"It's not the hand you're dealt, it's how you play it" - is going to be my motto from now on. Last week I went through every extreme of emotion, having to deal with the reality that things are not that great with Daisy. Her GOS consultant came to the hospital on Thursday and spent a lot of time both with her doctors and with us. Now we have a far clearer picture of what is going on with Daisy's gut - in some way it is a relief to know that our instincts all along were right and that unlike the other children with Costello Syndrome, Daisy was not going to grow out of her gastro-intestinal issues. She has a diagnosis of pan colitis, basically her entire gastrointestinal tract is inflamed and ulcerated, caused by her immune system going haywire and attacking her gut. If the ulceration was localised to her colon then that section could be removed and if needs be she could have an ileostomy. She is not a candidate for an intestinal transplant as she has too many medical issues making her odds of survival zero. The possibility of a bone marrow transplant to attempt to "reset" her immune system has not been ruled out but this is further down the line. At this stage everyone agrees that the current treatment with steroids does not seem to have worked and so she will be started on new, stronger immunsuppressant therapy (and all its scary side effects).
Of course ontop of all of this is the small matter of the MRSA. It looks like the very aggressive and toxic antibiotic therapy is holding it in check but in all likelihood her central line is now colonised - meaning that the bugs are now clinging to it. So it has to come out, and this can only be done at Great Ormond Street. I found out today that her local consultant and GOS consultant have been in contact all weekend trying to get things in place for a new line before Christmas. It has to be done be an interventional radiologist, and the man who everyone wants to do the job is trying to move his list to accomodate Daisy. She has a habit of getting people to run around after her, it amazes me that we have got to the point that she is well and truly a signed up member of Great Ormond Streets special patients!
Not only are her clinical consultants rearranging diaries for her and juggling appointments but several microbiologists are working on the best drug treatments, pharmacists are tweaking tpn preparations and geneticists literally all over the world are testing her DNA and trying to get to the bottom of what has caused these massive changes to her immune system.
Andy and I find it ironic that as the goalposts are moved by Daisy's condition, previous possible diagnoses look more acceptable. I remember when I was pregnant with her we were told there was a one in four chance she would have Down's - I was devastated at the time but if I knew then what I know now......And now it looks like Daisy has something going on beyond her Costello Syndrome diagnosis. When Daisy was in the neonatal unit Daisy was tested for everything possible and we were told that although at the time there was no test for CS, hopefully she would not have that. Of course when a test was developed she tested positive and we got on with things, embracing her uniqueness both as a child and as a child with CS. Now we are facing the probability that she has another mutation or genetic issue alongside the CS and we find ourselves wishing that she "just" had CS - oh the irony!
TPN looks like it will be very much part of our lives from now on in. In the new year she will have another upper and lower endoscopy to see if there have been any changes in her gut following the drug therapy. She will also have a jejunostomy - so that we can try feeding her via the very top of her intestine missing out her tummy altogether. Giving her senstivities to volume when she is enterally fed nobody believes we will be able to get adequate nutrition into her via this route to do away with the TPN altogether, although it may enable us to limit TPN feeding to night-time only, and have jejunal feeding during the day meaning she could go home for the day and return to the hospital at night. In the long term we need to get set up to manage TPN at home - this is a minefield in itself although I know others who have managed it so I know it is not impossible, just even more life changing! However if this is the only way we can get Daisy home then that's just how it will have to be - as I said, it's not the cards you're dealt, it's how you play them!
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!)
special needs (85) costello syndrome (43) disability (32) aspergers (19) TPN (18) cancer (18) Great Ormond Street (17) carers (16) parenting (13) autism (10) shooting star chase (9) children's hospice (8) Blogging (6) family (6) SEN (5) hospice (5) Mitrofanoff (4) diagnosis (4) BIBs (3) Britmums (3) Neuropathic Bladder (3) communication (3) holiday (3) benefit cuts (2) david cameron (2) gastroenterology (2) singing hands (2) travel insurance (2) BSL (1) Child Hospice (1) Colectomy (1) antibiotics (1) benefit scrounger (1) bladder (1) france (1) health (1) line infection (1) makaton (1) portage (1) siblings (1) sign language (1) sign supported english (1) ultrasound (1)