Day Twenty One

I have discussed my daughter’s fascistic tendencies before. I am convinced if we lived in different times that her love of rules would have seen her indocrinated into the Hitler Youth in a flash. There’s little doubt she would have sold me and her dad down the river, the little Nazi.

We get told off. For doing things we shouldn’t. For not doing things we said we should. For not doing things we said we should when we said we would. You get the picture.

We have a labrador. A patient, well-behaved, 11-year-old labrador. He’s only lived with us for nine months, but he’s part of the family.

My girl is in charge of his food. Boy, oh boy, is she in charge of his food. He receives a cup of dried dog food in the morning, meticulously measured to the line, and the same again in the evening. Once every three days, he is allowed a small treat. 

If my daughter hears the cupboard being opened and the rustle of the snack packet (which she can do from several rooms away), her accusatory voice rings out: “Jazz is NOT allowed a treat today. He WILL NOT get overweight.”

People with Prader-Willi Syndrome have to have their food intake strictly controlled, to prevent them becoming morbidly obese. Their bodies don’t convert fat to muscle efficiently, so have to be on less calories than average, which is especially challenging as they never physically feel full up. 

I think my PWS daughter, whose own mealtime and snack regime is so rigid, is enjoying being in control. She has no real agency over her food, so she’s channelling her inner frustration to take a controlling interest in the dog’s diet.

Today, Jazz, being a labrador, rebelled. At lunchtime, my son had a slice of bread cut up into soldiers sitting next to his soup bowl - a little too close to the edge of the table. The dog spotted his chance, and snaffled a finger of bread.

He got told off, in no uncertain terms.

“It’s just not good enough. You will be banned from the house for a day for this behaviour,” my girl informed him.

Harsh. So very harsh.

(I let him back in. I’m not a monster).

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Song is The Fall - Who Makes The Nazis?

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. If you don't do it, my daughter might report you to the SS or something. A huge thank you to the anonymous donor who I'm half convinced might have made a mistake with the amount yesterday. Get in touch if this is the case!  

Day Twenty

Once a biker, always a biker.

I first wrote about The Sons & Daughters Of Anarchy (East Bedfordshire Division) on this blog a few years ago (See the bottom photo).

There’s been a few tattoos and meths busts since then, and Knuckles and Pugface have had their share of beefs with the West Bedfordshire Division (splitters!)

Now my girl has some new wheels, the gang have got a bloodthirsty new attack dog, Fang, and today they were ready to hit the mean streets of... our small market town.

Their biker gang is so intimidating, people actively cross the street to avoid them. Well, they do at the moment, but that’s probably got something to do with Covid-inspired social distancing. 

But there’s no doubt they are dangerous. My boy’s levels of insouciance leave previously confident passers-by feeling strangely inadequate. And my daughter - who gets easily distracted by someone in her periphery and then unthinkingly steers that way as she turns her head to be nosy - has a good chance of mowing down anyone who steps within the 2m zone.

Today there were no hit and runs, and no run-ins with the law. I suppose I should be disappointed, seeing as I am the the gang’s leader, Bad Ass Mother Drake (number of tattoos - one; number of kills - at least three goldfish; time taken to squeeze into leather trousers - 45 minutes including 5 minutes of actual passing out).

I should protect my rep, and not get soft. But watching my girl on her trike, sorry, hog, makes me very happy. Due to her Prader-Willi Syndrome, her weak muscle tone and poor balance means cycling is a problem, but the three wheels gives her the solid base she needs. She’s a bit heavy on the braking, and you have to occasionally move like lightning to direct her towards the middle of a drop kerb, but once she’s on a safe flat path, she can pedal along in her fiercely determined way.

It makes me fiercely proud.

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Song is The Supernaturals - Motorcycle Parts

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. And if you can't, Mama Drake will send the biker gang round to persuade you, just as soon as I do up these leather trousers.

Day Four

Today, I’m going to break down some of the characteristics of Prader-Willi Syndrome for you. With some pratting about, obviously.

If your reading device only shows you the first few words of a blog before you click on it, then you will have just read the phrase: ‘Today, I’m going to break down’. It’s always a possibility, of course, but don’t worry, I’m still standing. Or “I’m Dill Danding’, as Elton John inexplicably seemed to be singing on that ‘One World: Together In Our Enormous Celebrity Homes’ concert the other night. 

We’ll leave Mr Dwight Divving His Dife Like A Dandle In The Dind for now, and concentrate on one part of PWS that has one of the biggest effects: low muscle tone.

It’s actually called hypotonia (a word which always makes me picture someone manically feeding hundreds of sheets at breakneck speed into a photocopier, but then I’m an idiot).

It starts BEFORE birth, with Sunday-pub-football-level-instead-of-World-Cup-winning kicks in the womb. High numbers of PWS babies are in an abnormal position at delivery, needing assisted delivery or a cesarean (my girl needed the latter, which had one upside I enjoyed - amazing drugs). 

