Friday, November 24, 2006

Great Ormond Steet

We finally made it to Great Ormond Street this week for the test that everyone has been waiting for - the duodenal manometry. This is a measure of the peristalsis, or muscular movement through the gut, in response to food. The visit was pretty eventful from the start. I was running late and harassed and stressed as ever in these situations, not helped at all by the fact that Paul still has his leg in a cast and I was travelling on packed commuter trains with William in his "Charlie Chair" (or rather, "Darlie dair") with everything we needed for the next 3 days on my back in a huge mountaineering, and very heavy, rucksack. I eventually decided that I was going to be late and it really wasn't worth rushing to be there for 10 when it was highly unlikely anything would happen before lunch time so rang the ward and apologised and began relaxing a bit. When we arrived near the hospital we saw police everywhere. I eventually asked what the fuss was about and was told that Charles and Camilla were in. Well, a big hospital, I thought and spent the next 20 minutes totally lost in it and wondering if I'd bump into them. I finally found our ward and noticed that there were more and more people in suits the closer I got. When we walked in, we found that our ward was the very ward the Charles and Camilla were in. We got to see them briefly but, had I not have been so late, we would have got to meet them properly. They were there to open the new wing the ward is in. Very exciting. I know this visit has been a long time coming but we weren't quite expecting a royal welcoming party! It is a shame we didn't get to have photos for William's scrap book. I don't think the photo would have knocked the photo with John Terry from pride of place!

William had 2 days of tests and spent 2 nights in. He had an endoscopy and colonoscopy (tubes down and up) to see if there is any inflammation that could be treated. He also had biopsies taken to look at the histology of the gut. The scopes looked OK, as they have done in the past. The following day was the manometry test we have all been waiting for for so long. The hope was that this would show us something that could guide future treatment, such as surgery to remove the worst effected part of the gut. At the same time, he had an EGG, measuring the activity of the stomach via electrodes, similar to an EEG but on the tummy not the brain. The photo shows the complex computers and machinery all around him for these two tests, and Thomas the Tank Engine, the most essential bit of kit of all, keeping him nice and still enough for the measurements to show anything other than wriggling. He was sedated throughout but, having been sedated the previous day, he wasn't about to loose a day to sleep again! The manometry is very clever. A catheter is inserted in the gut with a set of holes all the way down. At regular intervals, water is pushed through the holes and the gut reaction to the water measured via the pressure it exerts on the catheter. The gut should squeeze in repsonse in a rythmic fasion - peristalsis. They decided only to use one measurement for William as the catheter all the way down would have interfered with his own tubes and any information at all would be a useful start. They used his own tube that is inserted just below the stomach to measure the reaction of the top end of the gut to the water. Once he was sedated and lying flat watching Thomas it was clear that his gut didn't really react at all. He seems not to be able to squeeze his food and drink down the gut at all. We are still waiting a deep analysis and formal report but the initial view of the consultant from eye balling the initial data is that William's nervous system is failing to tell the gut to react to the water. This fits with his current neurological picture with the ataxic walk and deformed feet and, as all the gut has also got worse as he has got older (he did actually feed for a while as a baby) it sadly does suggest a degree of degeneration in his condition. We had hoped this test would reveal a solution but it seems that, unless something can be done to fix his neurology, there isn't really anything that could be done to make his guts work again. A transplant would certainly not help as the problem appears to be his nervous system, not the intestines themselves. We still have to wait for the more detailed analysis and, you never know, there may be a glimmer of hope in there, but it looks unlikely he will be able to come off the TPN at the moment. Luckily, TPN is keeping him really well at the moment but it does keep him tied to a drip 18 hours a day and means he lives with the constant threat of septacemia from line infections and the knowledge that this will, eventually, destroy his liver. We also don't yet know what the underlying neurological picture is and what else this will do to him over the coming years. All in all, we are a little closer to understanding what is going on but no closer to a cure or solution. We are seeing our usual gastroenterologist on Friday next week so don't have too long to wait to learn what comes next. We had predicted there would be another long hospitalisation after this one but it looks now as if there wouldn't be much to gain from that and it is more a case of getting on with it, enjoying every moment and hoping that something can one day be done. In the meantime, we have great support and therapy to make sure William gets the best out of life and his smiles and laughters show that he certainly does! The clown doctors certainly got a smile and a laugh from him (eventually, once he stopped being overwhelmed).

1 comment:

Becky said...

Big hugs Sarah,

Well done to William on being so brave and getting through all his tests, and I am so sorry that they had no good news to give you.

I really really hope that they can figure something out for him soon.

That seems like such a useless thing to say, and I'm sure it's useless to read, but I just wanted to let you know that I'm keeping up with William and his journey.

Lots of hugs,

Becky, Seren & Dylan xxxxxx
(from the CFTrust board)