Straight

People who can make stuff have my utmost respect.  People who can create things. Who can fix things. Most impressive of all are people who can fix people.

The picture I’ve posted today is the best example I can think of.

Have a look at these screws and trust me when I say they are very, very, carefully positioned. These have not been Black & Deckered into some rawlplugs and then given a hefty thump with a hammer to make them flush.

No. These little babies have been meticulously placed, to within an ‘nth’ of the spinal cord. I use the term ‘nth’ as the exact measurement escapes me. It was a ‘somethingth’ of a millimetre. Close enough to make me feel faint.

These are what have hoiked my daughter’s spine back to an angle which can medically be described as ‘still a bit wonky but by God a hell of a lot straighter than it was’.

It was a spinal fusion operation. After these rods and bolts were fitted, bone was laid over the scaffolding and given the chance to fuse with her spine, so it has now 'set' forever, at this new, improved angle.

This is what is inside my daughter’s back. This is what has stopped her spine continuing a deadly curve that would eventually have harmed her internal organs and started to crush her lungs. 

This is a genuine, 24 carat gold (well titanium), unequivocal, it blows my mind every time I consider it, will you look at the size of those bolts, they’re sticking in her spine for God’s sake, how the hell do they have the balls to even do that, the air conditioning failed in the operating theatre you know, seven hours it took, I have never been so frightened about anything, MIRACLE.

Did I mention it was free on the NHS?

Video is The Stranglers - Straighten Out

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.

Rise

All I’m posting today is a song. It’s The Decemberists’ Rise To Me, and it struck a chord with me from the moment I first heard it. When I later learned it was about lead singer Colin Meloy’s autistic son, I realised why.

In a few short, simple, defiant lines, it somehow manages to encapsulate the emotions I feel as the parent of a disabled child: my melancholy at what could have been, my pride in what is; and my hope for what could be.

Big mountain, wide river, there's an ancient pull
These tree trunks, these stream beds, leave our bellies full

They sing out, I am gonna stand my ground
You rise to me and I'll blow you down
I am gonna stand my ground
You rise to me and l'll blow you down

Hey Henry, can you hear me? Let me see those eyes
This distance between us can seem a mountain size

But boy, you are gonna stand your ground
They rise to you, you blow them down
Let me see you stand your ground
They rise to you, you blow them down

My darling, my sweetheart, I am in your sway
To cold climes, come spring time, so let me hear you say

My love, I am gonna stand my ground
They rise to me, you'll blow them down
I am gonna stand my ground
They rise to me, and I'll blow them down
Cause I am gonna stand my ground
You rise to me and I'll blow you down

Video is The Decemberists - Rise To Me

Spectrum

Yesterday, the following discussion took place when my daughter asked me what autism was.

ME: “Well, some of the boys and girls at your school are autistic. It means they’re a bit special, like you, and they think a bit differently from most people. They can’t really understand how other people are feeling and how that should make them feel.”

HER: “What do you mean?

ME: [Struggling to think of an easy example] “Um...well say if Mummy fell over and hurt herself and started crying, what would you do?”

HER: [Without missing a beat] “Laugh.”

ME: “OK. Not the best example. What would you do if you saw Grandma and she was really, really upset? Would you be happy about it or sad?”

HER: [Looking confused] “Happy?”

It was at this point that I thought, not for the first time, that although Prader-Willi Syndrome isn’t on the autistic spectrum, it sometimes rubs shoulders with it.

Mind you, later on the same day, when my toddler (who has neither PWS nor autism) was approaching a sleepy pigeon in our back garden, waving around a bamboo stick as he did so, I added to the day’s litany of ill-conceived explanatory examples..

ME: “Oi! Don’t even think about poking that bird with a stick!”

HIM: “Why?

ME: “Because it’s not nice and it hurts. How would you like it if you were sleeping and I came and poked you with a stick?”

HIM: “Ha! Funny!”

It could just be my parenting, you know. It might not be anything to do with PWS. I might just be raising two psychopaths.

Video is Talking Heads - Psycho Killer

Letter

Yesterday, I let my daughter post a letter for me.

Now, as she’s 12, going on 13, and the letter box is roughly 100 metres from our house, you might think this is not a particularly noteworthy or impressive feat.

You might think that, but you'd be wrong.

The journey, or perhaps I should call it an expedition, involved a five minute briefing from me on where exactly she was to cross the road (directly opposite our home, in a very quiet cul de sac), how she was to stick to the path (you know, along the lines of Red Riding Hood), and then how (having achieved her objective) she was to return along the exact same route, checking carefully for traffic at all times.

I watched through the window.

She looked left and right and ambled across the road, chatting away furiously to one of her imaginary friends as she did so. (I learned in the debrief that it was Freya, an old favourite).

