwhelming.

My life is a full time managment job, I definitely did not work as hard as this when I was working full time, even though I had three children at the time. Hence the long time taken to update this blog - every day is full on and Daisy's latest problems mean that an already full workload is now even fuller - I am counting the days till the next respite break at Chase just so someone else can take over for a while and Andy and I can get a break. Roll on October!

2010-07-31T16:45:00.000+01:00

Three weeks ago today Daisy had her ileostomy surgery and as we all know, three weeks is a long time in our world……

The surgery itself went according to plan, the anaesthetist spent over 90minutes going through everything about Daisy, checking her recent echocardiograms and preparing a plan for her pain management post surgery. Daisy, like many children with Costello Syndrome is not great with anaesthetic and this is where Great Ormond Street Hospital comes into it’s own. She was in theatre for about 3 ½ hours but spent longer than usual in recovery mainly because she developed an allergic reaction to one of the anaesthetic drugs and her face and lips were swelling up. She came back to the ward on morphine and ketamine in a pump and stayed on this for over a week.

Immediately post surgery the stoma prolapsed which means that the intestine was pushing out further than it should. So having looked a pictures of stomas on the internet to ready myself my first sight of Daisy’s stoma was not quite what I anticipated, infact it looked like a six inch sausage of intestine hanging off her abdomen.

Another trip to theatre loomed but fortunately the prolapse corrected itself and now at least I know the difference between a prolapsed stoma and a normal one. I also had not anticipated that Daisy would have a large scar from her navel to her pubic bone where the surgeons had opened her up to take out a piece of her colon. This scar infected and we have had a three week battle getting it to heal, not a great task when you consider how close it is to a stoma which is pouring out bowel contents…Being immunosuppressed slows the healing time and this has meant that the infection in the surgical site has been very slow to heal.

After a week we were told the results of the biopsies that the surgeons had taken. Dr Lindley our Gastroenterologist is a leader in his field of motility disorders and neuropathic bowel disease, however he has never seen a disorder of the nerves like those in daisy’s colon. His words were that we should have done the ileostomy a year ago and it was the best thing that we could have done. It has been bittersweet having the proof that Daisy’s colon is not formed properly and has never worked, she was born this way and all along Andy and I have said that we did not feel that she would grow out of her gut problems, our instincts have been proven right. It also confirms that she has been experiencing severe pain in her gut and we are so grateful to be under the Symptom Care team, working with them to manage Daisy’s pain. The problem now is that the ileostomy is permanent, the hope that by resting the colon it would get better and the ileostomy could be reversed has gone. Daisy’s colon will never work, we still don’t know whether it is part of her Costello syndrome that has never been seen in another child with the syndrome or whether it is something she was going to have regardless of her syndrome, the important thing now is that the ileostomy has to work – and this is now our biggest battle.

Normally an ileostomy output (of poo basically!) should be around half a litre – at the moment Daisy’s ileostomy is pouring out double and some times three times this amount and it is just watery in content (sorry but Andy and I are now becoming experts on stoma bag contents and this is our daily conversation, we’re immune to the grossness of it!!!). Daisy is still on 24 hours a day TPN and we have started a tiny bit of milk – the equivalent of 1 tsp an hour – this just pours out of the stoma looking the same as when it went in!

The doctors initially started treatment for bacterial overgrowth and have found some bacteria in her small bowel (the only bit that is left and working) that would normally only be found in her colon, however they are still scratching their heads as to why Daisy is losing so much fluid, their strategy is to wait and see and hope. Losing more fluid than goes in also makes her dehydrated very quickly and lose essential electrolytes so she has to have daily intravenous fluid top ups ontop of her TPN. Add into this the intravenous pain medication she needs daily and that means that there are four pumps she is attached to at any one time.

Because her stoma losses are so watery they were leaking from the stoma bag and reinfecting the infected surgical scar, so the wonderful stoma nurses have come up with a solution involving a urostomy bag (normally used for draining wee) hooked up to a urinary catheter – so ontop of the pumps she has a big catheter bag we have to manage. This means we are pretty much confined to her cubicle all day and all of us are slowly going slightly mad…..

Of course it is the school holidays now. Thankfully we anticipated a long hospital stay so some plans are in place for the children, most of which involve Andy or I picking up or dropping off a child on a weekend. While Daisy’s stoma refuses to work properly or absorb any feed then we are here pretty much for the long term. We also need to be here at the moment to get her pain management regimen fine tuned – now she is on maximum gabapentin for her age, daily IV paracetomol and ketamine when she needs it. We have not seen the hoped for improvement in Daisy’s pain at night, infact if anything she needs more medication than before but at least we have a co-ordinated plan driven by our symptom care (palliative team) from both this hospital and Chase Hospice.

Family life has still gone on over the past three weeks. Xanthe said goodbye to St John Fisher School, the second of my children to progress to high school. I vividly remember the day I took her to nursery, all pigtails and smiles and now she is growing up into a beautiful girl, with her fair share of pre-teen hormones! She amazed us in her school production of A Midsummer Night's Dream where she wowed the audience with her performance as Bottom, clearly she has the Nimmo acting genes. The children are all visibly more relaxed now school has finished and the holidays have begun, even if once again they are spent in different locations it is nice for them not to have the added pressure of early mornings and homeword deadlines to contend with. I hope I will get a chance before the end of summer to do the dreaded new school shoes shopping run and get the last bits of high school uniform for Xanthe before she starts the next chapter of her life as a St Philomena's Girl.

Daisy had a brilliant report from Linden Lodge and will be progressing to Year 1 when (emphasis on when) she returns in September, she also had a great report for her piano playing, and has suprised us all with her musical ability!

So we muddle through again, and make the best of things, I am so grateful that at the outset of our journey with Daisy Andy and I had a strong marriage as we have been tested time and time over, our children are growing into lovely, intelligent , self sufficient young people and at the tender age of 7 even Jules can made a cup of tea for his mummy!
We will just have to wait and go at Daisy's pace and hope the ileostomy begins to behave and function as it should. In some children, without the complications Daisy has a small bowel transplant can be an option to get a child off TPN however, while this has not 100% been ruled out it is highly unlikely that she will be accepted for transplant or do well with the surgery so TPN and an ileostomy is our only option. With gastro children there are often no magic fixes, what works with one child does not work with another – the mantra is always the same, one day at a time, we just have to wait and see if this ileostomy decides to work and hope it makes the difference we are looking for….

2010-07-08T00:00:00.000+01:00

Well it turned out that a weekend in Glastonbury was just what I needed to recharge my batteries and prepare for Daisy's impending surgery. I had spent the best part of a year trying not to look forward to it incase it didn't happen so couldn't actually believe it when I did get there. There is certainly something magical about that part of Somerset and it worked it's spell on me, I left my watch at home, switched my mobile off and moved to Glastonbury time. Those in the know, know that Glasto is more than just the headline bands and my best memories are just of chilling listening to some amazing music and eating some lovely food (and partaking in one or two somerset ciders!). We were blessed with the weather and the extra money I paid to camp in a location with decent loos and showers reaped dividends. All in all it was a wonderful opportunity to just pull myself together again, relax and unwind. I got to spend some precious mum and daughter time with Xanthe too which was a real bonus. I just can't wait to go to Glasto again next year, it's cast a spell on me... (and I can't wait to thank the nurse on Rainforest ward who told me over a year ago that I should apply for tickets as I would love it).

So since Glastonbury my time has been filled getting ready for Daisy's impending ileostomy surgery...sorting out a complex childcare spreadsheet for the summer has been the main challenge. It is not easy making sure that children of different ages and different genders all get to have a fun time while their parents are either in hospital or working. I have evolved my plans on the assumption that nothing will go according to plan and Daisy will do another long term stay, it does seem to involve Andy or I spending a lot of time driving down motorways dropping various offspring at various relatives/holiday camps at different times in the summer - just hope they appreciate how much planning has gone into them having fun!

I have also been getting everything ready for Xanthe's big transition to High School. When Theo went up to his high school little did we know that a few weeks in Daisy would go into hospital and not come out again nearly 12 months later. This time I want to be better prepared as it really impacted him. Now instead of after school clubs we have a lovely after school carer who comes to the house and can turn her hand to looking after Daisy or one of the other children if needed. I have also made sure that Xanthe's new school is aware of the situation so that if things get stressful at home they are aware of it. Having a child like Daisy has a ripple effect on the entire family, we plod along then suddenly something happens like a tube being pulled out, a line infection, a UTI and lives get turned upside down, We all become used to this rollercoaster but over the years I have realised that we have to put some safety nets in place and not assume that we will all be able to ride the storm.

I have a realisation today also that when you have a child with a complex medical disability you become disabled as a parent/carer - or differently abled. I have to create a new persona and take away a bit of me in order to operate in this world of disability. I am now Daisy's mum, or just "mum" as the doctors and nurses call me (not all of them, but enough for it to jar). I have to be nice to people in order to get things for my daughter, I have to deal with people who I don't necessarily like, I have to be diplomatic and swallow my tongue, I have to know my place. The other day our community nurse dropped around some medicine syringes for Daisy, it was only that evening when I was drawing them all up I discovered that one of the syringes she had dropped off was the wrong type to connect to Daisy's jej tube. I was planning to visit a friend who was in our local hospital with her daughter that day so drove up and asked a nurse if I could have some syringes - two were produced together with a telling off that I shouldn't get them from the ward but should be getting them from the community nurses. It was only afterwards when I mulled over this I realised the situation I was in, here I was a highly educated woman who had once had a successful career, now trained to care for an TPN dependent, medically complex child, having to beg for syringes and being told off for asking the wrong person....This is the situation many of my hospital friends find themselves in - we are expected to do so much for our child but we are so isolated - I am friends with the nurses on a superficial level but I am not a nurse, I can talk to the doctors about Daisy, but I am not a doctor, I can work with social workers, occupational therapists, speech therapists and all the multitude of people who seem to have a say in my child's life but the power is with them. I am dis-abled because I have had to become a different person in order to get my child what she needs. So next time, please give me the sodding syringes, in fact give me ones that fit her tube so that when at the end of the long day I am drawing up one of the 20 odd meds she needs during the day I have the equipment at hand I need to do the job I need to do.....

So now as I type it is the night before the hospital admission. Tomorrow Daisy goes back onto Rainforest. I phoned twice today to check the bed is available and so far so good although I have not packed at this point (I am an expert hospital packer now so not too worried about doing it at short notice). Tomorrow we should be talking to the surgeons, anaesthetist and pain team in anticipation of the surgery on Friday. Daisy's pain med which had lulled us into a false sense of security a week ago has stopped working so Andy and I know that this is the only route to go down to hopefullly alleviate the night time pain Daisy experiences. Our wonderful palliative care team at Chase have visited and there is a plan post op so now the only thing left is for us to get to the hospital and for Daisy to have her surgery. I hope and pray it will make a difference, I am realistic enough to know that things will probably get worse but maybe we will turn the corner over time and not regret our decision....

2010-06-23T22:44:00.000+01:00

So much happens between posts, it's no wonder I don't find the time to update as often as I should. So, since the last post Daisy had a two week stay courtesy of the rheumatology team for rehabilitation (!). The children thought it was hilarious that Daisy needed rehab, but I had to explain that this stay was to retrain and rebuild her wasted muscles with the aim of keeping her walking and mobile and ultimately not confined to a wheelchair. I had been apprehensive about the stay, was it right for Daisy? The information I had received seem to be geared towards older, more cognitively able children - however I was completely wrong, those two weeks under the care of the most fantastic chronic pain management team have got Daisy mobile and not only mobile but confident in her mobility. It is not an understatement to say that it is a real joy to see her pottering about the house again, mini-rucksack on her back with her mobile enteral pump, splints on her legs but independent. The team worked her (and the rest of the children in the group) really hard, no parents were allowed into the sessions although I was never far away as I had to deal with pump alarms. Children cried and complained and were made to work through the pain and stiffness in their joints so that by the end of the fortnight children who had limped into the physio gym were skipping along the corridor. Daisy had to work with 1lb weights strapped to her ankles and this work now needs to continue at home - leg lifts, bridges, sit ups - all aim to give her strength to deal with the problems in her joints and muscles. We also discovered during the stay that, ask we have always suspected, there is probably another syndrome which accounts for the severity of Daisy's symptoms. Most of the children in the group were hypermobile like Daisy and many had a diagnosis of Ehlers-Danloss syndrome a disorder of connective tissue which at it's mildest can manifest as double jointed-ness (a trait which runs in our family, both Jules and I have double jointed elbows) but in extreme cases can cause lots of other problems, including gut disorders where the connective tissue is not working properly to help the gut propel food along causing dismotility. Costello Syndrome has been show to affect connective tissue too so it could be that Daisy has a double whammy causing her intestinal failure.

Once Daisy has recovered from her Ileostomy surgery she will come back in for another week in rehab to get her back on her feet again. And so to the Ileostomy surgery - well at long, long last we have a date, which in the words of the Patient Liason Service "I can now become emotionally attached to!" - Daisy will be admitted to Rainforest ward on Thursday July 8th and Mr Curry will perform her surgery on July 9th - she has a bed confirmed for at least fourteen days with contingency to stay longer or if stable enough transfer back to Queen Marys. So we have begun the countdown and are keeping our fingers crossed that this is the difference Daisy needs. I don't hold out hope that it will get her off TPN but maybe we can get more enteral feed into her and reduce the dependency on TPN, some children just have it a few nights a week to give them the calories and fluid they need...maybe. However the thing we do know and are prepared for is that things will get worse before they get better after the surgery. Daisy's gut does not work properly and intestines do not like to be handled so it's going to go haywire for a while and we are going to have to wait until things calm down and it decides it wants to work. Then we will have to try and get the balance right with the stoma. The large colon is responsible for the majority of water absorption in the gut, as we will no longer be using it we want the small intestine to absorb more otherwise she will become dehydrated, something that happens even without a stoma at the moment. Anyway, at the moment this is the only option we have available to us, we have one of the best paediatric surgeons in GOS (therefore in the UK), fantastic anaesthetists who know how to handle a complicated to anaesthetise child and nurses who know Daisy well and are able to use some of the signs she uses.

We did have a brief sojourn on Rainforest last week. Daisy had only been back to school for one day following her rehab stay and we were waiting for her school bus to arrive when Xanthe tripped over her pump pulling out the 30cm jej tube. Regular readers will know this has happened once before in similar circumstances so I was very upset as I knew it could take two weeks for a slot to become available to replace the tube surgically. We put Daisy back onto 24 hour TPN to maintain her blood sugars but my biggest worry was getting her meds into her, the vital ones being her immunosuppresants and anti-inflammatories, blood pressure meds and the newly started Gabapentin to help with her pain. It was because of this need that an urgent slot with interventional radiology was freed up and Daisy went back into hospital 48 hours after the tube had come out to have a new one replaced. As it turned out it was due for replacement anyway which was probably why it had come out so easily. Daisy did brilliantly recovering from what was essentially a very simple procedure, the only problem was anaesthetising her. Unfortunately she seems to have a rare reaction to the combination of midazolam pre-med sedation and the anaesthetic drugs, the previous two times she has had this she had an allergic reaction afterwards. This means she cannot have a pre-med and as she is up to 40 anaesthetics now, she certainly knows what is going on and becomes really scared, add into this the difficulty in cannulating her and the fact that we cannot use the hickman line during any procedures involving her gut (to reduce any risk of infection) and anaesthetic is now becoming very, very traumatic for all concerned, with recovery not much different.

We also managed to fit in Jules' Holy Communion and Party between hospital stays, it was a beautiful day and such a fantastic opportunity to catch up with friends and to see how much everyone's children have grown. Theo's voice has now broken and he is now nearly as tall as me, Xanthe is counting down the days until she is allowed to have her ears pierced (at the end of her year 6 term) and Jules has now made his holy communion, all grown up in his suit, pretending he was James Bond....Daisy wore daisies in her hair and was clearly completely in love with her big brother. I just about got them to stay still and look at the camera together to take a picture, now the next challenge will be to get it from Andy's laptop to mine - I'll ask Theo, he's the house techy..........................

The best thing for me about Daisy's confirmed surgery date is that I can manage my long hoped for weekend camping at the Glastonbury Music Festival with Xanthe. As I type I am looking at the pile of gear to put into the back of the car tomorrow, as usual we are taking enough to sink a battle ship - the forecast is for good weather, but this is the UK - forecasts are wrong. A friend reminded me to take earplugs but three nights without Daisy and I will sleep through anything and after endless months in hospital rooms sleeping next to my Daughter and sharing grotty bathrooms with other parents I am used to roughing it. Glastonbury - I'm a veteran of children's hospitals, it will be luxury after that (I hope!!)

2010-06-03T00:08:00.000+01:00

So now it is June and still Daisy has not had her ileostomy surgery...we did have a near miss last Wednesday when I received a call while out shopping to say that a theatre slot had been found and her surgeon, Mr Curry, had freed up some time for Friday to perform the surgery - however there was one crucial link missing, a high dependency bed for Daisy...One look at her complicated medical history and the surgical ward decided not to take her and there was no room at the inn on Rainforest, the only ward she can feasibly go to. So we are back to square one, waiting, waiting and more waiting. Lots of liaising is going on between administrators and ward sisters but everyone who knows Daisy knows she does not do things according to plan things are more complicated that another average child, add into that a very busy surgeon and we are now looking at sometime towards the end of July I was told today. Bang goes our summer, but I guess it means that hopefully (fingers and toes crossed) Daisy will be stable enough to go back to school in September.

We continue in the meantime to live our lives on a knife edge, unable to plan, or enjoy any plans we do dare to make in case they get changed. This is a busy time of the year for us, both Theo and Xanthe have parts in school plays, there are various end of school parties and celebrations for Xanthe, school uniform to be bought for new schools, sports days, Jules' first holy communion....and the big event for Xanthe and I, our plan to go to Glastonbury. My hope is that if we get another short notice call for the surgery like last week it will be at least two weeks before Glastonbury with the hope that by then Daisy will be stable enough for me to go away for a couple of nights while Andy stays with her, otherwise I hope the date comes as soon as we get back from Glastonbury hopefully meaning that by the time of the school play performances and end of year school shennanigans Daisy will have transferred back to the local hospital...but then who knows? We continue as always to try and make plans, in fact we make things up was we go along and seize the moment . Now that we are getting more sunny days I relish those special times when we can have a barbeque or go out for a few hours - we all savour them as moments to remember when we are back in a hospital room, staring at the walls. I think over the past couple of years Andy and I have definitely mellowed, we used to resent the constant change of plan and the unpredictable nature of our life with Daisy, now we have learned that the only certain thing is the unpredictable element of our life. We always have a contingency plan, we have become experts at juggling things and rearranging at the last minute, all mainly so that the rest of the children have a chance of some fun, the ones that suffer are us, the parents, but even then Andy and I have found that we have to savour the small stuff and focus on what we have, not what we don't have. We are both avid facebookers and use this to keep in touch with family and friends and eachother, we find humour in the blackest moments and we constantly try to make sure we have some time together whenever possible. This time last year Andy had been made newly redundant, the future was uncertain and Daisy was still long term in hospital. There were some hairy points in the year with work and finances but now at last it is all coming home to roost, self employment suits our life well, Andy can be more flexible with his hours which means he can be around more for the children when Daisy is taken into hospital, and this has lead to lesss stress all around.

