Geezer

The people who EAT themselves to DEATH!

It sounds like a low budget, barrel-scraping, horror film sequel, doesn’t it?

It isn’t, though. It’s a stock phrase I’ve seen used in headlines about Prader-Willi Syndrome more times than a PWS person has dreamt of hot dinners. Well, maybe not that often, but hyperbole never knowingly underused, and all that. 

It’s probably heresy to admit it in the non-nuanced world of unwavering internet opinions, I have very mixed feelings about this phrase. Because it’s technically correct. People with Prader-Willi have a hunger that they cannot control. If allowed unrestricted access to food, then they could die, initially from a stomach rupture, or in the long-term because of the life-threatening levels of obesity and associated medical conditions that could follow.

It’s horrific. It’s shocking. It’s extreme, of course, because it’s a tabloid or magazine headline. But it’s true.

After 15 years of living with a child with PWS, the phrase doesn’t stab at me like it used to. But I still yearn to be able to add my own sub-deck:

The people who EAT themselves to DEATH!

But of course, this is very unlikely, because a lot of help, support and expertise is out there to help families prevent this happening, and with a bit of luck and a lot of hard work it’s perfectly feasible for someone with this condition to live a long and even a happy life.

Not exactly snappy, is it? 

Journalists have to boil complicated issues down. I geddit. I used to be one (local, not tabloid, I hasten to add, in an effort to emphasise that I have never rifled through a bin in my life, apart from that time I lost an earring, and anyway it was my bin). The focus on the ‘story’ of PWS for a tabloid will never be the families coping pretty well everyday with its challenges. It will always be the ‘volume turned up to 11’ end of the spectrum. And in some ways it should be. Awareness of the condition and the need to fundraise for research and support is never going to be improved by playing things down. 

A friend forwarded me a link to this recent Daily Mail* story about a ridiculously cute little lad with PWS. (*Obligatory warning: It’s The Mail. Do not, under any circumstances, look at the comments). Yes, this article is what sparked my EAT themselves to DEATH ramblings. It’s sensationalised of course. Buried near the end is a quote from the boy’s mum: “Michelle, who works as a part-time nurse, added: 'With the continued research in genetics and PWS, we remain positive about the future and hope that our little boy will be able to lead a happy and healthy life.'” This was immediately followed by four bullet-point examples showing bizarre and frightening examples of the very worst aspects of the syndrome, which in juxtaposition was like having a hug and then being slapped.

Read it. See what you think. Perhaps I’m being a bit cynical, but it has just struck me that the boy’s unusual name (a sure-fire internet ‘hit’ generator) might have been a factor in the story being published.

I’m torn. If more people become familiar with the syndrome, then it’s a good thing. On the other hand, it’s a pretty bleak read for anyone with a newly-diagnosed PWS baby. And on the other...well I’ve run out of hands, but, I’ll carry on anyway...the photos show a cheeky chappy who looks like fun. 

So here’s to Geezer (named, fantastically, after Black Sabbath bassist Geezer Butler). Life will not be easy for you. But that’s no reason why it can’t be good. 

Video is Black Sabbath - Fairies Wear Boots

Broadcasting

Sometimes my daughter proves to me she has more Random Access Memories than Daft Punk.

Today brought about another entry in the Stuff She Unexpectedly Remembers charts, just as I happened to be listening to a slice of magnificently nerdy, electronic music from a speccy, history-obsessed duo called Public Service Broadcasting. Thanks to some military-style planning on the babysitting front, me and my husband and my brother and his wife had actually been to see this band play live last night.

“Were Public Sir House good, Mum? What do they sound like?” my daughter asked me, after school.

I thought for a moment about how best to describe them. They mix up electronic beats and synth loops with guitar riffs, which underscore fascinating audio clips from old films, news broadcasts, propaganda, and public information films. Some of audio samples come with grainy, flickering visuals, which in the live show are beamed onto projector screens and small towers of valve-powered retro televisions. Now, I understand this sounds a bit odd, but it’s jolly well very entertaining.

I looked at my girl’s expectant face, and decided to let her hear for herself, rather than listen to a rambling load of old guff from me. “Public Service Broadcasting, sweetheart. They sound like this...” 

She listened intently. “I like it! This is good. What’s it called?” I told her the track I’d chosen was “If War Should Come”. Ignoring her little brother, who was jumping up and down and shouting that he wanted to change its name to 'Alex' (!), she listened to the rest of the song, which comes complete with air raid instructions to “extinguish all lights” and ends with the sound of Neville Chamberlain, solemnly declaring “I have to tell you now, this country is at war.” 

