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!!)
Websites close to my heart
- Shooting Star Chase (Daisy's Hospice)
- Great Ormond Street Hospital Children's Charity
- Torbay Holiday Helper's Network
- Sick Children's Trust
- Daisy's Angels - Facebook Page
- About Costello Syndrome
- Asperger Syndrome
- Caudwell Children
- Together for Short Lives (Children's Hospices in the UK)
- Costello Syndrome Support Group
- Give Kids The World
- PostPals - Putting Smiles on Sick Children's Faces
- Singing Hands (Daisy is their No1 Fan!)
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