Thursday, 24 April 2014

Any colour as long as it's red

I really wanted Amy's new wheelchair to be red. I'd become a bit fixated on it. Obviously I wanted it to support her in all the right places, but choosing a bright colour seemed a small bit of necessary compensation for all the deliberations about the exact positioning of lateral supports and all the rest of it. (I'm nothing if not deeply shallow.)

But the local wheelchair service was giving nothing away. I'd requested red, and been told firmly that the NHS orders these chairs in bulk - and only in boring black. The therapist said she'd see what she could do, but seemed a bit bemused that I cared so much. She said she'd call me, but didn't, and so I resigned myself to another black chair.

It was a good surprise, then, when we arrived for our long-awaited appointment at the wheelchair clinic this week. Amy and I both squealed. (I stopped sooner.) She fussed impatiently until we finally sat her in the new chair, and then she was full of smiles. Her patience - never extensive - was sorely tried while the wheelchair engineer (unnervingly young-looking) fiddled about at great length trying to get all the supports, and the troublesome head-rest, exactly right. I sang Chattanooga Choo Choo, on repeat. Abby - who had to accompany us, as she had no school that day - did the usual pink flustered thing that she does these days when I sing in public settings. (What choice, though: Abby being quietly mortified or Amy being noisily impatient - well, what would you do?)

So we are happy with this chair. Amy was full of smiles the first day she left the house in it, and smiled particularly broadly for the school bus driver.

I will be happier, though, when we've been using it a few weeks and can be sure that it's right for her. I have a nagging fear that she's leaning to the left rather more than she really should. This 'truncal rotation' situation can probably be managed with a chest harness, if necessary, and I have it ready to attach. But I'd really prefer not to.

It's not a chair that tries to hide away. It has, I reckon, a good deal more character than either of the previous chairs Amy's had.

It's also indisputably more like a 'real' wheelchair and less like a buggy that might be mistaken for something a typically-functioning kid might use. I have zero problem with this: I welcome it enthusiastically. Never do I want to have another discussion with some random jobsworth that begins, "You can't bring your buggy in here."

We are ready to go places.

Tuesday, 8 April 2014

The words are there

Yesterday Amy said the word 'assembly'. No, really, hear me out on this...

I was chatting with a friend, R, who had come to see us. Amy was sitting in her 'unsupportive' chair - a small chair that has only arms, no harness, so she's not strapped in. Just sitting, with her feet on the floor. This is a big deal. Since she started on the MOVE programme at school, her sitting has been gradually improving. The goal is for her to be able to spend increasing amounts of time sitting in a 'regular' chair - and she is slowly, gradually, achieving it.

I was explaining this to R, and telling her how well Amy's doing, while Amy sat right there beside us. (We were involving her in the conversation - not talking over her head.) I said she was spending more time at school in a regular-looking chair, and she now sits in it at the weekly whole-school assembly on Friday afternoons.

At this point Amy, who had been fairly vocal generally (as she quite often is), looked up at us, and vocalised what sounded to me like 'assembly', in the context of our conversation. Right number of syllables, high level of enthusiasm, clearly trying to join in with what was being said. That happens sometimes, and I think I don't always pay enough attention. But R stopped and looked at Amy and said, 'Did you say assembly? You did!' And Amy was full of smiles. R then said to me, 'The words are there! She has them.'

And it's true: she often shows us. It's not just that she responds 'appropriately' when we talk, indicating that she understands what's being said, but that she has the words there herself and tries to use them. Ed's sure she tried to say 'It does!' the other day. She has other clear conversation skills too - listening and turn-taking. (Even the most cautious speech and language therapists agree with us on that.)

I've been sure about it for a long time, but struggled to trust my own, unvalidated observation. There are times when I see her try to form words with her mouth. It can only happen when she's calm, alert and focused, but it does happen. When I talk to her, she often does one of two things: either she vocalises in a very purposeful-sounding way, or she silently moves her mouth to try to make the words. I can see her do it. She knows the words: they are in there.

Sunday, 6 April 2014

'Stop looking at my hair'

Every morning when I go into Amy's room to say hello to her and begin the process of getting her ready for the day, the same thing happens. She takes a few seconds to focus, then smiles, then looks up at my hair. Always.

It's been going on for years, and I have no idea why. I have seriously uninteresting hair. The style and colour change only very subtly, and never overnight. It's not long enough to be put up in arresting arrangements. So why does she stare at it? It's become a running joke. Every day I say, "Amy, stop looking at my HAIR!" And she thinks it's hilarious.