She walked along the path, clutching the letter to her chest with one hand, and gesticulating with the other, as the conversation with Freya got more intense. 

Then, for a minute or two, she was out of sight.

I think I held my breath.

And she was back. Still chatting, with that odd little wobble-headed, flappy-armed walk of hers, checking for cars as she crossed the road again, before she wandered up our drive and back through the door, her chest thrust forward and puffed up with pride.

Now all I need to do is train her to go to the off-licence.


By the way, the letter was a cheque payment for my Visa bill. If she somehow managed to post it down the drain by mistake, then any late payment fee is coming straight out of her piggy bank. 

Video is an excerpt from Hill Street Blues

Video is The Main Theme from Italian film Il Postino. Be warned, you'll be whistling it all day.

Wave

My daughter had a wave yesterday.

That’s how we refer to an episode where she is overwhelmed by her emotions. (A bit like me watching the end of Casablanca after four glasses of wine).

People with Prader-Willi are emotionally immature, and struggle to control their emotions. In some, this might be anger and frustration, particularly when food is denied to them. In my daughter, it takes a slightly different form.

Every now and again, she gets upset. And I mean really upset. A body-wracking sobs/ tears rolling down her face/unable to be consoled kind of upset.

The strange thing is, we can’t always work out what sets it off. It might be tiredness - but then there’s plenty of times when she’s tired that it doesn’t happen. Perhaps it’s anxiety - maybe when all the little worries and stresses swirling around in her head build up to breaking point.

The best way I have of describing it is that it’s like a wave. She might be paddling along, dipping her toes into difficult emotions, when suddenly, the tide rushes in and a wave of feelings crashes over her.

There is nothing we can do. My husband or I just hold her, brush her hair out of her eyes, and try and say calm and comforting things (probably to make us feel better, because she’s not really aware of them). The best we can offer is to curl up on the sofa with her for 20 minutes, if it looks like being a big wave.

Because all we can really do is wait. Then, as suddenly as it started, the tide goes out again. The wave retreats, with a whoosh. And with a wipe of the eyes, a sniff, and a deep breath, she’s back. And asking me why I don’t like Justin Bieber when he’s so good at singing. Telling me her Daddy is the worst dancer in the world. And asking if it’s really true that a man puts his penis into a ‘lady’s bits’ to make a baby. God, where’s a wave when you really need one?

Video is The Pixies - Wave Of Mutilation

Video is The Pretenders - Stop Your Sobbing

Bones

Question: How does someone know that they shouldn’t eat the bone on a chicken drumstick, if they’ve never had a chicken drumstick before?

Answer: They don’t.

If cooking chicken for my daughter, in accordance with my husband’s motto in life - "Always go for the breast" -  I’ll pick healthier, skinless cuts.

But there was this one occasion, running late, frazzled, and faced with a poorly-stocked Co-op shelf, when I grabbed a pack of chicken drumsticks.

Having cooked them, I plonked one down in front of my girl, along with some new potatoes and a mountain of salad, and then proceeded to knock over a glass of orange juice all over the table, chair, floor, radiator and wall. My attention was elsewhere for a good few minutes.

When I looked back, my daughter’s plate was clean, apart from a few splinters of bone.

Five hours later, the A & E doctor finally saw us, felt her throat and belly, and announced that it must have been a reasonably soft bone, and my daughter must have “munched the bejaysus out of it”, because there were no bits stuck anywhere they shouldn’t have been.

Prader-Willi Syndrome means you’re constantly, overwhelmingly hungry. And when someone is controlling your portion sizes, you’re damn well going to eat every last bit.

So she did.

Video is The Bees - Chicken Payback

Video is Elbow - The Bones Of You. 

Second

If you’re ready and you want it to happen and if all your bits and bobs are in decent working order, then the choice to have another child should give you a good feeling.

And it felt right, when my daughter was five, to chuck the old special smarties in the bin and say “Izzy wizzy, let’s get busy!”* 

*Please note, using a catchphrase from The Sooty Show here is merely for euphemism purposes. (Hand puppets were in no way involved in conception activities).

We’d said we’d wait until she was walking. Then when she took her first steps when she was three - thanks to her leg pads and walking frames, and physiotherapy sessions - the waiting period was extended to “when she gets a bit better at walking”. And then, as time passed, I realised the real reason for procrastination was fear.

I was afraid I wasn’t strong enough to cope if something went wrong.

I was afraid I wouldn’t be able to give a new baby and my daughter the attention each would demand and deserve.

And I was afraid that a new baby would grow up shouldering a responsibility they didn’t ask for: a sibling with a disability that will always need their support.

And then I thought: “Oh, sod it. It’ll be fun.”

Video is Marvin Gaye & Kim Weston - It Takes Two

Sheena

My daughter used to have a teaching assistant called Sheena.