We also have a great new carer for Daisy who comes to help after school some of the week, while she cannot do any of the medical stuff it is great having another pair of hands around and she can also help with the older children. We are in a better position than we were this time last year, not in terms of Daisy's health, but in terms of the support we have put in place to help us cope with challenges. So now to Daisy's health....it's been three weeks since she was discharged from hospital following her last drama, and now we are back in again, this time for a two week intensive rehabilitation stay under the rheumatology team at GOS. This is a different sort of stay and, if hospital ever can be, quite enjoyable. Everything is focussed on building up Daisy's wasted muscles and helping her cope with her hypermobility which, while a feature of Costello Syndrome, is very extreme in Daisy's case (well no suprises there). It is very intense for her and as we are staying in the Patient Hotel and not a ward it is intense for me staying with her as there is no nursing help at all, however already we are seeing the benefits and she is becoming stronger and more confident on her feet. Even though the ileostomy surgery will set her back she will have a good foundation for her recovery and her muscles will remember what they are supposed to do (I hope).

In the three weeks between stays we have made some progress with the management of Daisy's pain. This is still the biggest issue in her and our lives. Every single night she wakes with pain in her tummy and every single night we have to give her a maximum dose of calpol. It is ridiculous that she went from IV ketamine infusions, boluses, IV Paracetamol and Oramorph while in hospital to calpol via a non-working gut....however we have had a bit of a breakthrough, thanks once again to our wonderful hospice, Chase. Chase have employed a Palliative Care consultant as a shared role with the Royal Marsden Hospital, I met with her last week to discuss Daisy and her pain management - in fact the more palatable title for palliative care with children is symptom managment, which is exactly what we need. She has put together a pain management plan for Daisy for when she is at home, at the local hospital and at GOS. In the next week Daisy will start on a drug called Gabapentin, while it is used for lots of different purposes one of the side effects is to help manage neuropathic pain, which is the pain Daisy experiences from the incorrectly functioning nerves in her gut. This in turn may help her sleep better which may help her cope better with the pain. As I posted on my facebook page, you shouldn't be happy about meeting with a doctor who cannot cure your child, but I was over the moon, at last someone who understood the issue and impact of Daisys pain and managing it. Even if the Gabapentin doesn't work there are a whole other range of drugs we can try so I feel that we are no longer limited and alone. Ultimately we hope the ileostomy will help but taking the non-functioning colon out of operation but no-one really knows if this will work until we try. So in the meantime we will continue to muddle along, waiting for the surgery date, hoping it doesn't have too big an impact on family plans and keeping everything crossed the surgery goes well and with the desired effect. Maybe by the time of my next update we will have that elusive date - here's hoping!

2010-05-08T22:07:00.000+01:00

Dressed as Peter Pan during the celebrations for Peter Pan week at Great Ormond Street

Beautiful raspberry pink casts, her nails have now been painted to match by big sister

Listening to a professional storyteller who visited the ward, she got 3 stories out of him and did not want him to go!

Before going into hospital, looking at a lovely gift sent to her with her big sister

2010-05-04T16:29:00.000+01:00

Well guess where we are? Yes that’s right – still in hospital!!! In fact as I write we are on our home ward at Great Ormond Street Hospital – Rainforest. I always knew things would not be straightforward with Daisy’s orthopaedic surgery or TPN review, which was probably the reason why I had contacted our local hospital the week before to forewarn them of a possible transfer at the end of our booked week at GOS and also the reason why I had booked Daisy into the hospice for a few nights stay the following week. What I had not anticipated was Daisy being too unwell to actually leave GOS and needing a transfer to one of the high dependency beds on Rainforest!

The orthopaedic surgery itself went according to plan, a registrar drew two arrows on her legs so that the surgeons would know they were doing bilateral tendon lengthening and then he thought it would also be wise to draw arrows pointing to her ears so that the ENT surgeons would know where to put her grommets (hearing tubes) – I thought this was funny as it would be a bit worrying if an ENT surgeon did not know that grommets go in the ears. Annoyingly the arrows were drawn in indelible ink and Daisy spent the next week with them on her face and receiving puzzled looks from various medical staff..

She returned from theatre in her usual state – screaming and thrashing around. I always see other children in recovery sitting up taking things in but Daisy has a really bad response to anaesthetic and gets very upset afterwards. She had some respiratory distress for a couple of hours then flared up in a huge allergic rash requiring IV hydrocortisone – this is the second anaesthetic this has happened so the team are starting to get a bit twitchy about what to give her. She was clearly in some pain and we started her on IV paracetomol. The pain did not seem to come from her legs (which were now wrapped in lovely raspberry pink fibreglass casts) but from her tummy. As it worsened the night doctor started morphine into the mix. She transferred to the short stay ward the next day to start her TPN review and spent a morning in nuclear medicine having various tests and scans. By the afternoon the pain was getting worse and eventually she then spiked a 41.5degree fever and began vomiting copious amounts of bile indicating her tummy had shut down (we had only restarted minimal feeds anyway). We did blood cultures and started her antibiotic protocol assuming line infection. Her pain was becoming worse and worse and the pain management team decided to start her on a continuous ketamine infusion . To cut a long story short she needed the ketamine to be turned up more and more and she basically had 5 long nights without sleep and in considerable pain until it was controlled.

I was so please that she was transferred to Rainforest where she is so well known and where we could develop a plan for what to do next. Daisy’s pain at night has been increasing over the past few months and as you know we keep bouncing back and fore to A&E as she spikes temperatures requiring IV antibiotics. So far none of these episodes have been caused by a line infection and I was surprised that once again this was the case with this episode. This has left the doctors scratching their heads as they try and find out why Daisy is getting so many temperatures and why she is in so much pain at night. In a way it is good that she has done all of this here; for a start at our local there is no way that she could have received the level of pain relief offered here and she would have had to be retrieved to a more high dependency setting – wherever a bed was available which probably would not have been GOS. Also, we have been struggling to manage Daisy’s pain and these episodes of fever for a few months now and sometimes you feel like you’re a broken record telling the doctors about it. Without seeing it for themselves we have been told over and over it’s a UTI or winter bugs but thank goodness the doctors now can see the reality of what we have been telling them and agree there is a lot more going on.

The main decision is that an ileostomy is now absolutely essential,. No-one knows if it will work but at least the logic of reducing the length of passage of food in a very dismotile gut makes a lot of sense. So, having recently left a message for our surgeon saying we would like to go ahead but preferably at the end of our summer things are once again out of our control and the team here have been in touch with him to ask for it sooner rather than later....so much for the comment that the timing of the surgery was the one thing we had control over in my last post!. This has sent me into a real quandary as once again timings and plans are taken out of our hands. Now I hope that we get the date within the next couple of weeks – this would mean that hopefully by mid June Daisy would be well enough to be transferred to our local and be closer to home, mainly for Jules’ holy communion day. Later in the month or the beginning of June and we are in the danger zone, not only for things planned in June (including Xanthe & my much planned and talked about once in a lifetime very special trip to the Glastonbury Festival…..don’t even get me started on that one…) but also that Daisy will still be very much in hospital by the time the children break up for the long summer holidays meaning another summer spent juggling their needs with Daisy being in hospital.

I know how much Theo was affected by Daisy being in hospital for the entire first year of his new High School and we are experiencing set backs from this again now. Xanthe starts her new High School in September and I just want to be there for her as she makes the transition from Primary to High School. In a way Xanthe is in a better position, being a typical girl she has a lovely group of friends who support her and who she can talk to, she also uses her creative talents in art and writing to express how she feels and at the end of the day she was only five when Daisy was born and can hardly remember a time when hospitals were not part of our lives. The boys find it harder to express themselves, Jules loves Daisy completely and utterly, he worries about her when she is not at home and he needs his family to be together and Theo, who is now a teenager, veers from mature and sensible to completely exasperating. For Andy and I it is difficult to know if his behaviour is just normal teenager stuff or his way of dealing with his worries – my guess is it’s half and half.
And Andy and I struggle to find time for eachother like so many other parents of chronically ill children. Having been made redundant last year, Andy is building a very successful business that needs all his time. We spread ourselves thinly but both appreciate the importance of making time for eachother. We still find time to have a laugh and find humour in our situation, we are so fortunate that we had a good, solid relationship to start off with but it has been tested over and over in the past few years.
My biggest worry is that we are not moving forwards. Simple orthopaedic surgery has set Daisy back, her walking ability is worsening, normally children in casts after this operation are walking within a few days. Two weeks in and she can just about find the strength the weight bear for a few seconds. The dependency on TPN is increasing and her ability to tolerate enteral feeds is decreasing. The one area where she continues to progress is in her cognitive development, she is as bright as a button and signs constantly and often supports her signs with clear words. She has clear likes and dislikes, understands everything and when she is not in pain is so happy and contented.
I just hope we get a date for this ileostomy surgery very soon so that we can give it a try and Andy and I can be trained on yet another aspect of her care….clearly we missed our calling when we opted for careers in business, we were obviously meant to be nurses…

2010-04-17T22:35:00.000+01:00

After much soul searching and wishing we could see into the future, Andy and I decided that it was inevitable that the ileostomy is going to happen but the thing we have control over is when. Our wish, after five years spent in and out of hospital and Daisy's deterioration in the past two years, is that we just have some time together as a family. So we have contacted the surgeon and asked for the ileostomy surgery to be scheduled for early autumn as opposed to early summer which was the orginal timescale. At least this way we can manage the risk of Daisy spending all summer in hospital and at least (hopefully ) keep hospital stays a bit shorter.

I was very excited to get a phonecall from the orthopaedics team offering us a cancelled date for Daisy's foot surgery. However like a London bus, you wait and wait and suddenly they all come at once - the date offered was the same as the week of tests and procedures planned by the gastro and endocrine teams. However I am so impressed by the fantastic Great Ormond Street Clinical Nurse specialists who have been pulling together and liaising so that hopefully Daisy can have her foot surgery (and some new grommets inserted into her ear canals). On Monday morning I will drive up to Great Ormond Street for 7.30am and Daisy will have her surgery that day, after a night on the orthopaedic ward she will transfer to the gastro ward then have all her TPN and gastro investigations including another general anaesthetic on Thursday when she will have an upper an lower endoscopy. The ward closes on Friday (can you believe this, GOS has some wards which are only open on weekdays even though they take the sickest of the sick) so I am assuming they will not be able to transfer her over to Rainforest ward and have prewarned our local hospital to expect a possible (or probable) transfer - two anaesthetics, orthopaedic surgery and whatever they find when doing their procedures and investigations....I just don't want to take too many risks. We have also booked Daisy into Chase for a respite stay starting on Sunday evening...

I really hope Daisy gets to go to Chase after her hospital stay, for a start managing a child with both legs in plaster who has wasted muscles and vestibular (balance) problems is enough in itself but add into that the pumps and TPN and then the fact that still not a night goes by without the need for pain relief and you can understand why sometimes we need a break....even though going to Chase is sometimes not a break as I wait for the call in the middle of the night that has so often come to say she has spiked a temperature signalling yet another infection.

Jules is now exactly the same age that Theo was when Daisy was born, I look at him and wonder, how did we manage? She was born prematurely and by caesarean (my first c-section out of four deliveries) 3 days before Christmas - the other children were 7, 5 and 2 and I had already spent 3 weeks in hospital beforehand. We were apart for Christmas that year, Andy was at home with the children, I was in hospital recovering and Daisy was in intensive care. Yes things have improved - over the past month we have lost a member of our Costello Syndrome family suddenly and unexpectedly and five children who are also supported by the Postpals Charity have lost their battles, at least Daisy is still with us and fighting. But - much as we were relieved to have been given a diagnosis for Daisy so early on, what has this really meant? No other child with Costello Syndrome has the extreme intestinal problems Daisy has, no-one else manages TPN at home - one or two have been on TPN in hospital when their child was being treated for Cancer or was very ill but normally with this syndrome children are starting to lose their dependency on tubes and starting to eat orally by this age, or at least to be able to take feed into their tummys....Daisy is completely TPN dependent, she still only gets the equivalent of 3 teaspoons an hour of feed into her jejenum for 10 hours a day. We cannot use her stomach as it does not empty properly and the milk would just build up and build up until she was sick. Every single night she screams with pain, I will not be suprised to be told that her gut is inflammed again next week when she is scoped, I don't know of another child with Costello Syndrome who battles with inflammatory bowel disease and has to receive immunosuppressant therapy. And then the children I know with these sort of gut issues don't have the Costello issues - they are cognitively able, they do not have severe visual impairments, they do not have to be screened every few months to check for malignancies, their parents do not have to worry that tight heel cords mean that their spinal cord has tethered or a chiaria malformation has formed necessitating brain surgery.....Some days it is very lonely in this world and I cannot help but feel sorry for myself, every single day we disconnect TPN, we flush her line, we lock the line with drugs aimed at preventing infection, we deliver her meds into her jejenum - no nasty tasting meds into the mouth for Daisy, we make up the milk mixing in complex carboydrate powder to make sure her blood sugar does not drop, set up her enteral pump, we remember to get the new bag of TPN out of the fridge to come to room temperature, prepare her IV trolley, connect her up to the TPN, give her more meds, inject her with growth hormone to make sure her blood sugars remain stable, untangle her from her TPN lines when she thrashes around at night.......day in, day out. And each day we are rewarded by her progress, little steps, more and more words, a new obsession with Thomas the Tank Engine, a cuddle for her brother when he was feeling ill, asking to have her toe nails painted.....and this is why we are doing it, Daisy is not a syndrome or a patient or a set of symptoms, she's just another of our children, maybe a bit more complex and time consuming, but a five year old girl who keeps defying the odds and suprising us with her abilities. I have never compared her with other "typical" children of her age and I must remind myself that while she has a diagnosis, that only serves to get her the help she needs, that label still does not lay out the path of what she will or will not do, having Daisy in our lives is rewarding and wonderful and she is one of four unique, sometimes exasperating, children and that is what matters most.

Roll on Monday...........

2010-03-20T21:14:00.007Z

So we are nearing the end of March and still our battles with Daisy go on. This year we have already racked up 5 hospital stays, and the fact that we can now do things like TPN and bloods at home have kept her out of hospital the rest of the time.

We are facing some big decisions and seeing Daisy deteriorating in many ways so our mantra continues to be "will it improve her quality of life".....

The ongoing issue remains infections, she is chronically unwell a lot of the time with low grade fevers and "pyrexia of unknown origin" - we know that the taurolock line locks we now use in her hickman line are helping protect the line from infection but the large doses of immunosuppressants she is on mean that she picks up every bug going and ontop of that bugs move from her not very well functioning gut into her blood stream causing her problems. This has been the main cause of her recent hospital admissions as any bug also causes her tummy to go into "pseudo-obstruction" and shut down - this is happening more and more and I have had times where I have considered only buying yellow pyjamas and bedsheets for her to match the bile that pours out of her tummy during these times..

One of our admissions was slightly more dramatic than normal and for the first time ever I called 999 for an ambulance when I discovered Daisy soaked in blood pouring from her hickman line - to this day we still do not know why it started to bleed suddenly but may be something to do with her high blood pressure issues.

We have had two meetings with Surgeons since my last post - after a couple of cancellations because Daisy was too unwell to attend the appointment we met with her Orthopaedic surgeon and have at last got the ball rolling to get her feet sorted, this can't come soon enough as Daisy has now gone from being able to walk around with her little backpack carrying her pump to not being unable to walk unaided and mainly shuffling on her knees to get around the house. The tightening of her achilles tendons is so severe that her big toe joint is now at a right angle to the rest of her foot when upright. She cannot wear shoes or her foot orthotics and has considerable pain in her hips if she spends too long on her feet. Once she has the surgery to lengthen and stretch her achilles tendons, which will be in about two months time because of the waiting list, she will need to be in casts for quite a few weeks, hopefully once everything has healed she will then need to go into hospital for a two week intensive rehabilitation stay as her muscles in her legs have wasted and essentially she will need to re-learn how to walk again. I am so frustrated by this situation as I have been saying to physiotherapists for years that tight heel cords can come on very very quickly in children with Costello Syndrome but, if we ever actually saw a physio which was not often, I was accused of worrying about things before they even happened. We have now reached a point where what would have been simple surgery has now become quite complicated with no guarantee of a complete success....

The other surgeon we have met with was one of the Paediatric Surgery Consultants at GOS, our lovely gastro consultant referred us to him to discuss surgery to form an ileostomy. This is where the bowel is brought out through the abdomen to form a stoma, bypassing the large colon. The pellet study of Daisy's intestinal transit showed that the radio-opaque pellets she had to swallow took at least 3 days to reach her intenstines and then she took a total of 19 days to pass them all.... confirming, that she has very poor intestinal motility, particularly in the lower part of her colon. The severe pan-colitis she also has, has probably affected the nerves and in a nutshell her gut, which didn't work properly from birth, has now deteriorated massively. The hope is that by performing an ileostomy we create a smaller area for the gut to work and bypass the areas we know are the most diseased and dysfunctional - possibly to allow them to heal and one day work properly... This may reduce Daisy's dependency on TPN and maybe even allow her to be fed into her stomach instead of into her jejenum...These are all the positives, the negatives are that it is major surgery and there are no guarantees except for the fact that she will get worse before she gets better and we may after all of it end up having to reverse the stoma and reconnecting the bowel if it does not make any difference...

This is a huge decision for Andy and I, we know we have to try and give Daisy the best chance but it's all the unknowns....and, if I am completely honest, with the memory of our recent 12 month stint still fresh the thought of another long marathon stay in hospital fills me with dread. The surgeon has left it to us to make the decision while booking the bed and the theatre slot anyway so that we do not have to delay if we decide to go ahead. It is such a big decision for us because Daisy is just not straightforward - she is classed as being risky for anaesthetic because of previous problems and because of her heart condition, her endocrine issues mean her blood sugar mangement will be even more tricky, her gut does not tolerate change will definitely go into shut down for a while and then there is the risk to her hickman line as she will have a hickman, gastrostomy, jejenostomy and ileostomy which are all possible sources and sites for infection....

However both Andy and I know we have to make a decision soon, Daisy's condition has deteriorated massively over the past two years and is definitely not getting better. The amount of feed she receives into her jejenum is paltry, the equivalent of 3 teaspoons an hour and most of that is made up of sugar just to maintain her blood sugars while off the TPN. She is TPN dependent and this is not conducive to a long life, her pan-colitis is poorly controlled - at the moment she has a flare up which has slowed her gut down and is causing her significant pain. The immunosuppressant therapy she is on to dampen down her body's response to the inflammation is difficult to manage as her gut does not absorb the drugs well and she cannot take them orally as she risks aspirating them. I have to take bloods weekly to measure her cyclosporin levels - too high and she risks kidney damage, too low and she will have a flare up of the inflammation. In a year she has not had a consistent run of her levels being where they should be. For all these reasons and for many more I know we must give the ileostomy a try, if I could see into the future it would be so easy, but then life with a child with Costello Syndrome was never meant to be easy....