She does it to Abby too, though less systematically. (And Abby has much crazier hair - who wouldn't look at it. See what I mean?)


I'm convinced it's a deliberate game she plays with me. It's a little communication thing that happens between us every day. But she clearly finds hair, as a general thing, amusing.

Add her fascination with hair to her ongoing interest in babies, and you'll see why she loves pictures of her little second cousin so much. This gorgeous baby lives in the US, so we're unlikely to meet her any time soon, but Amy loves looking at all the pictures her dad - Ed's cousin - shares with us. A cute baby with lots of hair: she couldn't be more thrilled.




Wednesday, 2 April 2014

Spinal brace-love. Or not.

Just when, I've been asking myself, did I become chief cheerleader for spinal braces? How can this have happened?

Of all the things I hate about living with Rett syndrome, strapping Amy's little body into a rigid brace every day and feeling it dig into me every time I cuddle her is pretty high on the hate-list.

But I've had a few conversations recently that have gone something like this. Friend - fellow Rett-parent - tells me that their daughter's latest spinal review shows her curve worsening. A brace has been proposed, in an effort to delay the progress of the curve. Friend is devastated. I didn't think this would happen. I feel bad we didn't realise, didn't do more. I'm not ready for this. There must be an alternative.

And I - trying to be helpful and supportive, although perhaps not empathising as well as I like to think, because isn't the fact that a brace is necessary so utterly shit a state of affairs that it doesn't need restating? - start talking about how we've learned to live with it, and how we find it helps Amy. It helps stabilise her. Means she doesn't need to wear a harness in her chair. Seems to make her feel more secure. And, the progress of her curve has slowed right down. We're used to it now.

Other parents are not comforted or reassured. Because, really, why would you be. It's a sign of a scary physical deterioration that is very hard to accept. But I feel a bit (silently) indignant. Why did you think bracing was something my child would have to have and yours wouldn't?

Yes, apparently I am the doyenne of Rett-parents who are brace-compliant. My kid's been wearing one for so long, from such a young age, that maybe other parents think that's how I like it.

It isn't how I like it. I hate it as much as I ever did. Just as much as you do, in fact. But wanting something not to be happening doesn't make it go away. We've adjusted. Made the best of it, even. You will too, just like with everything else. Doesn't mean any of us have to like it.

Tuesday, 1 April 2014

Trombones are interesting, but the piano's still best, and a 'break' won't be 'nice'

Taking Amy to 'events' is not a thing we tend to do. Even though she's keen on music, she's never been keen on the atmosphere of something being staged or performed. Probably a combination of an enclosed, crowded space and an air of expectation. (It's hard not to put pressure on her to like things, sometimes.) So we avoid, for now, while telling ourselves that it won't - we do very much hope - always be this way.

But when I heard about an interactive music event that was happening near us last weekend, especially designed for disabled children and their families by Create and featuring an outstanding pianist, I threw my usual gloomy caution to the wind. I knew it was going to be good when we arrived - early enough to be inconspicuous, not so early that Amy's goodwill had time to expire - to find the pianist already playing, in a hall that had been set up to be mainly filled with space rather than seats.

Amy's eyes lit up. There were a few children hanging around the piano, but she managed to work her way right in. The pianist, Derek Paravicini - himself disabled - could play anything anyone wanted. This is the moment she realised he knew Chattanooga Choo Choo, which remains her favourite piece of music.




The main concert featured a brass quintet, and lots of interactive moments. She was particularly interested in the trombone, which she got to see up close and managed to successfully touch. I can see no alternative: one of us will have to learn to play it.



But the piano continues to have the most startling effect on her. It's what she loves above all else. Music seems to speak to her, and call forth a response, in a way that nothing else does. Everyone agrees. Including her music therapist, L, who called me last week to tell me how well Amy's been doing with the weekly one-to-one music therapy sessions she's been having at school this term.

It was good to hear this, but I had a feeling L's call wasn't entirely to share the joy. And so it was. After telling me how responsive Amy is and how much she enjoys the sessions, L said - bafflingly - "It will be nice for her to have a break." Turns out that the sessions will be coming to an end this month. For all the obvious reasons - limited resources, a waiting list. I understand that, even though it makes me sad. But why not just say so, rather than suggesting that the cessation of the service is in Amy's interests? A break may be necessary, to make sure that everyone has a brief opportunity to benefit from what is a frustratingly limited resource. But please, let's not pretend it will be nice.