Apparently, when she was at school, a certain Ramones’ song  was the bane of Sheena's life.

Fast forward 20 something years. Sheena the teaching assistant quite rightly thinks that the children in her class would not associate her name with a song from 1977.

Mwah, hah, hah. Not in our house, my dear. 

I have never seen my daughter quite so excited and so energetic before or since we played her this and pointed out it must be about her Sheena. The idea that Sheena had her own ‘shouty’ song appealed to something in my girl’s very core.

When my daughter was diagnosed with Prader-Willi Syndrome, when she was too weak as a baby to lift her head, and when I thought she'd never gain the strength to walk, I was I could have known that one day she would be pogoing like Zebedee after a double espresso.

Admittedly, after this danceathon she was so tired she had a two and a half hour catatonic nap and was pretty much wiped out for the next day, too.

But for a couple of minutes here, my daughter wasn't disabled. She wasn't different. She was an over-excited little girl, like any other, having about as much fun as it is possible for a little girl to have.

Hey, ho, let's go.

Wheels

It was a backwards Zimmer frame on wheels.

It was latest in a series of contraptions to help my daughter achieve physical feats other children managed with ease (see previous blog entry Cricket).

Called a 'rollator', which sounded like something you'd cut your lawn with, it was actually an open-fronted frame which provided enough support to enable my three-and-a-half-year-old daughter to walk.

What it also did was act as a big fat flag to anyone who saw her, teetering along painfully slowly, that this child had something wrong with them. It also marked her out to the other children in her playgroup as different.

So, we decided to add something else to the equation. We would make it an object of envy, not pity.  A trip to Halfords, and a few minutes work with a screwdriver and the frame had some custom additions: a drinks holder and flask, a shiny new bike bell, and last but not least, some furry dice.

Yes, we pimped her ride.                          

                                                   

"Well, I'm not braggin' babe, so don't put me down

But I've got the fastest set of wheels in town

When something comes up to me he don't even try

'Cause if I had a set of wings, man I know she could fly"

- The Beach Boys - Little Deuce Coupe

Whistle

It’s a tiny, pleasant little sound. A gentle ‘tinkle’. It’s the noise the pea makes inside a whistle. Not when it’s being blown, but when it’s being jiggled about.

My daughter ALWAYS has a whistle. (And, just to be safe, I always have a spare one in my handbag).

It serves a very useful purpose: it keeps her hands busy. Without something to fiddle with, she’d be displaying some of the traits she shares with some other people with Prader-Willi Syndrome - obsessive compulsive type behaviour like rubbing her gums, picking her nose, sticking her fingers in her ears, or picking any insect bite or tiny cut in the skin.

So instead, she fiddles. Everyone who knows her is used to her tinkle. You can hear her coming, like a cat with a collar on its bell.

Once, my daughter and her well-known whistle even saved the day at a kids’ football match. We’ve gone to watch my friend’s sons play at the local ground on a Saturday morning, and rolled up a few minutes late, wondering why the game hadn’t already started.

There, looking stressed out, was my mate’s husband, Fraser. Who was about to abandon the game before it had begun because although they had a ref, they had no referee’s whistle.

I believe he was actually in the middle of the phrase: “Where on earth are we going to get a....” when he broke into a huge grin as he spotted my girl ambling across the field towards him, absent-mindedly fiddling, as usual, with the whistle that was slung around her neck.

Video is The Beatles - Two Of Us (It was either going to be John Lennon or Roger Whittaker whistling. Lennon won).

Fractions

I have a confession to make. I don’t want my daughter to understand fractions.

I wish her to remain blissfully ignorant of how to split a shape up into five equal parts. I do not want her to understand why two thirds is greater than three fifths.

This is selfish, and purely to prevent a terrible confrontation.

You see, when your child has Prader-Willi Syndrome, a condition which means they are constantly hungry but have to eat a restricted diet, you quickly learn some psychological tricks which may convince them they are somehow being ‘allowed’ more food.

By having a small dinner plate, which is filled up right to the edge with food, rather than the same amount looking somehow more meagre in the middle of a larger plate.

By keeping an empty raisin box, and tipping half of the contents of another box of raisins into it. So your child can have a ‘second’ box of raisins, but is actually only having one box, split into two.

And, last but not least, by telling porkies about fractions. “Yes, sweetheart. I’ve cut it in half, so there’s twice as much now”.

She’s going to kill me when she finally works it out.


Video below is Mr Duey - Fractions. I can only apologise.

Oestrogen

It was just one little sentence. One, short phrase uttered by my daughter yesterday as she chatted, shyly, in a hospital consulting room.