My gut feeling (pun intended!) is that we will ask for the surgery to be pushed back to late summer to allow her to recover from the orthopaedic surgery which is essential to ensure she does not lose the ability to walk and allow us some family time over the summer. In the meantime Daisy is due a planned week long stay in GOS for a whole range of tests including another endoscopy/colonoscopy - perhaps the results will help tip the balance on how quickly we go ahead with the surgery...

Life at home still goes on - Daisy now has transport to take her to school and this has freed up so much time in my day. However she has not managed a full week at school, so much time is taken up with illness or hospital appointments. When she is there however she is at her happiest. She gets so excited when the bus arrives to collect her and her cognitive development continues to amaze everyone who works with her. The plan now is to move to "sign supported english" as she is starting to vocalise words more and more but will need the signing to get her point and meaning across. We all sign naturally with her and never have any trouble understand what she is trying to communicate.

Andy is still building his new business and work is picking up more and more, Theo is totally involved in his life at high school and Xanthe is over the moon having been offered a place at her first choice High School. Jules is busy preparing for his holy communion and we are all looking forward to that date as it is yet another excuse for a Nimmo Summer Garden Party...we continue to make plans, and tweak and change them to fit in with Daisy...this has become our life, living with uncertainty has become normality, we have learned not to fight it but to go with the flow, expecting the worst but sometimes being pleasantly suprised. Maybe it's not a textbook strategy but for us, it works....

2010-01-12T22:40:00.002Z

Every day my youngest daughter's capacity to overcome the odds that are stacked against her completely amaze me. Yesterday she had a dual sensory assesment at her school to look at the support she needs to take into account her visual and hearing impairments. In fact Daisy is multi-sensory impaired, all her senses are not wired like yours or mine so every day tasks take so much longer, she has to work twice, three times as hard as any other child just to do simple things like sit at a table and play with a toy. She has to remain balanced, focus her hearing and vision, explore using touch and taste and smell, all of these things she does with a system that does not function in the way it should. What we discovered, or rather confirmed that everyone in the room already knew, is that Daisy is a bright little cookie. There is so much going on in her brain, but she needs to be given the resources to help her use it. The biggest problem continues to be speech, which is limited, however, her communication (using signs) is coming on leaps and bounds - just because she does not speak it does not mean that she does not understand or need to tell you how she feels. One of the outcomes of yesterdays session was to really work on her signing, progressing hopefully to using British Sign Language. I hope that as she begins to use signing more and more this will equip her for later life and help her assert herself and get other people to understand that lack of speech does not mean that she does not have feelings and comments to make on what is happening to her.

Today Daisy wore her new bone conductor hearing aids to school - what a transformation, the teacher told me she has been so vocal, it's like a light bulb has gone on with her ability to hear more clearly...of course we are now waiting for the proper digital ones to arrive so that she can have even better sound quality...

Daisy started the new year in Rainforest Ward - just to make her mark that hospital life would continue, despite our wonderful birthday, Christmas, new year break....The stay was for a new hickman line, two repairs to her hickman line were too risky for infection so she was prioritised for a line change, removing one from the right jugular and placing a new one into the left jugular. Fortunately this was relatively straightforward inspite of the interventional radiologist (who has worked on Daisy before) commenting, yes I remember Daisy, she has very wiggly veins....don't you just love these doctores and their casual asides...

This year will see lots more hospital stays - the likelihood of an ileostomy to bypass Daisy's non-functioning colon is looking more and more strong. Tests that were conducted over Christmas seem to confirm this although we will get the official results when we meet with Daisy's main Gastroenterologist on Monday. This takes us into yet another new territory - another bit of plumbing to contend with and what essentially is very major surgery for a little girl with a lot going on. Our hope, if this is the route we go dow, is that this will put an end the awful pain that she is experiencing, mainly at night time.

This is not the only surgery she will have this year, we are still waiting for the date for the tendon releasing surgery - she now spends more time off her feet than on her feet and if this surgery is not done soon Daisy will be unable to walk. Following this surgery will be around 6 weeks in plaster casts - fun for all involved and then a two week intensive rehabilitation stay at GOS. The only good thing with all this surgery and hospital stays is that it is all planned, hopefully we will see less emergency admissions this year and instead have more planned stays focussing on improving Daisy's quality of life.

2009-12-24T23:02:00.004Z

Merry Christmas and a Happy New Year

2009-12-24T14:35:00.005Z

Well, after much cajoling by family and friends, I am back to blogging.... to be honest it has been difficult to fit in the time to write now that Daisy is at home, also, sometimes I feel like I am writing the same stuff over and over and maybe people don't want to read it but based on feedback when I stopped writing for a while it is obvious that a lot of you out there quite like my ramblings.

So here, instead of cards (do you think I have time to write Christmas cards???) - is my Christmas blog...........

Lots has happened (no suprises there) since my last post. two emergency hospital stays, lots of outpatient appointments, more surgery plans and continuing battles with bureaucracy. Lets start with the good news first however...

After the October half term our local education authority saw sense at last and agreed to fund a full time one to one support for Daisy so that she could attend school full time. The difference in Daisy has been amazing, she is more confident, vocal and her communication skills (using sign language) are truly fantastic. She ended the term being awarded the Principal's prize for excellent signing and also enjoyed playing an angel in her class assembly, sitting confidently and watching all the other children, joining in with all the songs and applause. School has been the making of Daisy and we are so grateful and happy that she has been able to attend this particular school, a specialist sensory school where the teachers are skilled in working with children with sensory impairments. The only downside is that although funding has now been approved for Daisy to be taken to school with the school transport service this has not yet happened (due to bureaucracy again) meaning Andy and I have to take her back and fore to school which takes up two hours of our day and a huge amount of juggling with the other children. I'm really hoping that this is resolved in the new year as the daily battles with other school run mums across Wimbledon are taking their toll and we are worried about the words Daisy is learning as we vent our frustrations on other mad drivers!!!

Another lovely thing that happened in November was Theo's confirmation. Some of you will know that I am a practising catholic and a confirmation is a really big rite of passage for a young person. It was such a lovely chance to be together as a family with the focus on one of the other children for a change. In catholicism you take a saints name at confirmation and Theo chose "John" which was my dad's name, particularly poignant as mum was his confirmation sponsor and dad's anniversary had been the week before . I know dad would have been so proud of all our children so it was so nice for Theo to honour his memory in this way...

Of course as always there have been the obligitary hospital visits both planned and emergency. We had a few trips to A&E in November as Daisy was experiencing really acute pain and screaming out, always in the back of our minds is the increased risk of malignancy that Costello Syndrome carries. It's so difficult taking Daisy to A&E now as staff change so frequently and it can be really frustrating going over and over things with new people. Theoretically she should have notes kept in A&E for what to do in an emergency - something that was agreed with the Great Ormond Street team on discharge - but we found on our visit there that the notes had not been updatede which made things very difficult. In addition it doesn't help that Daisy is so complex, some doctors can flounder a bit, particularly when faced with such knowledgeable parents. It took four visits before Daisy was admitted with a 40 degree temperature and quite poorly. As there were no cubicles at our local any more we had to be shipped to Epsom hospital which might as well have been Manchester as we had to start all over again with staff who did not know Daisy or us. They were however fantastic and Daisy was home after a week and then went to our wonderful hospice to recuperate...

A couple of weeks later her temperature shot up again, we always have to suspect line sepsis in this scenario and we had to rush again to A&E. This time she did get a cubicle at our local hospital so at least we were closer to home. This last stay in hospital highlighted some things to us which have changed so much since the days when Daisy was smaller and much, much less complicated (although we didn't think so at the time!!!). Daisy is the only child who normally is on the ward who is totally TPN dependent and a patient of Great Ormond Street. Part of the philosophy of the Home TPN programme at GOS is that in order to minimise infection risk only the parents should set up or take down the TPN or access the line for bloods. In theory this is fine but when you have a child like Daisy who never ever sleeps through the night and has lots of complications it puts a huge pressure on Andy and I, particularly when you add into the mix Andy trying to run and manage a business and three other children who are not allowed onto the ward because of swine flu precautions. We had agreed at a meeting at GOS that we would manage Daisy's TPN when in hospital but use a hospital pump provided by GOS, normally this works in every other hospital where their home TPN patients visit as the pump that was provided is a standard hospital one. Standard except in our local children's ward (even though it was in use in the rest of the hospital). So we had to cope with the scenario of nurses unwilling to use another hospital's pump, unwilling to put our TPN through their pump, sleep deprived parents who were having to stay awake for 18+ hours while also trying to juggle childcare and in Andy's case client work. Needless to say things were not good during Daisy's last hospital stay and while she recovered well we were disappointed that, without going into detail, we were seen as a problem. In five years we have got very close to the staff at our local hospital and I guess with the demands being put on us as parents of a medically fragile and complicated child our relationship with them has had to change as we have become more knowledgeable about Daisy's needs and she has become more complicated. Ultimately we would like to avoid any unplanned hospital stays and, now that Andy and I have been trained in lots of areas like giving TPN and IV medication hopefully emergency visits will be short and sweet.

However the planned visits are unavoidable and these all take place at Great Ormond Steet Hospital. Again since having time at home we have really come to appreciate how incredibly lucky we are to be able to have all of Daisy's specialist care at GOS. As she grows bigger other issues, more common in her syndrome, are starting to manifest. The one area which has crept up really quickly is Daisy's feet and general orthopaedic issues. Her achilles tendons are now so tight she walks in a permanent tiptoe and her leg splints are now causing her severe pain. She will have surgery in the new year to release her heel cords, followed by several weeks in plaster casts. After that is all over she will then be readmitted to hospital for extensive rehabilitation physiotherapy under the care of the rheumatology team as she has been found to have benign hypermobility syndrome complicated by fluctuating muscle tone (try saying that after a few drinks!) - the bottom line is that this causes her pain and puts things out of alignment which causes her even more pain and was probably responsible for the fluid on her hips during the summer.

We met with Daisy's Gastroenterologist a few weeks back and he now feels that the extreme inflammation she experienced with her inflammatory bowel disease has damaged her nerve ending in her gut which were not functioning properly, hence her dependency on TPN for her nutrition and her inability to manage more than 15mls/hour into her jejenum. Over Christmas we are faced with the fun task of trying to get Daisy to swallow four radio-marked pellets over four days to try and work out how slowly her gut moves - obviously if on x-ray the pellets show up in her lungs it will confirm that her swallow is still unsafe!!! We are then back in clinic in January to see if there are any further options for treatment, including, surgery, which may help her.

Despite everything, and even though she has kept us on our toes during the past few weeks Daisy managed to be well and stable for her birthday, for the first time ever she spent it out of hospital and hospice and we managed a short pre Christmas break to Center Parcs in Longleat. The Gods must have been smiling on us as considering we have not been further than our hospice in Guildford in the past 18 months Daisy was fit and strong and loved every minute of our time away, we avoided all the severe weather and the snow we had just made the forest seem even more magical than ever. I can hand on heart say it was the best birthday ever and whatever happens in 2010 the memories of happy family times in the run up to Christmas will sustain us all for a long long time.

Right - I need to get back to Christmas preparations, the presents are not going to wrap themselves. We have vigil mass in a couple of hours, Xanthe is playing an angel in the church nativity, Theo is altar serving and even Andy, the arch atheist, is coming to church (mainly to hear the choir sing a not-tongue in cheek version of Johnny Mathis' "When a Child is Born" - including the spoken bit in the middle....)

Hopefully this blog update will make up for the fact that I have not sent any Christmas cards, however to everyone who sent us cards and Daisy birthday cards, thank you so much - especially post pals who brighten our days!!!!

I'm going to post some pics later but in the meantime:

HAPPY CHRISTMAS AND A HEALTHY AND WEALTHY 2010

XXXXXXXX

useful links:

www.lindenlodge.wandsworth.sch.uk
www.postpals.co.uk
www.facebook.com/people/Stephanie-Nimmo/653854349
http://www.gosh.org/x-factor/the-charity-single/your-say/stephanie-nimmo/

2009-10-12T22:04:00.006+01:00

Apologies again for not updating more frequently - I'm sure I'm breaking the European Working time directive rules on the number of hours I work, on an unpaid basis, nursing Daisy, let alone looking after the rest of the family....

Again, so far, so good - apart from the usual dramas our lovely daughter throws in our path. This month's rare occurence which befell Daisy was a 1cm hole in her hickman line. As you can imagine a hole in a line which goes straight into her heart is not desirable so as always we had panic stations.... I noticed the problem when flushing her line on a Friday morning (it's always a Friday!), there was a strong smell of TPN in her room and when I flushed the line I saw the hole. I now know that repairing a hickman line is not straightforward, initially, in my naivety, I thought it was a case of calling the community nurses to come and carry out the repair, obviously not. There are limited people who can carry out hickman line repairs, and no-one in our local NHS trust could do this. In fact, I have since discovered that in some areas a repair would not even be attempted, instead it would be a case of removing the line and inserting a new one. As you have guessed, it was time for another trip to Great Ormond Street and a three hour stint with their fantastic nurse practitioners who, after a couple of false starts, repaired the line....

Of course it shouldn't have happened in the first place, holes in hickman lines are rare, especially in such a new line, but rare and Daisy go together! And as always her timing was impeccable - not only was it a Friday, but Andy's parents were due to arrive from Scotland having not seen the children in a year and Theo was due to return home from his Ocean Youth trust trip. Major multi-tasking and, as always, wonderful help from Chase hospice, and everything was sorted in time.....

Apart from this drama, our month has been spent trying to get back to some semblance of normality as a family again. We have all spent a year living on our nerves and separated from eachother, and the scars run deep. While superficially it looks like the children have coped well, Andy and I have realised how difficult it has been for all of us. Theo had his first year of high school with minimal support from his parents, often coming home to an empty house and cooking a meal for himself. Jules and Xanthe went to after school club or to friends houses. Andy and I were like ships that passed in the night putting all our energy into the children. Now we are all just relishing being under the same roof and enjoying the simple things....Theo is loving having his parents there when he comes home from school, Xanthe likes our pampering evenings, Jules is able to have friends back home to play. Daisy remains the centre of attention, but in a positive way. Andy and I are happy to be back home, able to catch up on TV together and share a joke....

Life is still tough but at least we are all at home. Every day I spend an hour in the morning doing the TPN and meds and getting Daisy ready for the day, the same again at night....It is physical work that requires maximum concentration. Before Daisy's deterioration she was on 24 milk feed through a feed pump and around 13 different meds a day. Now the feed pump time is reduced to 10 hours but she then has 14 hours on TPN and still the same number of meds, just different ones...ontop of this is her physical problems, her splints, without which she can no longer walk, the pain at night, the yet to come health problems.

She now goes to school for four hours a day and the benefits show on a daily basis. She is becoming more and more vocal and cognitively is really bright. The school is going to start progressing her from using makaton to British Sign Language as her brain works quicker than her ability to acticulate herself. We are really hoping that in arming Daisy with the skill she will benefit in the long term as she can really get across her feelings. Of course this will mean that Andy and I will need to learn BSL - we are really excited about this prospect.

After half term Daisy should go to school full time - paperwork and bureaucracy permitting! The next battle will be to sort out transport to get her to school and back as most children in special schools are taken there in buses and taxis . Daisy will need an escort, so this will be put to yet another committee for funding approval.....This is what drags you down as a parent of a disabled, medically fragile child - you spend your days fighting to keep them alive, fighting for the best for them and it all boils down to paperwork, panels and case conferences, none of which,you, as a parent, have a say in....

I do sometimes wonder if Andy and I were not so articulate, educated and determined to cut through all the red tape which prevents our daughter getting what is her right, how she would do......

Anyway, as I said, so far, so good, we are staying away from hospital and doing the nursing stuff at home, life is better, we are all together ....

2009-09-17T21:22:00.005+01:00

Well, so far, so good, I am pleased to report we are still out of hospital and only visiting as an outpatient. My workload has not eased however as now is the time to fight all the other battles that have been put on hold for a while. Examples of these included sorting out a proper bed for her which will enable me to do her IVs without breaking my back and which will keep Daisy safe when she thrashes around at night in pain. We have had a few false starts with our wonderful Primary Care Trust on this one and two, totally unsuitable, beds have been provided already. The third one will arrive hopefully in around 6 weeks so I am really hoping it is third time lucky. In the meantime we are improvising, my wonderful friend's wonderful carpenter husband has adapted a second hand cot bed with higher legs and a door which opens outwards - thank goodness for friends like this as I really do not know how I would have coped with an ordinary cot in the meantime.

Another battle is to get Daisy the support she needs in school. Prior to her massive increase in medical needs (when her needs were still very significant) Daisy had a one to one learning support assistant for 4 hours of the school day. We now have to demonstrate to the education authority why she needs a full time assistant - it's patently obvious, but unfortunately there are massive bureaucratic hoops to jump through to achieve this so in the meantime she is back at school, but only for 4 hours a day.

Daisy is loving being back at school. It is a specialist school for children with visual impairments in the most wonderful location between Wimbledon Common and the All England Lawn Tennis Club. The facilities and staff are outstanding and even in just a few days back in school Daisy is coming on in leaps and bounds. The staff are so happy to have her back, everyone seems to know her there and I just hope that she can have a really long run of time back at school so that she can continue progressing.

I have my usual battles with the medical issues, we now have a date for her rheumatology appointment, this is at the end of October. I am still trying to get a date and plan for her surgery on her feet. These are getting worse and worse, she can no longer stand up without wearing her foot splints and even with these on she is unsteady and falls easily. I am so concerned that this surgery needs to happen very soon as I don't want her to lose the ability to walk. We are waiting for a date to go back to GOS for the TPN/Nutrition Clinic. Despite pushing up the volume of TPN Daisy is receiving she is continuing to lose weight and I am trying not to worry too much but you can't help wondering if there is a more sinister reason behind why this is happening...

Anyway, the next couple of days are an opportunity for me to put all of these worries behind me. Tonight I am staying at Chase Hospice with Daisy, tomorrow Andy and I are going to Wembley Stadium to see Coldplay as our wedding anniversary treat to ourselves. This will be our first night off in a year as Gemma will stay overnight in Chase to do Daisy's TPN. On Saturday, Andy and I will stay in Chase without the other children (mum is looking after them for the weekend). After we have put the TPN up we are going out for Dinner! This will be the first time we have stayed at Chase without the other children and with Daisy now TPN dependent this is the only chance we get to have a proper break. On Sunday mum will drive to Chase with the rest of the children so that we can all enjoy the famous Chase Sunday Lunch as a family.

Being at Chase is giving me a break from all of the stress I deal with on a day to day basis. This service is provided completely free to our family and is funded almost entirely by voluntary donations - without Chase I really don't know how I would cope.

I'm off to bed now, no getting up to Daisy tonight and no school run tomorrow - bliss!!!!!

2009-08-21T22:52:00.006+01:00

We lasted 18 days at home before a return to hospital....I have been putting off updating this blog because I wanted to write that we were back home, which we are now, but also because managing the needs of four children during the school holidays and a husband who is working freelance means that there are even less minutes, let alone hours in the day to update. In not writing for a while I discovered how many people actually read this blog as so many people have contacted me to ask me to update it. It is so flattering to know people enjoy my writing and I promise to try harder to update a bit more frequently. In fact it will make my life easier to do so as so much has happened since my last post that it becomes it nighmare to condense it all into a manageable chunk of text for people to read, so in advance, apologies if this one rambles a bit but here we go.......

