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....




1 comment:

Robyn Faye said...

Dear Stephiane & Andy,
I dont understand how someone so adorable and brave can go through so much? Daisy is beautiful and your other 3 children are so lovely too :) i know i dont undertstand what your going through but i understand how hard it can be, your amazing parents you really are! I hope one day when i have children im as amamzing as you both are. everyday with Daisy is a miricle (excuse my spelling) shes just so extrodanairy. i dont know what else to say except you are amazing :)
Love & Support
Robyn
x x x x x <3

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