My daughter suddenly looked very excited. “THIS IS FROM THE OLDEN DAYS! THIS IS IN GOODNIGHT MR TOM! WHEN THEY’RE ALL LISTENING IN THE CHURCH!”

I was flabbergasted she’d recognised that the voice clip was the same one used in one of 'her' films.

"Wow. Yes, it is, you're right."

“Who says it, Mum? It is the same man as in Goodnight Mr Tom, isn't it?” 

“Yes. Well, it was on the radio and it was the Prime Minister at the time, who was called Neville Chamberlain.”

“Oh.”  Her RAM chip kicked in again. “I know who the Prime Minister is now, Mum. He’s David.”

“Yes, he is! And what’s his last name?”

“Er.....Beckham?”

Video is: Public Service Broadcasting - If War Should Come

Century

15/11/13 is a Significant Date.

Bear with me, and I’ll explain. Prader-Willi Syndrome is caused by a spontaneous alteration on chromosome 15. A little missing stripe. If you want to get all technical about it, geneticists label this region 15q11-q13. There are also two other chromosome disorders which have the same genetic alteration (Angelman's Syndrome and Dup15q) and which share some, but not all of the characteristics of Prader-Willi.*

Because this 15q11-q13 number resembles the date 15/11/13, which of course only rolls around once a century, the International Prader-Willi Syndrome Organisation and other people and groups involved with PWS used it to try to further awareness of the syndrome. Fundraising events were organised; blog posts posted; T-shirts printed; conversations started.

Today, of course, is the 16th. So I managed to mark the significant date of 15/11/13, which, if you recall, only comes around every 100 years, by remembering it 24 hours too late. I blame my poor organisational skills, which don’t have anything to do with any missing stripes on particular chromosomes, and are entirely due to me being a numpty.

See you back here next century then? Stick it in your diary - but good luck with scrolling through your smartphone’s calender to get to 3013...

*Click here to go to the IPWSO website page featuring a very clear diagram explaining the connections between the three 'chromosome 15 deletion' disorders.

Song is Blur - End Of A Century

Bango

My daughter has something in common with a large number of children with special needs: she doesn’t like loud noises. Certain big bangs, crashes or wallops can upset her, although she’s not affected quite as badly as the autistic lad in her class who wears ear defenders and as a consequence looks like a miniature DJ who’s lost his decks.  

She updated me with the latest chart, today. Here they are, in reverse order, pop pickers.

5) Fire bells

“You don’t need to worry about fire bells, really, sweetheart, because they only have a drill every so often, and they only last for a minute or two,” I said to her last year. The day before her school had a fire. The day before the bell went off for about half an hour. The day before the fire bell was topped by the ear-splitting squeal of fire engine sirens, just to add another level of wrongness to the How Wrong Was Mum scale.

4) Loud people, particularly [name redacted] and [name redacted] in her class

A bit specific, this one.

3) Daddy, drilling

My husband does not do much DIY. I do not encourage him to do more. When he does, the rest of us go out.

2) Pop concerts/music

My girl is very concerned about music being too loud. Her parents’ choice of music more so than her own, but even her beloved One Direction tunes are never turned up to 11, which is something to be grateful for, I suppose. A few years ago, she went to ‘The Big Gig’, a show organised by the Guides at Wembley Arena, and still talks about how it was too loud. Pixie Lott was on the bill, so I would have to agree.

1) Rheumatic drills

When she told me this I wondered, for a fleeting moment, where on earth she’d seen a Sergeant Major doing that marching Sound Off song with a bunch of arthritic pensioners, and how noisy would that be anyway? Then I realised she meant pneumatic drills, which is fair enough.

You may have noticed fireworks aren’t on the list. After a brief spell in the charts a few years back, they have consistently failed to feature in the affronted eardrum countdown. She’s decided she likes them. "They're a different kind of loud, Mum. Thunder's all right, too."

So tonight, we’ve been to a display (with a set of ear defenders stuffed in my bag, just in case of a sudden change of mind, but which weren't needed). We’re off to a friend’s firework party on Saturday, too.  And we’ll have a bangin’ time, because they're the right sort of decibels, apparently.

Song is Billy & The King Bees - Bango

Related posts: 
Kaboom
Blast

Vamps

Nosferatu, eat your heart out, sucker.

The annual sugar rush of Trick or Treating was not designed with Prader-Willi Syndrome kids in mind.

Children who don’t physically feel full up and buckets full of unsuitable sweeties and chocs are not a good mix.