I looked back over my last post and the signs were all there - the early return from Chase with high temp...of course it was not just going to go away... The day after our return from Chase Daisy was due for bloods so we decided to run some blood cultures also to check that there was no line infection, I think 18 days at home had made me forget too quickly as I was caught off guard when a call came through from the ward to say that the cultures had come through as positive and could I bring Daisy in to start IV antibiotics.....

Hindsight is such a great thing isn't it - she had been looking increasingly pale as the days at home had worn on and I think I was just trying to ignore it. Things went well in hospital for a while, she responded to antibiotics and the plan was for me to take her home and continue to administer IV antibiotics at home, but of course this was never going to be a smooth journey. To cut a long story short, big sister Xanthe accidentally pulled out Daisy's jejunostomy tube (all 30cms of it, sutured into place in her intestine) while Daisy was allowed home for a few hours before discharge. I had been out at the time and came back to chaos - Daisy screaming, Theo panicking, Xanthe very tearful and Andy with a 30 cm jejunostomy tube in his hands. Normally if a child just has a gastrostomy button, which Daisy used to have, it is possible to simply reinsert it - we have done this at home previously. A jejunostomy is a different matter as it has to be put back surgically....and so our problems began.

As far as GOS was concerned Daisy was not an emergency as she had a Hickman line and could receive nutrition via TPN, the fact that we had to run her TPN over 24 hours and switch her immunosuppressants back to IV infusions lasting for 2 hours twice a day, didn't seem to matter. We were frantically trying to get her back into GOS to get her jej tube back in as she had been so close to home after what had initially been just a blip. But of course once one thing goes wrong the house of cards just falls down, the fluid came back on Daisy's hips causing her massive pain and then no suprises she spiked another temperature. Of course Daisy couldn't spike a temperature in a safe controlled environment like the hospital ward, instead she waited until we had been allowed a few hours day release at Chase Hospice.

The plan had been to leave Daisy with Gemma and Rachel at Chase while I took the three other children for lunch. We had just settled down for a lovely lunch when I got a call from Gemma - Daisy's temp was up and she had become poorly - all in the space of an hour. Cue a frantic request to our waitress for takeaway containers, our meals were poured into them and we ran through the streets of Guildford. The children were so brilliant, taking it all in their stride as always. I was doing my quick mental scenarios of what to do - if she was dangerously septic then it was a 999 call scenario, if not well enough to travel back to Queen Mary's then we would have to take her to the nearest hospital, Royal Surrey, who not suprisingly have their own set of notes on Daisy. Fortunately when I got to Chase I felt I had enough time to get her back to Queen Marys. The lovely girls from Chase dealt with the issue of what to do with the other children by taking them swimming and arranging to bring them home separately.

Daisy was started back on IV antibiotics, yet again but at least GOS had come through with a date for the surgery. The biggest issue with having Daisy in over the summer holidays was not only that the children were on holiday but also anti- Swine flu measures meant that only two named adults could be with Daisy on the ward so I was left juggling children, Andy's work schedule and Daisy on 24 hour TPN. The agreement with GOS when we had been discharged had been that if Daisy ended back up in hospital again only Andy or I would access her hickman line. That worked well for the first few days, but as the days turned into weeks and the number of times her line needed to be accessed increased with infection the practicalities of this became more and more complex. The bottom line was that I was expected to be two places at once, 24 hours a day with Daisy administering 4 hours worth of IV infusions plus various antibiotics, taking bloods and managing her TPN as well as looking after our 3 other children on their summer holiday - clearly not practical especially as all my friends were on holiday (and why should they have to look after another child during the school holidays), by this stage all the holiday clubs were booked up and my mother had swine flu and couldn't come up. At one point I even had to leave the children for over an hour in the lift lobby outside the hospital ward door playing on their nintendo's while I sorted Daisy out! A complete nightmare!

I think our experience of looking after Daisy over the summer in the hospital shows that with Daisy you can have plans - for example being strict on exactly who can touch her hickman line, but the reality of our lives means that these plans need to be fluid. As I said to the Matron of the Children's ward would she expect a member of her staff to work 24 hours a day, seven days a week with no break? I think what really brought it home for me also was when Daisy developed an infection while on the ward and I was worried about her - I wanted to be worried about her as a mum, not also worrying about managing the medical side of her. The pressure of managing a lot more of Daisy's medical needs while she was an inpatient knocked both Andy's and my confidence as we realised how much pressure there is on managing Daisy's complex medical needs. The GOS policy is to minimise the number of people accessing a TPN dependent child's central line in order to prevent infection, but the reality is this needs to be balanced with the practical and emotional needs of the family. We are going to have further conversations with all of the teams involved in Daisy's nursing care to fine tune plans based on our experiences over the summer.

Anyway, to cut a long story short, Daisy ended up back on Rainforest ward and eventually she had her jejunostomy replaced. Having spent 2 weeks with just a naso-gastric tube holding the stoma open we were not even sure if the existing stoma would be viable. This was pointed out to me by the surgeon who came out of the theatre to inform me that if they could not use the existing stoma they would have to wake Daisy up and rebook her as they did not have enough time to do the more complicated procedure of opening a new stoma. Well, thankfully (and I say thankfully for the surgeon's sake as she seemed to think it would be OK to anaesthetise my child for possibly no reason) the stoma was viable and she came back from theatre with a lovely shiny new trans-gastric jejunostomy.....and an allergic rash to the anaesthetic which required IV antihistamine...

While we were in GOS Daisy had a scheduled appointment with her orthopaedic surgeon. This has to rate as the worst meeting with a consultant I have ever had, for so many reasons. At the time Andy was at home with a bad kidney infection, running a high temperature and trying to look after 3 children, I was completely sleep deprived and emotionally raw . Orthopaedic clinics are always chaotic and this was no exception, lots of wheelchairs means limited space, ontop of that we were late and Daisy's infusion had not finished, I forgot to pick up her notes when I left the ward so that caused further delays. When we got to the surgeon he did not even acknowledge Daisy and just leapt straight into "well you want me to look at her hips" - well no actually I would like you to understand some of the complex orthopaedic issues the children with costello syndrome have, currently manifesting as severely tightened achilles tendons causing her an inability to put her feet flat on the ground or walk unaided, her risk of scoliosis, and yes, the fact that she keeps getting this fluid on her hips which is not a costello thing and is causing her a lot of pain.....that's what I wanted to say but instead I blubbed some nonsense and burst into tears, something I never do infront of health professionals. It probably helped slightly as, although he still did not interact with Daisy (I guess this is true of most surgeons, they are used to seeing their patients asleep), he said her feet needed surgery and that her hips were a rheumotology issue and the gastro team on Rainforest should involved rheumotology in her care. Bang, consultation over, except it was only later I realised there was no plan for scheduling her foot surgery and the problem with Daisy's hips was not his problem....

We waited all day on Rainforest for the rheumotology team to come and review Daisy, possibly with the plan of booking her in to inject steroids into her hips under general anaesthetic. The plan had been to transfer back to Queen Mary's that day so transport was delayed until this review, the review that never came because at 5pm a call came through to the ward that the Rheumotology registrar could not make it that day (something to do with being the Friday before the bank holiday weekend perhaps) and our local hospital should refer and sort it out - bloody great. So there we were, no ambulances available to transport Daisy back to Queen Marys and no further forward on sorting out the problem with her hips...we got back to Queen Mary's at 9.30pm and as for the Rheumotology appointment, well we are still waiting, I'm planning to see what tomorrow's post brings and then it may be toys out of the pram time again.

The rest of Daisy's time in hospital was spent weaning her TPN down from 24 hours a day back to 14 hours and building up her jejunostomy feeds ml by ml to 15mls an hours for 10 hours a day - no hopes of any breaks from some sort of pump yet as her blood sugar drops as soon as they are stopped. But the good news is that after a month in hospital - in fact adding it up it was 12 months in hospital altogether with just 18 days at home - Daisy came back home again last Tuesday!!! Yippeeee!

We are seizing the moment as we strongly expect another crisis to hit, there is no reason not to expect this, so in the meantime while she is looking the best she has for months, she is back at school for 4 hours a day. And loving every minute of it of course. We are just loving having her home - everything is so much easier when she is at home; updating the blog, doing the TPN, doing the meds, helping the other children with their homework, catching up with my friends, spending time with my husband - it's just great and I really do savour these moments to keep me going during the inevitable hospital stays that will come.

Next week brings a cardiology appointment and the worry that brings with it that her cardiomyopathy may be worse still. But it also brings the normality of family life as the children get back into the full swing of school life, after school clubs, music lessons, swimming. With Daisy at school during the morning I can start attacking the cupboards which have been stuffed full of clutter and papers over the past year - the obsessive compulsive in me is looking forward to this greatly!!!!

Till the next time...which I promise will not be as long...

2009-08-10T21:31:00.006+01:00

Daisy has now been home for 2 weeks and, just like the pain of childbirth, memories of the endless months in hospital are slowly disappearing.... Just because Daisy is home does not mean she is better and the stress and worry of looking after her is still there, however this time we are in control and at least we are in our own environment.

The first week at home was spent getting into the swing of doing the TPN at home, clearly 10 months in hospital has institutionalised me as I have arranged Daisy's drug cupboard like a hospital cupboard and labelled all the shelves and boxes , sometimes the obsessive compulsive streak in me can be quite handy. We have now been trying to arrange and organise everything Daisy needs to have some sort of normality and for Andy and I to get a break. We have been lucky in getting our care package hours increased to get some more support during the day and we have relented and agreed to having night support which will mean having someone in the house for a few nights a week to manage Daisy's needs overnight. We have always shied away from this sort of support, preferring to rely on time spent at our hospice as overnight help, but as Daisy has moved the goalposts in her needs somewhat we have to accept more help with her. Of course this is all in the planning stage - now we need to get the right people in place to help us. When you have a child with complex needs you get used to having people come to the house all the time, over the years we have got used to a regular team of people - our friends from Chase Hospice and The Rainbow Trust, community nurses, key worker, social worker, physio. Now our team will be expanded even more.

We had a discharge videoconference with the Great Ormond Street Team and Queen Marys team a few days after coming home. Some people couldn't make the meeting but I was still amazed with how many people are involved in Daisy's medical care - and this was only to discuss the gastro elements of her life. We now have plans for managing future line infections and how to manage various other emergencies. The main plan to stay infection free however is for only Andy or I to ever touch Daisy's hickman line. This is a big emphasis with the GOSH home TPN programme, their research shows that the less people accessing the line, the less risk of infection. We also have to use anti-microbial "line locks" which have been proven to decrease infections in home TPN patients by 70%. The bottom line is to try and optimise Daisy's chances of remaining infection free we have to commit to only being the ones to put up and take down the TPN, take bloods from her line and do all the other stuff involved with managing a child with a central line on home TPN. At times over the past 10 months we were very close to not bringing Daisy home alive so this is a small price to pay for having her home.

Our second week at home has been spent at our wonderful hospice, Chase, in Guildford. Daisy stayed in her own room (complete with obligatory TV showing continuous Peppa Pig episodes) while the three other children, Andy and I stayed in an upstairs flat in the building. I did the TPN connections in the evenings and disconnections in the morning but apart from answering questions on Daisy's care my role was redundant which meant I could indulge in some retail therapy in Guildford and enjoy lovely walks along the canal near the hospice with Andy and the children. In fact tempting the children out into the fresh air for walks was really difficult as there is so much to do at Chase and the british weather even managed to shine for a couple of days so they could play in the garden. We left on Sunday, a bit worried as Daisy decided to spike a temperature and I had visions of putting the A&E plan into action. Her temp came down later in the evening and I am keeping my fingers crossed it was nothing sinister, although we did blood cultures this morning to rule out any infection. Daisy is massively at risk of infection at the moment as not only is she immunsuppressed because of the treatment for the inflammatory bowel disease, but she is also neutropenic, which means she does not have enough of the disease fighting white blood cells to cope with an infection. The GOS doctors think this may be a side effect of one of her medications, unfortunately this medication is working well to help calm down her inflammation so they don't want to take her off it, so for the timebeing she is having regular bloods done to keep a close eye on things.

We have a week at home now and I am still trying to get to the bottom of the mountain of clutter that has grown in all the cupboards since Daisy has been born. The best thing is just being able to spend family time together , Daisy's infection risk means that we can't plan too much but it's so nice all be under one roof - we regularly ignore the phone (sorry to all those that have experienced this) and the door bell because after months of having people around us all the time, it's just so good to be just us....

2009-07-24T20:39:00.008+01:00

Daisy is Home!!!!!!!!!!!

Obviously, given the time which has elapsed between this and the last post you can guess there were a few hitches along the way! We had been working towards coming home on Thursday 16th July and Andy and I had completed our TPN training and had done our first "live" connections and disconnections on Daisy. Very daunting as it really goes against your instinct as a parent to connect your child to a bag of fluids which while keeping her alive could also kill her if you get it wrong. Still, we did it and were all set to come home when out of the blue on Tuesday night she lost the ability to move her legs and was screaming in agony. Now I know that this blog is called "is this in the plan" but recently I have seriously thought about renaming it " well, we didn't see that one coming...."

There are times when I am so grateful that Daisy is already in Great Ormond Street Hospital, and last Tuesday night was certainly one of them as we had top doctors and nurse practitioners gathered around her cot scratching their heads and wondering what had happened to suddenly cause this acute episode. An ultrasound of Daisy's hips revealed she had fluid on both of them, so instead of going home we made yet another trip to theatre and the MRI suite to have her hips looked at and the fluid drained from them. Over the course of the weekend it was apparent that the only thing now keeping us in hospital was the 4 times a day doses of two different antibiotics to treat the infection in her hips....Andy and I were clearly competent in connecting and disconnecting TPN, taking blood, changing dressings, adding spec sets and changing smart sites, drawing up jejunal and gastrostomy meds and connecting enteral giving sets...we were more than happy to take her home on iv antibiotics. Cue visits from a few more hospital teams - infectious diseases, the unfortunate name of the team that look at children that have lots of infections (like Daisy)..Irony of ironies, the doctors from the infectious diseases team was the locum doctor who treated her in February when she nearly died of a line infection, the very man who phoned me at midnight and was treated to the spectacle of me in my Primark Leisure wear! He of course remembered Daisy, as anyone who ever meets her never forgets her, and ordered quite a few immunology tests. Although all of Daisy's recent infections have been from her hickman line, in common with many gastro children she also has a problem of translocation where the bacteria which normally sit in the gut move into the bloodstream, and being immunosuppressed to control her inflammatory bowel disease this is a major problem for Daisy.

We also met the lovely Dr from the Orthopaedic team (Xanthe insists he looks like Gary Lineker but in my myopic state I am reminded of a young George Clooney on ER). Daisy has been under the Orthopaedic Team at our local hospital for a long while now but this new change in events coupled with Daisy's need for Ankle supports and extra tight achilles tendons means that she is well and truly on the radar of this team now and will definitely need botox injections into her achilles tendons, probably surgery to lengthen her tendons and constant monitoring of her hips to make sure the fluid and inflammation does not come back.

Anyway, to cut a long story short, the fluid was drained from Daisy's hips, the physio's got her walking again, we were able to stop the IV antibiotics and around 3pm on Thursday 23rd July, almost exactly 10 months since she first went into hospital our daughter, Daisy Rose came home and our family became complete again......

I could blog about how wonderful the past 24 hours has been doing normal things like pottering around the house, going to the supermarket, cuddling up on the sofa, but the pictures speak for themselves. Needless to say it is difficult, even before this change our lives were diffficult as Daisy had considerable medical needs. These have now gone through the roof in terms of what we have to do and how we have had to adapt our lives but it means that our daughter is home with her brothers and sister and our family at long last is complete again!!!!!

I cannot finish this post without thanking and acknowledging the family and friends who have helped us on the rollercoaster - the secret gardeners who transformed our front drive, the ironing mother and mother-in-law, the friends who took children in at the very last minute, the people who texted, sent messages, called, sent cards, the nurses, doctors, health professionals.... - you all know who you are and we thank you from the bottom of our hearts, we could not have got through these times without you! Anyway, as Andy says, onwards and upwards, here's to the next chapter in our lives with our beautiful children......don't worry, I'll keep blogging, just want to smell the roses for a while......

2009-07-10T20:36:00.005+01:00

Yes - we're still in Great Ormond Street but hopefully the end of our hospital life is in sight as we have another possible date for release (prison analogies are so relevant) but I'm not naming it as I just don't want to jinx anything!

Our lives were brightened up by the recent visit of the the lovely Johnny Depp - I still can't believe it was really him (it definitely was) - and also a visit by Dr Linden Cullen from Holby City (or rather the actor who plays him!). Daisy was also well and fit enough to really enjoy some of the benefits of the hospital school including a cookery lesson with a West End Chef where she made delicious blueberry muffins and a strawberry tart, she also had a private music lesson with two musicians form the City of London Symphonia and has been a regular at the truly fantastic Singing Hands sessions. Doesn't this all sound like a holiday camp! Well believe me it's not, these little interludes just help us get through the rubbish bits which recently have included taking a good 24 hours to recover properly following a "short acting" anaesthetic for an upper and lower endoscopy, the trauma of having to have a canula to decrease the risk of her hickman line being infected during the endoscopy, being stuck on 24 hour fluids while trying once again to build up her milk feeds, constant "BM" checks to make sure her blood sugar is stable and vomiting meds from her jejunostomy (this shouldn't happen because this is not even her stomach).

Anyway - that is all behind us, Daisy appears to be relatively stable now, everyone is happy that she will only have 15 mls an hour feed going into her tummy and this is only to maintain her blood sugar, not to provide any useful nutrition, added to this a special carbohydrate powder giving her 20% carbohydrates in this 15mls or 3 teaspoons, specifically to prevent the hypoglycaemia. The growth hormone therapy, while not achieving the metabolic stability we had hoped for (growth hormone has a role to play in helping regulate blood sugar) has at least enabled Daisy to grow to the point that she can now wear a small backpack and carry her feedpump around with her for the 10 hours a day when she is not on TPN - this is absolutely fantastic as it means she is independent for the first time since she learned to walk and can now make decisions about where she wants to go. Today she ran up to Andy and gave him a hug when he walked into the room - this is the first time she has been able to do this without having to wait for someone to move her pump. Daisy has also been getting some physiotherapy at last and now wears splints on her legs and feet to attempt to stretch her achilles tendons which are now so tight that without them she does a fair impression of Darcy Bussell on pointes. The likelihood is that the next stage will be botox injections and casting to try and stretch her achilles so that she can walk with flat feet without her splints and longer term possibly tendon lengthening surgery. All this is fairly common in Costello Syndrome, but as her consultant says having the Costello Syndrome on its own or the Gastro problem on its own would be bad enough, but both together is a real double whammy as she just has to battle with so many issues. Which in true Daisy form she does with a huge smile on her face and massive determination.

