Thursday, 25 April 2013
Thursday, 18 April 2013
Caring for Jessica is completely different at night to how it is in the day. In fact, the only similarity is how difficult it is to get her to sleep at either time!
Jessica rarely cries in the day, or gets upset or frustrated (apologies here to any Rett families reading this whose daughter who cries a lot). She tends to be very easy-going, good fun and generally very placid. If she is upset, it is usually because there is something really wrong – she is in pain, unwell, hungry, tired etc.
BUT at night, it is a completely different story…
The history of Jessica’s sleep issues is that she has never been a good sleeper, even as a young baby. When she started nursery at 14 months old, I warned them that she was very difficult to put down for a nap, but they were very blasé - “oh, we’re used to babies like that, don’t worry”. They eventually admitted defeat, and said she was the worst baby for sleeping that they had ever had!
Up to the age of 9 months old, we were very clear that Jessica wouldn’t be allowed in our bed at night. But this involved me spending up to 2 hours trying to get her to sleep, only for her to wake up again after 20 minutes, and then having to repeat the process again. ALL NIGHT. Inevitably, this was not sustainable; we gave in and started bringing her into our bed to sleep. But this meant that she still wouldn’t go to sleep in the evening, so she would end up downstairs with us until we went to bed, as this was the only way we got to have dinner, and manage to do anything in the evening. We muddled along like this for a while, with my husband working long hours, and me working three 10 hour days a week and trying to do paperwork in the evening, until this too became unsustainable. The turning point was when Jessica was about 20 months old and going through the regression stage of Rett Syndrome, although we didn’t know it at the time (Jessica was only diagnosed with Rett Syndrome a week before her second birthday). Her regressive stage was fairly mild – you had to know her well to notice, but she was slightly more withdrawn than normal, and less happy to be in groups of people. To be fair, she had never walked or talked so she didn’t have those skills to lose. The main skill she lost was the ability to feed herself using her fingers/hands, and this was a big thing, as she had always been very independent about eating and would never let us feed her anything on a spoon. But anyway, back to sleeping (or rather lack of). Up until this point, Jessica would always sleep if she was in our bed, but during this stage, she would cry a lot, and started flinging her arms and legs around, which we now know was the beginning of her sensory-seeking behaviours. I was being constantly hit by Jessica’s flailing limbs, and remember, in the blur, curling up in a ball at the bottom of the bed, sobbing with tiredness and trying to stay out of her reach. In desperation, we ended up at a private sleep clinic, and they gave us the Gradual Withdrawal technique to use on her. And it worked…to some extent. Within 2 weeks, she was going to bed in her own cot at her own bedtime and with far fewer wakings in the night. However, if you were to look through Jessica’s baby photos, you would be forgiven if you got the wrong impression; it was so rare for Jessica to be asleep that we always took a photo –consequently it looks like she slept all the time. Which was so NOT the case!
Now we obviously know that she has Rett Syndrome, and a lot has changed from then. Jessica will go down to sleep really easily, and will want to sleep as she is tired, but she is continually woken up by a variety of medical problems that we are only just beginning to get our heads around. I know that a lot of Rett mums like looking at their Rett daughters when they are asleep, as it is one of the few times they look at rest; their hands are still, their abnormal breathing patterns calm down etc. But I don’t like putting Jessica to bed, as I know that for the next 12 hours, she is going to wake frequently, crying and distressed, and there is (almost nothing) I can do to help her.
From September 2011, Jessica experienced quite pronounced sleep apnea, often causing her to go blue around the lips when she was asleep. Jessica would be struggling to breathe while asleep, and would have to wake up in order to take a proper breath. She had a basic sleep study at our local hospital in July 2012 which showed her oxygen levels dropped to 80% when she was asleep. Jessica was recognised to have large tonsils, and it was suspected that she would need to have them removed, but in September 2012, a year after these breathing problems started, her breathing dramatically improved. She then had an advanced breathing study at Great Ormond Street in December 2012, which predictably, came back normal, although they did note how often Jessica woke up distressed, and questioned if Jessica had reflux.
Jessica also experiences strange shaking episodes that may be seizures, but we are not sure. They only happen when she is asleep; she wakes suddenly, and her breathing speeds up and becomes heavier. She will then start screaming – she is truly terrified and her whole body will start shaking. Hugging her calms down the shaking, and if you get to her at the point her breathing starts to change, you can halt the process. It also happens if she gets woken up suddenly. At the beginning, they only happened one in a blue moon, and gradually increased until it peaked in August 2012, with several episodes a night. Jessica had a 3 hour sleeping EEG (a tracing of her brain activity), but she didn’t have any shaking episodes while she was being monitored. What it did show however, was that Jessica has extremely abnormal brain activity when she is asleep. As she didn’t have any shaking episodes while being monitored, they don’t know if those episodes are seizures or not. Nevertheless, and because her EEG was so abnormal, she is being referred to a more specialist team.
And then there is general Rett sleep disturbance, where Jessica is just “awake” for hours at a time, with nothing apparently wrong. She is not sleepy, not distressed…just awake. Last week Jessica woke at 2 am for 5 nights in row. She then did not get tired until her bedtime at 7:30 pm the next night.
So all in all nights in our house are pretty disturbed. Especially when you add a new baby into the mix! We are trying to address all the above issues; a test to see if she has reflux, referral to specialist neurologists regarding her abnormal EEG, and we have been given Melatonin to try when she wakes for long periods at night. Such disturbed nights are really limiting Jessica’s quality of life which, given the limitations Rett Syndrome already places on her, she really doesn’t need. She is often too tired to do her physiotherapy, or to concentrate on other activities. She also sleeps through lots of fun things we try to do with her.
At the end of the day (no pun intended!) we need to get this sorted out because Jessica needs more sleep. And so do we!
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Saturday, 6 April 2013
I know that Jessica understands what we say to her, and I know that she wants to respond appropriately. And on days when everything comes together, she can show me that is the case. But in order to reassure myself that I’m not imagining it, and also to feel a “connection” with Jessica, I find myself constantly testing her.
But I worry that it may be very tiresome for her. I know that I wouldn’t want someone constantly asking me “which one is red?”, “where is the number 2?”, “can you find the pig?” etc. etc. etc. Add to that the fact that we really have no idea what her cognitive level is; she may be thinking “oh no, not again, I’ve been able to do my colours for years, you just haven’t realised it before now!” I feel a bit like I imagine a teacher would, who has been asked to write a series of exam papers but doesn’t know the age of the pupils, or what the subject is that they have been studying!
So I was really happy when Jessica “asked” to show me all the animals in her favourite book. I was trying to get her to choose her bedtime story, as we do every night. I normally give her a choice of two books, and she will indicate which one she wants by either reaching out to touch the book she wants, or through eye gaze (where she looks at the book she wants, and then back at me). However, on this occasion, she looked at both books on offer, and then very deliberately looked away. She also did this with the next two books I offered her. I then offered her a further two books, one of which was her favourite animal book. At this, she became very excited, and both touched the book, and indicated through eye-gazing that this was the book that she wanted. However, when I started reading the story, she couldn’t have been less interested. It was only when I got to the last page with all the animals on it that she sprung to life – she wanted me to ask her where all the different animals where, so she could point them out to me. And she did. All of them! I love moments like that, when she so definitely wants something, and manages to communicate it to us. And so does she.