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'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!