Sunday, 14 May 2017

Baps

Baps. We’ve been juggling some enormous baps.

Unlike most of my everyday conversation, this isn’t as rude as it sounds. I’m just being a bit free and easy with my brain’s random acronym generator. BAPs (in my head, anyway) stands for Bunches of APointments. And lately, BAPS are, indeed, what we’ve had our hands full of.

My daughter turned 18 towards the end of last year (dear God, I’m getting old). So she’s in transition. This is a word that makes me thing of gender reassignment, but it’s not that kind of transition; she’s at the age when she swaps over from child to adult services in health and social care, and that involves big BAPs.

There have been GP appointments, dental appointments, and hospital appointments. There have been social care assessments and visits, plus a new social worker, new physio, and new endochrinology team to meet. 

A bridging loan has been required to pay for all the exorbitant car parking fees. A small area of woodland has been felled to make the paper needed for all the letters, reports and forms involved in BAPs work. There's been an oak tree’s worth just for my daughter's EHCP plan. 

And all of these things disrupt the normal routine. They put the usual timetable out of kilter, and cause varying degrees of worry to my worry-guts daughter, from low-level anxiety to full on meltdown, and the myriad of stages in between. Although she’s pretty good at meeting new adults, she comes away from each appointment having hoovered up info that she blows back out again in the form of repetitive, repetitive, repetitive questioning. 

This is how it goes. There are stages in our PWS children’s lives when we tend to feel more like a PA than a parent. When we switch our work schedule, tag team with our partner, and start scaring the postman with a terrifying scowl whenever he approaches our house.

But I know something. BAPs aren’t constant, even if it sometimes feels like it. There are stages in PWS lives when things do go gloriously quiet, when things tick along, when you’re not having to invent a time travelling machine to be in three places at once.

Enjoy these times when they roll around. And try not to start rifling through your paperwork because of the niggling thought that somehow you must have missed something. 

Ignore the niggle. Embrace the peace. You’ll know when the BAPs are back.

Song is Blondie - Long Time. Chosen for the first line: "I've been running circles"... And also because it's a very cheery pop song.

This post first appeared in the latest edition of PWS News, the newsletter of the Prader-Willi Syndrome Association UK. They have a very informative website here.

Wednesday, 10 May 2017

Diary

I love my daughter's school diary entries. In them, she has to list what she's done that day. Grammar and punctuation can be an issue. Yesterday's curriculum seemed a little...well...'frisky' - and I'm not talking about the piglets. I'm not surprised her teacher felt the need to annotate.



_____________________________________________________________________________



Song is Beck - Sexx Laws

Monday, 1 May 2017

Five

In honour of May being Prader-Willi Awareness Month (and also Gin & Tonic month, just because I’ve decided, OK?), I’m going to share something.

I could talk about a missing bit of a chromosome. I could give you a list of the main characteristics of the syndrome: how it involves weak muscle tone, immature sexual development, an excessive appetite coupled with a decreased calorific requirement, learning disability, and poor emotional and social development.

But that’s impersonal. And a bit depressing. It’s the kind of summary that floors you when you read about PWS as a new parent - as we did 18 and a half years ago. It’s a blood-draining, gut-punching shock. It’s unthinkable, unimaginable, unfair.

I’ll share something else, instead.

Tonight, I’m sewing a badge onto a blanket. It’s a small thing, but it isn’t a small thing.

My girl, the one with weak muscle tone, the incredibly stubborn one who won’t do anything she has set her mind against, and who has for all of her life clung insistently to floats and woggles in the pool, has earned her 5 metre badge.

Her swimming teacher, an otter-like marvel, has someone channelled my daughter’s stubbornness and determination, and got her to swim free. My girl’s style may have been unorthodox, her feet may have been dangerously close to touching the bottom of the pool, and she won’t be winning any speed records, but she did it.

That’s it, that’s what I want to share. That phrase. Expected and unexpected. Familiar and fresh. A phrase that I’ve used countless times, after countless waits, in different contexts, but always in the same amazed tone.

She did it.

I bloody hate sewing, though.

Song is Agnes Obel - Fivefold

Saturday, 22 April 2017

Pitched

Today was one of those community days.

I don’t mean I had to don an orange jumpsuit and paint over graffiti - that’s every other Sunday, and I still insist I was provoked, officer.

No, it was one of those days spent with other members of the PWS community. This PWSA UK (Prader-Willi Syndrome Association UK) family day was at Thetford Forest, and proved as much of a grin-generator as previous events we’ve attended. And, as you may know, we’ve attended a lot. Once my daughter has got wind of a Prader-Willi meet-up - no matter whether the location is The New Forest, Manchester, London or The Lake District - I have to have a cast iron excuse not to take her. By cast iron, I mean basically a death in the family. Hell, no, immediate family.

So we punched in the postcode in the sat nav, entered into the usual Brexit-complication-level negotiations over song choices on the stereo along the way, arrived, parked up, disembarked, registered, and got our name stickers. (Incidentally, I’m thinking of standing as an independent candidate in the forthcoming General Election on the single issue of it being compulsory for everyone, everywhere, at all times, to wear a name sticker. I’ve essentially got to that age where my brain just has no more face-name neurotransmitters left. I've pondered over the cause of their destruction, and I've narrowed it down to over-consumption of gin). 

We spotted a few old friends from previous events, and made a few new ones. There was, as always, a mix of tiny babies (with parents with a tell-tale, slightly shell-shocked demeanour), wobbly toddlers, cute children, my own diminutive teenager, and a lovely 21-year-old chap with a charming heavy facepaint/light beard combo, who introduced himself to everyone at least twice, and was utterly thrilled as this was the first time he’d met more than one person with Prader-Willi ‘like him’.

