Thursday, 30 April 2015

A health update

A health update.....

I've not been updating this blog very often recently (for no particular reason)so I thought I would post a general update regarding Jessica's health.

I wrote here that in August we stopped Jess wearing her rigid spinal brace as it was exacerbating her reflux too much, and she was vomiting a lot. Without the brace, she wasn't vomiting as much, but was still bringing some stomach contents into her mouth, and we think she was frequently aspirating tiny amounts of stomach contents into her lungs.

Whilst this wasn't causing her significant issues from a chest infection perspective as she was never admitted to hospital for her chest or needed oxygen etc, (although clearly it's never great to be aspirating stomach contents!) what it did cause was her seizures to become very uncontrolled, which required her to be frequently admitted to hospital.

Previous to this, Jess had been quite stable on Epilim. Since this drug was started, she rarely had any seizures at night (up to that point she had only had nocturnal seizures) and all her other strange nighttime behaviours had calmed down. However, we have discovered that even the tiniest infection can cause Jess to start having seizures. It may be so minor an infection that on several occasions we haven't even realised anything was going on and the seizure was the first indication that something was wrong (it is a bit scary when your child stopping breathing with a seizure is the first sign of a separate health problem).

So, last September, when we thought Jess was constantly aspirating due to her reflux, her seizures went crazy. At least once a week Jess was being "blue lighted" to A&E in the back of an ambulance. The school would ring me to say she'd had a seizure and hadn't woken up - I would be at home, on the bus, in the pool for Anna's swimming lesson....  And then I would have to rush either to the school, or to A&E while keeping Anna calm and occupied.

We had assumed that Jess would need to have a Nissen's fundoplication in order to get control of her reflux (she was already on over maximum doses of reflux medication). This was not a surgical procedure we were exactly enthusiastic about, but we were resigned that it would be what she needed. However, Jess's consultant also wasn't keen, and wasn't sure it was the right procedure for Jess, and recommended we change her gastrostomy to a gastrostomy/jejostomy. This means that her feed goes into the jejostomy, which goes into the jejunum, below the stomach, meaning it shouldn't come back up. Her medication continues to be administered through the gastrostomy.

This has made a massive difference to Jess. Her feed now runs, very slowly over 19 hours, into her jejunum. She is allowed small amounts of oral feed - up to 15 spoonfuls (we tend to give this at lunchtime as breakfast is too rushed, and she is too tired in the evenings) which is more for her enjoyment than for nutrition.  And she does really enjoy it.

With this plan Jess's reflux seems under control and, as long as she is otherwise well, her seizures are also stable. She is also tolerating her rigid spinal brace again -most days anyway- and she will be reviewed in June to see if not wearing the brace for 9 months has caused a significant deterioration of her scoliosis.

However, nothing with Rett's is simple. We are now left with one major health issue that needs addressing -Jess' SEVERE sleep disturbance. Sleep has always been a massive issue for Jess (and us!). She has been seen by various specialists- since before she even had her Rett's diagnosis. However, every specialist has said we have to get on top of her reflux, her seizures, her breathing (Jess had her tonsils and adenoids out as she had sleep apnoeas due to her tonsils being too large) to ensure that these are not causing any of her sleep issues. Now is the first time we can say that all those conditions are finally under control and what we are left with is a true sleep disturbance.

Jess goes to bed on an adult dose of Melatonin. Without this, she cannot fall asleep. She will then sleep for a few hours, but will then wake and will usually stay awake for the rest of the night. The earliest she has woken is 23:30 (on the same day she went to bed!) but she will commonly wake anywhere between midnight and 0300. When she wakes like this, she is often distressed and clearly still tired, wanting to fall asleep again. She may doze off, but it is for seconds, before her body jolts her awake again.

This is so hard on the whole family. We are exhausted and shattered. I am regularly surviving (or not?) on less than 4 hours' sleep, and then having a hyperactive 2 year old to look after in the daytime. This is not sustainable, and I am having some serious health problems directly related to my sleep deprivation.

However, there may be light at the end of the tunnel. Prompted by my own health issues, and the fact that we have now ruled out any other cause for Jess's sleep disturbances, the doctors are now taking it seriously. Jess has been referred to a sleep clinic, and they are discussing starting her on proper night sedation in the meantime.

This lack of sleep has, for me, been pretty much the hardest thing to cope with out of everything Rett Syndrome has thrown at us. You can cope with so much more, so much better, when you have had enough sleep, and I will admit that I was in tears of relief when the doctors said they would consider sedation for Jessica. After nearly six years, this hell for me may be over, and I may get a semblance of my life back.

But it's not just about me, Jess needs more sleep too. She is tired, and you can tell she is far more able to concentrate, communicate, do her physio exercises, tolerate the spinal brace etc when she has had a good night's sleep.

Hopefully, the sleep issue will be addressed soon, and I feel we will then have made significant progress in dealing with the Rett symptoms that causes us the most problems and Jess the most distress.