Sunday, 11 June 2017

MRI

We kept a lid on it.

She was bubbling a bit, but nothing we couldn’t handle. 

My daughter was waiting to go in for an MRI scan at the Royal Orthopaedic Hospital at Stanmore.

The scan was so doctors could have a closer look at her back, as her posture has unexpectedly developed a little more wonk and stoop over the last few months.

I read my girl the explanatory leaflet, telling her exactly what the procedure would be. Despite having had a couple of MRIs before, my Prader-Willi Syndrome teenager was persevering with her perseveration (repeatedly asking the same anxious questions).

“If I start to dream, will I start to grind my teeth? Mum, we haven’t got my mouthguard!”

“Will it be really loud?”

I assured her that if she did have a little nap, she wouldn’t be in a deep enough sleep to dream, or grind. I also reminded her that she’d be given headphones, which would make the beeps and clonks quieter. 

After a few verbal laps of this, my daughter settled back in her chair, edgy, but reasonably content. And then a ‘helpful’ woman patient in a hospital gown - who I shall henceforth refer to as ‘Mrs Shut The F*ck Up’ - appeared, having just finished her own MRI.

Seeing my girl and obviously trying to be nice, Mrs STFU said the following. (Please bear in mind my girls two obsessive lines of questioning, as detailed above...)

“Don’t worry about it - after a minute or two you just drift off and start dreaming.”

AAAArrrrrrgghhhhh!  I leapt in with a quick qualification that the lady didn’t mean she’d been asleep and that she meant day-dreaming, shooting the woman warning looks utilising some meaningful Roger Moore eyebrow movements (RIP the great man. And his eyebrows).

The woman's supplementary f*ckery?

“You’ll be fine. Although there’s a bit of banging. It’s a bit like a giant hitting something with his hammer.”

A BIT LIKE A GIANT HITTING SOMETHING WITH HIS HAMMER! In what world would you think a sentence like this would reassure an anxious child? I pity any grandchild of hers who has Nanna STFU reading them a calming bedtime story!

Thankfully, the male nurse leapt in, noticing my eyebrows go from ‘Roger Moore’ setting to full ‘Bill Bixby turning into Lou Ferrigno’ territory (for anyone under 40, this is an Incredible Hulk reference, OK? Telly hulk, not sexy Mark Ruffalo Avengers hulk...)

Meltdown Prevention Nurse - picking up on the sudden escalation in anxiety and gamma ray levels in the air - ushered Mrs STFU off to the changing room, after telling my girl: “Oh it won’t be too loud, I promise. I’ve got nice big headphones, and you can choose some music to listen to if you like.”

She steadied. The thought of Taylor Swift did the trick. And, a few moments later, headphones firmly on, my girl lay underneath the clanking, doughnut-shaped MRI machine for forty minutes, still, compliant, and chilled out.

Meanwhile, I fell asleep in my chair in the corner. Dreaming of Mrs STFU and very probably grinding my teeth.

Video is opening sequence from the 1978 TV show The Incredible Hulk, Not dated in anyway, no siree.

Saturday, 10 June 2017

Bananarama

Seventeen thousand steps is a decent distance - around eight miles, depending on your stride.

If you’re a bit of an exercise nut like I’ve somehow turned into over the past 18 months or so, it’s a pretty common figure on my Fitbit.

But if you’re a teenager with Prader-Willi Syndrome, with weak muscle tone, and an aversion to exertion, it’s a marathon.

And yet that’s how far my PWS daughter walked today, on a trip to London Zoo.

It was a PWSA UK (Prader-Willi Syndrome Association UK) Family Day. One of those special days where us oddball, motley, marvellous PWS people gather together.

We took over the education centre at the zoo, where staff showed us cool stuff like a tortoise shell and a crocodile skin (the crocodile wasn’t in it, in case you’re wondering about the Health & Safety implications).

The face-painter was kept busy creating Spidermen and tigers, and my girl and her PWSBFF (Prader-Willi Syndrome Best Friend Forever) got their arms daubed with pretty flowers, beaming like drunken hen night revellers in a tattoo parlour.