Thursday, 27 March 2014

Keeping the moment from running away

"A good snapshot keeps the moment from running away." - Eudora Welty

One of the tasks that's been on my to-do list for approximately the last two years is 'sort out photos'. But it seemed that the more family photos we took the more undoable this task became. We had so many photos on our phones, plus hundreds on the camera, and hundreds more on the laptop. A good few had been uploaded to Facebook. But none of this is quite tangible enough for my liking.

Leaving aside the fact that the digital versions are woefully disorganised, I am old-fashioned enough to like real photos in real albums and frames. I find it easier to look at them properly, and to think about what was happening when they were taken.

So we made ourselves do it at the weekend. I started out feeling distinctly grumpy and overwhelmed - but then a strange thing happened. I started noticing not just how lovely my children are, but how totally central Amy is to our family life. Not just central in the sense that she constantly needs things and we have to design our lives around her (because that's obviously true), but that she is literally in the middle of all of us. So many shots of us looking at her, her looking at us, showing her things, doing things together, and including her the best we can.

Clearly it's not possible to be entirely objective about one's own family, but what I see is how much Amy is loved. For all that our daily life with her is tricky, and for all the endless dilemmas about what's best for her and what we should be doing, these pictures show, quite simply, what she means to us.

We put a selection of the best ones - family, friends, animals, cakes - in a frame that she can see from her bed. And she looked and looked, and smiled and smiled, for a long time.

We should have done it sooner.

Monday, 17 March 2014

That thing with her arms? It's not just her clothes

Dressing Amy has definitely become more tricky of late. (Even upbeat Ed agrees this is so.) But I thought it was probably my fault.

It's never been an easy task, putting clothes on a floppy person who can't help out by pushing her arms or legs into things and who can't even sit reliably while necklines are adjusted or buttons buttoned. But recently it's got harder. It's the sleeves that have become problematic, you see. They all seem too tight. Getting Amy's arms in and out of them seems to take longer than it used to. Getting her arms to bend at the right moment doesn't seem to work so well. Getting them to straighten again is even harder.

Clearly, she's grown out of her shirts. The loose, tunic-type tops she wears at weekends aren't so bad, and her stretchy under-shirts are okay. It's the school uniform polo-shirt that's particularly problematic. That's where the self-blame comes into it: she's grown a fair bit in the last few months, and I should have bought her the next size up before now (even though, once the shirts are on, they seem to look okay). What was I thinking? Just not paying enough attention... Really, when am I going to stop making it all about the things I have and haven't done?

We saw Amy's consultant neurologist today, Dr W, along with one of the paediatric neuro registrars. They took a long and careful look at Amy, before deciding - quite logically - that we couldn't make any useful decisions about her epilepsy meds without an up-to-date EEG. I knew before we went in that this was all happening in the wrong order - but the appointment system produced this consultation (long overdue) without scheduling an EEG, so I went along with it. They wanted her to have the EEG there and then. (The registrar said, do you know where you're going - it's just above where we are now. Yep, I said, we've been there before - opposite fetal cardiology, yes? Okay, I might have been showing off a bit. We've been EVERYWHERE. I still find it painful going back to that end of the first floor. There's a very particular atmosphere there, where couples wait with their burgeoning bumps and shellshocked expressions. I empathise hugely, but I'm sure the sight of Amy and me couldn't be less helpful.)

Anyway, back to the sleeve situation. It's all connected. Before we were sent off for the EEG, the neuros took a look at Amy's hands and arms and told me she's showing signs of dystonia. I know that dystonia - stiffness and tremors in the muscles - is a Rett 'thing', but I've always been a bit vague about what it actually looks like. The registrar asked, is she hard to dress? Her arms are stiff, it must be hard to put her clothes on. With this question, everything became more explicable. It isn't a simple case of maternal negligence over the sizing of clothes. It really is hard to get her to unbend her arms, or to raise them. She winces when we try, and that familiar look of confusion and discomfort passes across her face.

There's a drug they'd like her to try. It should make her arms less stiff. (And her neck. Yes, her neck too.) But it might make her all-round more floppy. It could help the non-stop dribbling as well, but may cause other problems. They want to wait for the EEG results, because the priority is to get the epilepsy meds right - and it's a bad idea to make more than one medicine change at a time. Every decision has to be balanced against a whole bunch of other decisions. So we'll hang on.

I finally got round to buying some bigger shirts this afternoon. Even though they're not really the problem, you never know, it might help.