We were seeing her endocrinologist* (*hormone-wizard, as I call her. This may, or may not be a precise medical definition). Her paediatric consultant was also there, plus a nurse. All of them have seen my daughter on numerous occasions, so she feels quite at ease talking to them.

We were discussing puberty and menstruation. As you do. Most girls with Prader-Willi Syndrome do have some signs of puberty, but do not have their periods, or if they do, they’re scanty, and irregular, and may not occur until they’re in their 20s.

Although this doesn’t sound like a terribly bad thing, it is. It means the body isn’t producing enough oestrogen, which is very good for bone density and protecting against things like osteoperosis. So at some point in the next few years, she may have to have her oestrogen levels artificially increased.

Of course this deficiency in sex hormones means something else. Something I’ve been open with my daughter about, mentioning it if it naturally comes up in conversation, so it’s not a big secret or surprise for her.

And this is what she referred to, in front of all these medical professionals, as we talked about her menstruating in the future. It made me desperately sad and desperately proud.

“Well, I can’t have a baby, but I might have a period. That’s quite grown-up, anyway.”

Video is Bandit Queen - Oestrogen.

Hips

Today's blog is a special guest post by Katy G:

When my daughter was born she had a hip problem. When we received her diagnosis and year-long treatment plan we were knocked for six. The ancient, grim-faced doctor gleefully called the traction cot in which she was interred for a month 'The Gallows'. I read an article in some shit magazine at the dentists with the headline: 'Doctors Hung My Baby Upside Down to Fix Her'. I read awful stuff on the web about the sores and stench that would result from her nipples to ankles plaster cast. If I had found your blog I would have been in heaven! My husband and I too joked about having T-shirts made for her (see blog entry Gillian). Ours were going to say: 'My Dad Chucked me Down the Stairs' or: 'I Fell Off My Motorbike' because we got fed up of the whispering in the supermarket, and the: "Oh, the poor little thing!"s.

The rays of sunshine were few and far between, but I remember each and every one vividly. The man who glanced at us in the bank queue, did the classic shocked double-take, but instead of following up with sympathy, stuck his tongue out her. The amazingly camp bloke working in the clothes shop who threw his hands up on clocking us and squealed "Ooooh! Look at you! Doing a star jump!"

After a week of my daughter in traction in hospital in her special cot (flat on her back, legs in the air, held up with ropes, never let out, or allowed to change position) we were allowed to bring her home, with the cot so she could carry on her traction in the middle of our sitting room. We slept next to her, on the floor, with a stout pair of scissors to hand in case she choked and we had to cut her free. So that was relaxing. Anyway, one day we had friends round, and it seemed rude to exclude her, so we sat on the floor around the contraption containing the perfectly cheerful strung-up-baby, drinking and chatting happily. A man knocked on the door collecting for charity. I was slightly jolly, with a beer in hand, and asked him in, while I found some cash. I didn't expect him to follow me into the sitting room. When he walked through the door he saw a bunch of youngish adults clutching cans of Stella, sitting laughing in a circle around a baby tied up in a weird, metal frame. He looked utterly shaken and appalled. I still can't think of that without giggling. And feeling slightly guilty for ruining his day.

Video is The Swinging Blue Jeans - The Hippy Hippy Shake

You can follow Katy on Twitter at @ksg123, or on the Word Magazine blog (username: katyg). Or on the Westmount Music blog.

First

My daughter spent an amazingly exciting week away at camp, sleeping in a tent, going to Thorpe Park, swimming, having campfire sing-songs, and even caving. So what was her answer when I asked her what the “best bit” of her trip was?

“Spag bol. We had spag bol, Mum.”

Similarly, when she went to a pop concert at Wembley Arena with the Guides, and saw JLS and a host of other young chart-botherers, what was the highlight for her, when quizzed? 
“That chicken pasta meal you packed for me, Mum.”

Our recent holiday on The Isle Of Wight? 

“The sea food platter”.

Can you see a pattern emerging here?

Today, we were round my Mum and Dad's. My husband was working, so didn't know anything about a little gift 'Nanna' had given my daughter: a signed photo of young Arsenal and England star Jack Wilshere, sorted out for my little Gooner by a friend of a friend.  (By the way, my husband, despite being a Spurs fan, manages manfully to curtail his instinctive hatred of the Arsenal when it comes to my daughter's love for them.)

When he arrived back home and settled down for a catch-up chat with his favourite girl, and asked her if anything good had happened today, her face lit up like a beacon. "Ah, bless," I thought to myself. "She's so excited about that picture, she can't wait to tell her Dad!"

Grinning from ear to ear, she looked up at him, her eyes shining.

"We had a LOVELY lasagne."

Now I enjoy a meal as much as the next chubster. But for people with Prader-Willi Syndrome, food is such an all-encompassing force in their lives that it really is their first, their last, their everything.

(Cue Barry:)