My daughter did her usual quizzing of parents of PWS babies/tots with questions about their age and what they eat. But there wasn’t time to speak to everyone - the teen queens had a zoo to explore, and explore it they did.

The duo became a trio with the addition of NPWSF (New Prader-Willi-Syndrome Friend), a delightful fellow teen who joined the Tattoo Club, grinning just as widely, and accompanied us on our safari trek with her dad. I christened the girls Bananarama (but only in my head, because there’s no need to talk about food unnecessarily in front of teenagers with genetically-led insatiable appetites, is there now?). 

We peered at penguins, goggled at giraffes, beheld bats, and I would say leered at lions, but I’ve run out of alliterative synoyms for ‘looked at’, and that one’s just silly.

My boy, the one without PWS, and the one with perfectly springy, tough muscles, was the one to run out of steam because “his feet hurt”. We called it a day, and caught the train back home, accompanied by a pigeon who hopped on at Kings Cross, and hopped off with us 45 miles later. He could only have exuded more of an air of ‘experienced commuter’ if he’d been clutching an ovepriced cappuccino whilst reading a copy of the Evening Standard.


Thanks to the PWSA UK for arranging, and for the lovely staff and volunteers for their friendly welcome and hard graft. Thanks to the zoo staff, too. 

And finally, most importantly, most memorably, thanks to our own version of Bananarama for their smiles - the only things today that blazed brighter than the sun.


Video is Bananarama (Jesus, that's a word you get lost in when you're typing) - Love In The First Degree. This video, though. I repeat, flabbergasted, this video, though.

Thursday, 8 June 2017

Vote

Today, I took my 18-year-old daughter down to the polling station to cast her first ever vote. I’m happy, proud, and a little bit teary. 

Yes, the smile will be wiped off my mug tomorrow, when we know the result of the UK General Election (or as I like to call it, another demonstration of the average British voter’s inexplicable desire to shoot themselves in the foot, repeatedly).

Like the morning after the Brexit vote, I’ll be feeling so utterly out of step with an insane world that I will most likely be out running at some ridiculous hour (I might as well do a physical version of my emotional huffing and puffing).

But today I’m happy.

I did the responsible thing. Taking into account my girl’s learning difficulties, I tried to give her a simplified guide to each party's policies. And I did try to disguise my own preferences, honestly, m’lud.

She’s watched the Newsround special. She’s talked about it in school (and told me that one of her teaching staff will definitely be voting for the ‘craps’. After some discussion, I realised she meant ‘crats’ as a diminutive of the Liberal Democrats, which came as something of a relief).

She chose ‘the red ones’. “Because of more money for the hospitals, and Grandad is in hospital, isn’t he?”

I explained how she would have to tell the polling station staff her name and address, take the ballot paper into the booth, put one cross next to the name of the person from the party she had chosen, fold it up, and post it in the special box. (I broke this down into each step, obviously, as otherwise she would have been lost at “name”).

And we did it. She tentatively walked to the voting booth, and took an age to read everything on the paper and find where she needed to mark. Then she walked, uncertainly, over to the ballot box, where the chap showed her where to post her slip. I watched all of this, standing a few yards back by the door, having already cast my vote. In the meantime, I’d had a little chat to my eight-year-old son about the process, and explained how important it was to vote, and how some people had died in order to give everyone the right to choose the people who ran things.

He didn’t look especially impressed.

“Why don’t they use computers instead of bits of paper?” he asked.

“Well, when you have computers, you might get hackers,” I said.

“Yeah, but you could get burglars stealing the box,” he replied, looking thoughtfully at the windows and doors, possibly checking for ease of unauthorised entry.

“Blimey, are you planning to hijack the election yourself? You’ll get locked up, you know.”

The polling station staff had overheard us, and were smiling.

As I ushered him and his sister out of the polling station, my boy was still pontificating.

“No. I’m not going to steal the box, Mum. But I think I know who will win, anyway. They’re called the Conservatories. They’re a bit like that baddie, Trump, aren’t they? He’s horrible.” Then he winked, put on an American drawl, and added: “And they WILL BUILD A WALL.”

Song is The Move - Vote For Me

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