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