TPN training had been put on hold with all of the setbacks but now it is completely back on track and the big day for Andy and I is Monday when we will be watched by all three Nutrition nurses doing a dummy run through of a connection and disconnection of TPN in preparation for doing a "live" connection on Daisy. Just to add to the pressure one of the nurses popped her head around the door this afternoon to remind me that if either of us did anything wrong we would not be able to do a live connection and Daisy's discharge would be delayed. This is worse than any test or exam either of us have ever had to do as so much is at stake - essentially our daughter's life. If we make a mistake when putting up the TPN we could cause a fatal line infection or air embolism which could kill her - the fact that Daisy has already had so many touch and go line infections makes us fully appreciate what a major deal going home on TPN is, you cannot cut corners and you cannot have any lapses in concentration when doing it as it really is a matter of life and death.........

After 10 months going home is really daunting, we have been so used to having nurses and doctors around if we have any concerns, now we will have to dial 999 if we have any worries - the days of me piling Daisy into the car to go to the hospital while Andy stays at home with the children are gone, it's a good thing Daisy is used to ambulances as this will be the emergency transport if she spikes a temperature as it probably is a line infection and we cannot risk her deteriorating on a car journey...

So now the end is in sight the next thing we are having to contend with is the worry of Swine Flu - blimey there's always something isn't there? We can't wrap her up in cotton wool, so we just need to be aware, the plan is that if she has know contact with Swine Flu we have to start Tamiflu immediately, hopefully this will nip it in the bud as the last place she will need to be is hospital - apparantly Queen Mary's is full of Swine Flu cases at the moment so I don't think we will be making any social visits when we get out of hospital!

My plan for the weekend is to swot up on my TPN drill , I know from talking to other home TPN parents here that it will become second nature once we get her home but at the moment, like learning to drive, I am having to think through each move. I also want to go to Patisserie Valerie and order a massive cake with "Welcome Home Daisy" to take home with us, there's a bottle of Bollinger in the fridge waiting at home although I doubt if Andy and will be drinking it, we'll be tossing a coin for who does the first home TPN connection......

2009-07-05T09:50:00.003+01:00

When Johnny met Daisy...............

an unexpected visit from Johnny Depp dressed as Captain Jack Sparrow.....he has strong links with the hospital after they saved his daughter's life a couple of years ago. Obviously he was very taken with the lovely Daisy, particularly when she blew kisses at him although when he walked through the door she signed "where's Peppa Pig?"...

More blog news to follow tomorrow - home for a few hours to unpack the delivery of all the equipment we need to manage TPN at home...

2009-06-24T22:51:00.002+01:00

Same old, same old - Daisy has infection, Daisy recovers from infection, gut becomes inflamed again, feeds not tolerated - and on and on and on we go.....next month it will be 10 months since we first were admitted to hospital........

Daisy is clear of the last line infection but her gut is clearly still inflamed so she will definitely have an endoscopy next week and I'm hoping they will be able to get some more biopsies this time to give us some more answers. She is still on IV antibiotics as they are all so twitchy about taking her off them and opening the floodgates for another infection. Her consultant came around today and he did refer to Daisy as a miracle child given all that she has been through, he said the plan is to get her stable enough to get home and then try and work towards - if at all possible- a cure.....yes he said cure so maybe he has something in mind??? One of the last resort treatments for Daisy's gut condition is a Bone Marrow Transplant, maybe he was thinking of that??? The problem is with us parents of chronically sick children, we hang on every word our consultants say looking for a little source of hope, they do not realise how much we churn over the conversations we have had with them, pinning our hopes on maybe just one word...

As always in the plate spinning world that is Daisy there is a problem, and this week it's back to her old problem of blood sugar regulation. One of the reasons she is on 24 hour iv fluids is that she cannot tolerate any time without sugar and we cannot get enough into her tummy to keep her blood sugar up. In fact if there is any delay in her IV being changed her blood sugars drop and she goes "hypo". Fortunately Daisy's endocrinologist also covers this ward so he will try and work out what we can do to try and maintain her blood sugars without this dependency on a constant supply of glucose into her bloodstream. The reason he had originally diagnosed for her problems was insufficient growth hormone, as this plays a role in blood sugar regulation. However Daisy has been having Growth Hormone injections for quite a while now and while she has grown to nearly 90cms the sugar regulation problem remains.

In herself she is really happy and enjoying the benefits of staying at GOS as it is so well geared up for long stay patients - she has a visit every day from a teacher who is actually able to use sign language, and she also goes to Singing Hands sessions and Music Sessions in the hospital school as well as spending time in the activity centre playing with other children. Then there are the visitors to the ward - neither Daisy or I are very enamoured with the Clown Doctors finding them deeply unfunny but we did enjoy a recent visit from the living legend that is Sir Terry Wogan.

So on we plod - Daisy has had a few tests this week, a video fluoroscopy which had to be abandoned as she refluxed up all the contrast fluid used to show up her gut, the outcome of which confirmed what we already knew, her tummy is now redundant and cannot be used at all. She also had a DMSA scan of her kidneys today to check for scarring following lots of urine infections as this may be the cause of her elevated blood pressure. We restarted feeds into her jejunostomy today, slowly, slowly as always at a rate of 5mls an hour, so it will be a while before she is on enough volume to take her off daytime iv fluids. Of course all of this delays us getting home, TPN training has been delayed and now it is getting really close to my deadline of the long school holidays to be home. I am reaching the end of my tether - I don't mind hospital when Daisy is acutely unwell, she is safe there, but as her condition is chronic, Andy and I can manage her at home. I hate that she is referred to as a patient and that we are so institutionalised, I don't enjoy my goldfish bowl existence or this complete lack of privacy, I crave the chance to sit on the sofa, cook food in anything other than a microwave and shut the door to the rest of the world. Most of all I am fed up of our family not being together - I want to be able to help Theo with his homework face to face, not via the phone, I want to make sure that Xanthe is remembering her Irish Dancing shoes on a thursday, not texting Andy to remind her and I don't want to miss any more of Jules growing up....

2009-06-15T23:25:00.004+01:00

A friend of mine in a similar situation once described this life as like a game of snakes and ladders - you climb a ladder and make some progress towards your goal then when you least expect it you hit one of those pesky snakes and back you go again.... Over the past few days we have been climbing some big ladders, Daisy was managing 10 hours off TPN, she was enrolled in the hospital school and loving it, Andy and I were enrolled in TPN training school and feeling one step closer to bringing her home, we had even discussed a date of 24th June to discharge Daisy back home for good - I use the past tense as when we least expected it we hit that great big snake you get just before the finish line, the one that sends you all the way to the bottom of the board again....

Daisy started vomiting on Thursday evening and by Friday evening it was clear that her gut had gone on strike again (technically called pseudo obstruction) just as it had when she was first admitted to hospital all those months ago, her temperature also shot up and blood cultures show that once again she has a line infection. The bottom line is that she has had another flare up of her gut inflammation which has caused her to gut to stop working again, this has meant that we have had to stop anything going into her gastrostomy or jejunostomy. She is back on 24 hour IV fluids/TPN plus IV antibiotic infusions and her immunosuppressants have also been switched to IVs. Her consultant came to see her today and confirmed what we already knew, Daisy will be on TPN for the rest of her life. The plan now is to try and manage the inflammation again and then she will have another scope (where a camera looks at her gut to see what is going on), she may have to go onto even stronger immunosuppressants which would definitely only be IV infusions and at this stage we really don't know if getting back onto feeds into her jejenum (top of her intestine) is a possibility. All we can do is hope that in the same way Daisy has suprised us with the sudden turn of events she will suprise us by picking up and getting back to where we were before.

As always we hope to save her hickman line but the infection is pretty bad as it is also around the entry site in her skin as well as in the line. It's just a waiting game to see what the next few weeks bring. Andy and I are continuing our TPN training and all the equipment we need is being delivered to the house so at least we will have everything in place although it is looking likely that the hospital will have to bend their own rules and allow Daisy home with minimum breaks from the TPN.

The rest of the children are really disappointed - I was supposed to have brought Daisy home for the day on Saturday to have a belated birthday party for me (it was my birthday on the 10th) - instead I came home on my own and then spent most of the day catching up on sleep as I had spent the previous night up with Daisy.

I guess I was a bit taken aback when the hospital were talking about a discharge date of the 24th - always in my mind I had imagined that Daisy would get home to coincide with the rest of the children breaking up for the end of term so perhaps if I keep that in mind then I won't be disappointed and we really will get home in time for the summer holidays.....

2009-06-08T21:58:00.004+01:00

So we got Daisy over her latest line infection and managed to save the line and life was ticking along, with the big focus on her next admission to Great Ormond Street. I had a call early last week saying that we were booked into a 5 day bed for her to have two procedures on two separate days under general anaesthetic. In complete frustration at the slow progress in moving Daisy from 6 hours off TPN to a regime where she would be able to come home I phoned the nutrition nurses at Great Ormond Street and basically said that Daisy, all of us, needed a life and we need to find a way to get her out of hospital and home.

Cue a second phone call from Great Ormond Street late last week, can we bring Daisy in that day, in fact asap, as a long stay bed had become available. Sometimes you have to be careful with what you wish for – one minute I was plodding along enjoying the few hours freedom in the day with Daisy and gearing up for a short term stay at GOS , leaving hopefully with a date to start TPN training and bringing her home, next minute I am frantically rushing around, phoning friends to pick up the children, ironing clothes and sorting out the million and one things that need to be sorted out when you have a family of 6 so that Daisy and I could transfer from Queen Mary’s to GOS quickly enough not to lose the bed. We arrived last Thursday at 8pm, not really knowing why we were there…

The first thing that needed to be sorted was another blood transfusion. Daisy has been chronically anaemic for a long time now and while she copes well with a haemoglobin level well below target levels every now and then this drops very low and she needs a transfusion. The levels of immunosuppressants are currently low in her body and the doses are needing constant tweaking and changing. The most exciting thing is that following my call to the nutrition nurses Daisy became the priority for home tpn training and set up and we arrived her to find there was actually a plan to get her home!

For the past few days we have been weaning down the hours Daisy spends on TPN – it’s early days yet but so far so good as she needs to be 8-10 hours a day off TPN to be allowed to go home on TPN. The dietitian has been keen to try and increase the amount of feed that is going into Daisy’s jejunostomy, however sometimes an increase of even a couple of mls and hour can upset the applecart with Daisy and for the past two nights she has vomited so we may not be able to push these feeds too much. However she is now allowed a two hours break from her milk feed pump which means for two whole hours a day she is completely free of tubes and wires and skips around like a little spring lamb – it’s the best sight in the world!!!!

The plan for the week coming is for Andy and I to start TPN training, fitting this around the two general anaesthetics that Daisy needs this week plus any thing else she throws our way (another transfusion is a possibility). Given that this is the first week of Andy leaving his old company on a redundancy package and starting up his new business this is a huge commitment on our part as he has had to completely rejuggle everything to fit this in. We have also had to completely rejuggle the children’s schedules to take into account Andy and I both spending all day at the hospital. But to get Daisy home this has got to be worth it.

So as I said at the beginning, be careful what you wish for.........I am so excited at the prospect of having all my family under one roof, but at the same so daunted, not by having to do the tpn, we will do whatever it takes to give Daisy quality of life and both Andy and I are dab hands at changing gastrostomy buttons, checking blood sugars, placing ng tubes....It's more the fact that for the past 9+ months we have been cocooned in this world of hospitals, desparate to get out and reclaim our role as parents, and reclaim our daughter. Now this is about to happen we realise how much we have depended on nurses and doctors being round us all the time, providing us with a security blanket, now it's over to us. I guess the thing I am dreading more than anything is night-time. Children with Costello Syndrome are notoriously bad sleepers, add into that reflux, tummy pains, beeping and leaking pumps, itchiness from excema, oversensitivity to noise and you get the recipe for broken nights. Theoretically we are entitled to some sort of night respite package but whether that will be forthcoming in the short term is another matter. And then there's the twitchiness about line infections and illness - we have seen Daisy go dangerously downhill very very quickly, this makes you very overprotective as parents and it will take me a while to feel safe without the comfort of a resus trolley outside her door and a picu down the corridor. The children will have to adjust too, they will get a full time sister back and parents who need to focus on her needs while in the house. The time we need to put TPN up is the worst time of the evening for parents - 6pm -7pm, right in the middle of tea time, activities, homework, bath time - our already very self sufficient children will have to become even more so. It will be so good to get back to being a full time mum to all of them, we are realistic in knowing that we will still get stays in hospital but nothing should ever be as long as this stay, who knows, maybe next year I can even get back to work!!!!!! (those who know me well will know this is only half a joke, watch out, maybe I will sign up for med school!)

2009-05-21T18:27:00.003+01:00

I guess I blogged too soon......at 6am this morning I had a call from Chase to say that Daisy's temp had spiked to 41.5 and they had called an ambulance to transfer her to the nearest hospital. I woke the children and delivered them to my wonderful friend Maria (thank you so much Maria!!!) and bombed down the A3 to the Royal Surrey Hospital in Guildford.

In true Daisy style she was still smiling, however her blood pressure was unstable and she needed one of her favourite IV fluid boluses to stabilise her. Blood cultures have been taken but we are working on the assumption she once again has bacterial sepsis caused by a line infection. Antibiotics have been started (2 weeks and 1 day since the last lot were finished - a record!) and by late afternoon she was stable enough to be transferred back to our wonderful Queen Mary's Hospital.

As always it's two steps forward and one step back and at least this time she did not need to go into Paediatric Intensive Care....and she did get to go to Chase at last, though leaving by blue light ambulance was not quite in the plan....

2009-05-19T22:26:00.004+01:00

We achieved another milestone yesterday. Since Christmas every time we have tried to get another break at our hospice our plans have been scuppered at the last minute, mainly by Daisy getting an infection and becoming very poorly. Well we managed it at last, and I took Daisy to Christophers, the Chase Hospice in Guildford, yesterday afternoon. I did not want to jinx anything so did not pack for her until the very last minute and there was a little glitch when she developed four strange new spots on her face (which the doctors think are some sort of allergy reaction).

She is there for two nights until Thursday lunchtime - theoretically this should give me a break but the reality is I have had a day of taking rubbish to the dump, sorting out the children's clothes, tidying cupboards and taking bags to the charity shop. I know to some people this will not be the ideal day off but for me it is a chance to start gaining some control back, over the past few months (and years) we have lived off our nerves, ultimately Daisy's condition is incurable and the uncertainty of what will be around the corner is always going to be there but at least we have learned to go with the flow of it now. This way of life is the norm for us now so rather than putting off sorting out the house until things get better I have decided to deal with things now. Things have not got worse and that's the best way to look with things. so rather than putting off all those house tidying projects I'm going to tackle them now....I have black bags at the ready and I'm braced to sort out the children's wardrobes once and for all!

I did speak to the nurses looking after Daisy this evening and she has a low grade fever so I'm keeping everything crossed this does not materialise into something more sinister. As always I sleep with my mobile and the home phone next to my bed so will not be suprised if I do get a call in the early hours of the morning. It will be a bit of logistical nightmare if the hospice do call to say that Daisy needs to be back in hospital however as Andy is in Manchester tonight so I am on my own with the three older children. We are blessed with the most wonderful friends and I know I can phone any number of good friends in the early hours if needs be to sit with the children if I do need to drive to Guildford.

Andy is in Manchester for a couple of business development meetings for his new business. He officially leaves his company on a redundancy package next week and we both agree that this is the best thing that could happen to us. He was looking increasingly tired and stressed and could not have continued at the pace that he was working, it is so nice to see him looking relaxed and even losing a few pounds in weight as a result of being made redundant! The one thing Daisy has taught us is to really put things in perspective - the most important thing is family time and creating happy memories. On Friday Andy will be able to attend one of Xanthe's school assemblies for the first time in years, that is worth all the bonuses in the world!

2009-05-14T15:23:00.002+01:00

Daisy's first afternoon back at school

We are trying to take Daisy to school for 2 -3 afternoons a week to coincide with her breaks from TPN - I stay with her for the session as she is still so medically fragile but as you can see from the picture she is so happy to be back, even though all she can do is sniff and lick the crisps on her plate! (you can see the line to her enteral (milk) feed pump at the back of the chair - my role at school is basically to follow her around with the backpack containing the pump - from Global Brand Manager to official bag carrier....)

2009-05-13T20:04:00.003+01:00

At last we have moved into a new chapter in Daisy's life. She is now able to tolerate 6 hours off TPN!!! This means for 6 hours of the day she does not have mixture of vitamins, minerals, lipids and other essentials to life dripping into her via a hickman line into a main vein - which means that she can leave the hospital for a few hours. This has made such a big improvement to the quality of Daisy's life as while she was on 24 hour TPN and a hospital based pump she was essentially tied to walking up and down the ward, now, we can get out and about. And the best thing is that just over a week ago we brought Daisy home for the afternoon!!!! This was the first time she had been home in nearly 8 months so it was a huge step for us all.

Daisy amazes me at how adaptable she is, she was so excited to come home and now takes it in her stride and is not even upset when it is time to go back to the hospital (at 5.30) - she even gets excited seeing her room in the hospital again as it is also such a part of her life. I'm so relieved that Daisy has transitioned into this new stage so easily, the night before she was due to come home for the first time I lay in bed worrying - for nearly 8 months our lives have revolved around Daisy being in hospital and we have adapted our home life to fit with this. Would it feel strange having her around the house again? Of course, I needn't have worried, from the moment she arrived home the traumas and worries of the past few months melted away. Lots of people have commented how the other children look so much happier too, they love to come home and play with Daisy and we have settled into a routine of sorts. The TPN can be taken down at 12pm and goes back up again at 6pm, it's amazing how much we can fit in during these short hours. One of the real bonuses is that I have been able to take Daisy back to school for a few sessions, it's a safe environment for Daisy infection wise as there are only 2 other children in her class, she also gets to spend time with the class next door which only has around 6 children. Not suprisingly we have seen huge leaps in Daisy's development since this new phase has begun, she is more vocal, is getting stronger on her feet and her sign language is racing ahead.

One of the unexpected problems has been a massive increase in Daisy's summer allergies, having been confined to a room for so long to suddenly be released to a pollen filled outdoors has meant that her nose is constantly streaming and any effort leaves her wheezing. Hopefully this is a small price to pay for a taste of life in the real world!

The reality is however that Daisy is still attached to a pump 24 hours a day. From a few months of age she has been feed her prescription milk feed via a pump into her tummy for 20+ hours a day. In order to maintain her blood sugars while off TPN and continue to stimulate her dodgy gastrointestinal system Daisy is attached to her feed pump all the time, running at a fantastic rate of 35 mls an hour - the highest she has managed since last summer. The feed all goes into her new jejunal tube at the top of her intestines, we know that not all of it is absorbed and she still has really runny nappies but this too is a step forward. The truth is though that being attached to a pump all the time is limiting her new freedom, I would love to see her make her own way around the classroom without an adult walking behind with her backpack containing the pump. She is too small and weak yet to carry the backpack so for the timebeing she gets freedom, at a price...