Strictly speaking, as someone who has just had to peel my pumped up, jitterbug, vampiric son from the ceiling, that much sugar is not really a good mix for kids who don’t have PWS, either.

But our daughter, the one who has to have her diet strictly monitored, and the one whose calories we have to limit, loves Hallowe'en - the whole bat to pumpkin lot of it.

So we do what we always do: prepare, control and substitute.

We only target a few select Hallowe'enie homes: neighbours, aunties, uncles, and grandparents. Instead of Haribo multipacks, or kiddy meth delivery systems as I like to call them, savvy relatives dish out lower calorie snacks (raisins, breadsticks, Flump marshmallows, etc). If anyone sticks in normal sweets or chocolates, I do a quick switcheroo, delving into a ‘here’s one I made earlier’ bag of no-sugar substitutes. I let my daughter have a rummage through the treats and choose one thing to eat when we get back home, and then we save the rest for snacks to eke out (or as it’s Halloween, that should probably read ‘eek’ out) throughout the week.

The kids are settled down now, in front of a film called Corpse Bride, which isn’t quite as horrific as it sounds, and is for children, honestly.  

It’ll soon be time to tuck them into their coffins.

It’s been blood-sucking, stake-avoiding, crypt-creeping, pointy-toothsome fun.

Video is Jace Everett - Bad Things (the theme from vampire series True Blood). "When you came in the air went out." That's a killer first line, isn't it? 

Related post: 
Hallowe'en

Wind-up

Winding back the clocks unsettles my daughter. She can’t move the little hands round in her brain and click the concept into place.

It didn’t help that I used the phrase: “We’ve lost an hour.” The response of: “But where’s it GONE?” was, of course, inevitable.

It’s a difficult enough concept, anyway. Add in the rigid mealtimes required in a semi-militarised Prader-Willi Syndrome household, and it’s a ticking time bomb of confusion.

Luckily, she was tired this morning and had slept in a little, so it was relatively easy to stagger the breakfast/dinner/tea times to fit into an artificial approximation of her usual pattern.

And, thankfully, at the moment, timings don’t have to be quite as exact as they were when our daughter was little. In previous years, when she’s been up with the lark and feeling particularly hungry, we have been known to refer to the clocks going back to something called ‘Greenwich Really Mean Time’).

Our girl understands more now, can be reasoned with to a certain degree, and is more capable of making choices. She’s willing to have her dinner or tea an hour or so later than normal if she knows she’ll be eating out somewhere as a treat with family or friends. Counter-intuitively, despite her fierce hunger, she sometimes gets even more agitated if mealtimes are brought forward, but if it’s for a particular reason that you explain clearly and give her enough advance warning about, she will accept it. Changes to the food timetable in whatever form they take aren’t something you can just spring on her: they have to be carried out in accordance with the detailed blueprints.  And, just like on Grand Designs, you should really try to stick to the architect’s drawings wherever possible, otherwise the ceiling will fall in.

So we had a morning of discussions about time, hours, time going backwards and forwards, how clocks change, why some change by themselves, why I’d forgotten to change the kitchen clock, why her watch was wrong, why her snack time was 10 o’clock but not really 10 o’clock, what time it was now, what time it would have been, and when will it be dinner time.

The clocks weren’t the only tightly wound-up things in our house today.

Video is Dr. Feelgood - She's A Wind-Up

Video is Cher - If I Could Turn Back Time. Just because I turned back time today, and it gave me an urge to wear fishnets and straddle cannons.

Related posts: Clocks

Head

My daughter came home today with her head in a bag. It’s either charming or terrifying, I just haven’t decided yet. 

It was the last day of school before half term, so I was expecting her to bring home her PE kit, which only comes back for a wash during school breaks, and therefore is a) a bit whiffy and b) more wrinkled than the testicles of an elderly elephant that's spent too long in the bath.

Her brother was also weighed down with the drawstring bag/smelly trainers/shorts combo, and in addition a cardboard item which I think was supposed to be a telescope, but he insisted was a ‘fart torpedo launcher’. All fairly par for the course, to be honest.

The head took me by surprise.

“It’s my head mum. My actual head.”

“Well, when you say your actual head, that’s not quite right, because you’ve still got your actual head, this one is just a copy, sweetheart.”

“It’s not, it’s my head. My actual one.”

“Well, it looks just like your head, but you do know it’s not real, is it?’

“It is real. It’s got my glasses and everything.”

“OK. Whatever you say.” 

I decided to capitulate over the decapitation before I got a headache. An actual one.

Video is Goldfrapp - Lovely Head