You might think life is tough for a child with Prader-Willi Syndrome.
There’s the hunger, the anxiety, the learning difficulties, and all the rest of the blah blah blah.
There’s also the poor circulation, giving rise in my PWS daughter’s case to really cold hands and feet.
But I really don’t see what she’s got to complain about. She gets to wear cosy thermal socks. Not only that, this morning, when we awoke to snow, and realised her boots had been stored in the outside shed overnight, she sat on the stairs admiring her nail polish, whilst her dad knelt on the floor hairdrying her wellies warm.
As a season ticket holder over many years at several hospitals, she’s had this done a fair few times.
It started with her successful, if prolonged, audition as a baby-sized pin cushion in the hospital’s Special Care Baby Unit. This was just after she was born when doctors needed claret donations from her so they could diagnose what was wrong. The unit was referred to jauntily as ‘Scu-bu’ by the hospital staff (which always set off an appallingly inappropriate voice in my head singing: “Scooby Dooby Scu-bu, where are you?”).
Since then, as the years have rolled on by, whenever she’s had to have blood taken, the same thing has happened: nurses have struggled to find suitable veins in her arms. Luckily, her high pain threshold and consequent indifference to needles means this hasn't been as traumatic as it could have been. But it can be frustrating, and sometimes she starts to get worried when the process takes too long.
Yesterday's nurse, a splendid, super-efficient, chatty woman, who also happened to be clued up about all things Topsy and Tim (to my daughter’s delight), had one go on one arm, and decided not to go the usual route of trying the other one, coming back to that one, and eventually getting it on the fourth or fifth try.
Instead, she felt my daughter’s hands, which of course, were like little blocks of ice. Her feet and hands are always cold, thanks to her poor circulation (a common PWS trait).
“Cor, they’re freezing,” she remarked, and bustled off, returning with a magic trick, in the form of a bowl of warm water. “Stick your hands under the water, sweetie, and we’ll make those veins come out to play.”
Sure enough, a few faint, thin, spidery tributaries began to appear the pale skin on the backs of her hands. In went the needle, out went the blood, and my girl looked suitably awed. So did I, to be honest.
I think there’s a medical term for what happened. It’s called common sense.
Video is Nick Cave & The Bad Seeds - Red Right Hand
She snaps her fingers. Not to click along in time to the beat of music she’s listening to, nor to summon me to do her bidding (she has other methods for this). No, she has a very specific reason for doing it.
It’s a test.
She took her gloves and coat off the other day, looked carefully at her hands, and started snapping.
“What are you doing that for?” I asked.
“I’m seeing how cold my hands are,” she explained, giving me a look. “If I can click my fingers then they’re not too cold.”
Having Prader-Willi Syndrome means my daughter’s circulation is poor, and her extremities are rarely warm, even with thermal socks and gloves on. She’s not happy being outside in wintry weather for more than a few minutes; if we make a snowman, we make it small and we make it damned fast.
So she seems to have developed a little totemic reassurance for herself. Each click, click, click, is accompanied by a satisfied nod, nod, nod.
I’ve decided to call it her cold snap.
Although it’s just struck me that I really need a contingency plan for when the weather turns really cold. What’ll I do if she can’t click? The title of one of her favourite DVDs seems strangely prescient: Ice Age - The Meltdown.
So I’m off to stock up. Glove warming heat pads, hot water bottles, and patience. Unfortunately, I'm guessing the last item might be out of stock at Sainsbury’s.
Video is Stiff Little Fingers - Alternative Ulster (The eagle-eyed among you might have noticed I originally posted this mistakenly with yesterday's post. Just keeping you on your toes.)
Sometimes I fail. Sometimes I don’t go the extra mile for my daughter. Sometimes I take the easy option. Like today.
Tonight, my daughter should be at her last Girl Guides meeting before Christmas. But she won’t be.
It's an outside meeting, involving a long walk in the cold to look at the Christmas Lights, and ending up with a pit stop for chip shop chips on the Market Square.
I started my usual stream of consciousness plan of action in my head: “Right, she’ll need her thermal gloves and socks on, but her hands and feet will still be cold, because of her poor circulation, which means she’ll feel more hungry and more tired, because the cold does that to her, also she obviously can’t have chips, so I should send her with a bag of Skips as an alternative because they’re reasonably low calorie, but what about the smell of the chips, that'll be torture, and then they said they’re also having a hot chocolate, well, where from, and will they be able to give her ‘light’ hot choc if I provide it, and where exactly will they be at 8pm, because she's so obsessive about her bedtime lately I've been picking her up from Guides half an hour early , but if they're out and about how easy will this be?”
And then I stopped.
I was tired. I should have organised this before. It would just be easier if she didn’t go.
This is why I looked at my daughter and I said: “Do you really want to go? And watch all the other girls eat chips? And get cold and exhausted?”
Of course, when I put it like that, she said “No”.
I’m not very proud of those questions. They were too harsh, too leading, and too cold. They go against the grain. Against our usual ethos of sorting stuff out, working out wonky, roundabout solutions, and finding alternative ways for her to join in.
