A beautiful gift of family time

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 .

Trying to look on the bright side

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!)

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)
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!

Gut Instincts

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….
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....
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!!)
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!
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

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…
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...........

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....

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.