It's been a looooooong day!

Had a lot of fun this morning! Realised last night (after our first fundraising car boot sale yesterday) that I've spent so much time and energy lately trying to organise ways to raise money, that I've hardly spent any quality time with Alyssa in the process! She stayed with her dad on Thursday night this week, and then with her gran on Friday night - to allow me to get up and organised to car boot sale early Saturday morning - and I couldn't believe how much I missed her! It's the first time she's ever been away from me that long, and it was really weird .... and not in a good way!

So ... I took her to our local soft play centre this am (Kidzworld) and we both had a great time, can't remember hearing her giggle so much as I did this morning as I chased her about and repeatedly caught her - just as she was falling over from laughing so hard and looking behind her, to see if I'd nearly caught up with her! Lol! An hour of that is unfortunately quite hard work with her extremely low muscle tone and hypermobility, so we were ready to go by then and she was already drifting off to sleep in the car by the time we were halfway home!

Annoyingly - and something I forgot to mention from Tuesday's update - is that we agreed to stop the latest drug trial (Thalidomide) because it really wasn't doing enough to warrant keeping her on it, with it's strict guidelines for administration etc. And - of course - within 2 days, she was scratching more .... but she also had a cold and resulting low-grade fever, so I was trying to put it down to that ....! However, the excessive sweating has now kicked back in so the Thalidomide is clearly getting out of her system now, and I've spent most of the day feeling like part of the WWF team in wrestling her to the ground at times just to stop her attacking the back of her head! *sigh* I'm not convinced it's actually the Thalidomide which stopped the itching as such ... but that because it reduced the excessive sweating, it in turn made her less itchy! I shall now have to update the specialists and see if there is something we could try to test this theory - ie putting her on something that will reduce her sweating, without attempting to touch the itching like the Thalidomide was meant to be doing - so we know for sure which is making her worse. I obviously don't want her on Thalidomide longterm if it's not having a significant improvement to her symptoms, although her symptoms are so many ..... it's hard to know what's best for her sometimes!

She's not been too bad today with respect to her guts, but am still having to help her pass motions using baby massage. She has also just automatically crying out for me to "help" as soon as she feels she needs to go, poor girl. It's just so much effort for her to do it by herself, not to mention the pain she feels at the time. Here's hoping this procedure which is now planned will be sooner rather than later! And with that in mind, I spoke to the anaesthetist on Thursday and we both agreed that it's sounding very much that this procedure will help resolve or at least reduce the pain she's in with bowel movements. It should also help reduce or eliminate the straining she always has to do, by reducing the pressure that builds up there. It's not clear (to me at least) if her rectal/anal passage is too narrow, or if the autonomic nerves used in that area are just not working as they should to aid defaecation but hopefully the procedure to stretch the anal area will still make her more comfortable. This procedure is mainly used now for children with chronic constipation, so fingers crossed.

The teeth are still an annoying factor, as I'm now fairly sure that the ulcerated area is now either bigger again or more damaged than it was on Tuesday. Alyssa is meant to be being reassessed in a couple of weeks from our visit but - as per usual - the dental team are so busy, that it's actually 4 weeks from our appointment last week. Presuming the operation goes ahead before that (as we're hoping it will) that leaves me in a difficult position. If I ask for someone to assess it on the day of the op, we may decide that the teeth need to be removed .... but they may not have a dental surgeon available then, but if I try to get her assessed before that it's a lot of hassle to actually get someone who knows what they're looking for ... AND someone who is actually able to make the decision to remove them - with my permission of course. Hmmm ... think I'll e-mail the specialists and ask if a dental reassessment could be made prior to the GA for the op and see what happens!

Ok, rambled long enough. Will update again as soon as I can. Life here is even more hectic than normal with all the fundraising organising going on!

Today's visit to the hospital ...

was pretty good actually. Alyssa had Nerve Conduction Studies (NCS) done today because of the drug she's been trialling (can pretty much say now that it's Thalidomide, which is a drug that most people are shocked over ... but has amazing results when used correctly). It can cause a type of peripheral neuropathy and - because we already wonder if Alyssa has this anyway - we're not sure how we'd know in Alyssa. The usual symptoms are "intense burning pain" ... which Alyssa may or may not feel so the NCS studies the electrical signals that the nerves are giving and receiving. Unsurprisingly (and positively) her results today were normal, and despite me thinking that we were abandoning the Thalidomide trial today because after 6 weeks on it, it's still not helping - the neuro still wants to give it a chance. So, we're sticking with it for the time being and she'll have the NCS repeated in 2-3 months.

As per usual, he was delighted with Alyssa's progress since we last met and asked how I felt the TSE was doing. I advised him that I am pretty sure it's making a big difference. She's eating better, complaining about a "sore tum/bum" far less and doesn't have the constant "frown" she was beginning to wear all the time. She IS being a typical 2 year old now so some of that was hard to distinguish between what was her just being "normal" and what was her being miserable due to her condition. Alyssa is still complaining for quite a long time about going to the loo, and still having problems actually passing motions but she's relatively happy the rest of the time - which is still an improvement. Will ask the anaesthetist next time I have contact if we can attempt to reduce the Gabapentin again and see if she really is coping better now with the TSE ... and hopefully get her off the Gabapentin altogether.

Saw the dentist; who was very pleased to discover that Alyssa has not bitten herself or caused oral damage recently, and who is now very hopeful that Alyssa is now old enough to be a bit more aware of what she's doing ... so possibly may get to keep the molars after all. Told her that Alyssa has literally just started chewing her hands - in the usual places - but that she does stop if told to (and as she is wearing arm restraints overnight to stop her tearing her skin off, she is also unable to bite her fingers/hands then either, which is when she used to do it!). So we'll see, and hope that the worst of the biting is over and that she may get to keep these teeth. She is eating so much better now that she can just chew easily with them, compared to tiring herself out "gumming" everything hard. They have suggested going back to see them again in 3 months ... but sooner if nec, as usual.

Saw the dermatologist. Discussed that neither the Phenergan nor the Sinepin (the two newest drugs introduced to try and sedate her but also reduce the itching) did not seem to be having much if any effect on her. The derm has suggested trialling two different types of cream - not really designed for Alyssa's problems, but which may work in a roundabout way. Something else worth trying is always a good thing!

All in all, a good hospital day! Will have to wait a few days for one of the creams, and a couple of weeks for the other one but will post as I learn if/how well they help.