Then, as newborns, PWS babies are very weak, requiring special feeding and arousal techniques. (It’s a coincidence, because as an adult I also require special arousal techniques, but that’s a whole different thing...).

The low muscle tone has consequent motor developmental delays (this phrase makes me think of being stuck at a roundabout in my battered old Honda Jazz automatic cursing the ‘motor developmental delay’ of it having the pedal-to-the-metal acceleration of a roll-along lawnmower).

So a PWS baby might not sit up until they’re one. They might not walk until they’re two (my daughter was three and a half, but more widespread and earlier growth hormone treatment has brought the average age down since she was a tot).

And children with PWS continue to have problems with strength, co-ordination, and balance. They have delays in activities such as jumping, climbing, and catching. They also struggle with their fine motor skills of drawing and writing.

Physio and orthotics (specifically supportive shoes, splints, and braces) help. Complications, such as scoliosis, may require an operation. If you think you know what stress means, book your kid in for a spinal op or two and you will NEVER sweat the small stuff again.

This bastarding hypotonia is for life. Adaptive and modified physical education programmes are recommended to build and maintain strength, tone and function. That means I spend an inordinate amount of time trying to think of sneaky ways to get my daughter up off her arse and doing stuff without it seeming like exercise. It will not surprise you that we have a fit dog.

One interesting fact you might like to know is that my girl is on drugs favoured by bodybuilders - daily growth hormone injections. She has yet to express any desire to don swimwear, sheen herself up with baby oil, and clench her buns and guns. But, incidentally, that is what I ask my husband to do for me as one of my aforementioned special arousal techniques...

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Song is British Sea Power - It Ended On An Oily Stage

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page. THANK YOU to everyone who has already donated - we've already broken the £500 mark, which is amazing! If you can spare just 26p, it would be help. £2.60 would be brilliant. If you donate £26, I'm offering sexual favours to be arranged when social distancing is relaxed, which may or may not involve baby oil. If this lockdown goes on for much longer we could possibly improvise with a rubber glove on the end of a broom. What the hell, it's for a good cause. 

Imperial

It’s halfway through the Easter break and I haven’t killed the kids yet.

That’s a successful school holiday in my book. Well, so far.

Today we visited baby Scarlett, a rosy-cheeked bundle of cute, who is missing the same tiny part of a chromosome as my daughter.

We’d met for the first time last year, and had a girls’ lunch, when Scarlett still had that wobbly, floppy-limbed, Prader-Willi Syndrome baby muscle tone.

Today, just a couple of weeks before her birthday, the little charmer was sitting up with a straight back, holding her head up, and clapping proudly. She’s got a lot to be proud of.

My daughter transformed herself into Prader-Willi Guest Expert to offer Scarlett’s mum advice on what constitutes healthy snacks and which TV programmes are babyish or not.

And my boy even managed to play nicely with Scarlett’s sisters without breaking into his current favourite song: a never-ending 100 decibel rendition of Darth Vader's Imperial March from Star Wars, each note sounded out as the word ‘bum’.

It was a good day. 

Damn. 

I had to mention it, didn't I?

Bum bum bum bum-bum bum bum-bum bum.

Music is "The Imperial March" - Darth Vader's theme from Star Wars

Maracattack

I started off doing the Crazy Crab and the Travelling Chicken with her, jealously eyeing up her maracas. (Gravity-induced diminishing pertness has meant it’s been a while since anyone jealously eyed up my maracas, but I digress...)

Before long, however, I just stood back and watched, amazed at the astonishing effect
Miranda Hart’s Maracattack workout DVD was having on my daughter.

Prader-Willi Syndrome means it’s hard for her body to convert fat to muscle, so she has poor muscle-tone. It means she has to be on a strict low fat, healthy eating regime, despite being hungry all the time. And it means she needs to exercise, despite not really liking the idea of ‘exercise’ very much at all.

This DVD, starring the tall and bumbling Miranda (whose slapstick TV show my girl finds very funny), turned all that upside down. The music began, and my daughter was off, in maraca heaven, shake shake shaking her thang.

She completed the 10 minute warm-up, and I took her jumper off, as she was actually putting some real oomph into it and looking a little flushed, a rare occurence when it comes to physical exercise, when her default setting is usually ‘amble’.

Then, for 20 minutes, she enthusiastically copied the workout routine, kicking her legs, circling her arms, hopping around, and giggling at Miranda’s silly comments, crabbing and chickening for all she was worth.

Mir-marac-ulous.

Video is The Rolling Stones - Jumpin' Jack Flash

Hogs

The newest chapter of the Hells Angels took to the streets today.

The Sons & Daughters Of Anarchy (East Bedfordshire Under 15s Division) let loose its two latest 'prospects', on a recce to the rec.

My boy was racing around on his new £10-from-ebay bargain bike (quickly picking up the pedalling technique and balancing required when utilising a tiny pair of stabilisers).