My girl was adored at by adoring Polly, who has a strong track record of previous adoration. They wandered around hand in hand, stopping off for my daughter to throw a little adoration of her own at Polly’s new baby brother (see picture). And my PWS girl - you know, the one with the insatiable appetite - insisted on not starting her picnic like everyone else until Polly had finished having her face painted and could sit next to her.

My boy behaved impeccably (by his standards), which meant he only burst half a dozen balloons, which wouldn’t have been so bad if they hadn’t sounded for some reason as though they were filled with gunpowder as well as helium. (Actually, in all seriousness, he was remarkable, playing a mini football match with a PWS boy, which awakened in him hitherto dormant traits of patience and magnaminity. Unlike when I play him, and he rugby tackles me, feigns injury, and bursts into tears if he doesn't beat me by ten goals).

It was a sun-kissed day, and a meeting of families who are all on the PWS map. We may camp out most days in very different topographic areas, from the beginners field to the SAGA cruise ship harbour, but today we pitched up together. And it was pitch perfect.

Song is: Yeah Yeah Yeahs - Maps

Monday, 6 February 2017

Pithy

I wish they could meet in the middle.

At one end of the dining table, I’ve got an 18-year-old girl who savours every last morsel of food. The obsessive hunger that comes with Prader-Willi Syndrome means that she eats everything on her plate, down to the very last grain of rice.

At the other end, I’ve got an eight-year-old boy who doesn’t want anything much apart from plain pasta. Any attempts to shovel other valuable nutrients into him require bilateral negotiations that make Brexit look like something I could wrap up easily in an afternoon, and still have time for several cups of tea and a haircut.

Earlier today, I was telling my nephew and his girlfriend about my boy’s culinary stubbornness. At least I was, until the fussy eater butted in with a scene-stealing interjection.

It was pithy.

“Don’t judge me.”

Cocky little git.

Song is Deerhunter - Back To The Middle

Friday, 27 January 2017

Extraction

Yesterday was a day where my daughter conformed to a great load of PWS clich├ęs, and defied a whole lot more.

We’d been at the hospital from morning long into the evening, on account of her having an operation to have all four of her wisdom teeth removed.

She was allowed breakfast, but no food after 7.30am. 

I’ll just give any Prader-Willi Syndrome parents reading this a couple of moments, as there’s a high liklihood they’ve just fainted with horror...
-
-
...There you go, pick yourself up and sit back down, breathing deeply, you’ll be fine.

To be fair, my girl was remarkably good about the lack of food. She did talk about it pretty much constantly, and checked with me numerous times what soft, liquidy snacks I’d stowed in a packed lunch box to give her when she came round after the op.

We recognised the dental surgeon who spoke to us about the operation he was about to carry out. “Where do I remember you from?” he asked, and then we all twigged that he was the same fellow we’d seen when we were referred to have her teeth out in his dental practice, and who had unwittingly instigated the mother of all meltdowns from my girl when he decided that the procedure instead needed to be carried out at hospital. (I wrote about it here, along with a random sheep and genitals-related anecdote. Don’t ask).

But yesterday, my daughter was pleased to see him, and was keeping a lid on her anxiety. The lid did start rattling alarmingly when the anaesthetist was having trouble finding a vein for the cannula, and mentioned ‘maybe needing to use gas’. One look at her horrified face (you can stick needles in her all day, but a hint of a mask gets her all wibbly wobbly), coupled with me aiming my special Laser Focused Red Alert Warning Raised Eyebrow straight at him, and he reconsidered. The cannula went in on the second attempt, the drugs went in, and she sank into unconsciousness. I told my eyebrow to stand down. 

The op went smoothly. 

My girl was in recovery for quite some time, as there was a bit of a logjam of patients and not enough beds on the female ward. I’d already read a couple of hundred pages of a book, and chatted with a couple of fellow waiting room attendees, one of whom amazingly turned out to know TWO children with Prader-Willi Syndrome. My girl told me later she’d been a little bit upset in recovery and had wanted her mum, but she hadn’t cried “much”. I wondered about this uncharacteristically touching affection towards me, but understood more fully when she mentioned the man had told her to “listen” and that she “really must stop taking the bandage things out of her mouth”. I could just imagine her stubborn face as she awoke to find two big bits of gauze in her gob, soaking up the blood, and her thinking, “Well, for a start, I’m not having these in...”

But back on the ward she was in surprisingly good spirits. The surgeon appeared and told me the potentially tricky bottom two gnashers had come out far more easily then anticipated. My daughter smiled shyly at him, and he wagged his finger and in a mock strict voice told her: “Just you leave that gauze in a bit longer, Madam!”

She had some nectar from the gods (otherwise known as a yoghurt in a squirty packet thing). I gave her another. She rolled her eyes in an even bigger ecstatic reverie. We waited around for another two hours for the pharmacy to prepare her antiobiotics, and then we were out of there. Home, for a mashed potato and gravy supper. The ‘squishy’ diet for 48 hours has and will continue to provoke much conversation, but she’s getting her food at normal food and snack times, even though it’s ‘different’, so in the grand scheme of things, it’s OK.

She’s been off school today. She doesn’t seem to have any swelling, and has only had a couple of painkillers. I took her for a haircut, then she basically Netflix binged on Call The Midwife.

She discussed it with me, having worked her way through most of Series One. 

“Mum, it really, really hurts having a baby, doesn’t it?”

“It certainly does. I take it there are lots of women screaming in Call The Midwife?”

“Yep. It’s full of ’em.”


Song is Timmy Thomas - Liquid Mood

Wednesday, 25 January 2017

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