The next milestone would be to have a complete fortnight off iv antibiotics without any more line/blood infections. Next week she will hopefully reach this point so fingers crossed! We are waiting for the funding panel at the hospital to approve the use of specialist line locks which we can put into Daisy's Hickman line and decrease the risk of line infections. In fact waiting for funding panels to approve various things is going to be the common theme for us now. Despite our excitement at the thought of getting a mobile TPN pump following our last GOS visit, this has been stalled a bit by the fact that in todays wonderful NHS, devolved budgets means that no-one actually wants to pay for anything as it is always someone else's responsibility... So in the meantime we are still waiting for some appropriate funding panel to approve the mobile TPN pump which would ultimately mean that Daisy could have even more time at home or school and improve her quality of life even more. Another example of this crazy situation is Daisy's enteral pump which we use to give her milk feeds. She has had a mobile enteral pump for many years but while in hospital tends to use the hospital feed pumps. As we are now out and about for a few hours we use her mobile pump reverting the the hospital pump for overnight feeds. When I tried to order a box of giving sets (the tubes which attach to Daisy's tummy) which we use with the mobile pump I ran into a situation worthy of a Monty Python sketch - as Daisy is in hospital we cannot order equipment (ie giving sets) for use at home, but the only way she can be mobile at home is to use the mobile pump -again this is all down to who owns the budget for what. Much as I love our wonderful NHS, Daisy is testing their systems to their limits!

A while ago, in the early days when Daisy was still tiny I would have got angry and thrown my toys out of the pram at this situation but the years, and particularly the last 8 months have taught me patience and perspective. We are inching towards the day when we don't have to put her back into the car at 5.30 to go back to the hospital, and when the juggling of children, activities and Daisy will be focused around the home a bit more so these bureaucrats and their inconveniences are quite low on my list of priorities - there is always a solution as long as we stay focused on the goal. And in our case the goal is to be home for the summer.

2009-04-29T22:16:00.002+01:00

Well the good news is we are back at Queen Mary's! Daisy had a new Hickman line placed and her picc line removed last Tuesday and was transferred back from GOS last Wednesday. It was wonderful to arrive back, everyone was so excited to see her and the staff had arranged all her toys and written "welcome back Daisy" on her white board. I could sense Daisy visibly relaxing, it's not quite home but at least it's closer to home and at Queen Mary's everyone knows her so well, she was innundated with visitors from the ward for the rest of the day.

The next day she was very subdued and I had a sneaky suspicion something was not quite right, this was confirmed later that afternoon when she fell asleep, something she only does during the day if she is ill, and woke up with a 40.2 temperature. It was actually one of the few times I just felt like bursting into tears then and there - not another line infection, and only 36 hours since the new one had been placed. She also developed copious runny nappies so I was also panicking about Cdiff, which (of course) Daisy has already had! Well thankfully she does not have Cdiff although the runny nappies are still continuing and look more like her gut deciding it's not going to work (not that it actually works anyway) but she does have a line infection and her gastrostomy site is colonised with staff aureus. Now having managed against all the odds to clear her of MRSA (methycillin resistant staff aureus) I don't want these little bugs to go and mutate into it so we are trying to be really rigorous with infection control procedures - not that we weren't anyway, it's just we all know what Daisy can do. So to cut a long story short she is back on IV antibiotics . This is such a familiar patter, she had only been off antibiotics for 8 days before the new infection, we have not managed a fortnight off antibiotics since the summer, that would be a real milestone!

We are, in Daisy fashion, making progress - ie; two steps forward and one step back but at least making some progress. The exciting news is that we are now on the radar of the Nutrition Nurse Specialists at GOS. To be able to go home we have to be on their radar as they organise the home TPN package, they also insist on children being a minimum of 10 hours off TPN a day(they have moved the goalposts, I had heard it was 8 hours!). The best thing is they agreed with our need to get Daisy out of the hospital and getting some sort of life outside of her isolation room as 7 months in isolation is not conducive to neurodevelopment and probably some sort of crime against her basic human rights! So we have actually left GOS this time with a plan - both to start giving Daisy breaks from TPN so that she can get outside and to try and get a mobile pump for times when she is on TPN.

The former is probably easier to achieve than the latter. This week, we implemented the TPN weaning plan with the aim being to get her to tolerating 6 hour breaks off the TPN. She still will be permanently attached to her other pump which drips milk into her jejunostomy, but that has been a permanent fixture 24/7 since she was just a few months old (and I promise never to complain about being tied to a pump again knowing now what the alternative is!). As of today she is tolerating four hours off her TPN and her blood sugars, though on the low side are reasonably stable, I am just hoping that we can achieve 6 hours by next week as I would love to be able to take her back to school for a couple of hours each day. I am also tentatively hoping we can bring Daisy home for a few hours this weekend - I just hope nothing scuppers this plan as I mentioned the possibility to the children and they are so excited at the thought.

Getting a mobile TPN pump is, as always, a lot more complicated than it should be. The plan is to start Daisy on the home delivery service while still in hospital - this means that her TPN would be made up by a pharmacy and 2 weeks supply delivered to her at a time to be used in conjunction with a mobile pump. This will work fine when we are at home but having a child still in hospital accessing a home delivery service is proving a bit difficult for the good old NHS to get it's head around. The issue as always is who pays for it. It Daisy was at home it would be the Primary Care Trust (PCT) but she is in hospital and so they insist that the Hospital Trust should pay, as the delivery is to a hospital and not home the delivery company say that VAT is applicable on the TPN and anyway the hospital trust are saying they have their own TPN pharmacy and so will not pay for something they can provide (albeit without a mobile pump), as Daisy is not currently an inpatient at Great Ormond Street they are not going to pay so we have reached a stalemate until someone out of the three trusts sees sense. I'm just focussing on getting enough time off TPN for Daisy to have some time out, if these silly conversations are still happening by this time next month I may consider a major toys out of pram episode but you never know, maybe it will be all sorted!

The other useful tip we got from the nutrition nurse was the use of line locks to help stop infection. Basically when Daisy's hickman line is not being used we can put a drug called Taurolock into the line to kill any bugs which may be pushed into her blood stream when the line is used again. Apparantly since using this drug they have seen a massive drop in line infections. The pharmacists are on the case and I hope we can start using it soon as we cannot risk any more line infections or losing any more lines.

Anyway, inspite of all the continuing issues, it's fantastic to be home, sleeping in my own bed and spending quality time as a family. The children have been so pleased that Daisy is back close to home - both Jules and Xanthe wrote about it in their school diaries. They are definitely more relaxed now we are out of GOS as they know that the only reason we are ever in that hospital is when things are not so good. I'm just really hoping we get to bring Daisy home for a few hours this weekend and do normal things like sit on the sofa and watch TV, these are things that we all take for granted but when your family is torn apart for so long like ours it's the little things that are so important.

2009-04-16T21:55:00.000+01:00

So, you know what it's like, you transfer to Great Ormond Street for a couple of days for a quick upper and lower endoscopy and end up staying a month....in fact it is now seven months (give or take a few days) since Daisy has been home and coming up to a month since we came to Rainforest Ward.

The good news is that she is so much better than a week ago, her additional potassium is being weaned down and she is off all antibiotics and anti-fungals - this makes me twitchy, she does not last long without needing some sort of weapon of mass destruction coursing through her veins - I will remain positive however, maybe this time the line will last....

The plan now is to take out her picc line and put in a new Hickman line early next week, theoretically then we should be good to go although we will let Miss Daisy decide on that. It has been nice to have Daisy reasonably well over the past few days as we have been able to use the fantastic play facilities at Great Ormond Street. The best thing is the other children have also been able to use the play centre too which has taken some of the pressure off entertaining them. Today Daisy decorated fairy cakes with a group of other children including a young boy on a "Berlin Heart" machine, which is an artificial heart. Great Ormond Street is probably one of the few places in the UK where a little girl being kept alive by a complex mix of chemicals dripped into a central line into her blood stream can play with a boy being kept alive with an artificial heart and no-one bats an eyelid!

I had a good chat with the gastro dietitian today. Understandibly they are very twitchy about pushing Daisy too quickly particularly given her recent experiences. For Daisy to be able to have night time only on TPN she would need to tolerate 34mls an hour of feed a day, with possibly more fluid during hot weather because of her heart and kidney problems. This currently seems a long way off so we are aiming for 2/3 time on TPN which would make us eligible to come home as the minimum that is allowed is 8 hours off TPN. At the moment she is managing 14mls/hour of feed into her tummy over 24 hours but it is difficult to push this any more at the moment as she will need to be starved again for another general anaesthetic next week when her new Hickman line is put in, and we will be back at square one as Daisy cannot go straight back onto full feeds after being starved and will need to build up ml by ml. As always, two steps forward and one step back, however I am optimistic that with the new feeding tube directly into her intestines we can try and aim for our 8 hours off TPN and get home (at some point this year!)

I have now given up on the bed in Daisy's room - basically she is going to wake up and cry whether I am there or not and I need a decent night's sleep to keep functioning, whereas the night staff can go home at the end of their shift, I cannot. For the past few nights I have left the hospital at 8.30pm and slept at home leaving for the hospital at 7.30am the next day. I am looking forward to being back at our local hospital, hopefully next week, it is only 12 minutes drive away and suddenly that life seems so much less complicated than a Northern Line commute to Great Ormond Street. It's over to you now Daisy, show us that you can turn the corner and get some time off that TPN. I'll do all the training I need and put in as many hours as required in the hospital just to get her home.

2009-04-06T12:59:00.000+01:00

People keep asking me how come I am so chilled about things, I guess over four years of this bumpy ride with Daisy has taught me to try and go with the flow and get through it in one piece for both our sakes, I have to take each day and trust that Daisy will continue to defy the odds. I was told by one of our geneticists who knows Daisy well to forget about what the books say and look at Daisy for my strength, this morning she was sat on my lap smiling and waving at her consultant and to look at her you would not believe what she has been through over the past few days (unless of course you looked at her blood results, which tell a very different story).

So last Monday she was scheduled to have her infected Hickman line taken out following a weekend of high temps and lethargy. Unfortunately and in a bizarre way our old nemesis of MRSA came back to haunt us. Daisy is still categorised as MRSA+ in this hospital inspite of all the clear swabs and cultures, the infection control rules here state that the child must be out of hospital for 6 months for this category to be lifted. No-one believes for one minute that Daisy is still colonised with MRSA but unfortunately while she has this against her name she has to be isolated and when it comes to surgery she has to have the last theatre slot so that the theatre can have a "level 2 clean". This situation was becoming farcical, as Daisy was last on the list , the interventional radiologists did not have time to take her line out and her condition was rapidly worsening, her peripherals were shutting down and she needed fluid rescuscitation....cue another trip to the Patient Advice and Liaison Team (Pals) for me and strongly worded emails to infection control from Daisy's medical team. Thank goodness everyone saw sense and her MRSA+ status was lifted and she was scheduled for the next day, second on the list. Not a moment too soon as she was really becoming poorly and also needed a blood transfusion as her haemoglobin had dropped. So that all happened on Tuesday - infected Hickman line whipped out, temporary picc line placed and a blood transfusion given

That should really have been the end of it, and in some ways it was, her temperature came down, but normally a blood transfusion would give Daisy more energy and colour and in this case it didn't. She looked grey and lethargic the next day and her blood gas results showed that her her potassium was dangerously low and her sodium was very high. She was started on extra fluids but we have not been able to bring her potassium levels up. Some of the meds she is on lower potassium and some of them are damaging to the kidneys which play a role in how potassium is used in the body. The past week has been a cycle of taking bloods, running a blood gas, finding her potassium is too low, putting up massive potassium infusions, checking her bloods again....... This is risky, too little potassium can cause the heart to stop, Daisy has cardiomyopathy anyway so this is extra worrying, too much potassium can kill you and we have been pumping maximum doses into her. She has been permanently wired up to an ecg monitor and the doctors have been walking around with worried looks on their faces. One of the meds which suppresses the potassium has been stopped and her immunosuppressant iv infusions have been stopped as they can interfere with the kidneys. This has given us another conundrum - stopping these meds will hopefully help Daisy rebuild her supply of potassium and hold onto it, but not having the immunosuppressants may cause the inflammation to flare up taking us right back to square one and undoing all the progress of the last few months.....

This plate spinning is too much for me, I'm leaving it to her big cheese consultant to work out. He is the expert and Andy and I have confidence that he will do the best thing for Daisy, always aware that our number one priority is to get her home at some point. At his point however we begin our third week at Great Ormond Street, today we had the first potassium level which was in normal limits, in my new found understated manner I did not jump for joy as I need to see a few of these results before being convinced that she is holding onto her potassium and turning the corner. The levels that are required for her immunosuppressants to work are now too low and so a decision needs to be made very soon about what to do next before her gut flares up. Feeds through her jejunostomy seem a long way off at this point but this is the only solution and at some point very soon we need to try again as this is the only way to reduce the reliance on TPN and the vicious cycle of infection and damage it brings...

I'm waiting for someone in the street to say to me "cheer up love, might never happen" then I can floor them with our story and add the additional icing on the cake that Andy was told on Friday that his role is redundant and he will have to apply of another role within the company or accept redundancy, still, it could be worse I suppose, we have just got through another week where Daisy put the frighteners on us and we have lived to tell the tell.

I always give up alcohol and chocolate for Lent, I am particularly proud that I have managed to see it through this year because quite frankly, come the end of lent I am going to enjoy the biggest chocolate and red wine hangover known to mankind!

2009-03-28T19:33:00.000Z

I suppose you could say that we are having a typical Daisy stay at Great Ormond Street in that nothing is going to plan...The good news is that there has been a major reorganisation on Rainforest and the ward is like a different place, everyone is friendly, caring and proactive and really representing the best of one of the leading children's hospitals in the world. This has been such a relief, particularly as you have probably guessed we have overstayed our allocated time on the ward...

Daisy has developed another bacteria infection of the blood ontop of the candida in her bloodstream and urine infection. She is now on antibiotics I have never heard of and apparantly starting to move into last resort categories as the bug she has developed here is one she has had before and seems to be resistant to some of the usual antibiotics she is on. Her hickman line clearly has to come out and this is now scheduled for Monday with insertion of a temporary picc line to give her chance to clear her infection. Regular readers will know we have not had good success with previous picc lines, they should last a few months (they are like semi- permanent lines) but Daisy's only lasted a week resulting in another blue light dash to GOS for a new line. I am wondering if they will keep her here now until the infection clears and they can put a new Hickman line in . This will really test the new Rainforest happy attitude - previously I would have heard mutterings about needing the bed space but instead the staff have been fantastic and really concerned just for Daisy's well being and the fact that things have not gone according to plan for us.

Logistically things will get difficult if we stay any later than the middle of next week. Mum goes back to Wales tomorrow and Andy and I will juggle staying at the hospital and managing the school run - probably Andy will stay here for nights and I will commute back and forth during the days with the children at after school club and at friends houses...

There has been good news though. Daisy had her upper and lower endoscopy on Thursday and the inflammation has decreased which means that the immunosuppressants and steroids are working - yippeee!! Her doctor wants to keep her on this therapy for the forseeable future so her risk of infection will still be as high. He also placed a jejenal tube for feeding Daisy at the top of her intestine, bypassing her stomach altogether. We are hoping that we can increase some of her feeds using this method and get more calories into her rather than just via her TPN. The endocrine doctors also feel that Daisy should be able maintain her blood sugars for small breaks from the TPN with just milk feeds. The plan, once Daisy is over this infection, is to try and achieve longer and longer breaks from the TPN with the aim of 8 hours a day off. She will still be attached to a continuous feed pump for milk feed but this was Daisy's life before TPN and I promise I will never complain about carrying a pump around again having seen the alternative!!!

The home word still has not been mentioned - we need to achieve these breaks from the TPN before this can happen so the reality still is June/July. Being superwoman I am already contingency planning for Daisy being in hospital during the long school holidays although it would be nice for her to have long enough breaks from TPN to be able to come home for the best part of the day and go back to the hospital at night.

Andy stayed at the hospital last night and I was able to get home overnight, fitting in a quick meal out with some friends and a chance to catch up on some gossip - these moments keep me sane and de-institutionalise me for a few hours! It was great to wash my hair, the shower on Rainforest still runs on lukewarm and you have to brace yourself to get into it so hairwashing is out of the question. The best bit was sleeping in my own bed - luxury! Did a quick Primark shop with Xanthe in the morning to pick up dungarees for Daisy as they are great at holding Hickman lines and enteral tubes in place, then we all drove up to GOS. Fortunately siblings can go to the Activity Centre here on the weekend so while mum sat with Daisy Andy and I were able to have a quick bite to eat and a coffee together, a rare treat these days. The combination of a sick child, 3 hyperactive siblings plus tired parents/grandma is not a great one so Andy has now driven everyone home and I am back to my hospital bed and prison regime!

Xanthe is singing at the Albert Hall on Monday evening and we have been promised that Daisy is first on the afternoon theatre list so if our plan works out of Daisy's respite nurse sitting with her for the evening and various children being delivered to the Albert Hall we may just get to all be able to go. As we know, the best laid plans........

I am relieved that Daisy is at GOS at the moment, if we need it paediatric intensive care is just down the corridor, hopefully she won't need it, but as always her happy smiles really don't show how ill she is. But inspite of it all we are making progress, and slowly, very slowly, we are inching towards the day when we can come home.

2009-03-22T18:13:00.000Z

So, one minute I am blogging about taking each day and enjoying the moment , and the next minute I walk into the ward one morning to be told by the nurse looking after Daisy that she had spiked a 40.4 degree temperature and was unwell.........great, another white knuckle ride again.

Of course everyone who knows Daisy well knows this pattern, we plan something, in this case, Andy going for a weekend to France with one of the children or we have another inpatient stay at Great Ormond Street booked and you can guarantee that Daisy will throw a spanner in the works. Of course this week it was a double whammy, weekend plans and inpatient stay so Daisy threw a double infection - candida in her bloodstream (you will remember from previous posts this is bad, bad, bad!) and a really bad urine infection , and for good measure streptococcus was isolated in her gastrostomy site. The bottom line is the Hickman Line (no pun intended) will have to be removed, candida sticks and you do not want to risk it creating fungal balls which settle in the brain, heart, lungs, kidneys - as I said, this one is bad.

She is smiling and gorgeous as ever which really belies her state - the infection is systemic, ie everywhere and requiring big doses of anti-fungal infusions which have really nasty side effects. On top of that she is back on antibiotics for the urine infection. These drugs together with the steroids and immunosuppressants do not make for a good combinations so her kidneys and liver are being monitored constantly. The only good thing is that we are going into GOS tomorrow so hopefully they can fit in a line change then, if the ward doctors can get their act together, I'm just keeping everything crossed that the endoscopy and jejunostomy can still go ahead as without these two procedures we will not know if we can stop the immunosuppressants and try and increase her feeds to get her time off the TPN. And time off the TPN means we can become eligible for the HPN programme - Home TPN and maybe a plan and a timeframe for coming home....

So many things keep moving and changing, many not under our control, the stress is starting to get to us all. Thank goodness Andy had a chance to get away and recharge his batteries, I do not want to be away from Daisy so knowing that at least one of us is getting a break is a comfort. I am getting fed up of lots of things about our institutionalised life however and have started to compile a list, many of which will be familiar with friends who read this blog:-

In no particular order...