I used the opportunity to mention, casually, to my daughter: "Haven't we still got your trike somewhere?" The battered red three-wheeler had been gathering dust in the shed since our house move, forgotten about and unloved - but the idea of joining her brother on a biker rally had my girl asking me to fetch her Hog from the shed.

We bought it a few years back from the mum of a Prader-Willi Syndrome boy who was selling it because her boy had outgrown it. Ideal for someone with poor muscle control and balance, it gave our daughter the confidence and stabillity she lacked on an ordinary bike.

So out it came. Tyres were pumped, the saddle raised a little, and soon she was steadily and sedately sailing along on the tricycle, serene and happy alongside her fearsome-faced brother who was making machine-gun noises and yelling: "TURBO BOOST!" every five yards.

People with PWS need exercise to help control their weight, as their bodies cannot convert fat to muscle as efficiently as others. But they're stubborn. Really stubborn. So 'forcing' them to exercise is impossible. Sparking their interest, however, varying things up, and making them think the activity is their idea, works a treat.

I reckon the Mini and Micro Hells Angels will be burning a lot of rubber - and calories - this summer, which is fine by me. Just as long as they don't ask me to let them have Death's Head tattoos.

Clip is Steppenwolf - Born To Be Wild, from Easy Rider

Steps

My daughter has pioneered and perfected a new propulsion fuel. It's a little unstable, but supply is effectively limitless, free, and there are no harmful emissions, unless an egg sandwich has been involved at any point.

It’s called willpower. 

Being born with Prader-Willi Syndrome means your muscles are weak. Everything physical is harder for you than other people. So from birth, when it came to getting about, my daughter was at a disadvantage.

It wasn’t a level playing field: when other children her age were crawling, she was still so floppy she was unable to sit up. And when she did finally manage it, they’d outstripped her efforts and were already walking. As she got older, she couldn’t tackle the steps leading up to a slide, or walk across that wobby rope bridge in the adventure playground, while her friends clambered like monkeys and raced around. She still needed pushing, when they were pedalling. She often needed rescuing when she became stranded on the top floor of the indoor play area (or the inner sanctum of hell, as I preferred to call it). Now she’s a teenager she can’t break into a sprint on sports day, do a somersault on the trampoline, or bound up the stairs two at a time and leap onto her bed in a strop.

But when I list the things she couldn’t and can’t do alongside her peers, it doesn’t fill me with sad memories.

Because I immediately think of another list. What pops into my head are the alternative milestones she fought to achieve. Like how when she couldn’t crawl or walk, she was still so determined to get around independently that she developed this amazing rolling technique, lying flat on the smooth wooden floors of our house. We called it the Runaway Sausage Roll; she could turn 90 degrees and be out of a doorway in an instant. Then there was the day she completed her debut lap around the park on her oversized trike. And the first time she she painstakingly wheeled herself up the hill to playgroup using her rollator (a kind of zimmer-frame on wheels) - her wobbly-legged, head-held-high gait a strut to behold. Let’s also not forget the astounding slow-motion climb up a mountain at Chamonix.

I’m recalling all this because she gave herself another turbo charge of willpower during a school trip to Warwick Castle, this week, when to the astonishment of everyone (and I’m sure to the brain-popping frustration of the poor buggers stuck behind her) she ascended, slowly but surely, 530 steps to the top of the castle ramparts.

The view must have been spectacular. It’s pretty good from here, too.

You were my heart-flower, 
My poetry, my song,
Just one look at you and then I knew,
On that day,
Serendipity smiled down at me.

Video is Asgeir Trausti - On That Day

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Wheels
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Musclebound

I thought she was terrified. For a moment I had no idea what to do.

It was when I went back to work part-time when my daughter was a toddler. (I say 'toddler', but that word was never quite appropriate for her, because she never toddled until she was three and a half. At that stage, 'roller' would have probably described her more aptly).

A good friend of mine had also returned to her job two days a week. So we did a child-share, looking after the kids when the other person was at work.

Amy was a little poppet. And as she knew me well, I was convinced she’d be fine about staying with me when her mum wasn’t there.

So the first day I had her, I picked her up to give her a big hug. And she froze. I mean, really froze. Her body was rigid, like every muscle in it had snapped to attention. She felt like a wooden board, she was so stiff.

I looked at her face, convinced I’d see two wide, frightened eyes and a mouth wide open, about to let out a scream.

What I saw instead was a happy, smiling girl, who started giggling, presumably at the startled expression on my face.

She wasn’t scared at all. She wasn’t “frozen” either. What I had felt was the body of an average 18-month-old child. Because my daughter was hypotonic (a fancy way of saying she had weak muscle tone) I was used to a squidgy, soft, floppy body. Amy’s toughness was just the way muscles should feel.

Video is Spandau Ballet - Musclebound. In my defence, the video is a) deliciously terrible and b) extremely funny.