Bitch-slapped

My daughter is not one thing. She is not defined by Prader-Willi Syndrome; it is a huge part of her life but it isn’t everything.

When I write about my girl, more often than not, I focus on the positive. It’s how I deal with life in general - get through the bad stuff and enjoy the good.

But it does mean that sometimes people don’t quite realise that PWS can be a bitch. The first photo doesn't look like my daughter, the second does. They're both her. It's just that in one, she's been bitch-slapped.

Having PWS means my daughter has random, anxiety-induced meltdowns that she cannot be talked out of, reasoned with, or distracted from. People with PWS are emotionally immature and can often be overwhelmed by their feelings, which they struggle to control.

When my girl gets swamped by a wave of emotion it soaks me, too. I sit next to her, unable to ease her distress, even though I know the flash flood will recede and she’ll be smiling her amazing smile again, sometimes after five minutes, sometimes not for a couple of hours, and sometimes not until she’s had a good night’s sleep.

There are people who’ve known my daughter all her life who have never seen this happen. It’s difficult and gut-wrenching, and yet, like an awful lot of weird and sometimes not so wonderful things you deal with when you have PWS in your life, you get used to it. Even though you want to slap the PWS bitch right back, you get used to it.

May is Prader-Willi Syndrome Awareness month and it’s something people should be aware of. 

We've been helped hugely over the years by the PWSA UK (Prader-Willi Syndrome UK) - an amazing charity who do tremendous work supporting people with PWS, their families, and professionals who work with them. To find out more about the condition and the charity click here and if you can spare a few quid then click on the Donate Now button on the right hand side of their home page.

Video is Talking Heads - Swamp

Re-up

We’ve re-upped. I’ve made contact with our new supplier*, given him the readies**, and my daughter is shooting-up*** again.

*been to the chemists

**handed over a GP’s prescription

***having a daily injection of growth hormone
(Do you ever think you might have watched Breaking Bad and The Wire a little too fervently? No? Just me, then).

She was on growth hormone for just over a decade from around the age of around five.  Although it was slightly more uncharted territory when she was little, it’s since become widely accepted to be beneficial for people with Prader-Willi Syndrome, and there’s some solid research to back this up. It helped my girl's body composition, strengthened her muscles, improved her motor function, gained her some height (before her spinal fusion put the brakes on this) and increased her energy levels and alertness.

But, a couple of years ago, my daughter's endochrinologist took a decision to stop the drug. I have no idea how I sleepwalked through that. I took my eye off the ball. Hell, I took my eye off the ball, left the field of play, showered and went home to Netflix and chill. My usual squeaky-wheel stubbornness was inexplicably oiled over. Enough of the euphemisms: I allowed a poor decision to be made and didn’t challenge it as I should have. 

However, my niggling doubts about the course of action - or rather course of inaction - got nigglier, and my wheel started squeaking again. After a series of discussions* with her endochrinologist (*‘discussions’, ‘pleadings’ pick a word), and after a test which proved that she did have growth hormone deficiency, my girl is back on Genotropin.

My daughter has absolutely no anxiety about being jabbed in the thigh with a needle every night. She did have a slight wobbly ‘6 on the Panic Richter Scale’ episode in the GP’s surgery when she found out that we no longer need a big dial-up plastic pen thingie to load a cartridge with her entire week’s dose into and that each of her doses now comes in its own throwaway syringe. (No, I don’t know why this was so important to her, but it was finally solved by referring to the syringes as ‘cool little mini pen thingies’).

So under supervision, before bed tonight, she pushed the plunger on her cool little mini pen thingie. And as the liquid flowed into her, the relief flooded into me. 

Talking Heads - Drugs

Indentured

I’ve got a virtual suit of armour. It was forged during the long, wild, terrifying day when surgeons bolted titanium rods into my daughter’s spine. It resides in a box in my brain, where I can rummage around and find it when I am in need of a bit of protection. There are a few other crazy items in the cranium cupboard, including a sink plunger and rubber chicken, but I have no idea where they came from or what I might need them for. But the armour is fished out regularly, and I’m only just packing it away now after its second airing in the space of a week, during which time I became an indentured* knight.  (*Teeth. I’m going to talk about teeth, you see).

My daughter needs to have braces fitted, and this week she’s had two visits to the dentist in preparation, to have two lots of two teeth removed. And I had to sit there about three feet away whilst they stuck an enormous needle in her gums and pulled out a couple of teeth, plonking them in a little tray right next to me, the roots covered in blood. Then watch it all over again seven days later.

It wasn’t actually squeamishness on either mine or my daughter’s part that had me preparing to don my battle armour, though. Her syndrome comes with a high pain threshold, which sometimes is a Good Thing. Needles and blood don’t especially bother either of us. But I was expecting tears, tantrums, and a general mêlée (wasn’t that the name of the car in the Dukes of Hazzard?) - simply because of a number.

Over the last few months, as my girl has struggled to cope with her anxieties and emotions, she’s been turbo perturbed by the idea of having teeth out. Subtraction has been at the root of her root subtraction. She’s been worried by the idea of having less than 28 teeth. She loved having 28 teeth. That specific number. She likes to ask everyone else how many teeth they have, and a common sight if you watch her talking to someone is how quickly into the conversation she will persuade them to stick their fingers into their mouth and dutifully count up their pearly whites. 

And yet, a meltdown didn’t happen. I think a slight Eureka! moment a few weeks back when we saw an X-ray of her mouth where her wisdom teeth were visible may have helped. “Look!” I told her, grasping at the opportunity that had suddenly presented itself to me. “See those four ‘secret’ teeth there, that you don’t normally see, well, if you count them that means you WILL still have 28!”

Cometh, the hour, cometh the man, or girl in this case. We went in, the uprooting was done, and we went back in the next week and repeated the removal process. Not one word of protest, not one flinch, not one conversation expressing dissatisfaction or worry about the whole thing. 

I shouted out a checklist to her this morning before school.

“Got your bag?”

“Yes.”

“Got your coat?”

“Yes.”

“Got your teeth?” 

“Yes.”

Well, she had to take them in and show them off, didn’t she?

Talking Heads - Pull Up The Roots

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