- having to ask permission to do things, eg like getting Daisy's growth hormone out of the fridge

-not being able to take my cup of tea into her room

-dragging a huge IV pole with me and holding 3 lines to make sure they do not go on the floor everywhere we go

-trying to keep 3 other children entertained while spending time with Daisy after school

-not being able to spend any time with Andy, particularly on the weekends when one or other of us is at the hospital and the other is at home with the 3 children

-having to leave Daisy at night

-not being able to have her home and to be together as a family

-living our lives in a goldfish bowl with no chance of any privacy

Anyway, whinge over. I am hoping that this week we may leave GOS with a bit of a plan and way forward. The longer Daisy is in hospital the more susceptible she is going to be to infections, but she cannot come out until she is stable. We have to go at her pace but at the same time I want the doctors to remember that she is a 4 year old girl with a life to live and she cannot continue to live it confined to a cubicle in hospital. If her life is going to be short then I want it to be full of memories of fun with her family at home....

2009-03-16T22:50:00.000Z

Fingers crossed Daisy is continuing to do well, not of course without her little ups and downs, which once upon a time seemed such a major thing and are now just another thing...... Her recent cardiology appointment showed that her heart is enlarged and the mild cardiomyopathy she has always had has become a bit worse, probably as a result of the infections and medications. We are now looking at starting heart meds, this was inevitable at some point in Daisy's life so it will be just another thing to add to the melting pot. Before starting these however she will see a renal specialist to discuss her high blood pressure. Yes - you read it correctly, when you are a child and your bp is high you are referred to a kidney specialist....it's because of the kidney's role in managing some of the chemicals which play a part in how the heart functions - especially things like sodium and potassium levels in the blood stream.

We hope to fit this in during next week's Great Ormond Street stay. We are now officially in countdown for this stay, which regular readers of this blog will know that I am not looking forward too. While it will be great to get some more biopsies taken and also see whether the drug regime has helped with the massive gut inflammation and scarring, I just hate being on Rainforest Ward with Daisy. Our previous stays have been so depressing, not just because they have always involved bad news and emergencies, but also the staff seem to be so demotivated and the ward morale is just awful. I did phone Great Ormond Street today to check that they now had 3 clear MRSA swabs from Daisy's gastrostomy site - 3 clear swabs means she can leave her cubicle and take advantage of the play facilities available at GOS, and as we all know, 3 clear swabs from another hospital do not count. The good news is that all 3 of her last GOS swabs are now clear!!!! The bad news is that the last swab grew pseudomonas - another hard to eradicate bug. Isn't it great how they made sure Daisy's local hospital were informed of this (not!). She will be reswabbed at the local hospital tomorrow to see if she is still colonised with this bug. Being colonised is fine, in a person with a normal immune response, but in Daisy's case, as with the MRSA, if these bugs get into her blood stream it could be fatal. Life once again is like a game of Russian Roulette.....

I am still pushing to get sign off to be able to reset and manage Daisy's hospital TPN pump - this would mean that we could leave the hospital for a couple of hours without a nurse. I could take her to school for a few hours, or down to our hospice or even home.....The problem is this has never been done before so all sorts of risk assessments and disclaimers have to be worked out, I'm going to keep pushing for this as Daisy needs to be in school occasionally, she needs the social interaction and she especially needs the access to the specialist facilities her school offers (it is a specialist school for children with vision impairments).

Last Friday the ward did a fundraiser for Comic Relief Red Nose Day, Daisy and I had the best day. The nurses made her a cute T shirt and she dressed up in Mini Mouse ears, a ballet tutu and wings. We love watching the Comic Relief on TV and again I was struck by how fortunate Daisy is in the lottery of life. Had she been born in the third world there is no way she would be alive today, I know I knock the NHS sometimes but I am so grateful for the fantastic free treatment we get. Our hospital is only 12 minutes drive away and then we have access to many world class facilities in specialist hospitals in London..... anyway, watching Comic Relief made me realise, sometimes I get down about things but the bigger picture is that Daisy has truly enriched our lives, and changed them. I found this quote the other day that sums up how Daisy has taught us to cherish the little things and live for the moment;

One day at a time- this is enough. Do not look back and grieve over the past, for it is gone; and do not be troubled about the future, for it has not yet come. Live in the present, and make it so beautiful that it will be worth remembering
(Ida Scott Taylor)

Yesterday we had a beautiful thirty minutes when Daisy was allowed off her TPN and was just attached to her enteral pump - we walked a circuit around the hospital as a family. This was the best thing ever, enjoying an early spring evening with my husband and all four children - a beautiful moment to keep us going through the tough times.

2009-02-28T21:26:00.000Z

I was affected in a way greater than I realised at the time by the news of David Cameron's son's death this week. Here were two parents given every privelege in life but still facing the struggles Andy and I face week in week out. The frantic dashes to hospital, the endless hospital appointments, the meds, the waiting for results, the hope, the worry...... and for them to leave hospital for one last time without their precious son. I thought back to our worrying time with Daisy a few weeks back and how it could have been the same. The reality is that illness and disability does not discriminate .... so many families like ours face the same challenges week in week out, and yet we manage to get on with life and keep our heads above water. Following Ivan's passing there were lots of interviews with parents of disabled children on the TV and in the papers, over and over the same answers came out - how do you cope? - because there is no other choice, you have to - whether you are the leader of the opposition, the Prime Minister (who himself lives each day with the memory of his first child who died prematurely and the challenges faced by his youngest child who has cystic fibrosis) or whether you are Steph & Andy Nimmo of Wimbledon. Andy goes to work , I go to the hospital, we get on with things, and colleagues, friends, family don't really know what we are facing day by day - apart from that small group of friends we now have who are also facing their own challenges with their own child. In so many ways I feel blessed to have Daisy, suddenly the spotlight was on families of children with disabilities last week and I felt that I was a member of a very special club who could really understand and empathise with the Camerons because there but for the grace of God...........................

We have had our usual share of ups and downs with Daisy since my last post. We were very excited to get her enteral feeding to 28mls an hour, however as always my daughter does not do things by the book and this includes showing us whether or not she is tolerating her feeds. After lulling us into a false sense of security for a week or so, she started to vomit and her tummy bloated up. Hoping that it was just a tummy bug picked up as a result of being able to leave her room, we waited for test results - all of which came back negative for every bug that she may have caught. So we pushed her feed volume back down to 21mls an hour again and have gone back to the drawing board. She seems to be tolerating the lower volume much better confirming that her stomach does not work properly and that she would benefit from jejenal feeding, bypassing her stomach altogether and feeding her directly into the top of her intestine. The procedure to create this new opening will be done at the end of March when she goes back to Rainforest ward at GOS. At the same time she will have an upper and lower endoscopy and we will know if the drug treatments she has been on have worked. I'm really hoping that we will start to make some plans for coming home as well. It will take such a long time to get everything in place that I want the planning to start now otherwise we are in real danger of hitting the summer holidays with Daisy still in hospital.

We were supposed to go to Chase this weekend for our re-booked respite following Daisy's near fatal infection a month ago. This time it was Xanthe's birthday and for the second time I had to meet the children from school and tell them that our break to Chase had been cancelled. This time it was cancelled because there were two emergency admissions for end of life care. Ultimately this is what Chase is all about, we may be in that situation one day so I really did not mind us being cancelled, I'm just focussing on the the next GOS visit. To make things up to Xanthe I rapidly organised a birthday party for her and while Andy sat with Daisy, Jules played at a friend's house and Theo plodded on with his homework I had a nice girly afternoon with Xanthe and her friends at the cinema. Chase has been rebooked for April so I'm really hoping we can actually make it, Christmas seems such a long time ago and we are in dire need of some family time together, the children are fed up of having to be dragged up to the hospital and I'm fed up of trying to divide myself into a million pieces for everyone. Sometimes I just wish I could just wave a magic wand and this would all be over and Daisy would be pottering around the house again just like she used to.......

2009-02-23T21:57:00.000Z

Given the time elapsed between this post and the last one you would be right in guessing we have just had half term (let the juggling commence!). As always the complete lack of strategic planning on our behalf (suprising when you bear in mind we both have - or in my case, had, roles involving strategic planning) meant that Andy had not booked any holiday and I was very light on respite time (of course everyone wants respite during half term). Anyway, Grandma (my mother - think the Jennifer Saunders character in abfab with a welsh accent) came to the rescue and had Theo to stay for a week which included attending a Blues Brothers tribute night (he fell asleep at the table!), sitting in a Ferrari - he's moved his obsession from Dr Who to Top Gear and viewing 1,600 videos on You Tube! Xanthe had two sleepovers and a day with a friend - all of which involved experimenting with copious amounts of make up.

Jules made it through half a day at the holiday club before nearly knocking himslef unconcious on a radiator while diving for a football. Having spent considerable time in hospital now I know that the fact that he cried straight away was a good sign and hopefully no permanent damage has been done. It is however difficult to tell the difference between genuine delirium and just stream of conciousness with Jules , although I'm opting for the latter.

In massive contrast to my last post, Daisy has had a fantastic week. We have been able to push her milk volume up more and more (this is the feed she receives via her gastrostomy button directly into her tummy). She remains on 24 hour TPN with some gastrostomy feeds. This in itself is a massive breakthrough. She is still on her nuclear strength antibiotics, and, although I hate her being on them I have to admit that the IV immunosuppressants and steroids seem to be doing the trick - I hope. Her latest blood cultures are negative which is great as hopefully she has cleared those nasty bugs from her bloodstream. I say hopefully as the problem is no-one wants to give the nod to take her off the antibiotics. The pattern seems to be with Daisy is for a week or so without antibiotics before another infection kicks in. This was the case over the summer before her hospitalisation so it's not even since having a Hickman line. Her main consultant is back tomorrow (having also had to juggle her childcare arrangements last week, it's why we get on so well, in many ways we share similar worries!) and the consultant microbiologist wants to brainstorm a plan with her. I feel I need to send this consultant microbiologist a picture of Daisy, he has received so many samples of bodily fluid from her over the past few years and has never met her, but he, like so many of Daisy's team, is caught up in her life.

Daisy feeling better and stronger brings its own challenges, she is no longer content just to lie in bed and watch Cbeebies, she wants to get up and be busy. Just like the rest of my children she is not a fan of daytime naps so the days are long. I am her teacher, physiotherapist, speech therapist, vision teacher, interpreter, nurse (for some things!) as well as her mummy. I am waiting for the powers to be to sort out the bureaucracy so that I can just have one role of mummy but in the meantime, and in the interests of not losing any more time with her development, I do everyone's job (for no financial return other than a smile). The best thing is that we have been able to venture out of her cubicle. This is such a milestone and for Daisy is like discovering a whole new world. We have visited the nursing station, looked at the children in the bays, taken in the parents room , checked out the doctor's office and most exciting of all, been to the playroom!!!!! Of course now she wants so spend all day out and about on the ward, with someone behind her wheeling her IV pole loaded with TPN, enteral feed, syring driver and someone next to her holding her hand as she is so unsteady on her feed (did I mention that ontop of everything she has a problem with her vestibular/balance function?).

Being immunosuppressed means that Princess Daisy cannot always have her wish, particularly as the ward is currently full of cases of diarhea and vomiting. So the onus is on me to be children's entertainer again. This is proving somewhat difficult as the TV in her room is once again stuck on Channel 5 and I'm not sure that the storylines in Hollyoaks are quite a suitable replacement for "In the Night Garden" on Cbeebies.....

2009-02-11T20:15:00.000Z

This post so very nearly could have been the worst one I ever had to post, as it was, it has taken me a while to think back over what happened last week and how close we were to losing Daisy...

The beginning of the week had been lovely, the snow had really brightened everyone's spirits and Daisy enjoyed looking at (if not touching) the bowl of snow the nurses brought in for her. We had a great time with all the children together on Tuesday as Andy managed to get into work and I spent the day together with all four children in Daisy's room which was suprisingly manageable. On Thursday Daisy was quite clingy and pale and I kept meaning to ask the doctors what her haemoglobin level was as I wondered if she was at the point of needing another transfusion. I thought if it was the case it would give her a boost ready for our weekend at Chase Hospice.

It was Jules who discovered she had spiked a bit of a temperature. I brought him and Xanthe to the hospital straight after school and he was taking our temperatures and playing around with the stethoscope (as you do when you have spend the best part of your life since the age of 2 entertaining yourself in your sister's hospital room). We gave her some calpol and when I came back later in the evening she was sleeping peacefully but still a bit hot, after more calpol the temp came down a bit and as it was 11pm and I hadn't packed a thing for our trip to Chase I went home.

Within half an hour of my head hitting the pillow the phone went, thank goodness Daisy's consultant was on call that night as a locum registrar was covering the ward. Daisy's temp had gone through the roof (40.5) and her blood pressure had dropped suddenly. She had been given a saline fluid bolus to restore her blood pressure and started on 2 of her usual antibiotics and an antifungal to cover all bases before the blood cultures came back. I rushed up to the hospital (greatful for my recent investment in Primark leisure wear which meant I did not have to change) to find her fast asleep and the room like a fridge to keep her temperature down. She slept quite well that night and by the morning just looked completely washed out.

I was still half hopeful that this was just another Daisy blip and we would be allowed to go to Chase. If Daisy was our only child then it would have been easy to cancel but it was the thought of letting the other 3 children down who were so desparate to spend some time together as a family and not have part time parents/siblings. However I knew things were serious when Daisy's consultant arrived and said she was worried about Daisy. I know as a mum of 3 herself she would be the first to ensure we had quality family time together, so if she was vetoing Chase we know it was bad.

I called Andy to ask him to get home early as the children would be disappointed and left Daisy at 3pm to collect them from school and tell them the bad news that we would not be going to Chase. Daisy was not well at all at 3pm and I really did not want to leave her but this was again my usual scenario of leave Daisy to deal with my other children or make frantic arrangements with very accomodating school-run mums to pick them up until I could get to them. Andy and I decided that this time we would break our rule of trying to be as open and honest about the situation with Daisy as possible and tell a white lie as to why Chase was cancelled. The children are still indignant that the staff at the Hospice could not get themselves organised to dig their way out of the snow (one day I will tell them!) - we did not want them to resent Daisy for scuppering yet another weekend although without Daisy they would not have been going to Chase in the first place (I guess without Daisy they would not even experience the number of ruined weekends however....).

By the time I returned to the hospital Daisy had deteriorated really rapidly - she was shaking with rigor, her extremities were freezing and her bp had gone through her boots again. Two consultants were on the ward discussing a plan for her and nurses and registrars were in and out of her room - I took one look and phoned Andy to get to the hospital straight away.

Daisy has had lots of tricky situations in her life but this is the first time ever I have looked at her and thought she was not going to make it - I have never seen her deteriorate so quickly and be so close to death. The doctors were trying fluid boluses to bring her BP back up but this was not working, she was shutting down, overcome with septic shock. An anaesthetist was bleeped to intubate her and the search began for a PICU bed. Of course to add to this it was Friday night and snowing, roads were in chaos and major motorways closed (as always happens when this country has extremes of weather) . There was no bed at GOS, Daisy's main specialist hospital, at one point Manchester was mentioned - good in that there is a specialist in her syndrome there, not so good in that it would have been a 4 hour trip in atrocious conditions. Eventually a bed was found in St Georges, 10mins by blue light down the road. Daisy was deteriorating rapidly and dopamine infusion was started, and, when the paediatric intensive care transfer team arrived, noradrenaline. This seemed to do the trick and suddenly she turned the corner and we dared to breath a tiny sigh of relief. It was decided she did not need to be intubated and she was blue lighted to St Georges PICU, even managing a smile as she was wheeled on the stretcher out of the ward.

In the same way she goes downhill, Daisy bounces back very quickly. We were really worried that the infection was in her Hickman line and it would have to be taken out even though it was only put in two weeks ago. However she responded well in PICU and the bug was found to be one from her gut that had leaked into her bloodstream and overwhelmed her body. We have subsequently found out that if it had got into her lungs death was a 100% certainty, as it was we had been dealing with only 50% odds of survival. And once again the girl with 9 lives beat the odds, but the cost to Andy and I was considerably more grey hairs and worry lines.

So Daisy is back in Queen Mary's now, having charmed the team at St Georges, and back on lots of horrible antibiotic infusions which have toxic side effects which I try not to think about. She is amazing however - anyone else who had cheated death like she had would still be lying in bed, weak and feeble, but she has lots of living to do and is raring to go. She is back on feeds into her gastrostomy and as of today is on the momentous rate of 6mls an hour. In fact during all of last weeks problems the one thing to keep working was her tummy (albeit with only 3mls an hour going it), so Andy and I are taking some comfort that this may be improving.

We have been quite shell shocked since last weeks events, at the time you are carried along with the adrenaline of the situation, and on reflection wonder if it is only the fact that your love as parents makes you think that the worst could happen. However reality set in when each of the doctors who had been involved in rescusitating Daisy told me how close a call it had been and that they had thought she might not make it...........

We are so determined to get on with things now and get Daisy home at some point this year. We now have a date to go back to GOS at the end of March for another scope to see if the damage to her gut is improving and in the meantime we can try and push the gastrostomy feeds to help her system work and allow her the possibility at some stage of time off the TPN.

It's almost business as usual again now - this weeks problem remains high blood pressure and the plan now is for her to have a 24 ambulatory blood pressure cuff fitted to look into the pattern of her highs and lows, and then send her to a renal consultant at GOS to decide which drug to start her on to help manage it. She is also due her annual cardiac check up on her mild cardimyopathy and this is always preceded by a 24 hour ecg, so there will be at least a couple more nights of her being attached to extra monitors and tangled up in wires.

Now we know she is in hospital at least until the summer we have been able to get more permanent plans in place for Daisy's education provision. We held a multi-disciplinary meeting today to discuss her care - 18 people attended all involved in different areas of her life and this did not include all the medical doctors she sees. It is incredible the impact she has not just on our lives but on the lives of those involved in her care, this was so evident last week when we nearly lost her, so many people on the ward told me how worried and upset they had been and how relieved they were that she was alright, in four years Daisy has touched a lot of lives and while she is determined to keep us on our toes she is surrounded by the most fantastic team of people who just want her to win this battle and get on with life

2009-02-04T21:16:00.000Z

I have decided that even if we gave Daisy sugar tablets she would have an unusual reaction to them (probably drop her sugar levels...), and so it is with her new drug cocktail - the potent combination of iv immunosuppressants and steroids. So this week's symptom I have had to become an expert on is (drum roll)......dangerously high blood pressure. In fact I have now become so obsessed with blood pressure readings and trying to find some sort of trend in why Daisy's bp has been going sky high that I have been taking the whole family's blood pressure (I am please to report that mine is in the very healthy range). So we have been tweaking the doses of meds and today following a conversation between Daisy's local consultant and her GOS consultant we have switched the steroids from IV to enteral (through her gastrostomy tube). Her bp has lowered a bit as a result but it may take a while for it to normalise (hopefully). We are also trying the achieve certain levels of the immunosuppressant drugs in her body in order to counter the out of control immune reaction in her gut (basically her body is attacking itself which is causing the massive inflammation and ulceration). Of course this being Daisy establishing these levels is not a test that our hospital microbiology lab performs day in day out, they run them twice a week so we all wait with bated breath the see if she is getting the right level of the drug to do the trick.... The word from GOS is that she needs to be on this therapy for 6 weeks before they can have a look at her insides again and see if it has done the trick.

All this means that inspite of now being absolutely clear of the MRSA (I promise that's the last time I mention it), Daisy remains confined to an isolation room for a while longer because of the risk of her picking up an infection. This is becoming increasingly difficult both for Daisy and for me - I am essentially Daisy's teacher, entertainer, carer and mother (replaced by Andy on the weekends) and sometimes I just go a bit stir crazy, particularly as prior to all of this I was working full time with a successful marketing career. Daisy is doing really well at the moment and, although she is physically very weak, needs more social interaction and opportunities to move around without the 4 tubes that are connected to her more or less permanently. There is a bit of light at the end of the tunnel in that we have re-started gastrostomy feeds - initially at 1ml an hour we now have pushed it up to 2mls and so far so good. Last time we tried this Daisy's tummy bloated up and she was vomiting and full of wind - after a full week on gastrostomy feeding she has been symptom free so this is a good sign. Fortunately the liver function problems she had previously seem to have reversed and she does not look jaundiced and is not pouring bile out of her tube.

I'm starting to get impatient (or in-patient) and wanting to get home but I know from bitter experience that if we push Daisy too much then we will undo all of the good work. At least this weekend (weather permitting) we'll get a little break as we are all going to Chase Hospice. Daisy will go by ambulance on Friday morning and I will follow on Friday evening with the rest of the children, closely followed later in the evening by Andy. I asked Jules what he was looking forward to doing at Chase, thinking he would say "playing Xbox all weekend" but his response was "hug Daisy all day and all night". This time has been so hard on them, Andy and I can at least talk to doctors, search the internet and focus on fighting Daisy's corner but all our other children get is an occassional visit with Daisy when she is up to it and to wait and wait for news on when she will come home. It's like being in limbo - we can't give them answers, all we say is that everyone is working really hard to make it possible and when she is ready and the doctors agree, then Daisy will come home.

Of course coming home will be so different - we have to get to a point where Daisy can tolerate 8 hours off her TPN, meaning that for 16 hours of the day she will be on it. This is not a straightforward thing, Andy and I will need to be trained beforehand and signed off by GOS and Daisy's bedroom will need to become more like a hospital room and less like a playroom. However we will do anything now to get her home and I know that Daisy will suprise us in time by becoming stronger and stronger and overcoming all these obstacles that life is throwing her. While this time is so trying, it's Daisy and her wonderful fighting spirit that is keeping all of us going....

2009-01-27T22:36:00.001Z

Daisy having a good day - long may it last!!!!

2009-01-26T23:08:00.000Z

I only stay at the hospital if we are at GOS or if Daisy is really poorly and at risk of being transferred during the night, we only live 10 mins drive away so we are really lucky. I leave most evenings at around 10.30pm and I drive back at 8.30am in the morning after dropping the other children at a friend's house.

Every morning I drive to Queen Mary's wondering what will greet me when I arrive, sometimes I even know in advance as I will have had a call from the nurses before I have even left the house..........

Last Friday I drove in hopeful that this would be the day that we would have the final all clear for Daisy's blood cultures & a third set of clear MRSA swabs, this was also the first day that she had stopped the 10 hours worth of toxic drug infusions and we could start moving on with treating her underlying bowel condition......yes well clearly this was not going to be a good day, I walked into Daisy's room and she sat crying her eyes out pointing at her arm which was swollen up like a Michelin Man - her picc line had tissued, meaning in short that it had to come out, we had to stop the TPN, get a cannula into her before her blood sugars dropped dangerously low and get her into a hospital that could place a central line asap. Once again my plans to leave the hospital a little earlier for a change that evening so that Andy and I could have a curry and a bottle of wine infront of the TV were clearly not meant to be.

As always Daisy loves to cause a drama, the doctor's ward round was put on hold as they attempted to get some line access into her and start fluid and her consultant delayed her clinic as she contacted our gastro consultant at GOS to get her transferred asap. I was dreading having to go to Rainforest so was very pleased to be told that as Rainforest had no room for us (and in their infinite wisdom had suggested that perhaps a new line could be placed at the local hospital - do these people not read her notes?????) , instead bed management had got us a cubicle on Tiger ward. This was good - it's in the new modern part of GOS so all cubicles have en suite bathrooms and Daisy has spent a week on this ward last year when she overstayed her welcome with breathing difficulties following an MRI under general anaesthetic. During that time the staff were fantastic and unlike the staff on Rainforest I was completely unaware of any difficulties Daisy overstaying her welcome may have caused to their bed management plans. In fact the nurse assigned to Daisy that evening had remembered her from before and even had an "In the Night Garden" DVD all primed and ready for her!

Daisy's cannula only lasted a few hours and she had to be recannulated at GOS, then overnight she detatched herself from her IV line and lost a considerable amount of blood, this meant that she needed yet another blood transfusion in the morning, delaying surgery for the new line even further. During the course of the day she spike a temperature and developed an odd rash and my heart was in my mouth that she had become septic again. She went to theatre at 8pm and came back with a lovely new hickman line which the surgeon has promised he has done every trick in the book to prevent infection - well we'll ask for him specifically if it does get infected!

We came back to Queen Mary's on Sunday and by Monday Daisy had really picked up, her blood cultures were confirmed as clear and she is now up to 3 clear MRSA swabs at Queen Marys and 2 clear swabs at GOS - still not enough to get us out of the room however, the concerns now more are for her infection risk as her immunity is so compromised.

Daisy had a lovely day today, she slept well last night - probably a combination of antihistamine for her rash (which is now disappearing and appears to just be another odd Daisy thing), calpol and melatonin. A good night's sleep is a real rarity for Daisy - she hardly ever gets restful sleep and wakes in pain a lot at night so it is lovely when she does sleep well. She has been really happy all day and I have asked the doctors to consider whether we could try and restart a tiny amount of feed again. Of course this has to be run by a huge team both here and at GOS so I will wait for them all to come to a consensus. My gut instinct (no pun intended) is to try and restart the feed while she is so well as she has not been this well in a while.

Of course part of me can't help wondering how long it will last, over the next couple of days we will restart IV steroids and start the big league immunosuppressant therapy, cyclosporin, to try and calm down the autoimmune reaction in her gastrointestinal tract. Once she has been on this therapy for 6 weeks she will return to GOS for her upper and lower endoscopy and at that point we will have a better idea of when we might get home. In the meantime, I am teaching Daisy to click her heels together three times and say "there's no place like home, there's no place like home...."

2009-01-22T21:32:00.000Z

Given the space between this post and the last one you will probably guess that things have moved on. Daisy eventually got her port taken out and new temporary picc line put in. She went to theatre last Wednesday evening at 8.30pm and the surgeons took the port out (leaving yet another scar) and put a temporary femoral line into her groin (stitched in to add insult to injury!). Our warders (aka nurses) at GOS thought that this would all be fine despite consultants both at GOS and at our home hospital insisting that a femoral line was only a temporary measure (they are not meant for long term use and come out easily) and was just to tide her over until the picc line could be put in, hopefully within the next couple of days. Once again the issue of bed management and Daisy overstaying her welcome reared its head on Rainforest ward.

Sadly, the system they have in place of booking complex children in for minor procedures on short stay beds is not working in Daisy's case - every single time this has been tried Daisy has needed the bed for longer than the allocated 3 days. It may come as a suprise to many of you to learn that God's Own Special Hospital (as Great Ormond Street is known in the trade) doesn't cope well with children who do not do things according to plan - actually I'm being unfair, it seems to just be Rainforest ward....Our situation last week was that nurses were trying to ship Daisy back to the local ward with an unsafe line against the recommendations of her consultant just to free up the bed space. This was very obvious when I saw Daisy's file open on the nurses station outside her room, there was a note on the front stating she was in for a procedure on Monday, if well enough to be discharged back to local on Tuesday and (in capital letters) must leave ward on Wednesday. It's a sorry state of affairs when the bed managers have precedence over clinicians....

Anyway, clearly Daisy was not going to be shipped back to her local with an unsafe line - the local doctors would not accept her back, her gastro consultant at GOS was furious that it had even been suggested and I completely threw my toys out of the pram! I told the nurses that the bed issue was not my problem and to stop treating my daughter once again as an inconvenience, I tried to slam the door of her cubicle, forgetting that it was on a self closer and so the drama was lost a bit as the door slowly closed shut. Our GOS consultant had made himself even more unpopular with the bed managers for Daisy's sake and then had moved heaven and earth to get her a theatre slot with the interventional radiology team later on Thursday to have the picc line fitted. His registrar told me that he had even taken on some of another consultants cases just so they could fit her in, I am so grateful to him for that.

So by Friday morning Daisy was good to go - only I did have to remind the nurses that Daisy would need TPN over the weekend and had they contacted the local pharmacy with her prescription (of course they hadn't!). We had to be blue lighted back to Queen Mary's as there was only enough dextrose provided for an hours journey and south London on Friday rush hour was certainly going to take longer than an hour!

Daisy's temperature came down as soon as the infected port came out and the plan is to continue all the horrible antibiotic and antifungal infusions for a week so today is the last day. Blood cultures were done yesterday and I am keeping everything crossed they are negative, meaning that the bugs in her blood stream are gone. She also had another MRSA swab done in GOS and that was negative too - of course this being the NHS 2 Queeen mary's negative swabs and 1 GOS negative swab do not add up to 3 negative swabs, each hospital likes to have their own set, so Queen Mary's swab 3 is currently being processed, if it is negative for MRSA it means that Daisy will be able to leave her cubicle. This will be so fantastic as she will be able to visit the playroom, obviously being immunosuppressed this can't be when other children are there but the Play specialist has said she can have "Daisy time" in the playroom each day...

The next stage had been to restart the steroid treatment and start on infusions of a new, stronger immunosuppressant drug, I use the past tense because as always the proverbial spanners have been thrown into the works. Both Andy and I noticed independently that Daisy was looking a bit jaundiced, her blood results today confirmed that there is a problem with her liver, probably due to the TPN and the fact that she is not being fed into her tummy. An ultrasound showed her bile duct was full of sludge, she has been vomiting lots of bile and is in a lot of discomfort at night so this is probably the cause. The docs here are speaking to the docs at GOS and tomorrow she may start on yet another medicine to try and put this right. She really needs to be fed into her tummy to stop this sort of thing happening, but it just does not work properly, not only does she have the massive inflammation, her stomach does not empty properly and her gut does not move as it should....the answer is, who knows????

So until her liver function stabilises once again we have to delay the treatment for her underlying condition and this all delays getting home at some point.

Still, it could be worse, we could be stuck in Rainforest, at least this way we are in a hospital only 10 minutes drive from our home with staff who know and love her and don't treat her as a bed blocker!

2009-01-13T20:21:00.000Z

Day two comes to an end at Great Ormond Street and Daisy still has an infected line in place......grrrrrrr! The fine balance of operating theatre lists and the availability of specialist teams was thrown by Daisy needing an urgent blood transfusion yesterday and this has thrown everything out of kilter. As predicted we had the night from hell with Daisy - a combination of late meds, transfusion, being clinically unwell and nurses who do not know Daisy as well as our local nurses meant that both she and I are a bit sleep deprived at the moment. The interventional radiology team who are the experts in putting in lines had a full list today so the plan had been for the surgeons to take out Daisy's infected line and for an interventional radiologist to come in to place the new one. This all had to be done on the emergency surgical list and as Daisy is still classed as MRSA positive she would have to be done at the end of the list. Problem is with emergency lists its difficult to plan timings and with the surgeons running late it meant that the Interventional Radiologists had finished their shift and gone off duty. Daisy's consultant was not happy with a surgeon placing the new line in a late night theatre slot as there were too many risks both as she is difficult to access and with her anaesthetic risks. So now we are on to plan C - tomorrow the surgeons are going to take the line out and a very temporary femoral line will be placed in her groin (ouch!) - the reason for this is that the infamous Interventional Radiology team are not available tomorrow (at this point I start chanting my mantra about why doesn't the NHS get its head around the fact that people are ill 24/7 and not 9-5 and weekdays only...). This will mean that she will then need another anaesthetic in a few days to place a more permanent line.......even I am losing the plot at this point.

The bottom line is that as per usual Daisy will be outstaying her welcome on Rainforest ward, we will probably lose our bed back at the local and Andy and I will have to do some rapid juggling to work out domestic arrangements, which includes getting Theo a warm jacket for his first Scout Camp of the season on Saturday and getting Jules and Xanthe to their siblings day at Chase on the same day. Being superhuman we will of course achieve this and hopefully still smile at the miserable ward sister on Rainforest who will hate us for throwing her bed management arrangements yet again! Good news is that the big cheese Gastroenterologist Daisy is under is coming to see her tomorrow - he tends to be able to get things done and I am pinning my hopes on him having a good plan for how we can move forward. In the meantime I am going to nip out and treat myself to some throat lozenges for my sore throat, a "tasty" norma-no-mates microwave meal and a small screw top bottle of red wine - after all, tomorrow is another day...........(we just have to get through another night)

2009-01-12T20:46:00.000Z

At long last we are back at Great Ormond Street - never thought I'd say those words but the worry of the infected line was becoming overwhelming - however we are not out of the woods quite yet. Daisy has a lovely upper respiratory infection and has been vomiting and coughing for the past few nights, she has had a temperature for eight days, due to the new line infection and is not well at all. Inspite of all of this the anaesthetist was happy to go ahead as the risk of the infected line outweighed the other anaesthetic risks. However Daisy being Daisy decided to throw yet another one of her spanners into the works - her haemoglobin level has dropped dangerously low and that risk now outweighs the line removal as it is too risky to do while she is so anaemic. So as I type she is just coming to the end of another blood transfusion, the second in about 3 weeks. She is a lot better for it and this will probably help her cope well with tomorrow's procedure - now assuming that they can fit her in on the morning list as promised....

We are all behind with her drug infusions and TPN tonight so I am gearing up for yet another disturbed night as infusions go on and off and TPN gets put up at some unearthly hour. Her blood sugars have been all over the place too - to get her through the transfusion she was given a glucagon injection at the start, in any other child this would have sent their sugars up to the roof but for Daisy it just pushed them into an acceptable range. Her endocrinologist is going to come and see her tomorrow (assuming she is not in theatre at the time) to discuss restarting her growth hormone therapy which should help her regulate her blood sugars and hopefully give her more muscle strength to cope with all of these infections.

I heard an ad on the radio the other day about giving blood and that blood banks are currently low on type O blood, Daisy's blood type. Each time Daisy has a transfusion I do say a mental thank you to the kind soul who gave up an hour of their time just for the promise of a free cup of tea and a biscuit to donate some blood, suddenly those radio ads seem so much more poignant, Daisy needed blood and it was there for her and looking at her colour tonight it makes the world of difference...

2009-01-08T23:51:00.000Z

Daisy's 4th Birthday and our Christmas at Chase Hospice

2009-01-07T20:55:00.000Z

Still have not downloaded those Christmas pics - must try harder.......I'll get on the case this weekend.

New Year for Andy and I started with a bit of a hangover and hoarse voices from singing karaoke at our neighbour's house, the best therapy we needed was to belt out some Abba numbers at the top of our voices until the early hours! Daisy's new year started with a snotty cold, but she was happy enough without a temperature, just an annoying cough until last Sunday when she suddenly spiked a 40 degree fever. Previous blood cultures taken just before new year had been negative so the docs felt that it was probably just a virus, an x ray showed a bit of phlegm on her chest and they took some more blood just in case. Her temperature has stayed up for the past few days and although the ward doctors kept saying it's probably just a virus my instincts were, as always, to prove me right. Yesterday afternoon the microbiologists (who are very au fait with Daisy's various bodily fluids) phoned to say that the first culture was positive for candida and the second culture was also growing something. I was dreading a return of the MRSA nemesis but it was it's marginally less scary cousin enterococcus. A quick call to Andy to update him, he googled candida sepsis and we have now realised that this is our new nemesis. Don't google it, you'll scare yourself! Daisy's port has to come out! This is where Great Ormond Street for the first time ever have come good. With impeccable timing I had a call from Rainforest ward to inform me that her port would be removed on Monday, in fact I have never known such a flow of communication between our ward here and Rainforest, they must have made a New Year's resolution to be more proactive!

The doctors here have tried to get her in earlier as she is not well at all - her temperature remains high and she is not a happy bunny. It does look like Monday is the soonest it can be done and I don't want her transferred to St Georges for the line removal as this adds another hospital into the equation and a set of anaesthetists who do not know her well (she can keep anaesthetists on their toes by throwing little wobblies now and then). In the meantime she has been started on two anti fungal meds as well as her antibiotic infusions, it feels like she spends more time on medicine infusions than on her TPN - no wonder she feels grotty. Her vancomycin requirements are now officially those of a 70kg man and are probably contributing to the existing Epsom & St Helier Trust Budget Deficit.

Our nurse from The Children's Trust at Tadworth started working with Daisy this week. After a year of pushing by Andy and I we eventually got social services to fund some short term respite at Tadworth to give us a break occasionally. The initial agreement was 2 nights a month but of course as Daisy has been in hospital for so long she has not been able to access it. Fortunately the funding was moved to provide outreach so now we get 7 hours a week from a named nurse. It is so nice to know that I will get a day off one day a week - Jane our nurse will work alternate early shifts and late shifts so that one week I can get time off during the day to catch up on things at home and one week I can get an evening at home. My life at the moment is spent dropping Xanthe and Jules off to our neighbours to be taken to school and them coming to the hospital, staying here until about 5.30pm, picking the children up from after school club, feeding them and then returning to the hospital when Andy arrives home at 7pm and staying here until 10pm (ish) or whenever Daisy settles. On the weekend Andy and I try and juggle our time here between us. When Daisy is really poorly or staying in GOS I stay with her and Andy juggles things at home. The reality is Daisy cannot be left for long, she does not have one to one nursing at the hospital, there are other patients besides Daisy here after all!, and although the nurses keep an eye on her when I am not here they can't be with her all the time and Andy or I have to try and be with her as much as possible, both to keep her entertained and stimulated as she is still in isolation but also to do all the nappy changing/bathing & personal care she needs, and often to untangle her from all wires as she is so active! It makes me really cross that the government seems to thing that when a child with complex needs is in hospital they are being looked after and the carer is getting a break, if anything my job is more difficult as I am trying to be several places at once all the time and never quite managing it!

With the new year we have been focusing on trying to get home at some point. I keep saying to people that it will hopefully be by the summer, but it may even be around May half term time. When we are at GOS next week we hope to see the TPN nurses to get the ball rolling with getting set up for long term home TPN. The homecare team here is happy to set things up but it has to be done in collaboration with GOS as she is their patient. Knowing how slowly the NHS moves it will take a while to get the funding in place but hopefully we can start slowly by having some day release. I would love to be able to take Daisy to school for a few hours even if only once in a while. Daisy's key worker is setting up a multidisciplinary meeting with everyone who , is involved with her , from the school, doctors, therapies, social services - such a small girl, such a large entourage.....

In the meantime amid all these line infections and worries I thought we should all have something to look forward to so I have booked some more time at Chase. We have the weekend of Theo's birthday in February booked and I have also booked a week for the first week in August - the children are just over the moon at the thought of a summer holiday at Chase. It will give us the chance to have some family days out and have some quality time with Daisy without having to manage her care for a while, something lovely to look forward to later on the year while temperatures go sub zero outside and Daisy battles with another line infection while waiting for her port to be removed!