Ouch

It's not often you see your face on the BBC News website. Not unless you've murdered someone. Or put a cat in a bin.

A journalist from the BBC's Ouch Disability Blog contacted me and asked if I could write something about Christmas in a Prader-Willi Syndrome household. A version of it appeared on the BBC site today and my social media feeds went a bit lively (in a good way).

There were no sensationalist headlines, the edits were thoughtful and prioritised the facts about the syndrome, and included in the piece was a link to the Prader-Willi Syndrome Association UK website. These are all Very Good Things. But that's not what has made me smile the most today. What made me giggle, repeatedly, was seeing our photo published. Because, Dear God, the shoot wasn't smooth.

Because of work shifts, we only had a 30-minute window of opportunity when all four of us would be together before I had to send the photo off in time for the deadline. This was not long, when you consider I was introducing my mum to the intricacies of my heavyweight SLR camera. I'm not saying she's a technophobe, but her mobile phone is a plastic cup and a piece of string. And she never remembers to charge her string.

"Just press the button halfway down and you'll see a little square, which shows you're in focus," I told her. "Why is the square around the picture on the wall?" "Point it down at our faces!" "Ooh, now there are lots of squares!" "Right, press it now!"

Meanwhile, my son had a face like thunder. He had announced as I collected him from the school gate, a few minutes before, that he had "hurt his willy." I didn't ask why. Sometimes, I have found, when time is short, being incurious can save your sanity. The mysterious willy injury gave rise to a limp to Nanna's house which can only be described as epic. It was one of those terribly painful 'switching from left to right when he had forgotten which side was supposed to hurt' limps. 

So it took about 30 shots. The camera battery was about to run out. Nanna was about to run out of batteries, too. The boy was very close to me giving him a festive Homer Simpson tinsel throttling. 

Then finally, we got one. One shot. "I think he sort of smiled!" said Nanna. And lo, so it came to pass that her family portrait was published on the website of the British Broadcasting Corporation. 

You can see the article here. 

And if you're interested, you can read my original version below, which has the full quota of silliness, stretched analogies, and mild profanity that regular readers are used to...

C-Day is coming. We’ve boot-camped ourselves into a crackling squad of commandos, and Operation Christmas is go.

Excuse the military parlance. You’ve heard the phrase ‘An army marches on its stomach’? Well our household cavalry would collapse if we didn’t have our provision of provisions finely tuned.

We’ve got a special, 16-year-old squaddie, whose world revolves around food. And Christmas has the potential to spin out of control.

Our daughter Josie has a rare chromosome disorder called Prader-Willi Syndrome. Although too weak to feed as a baby, children with PWS grow to have an insatiable appetite, and never physically feel full up. But because their bodies can’t convert fat to muscle efficiently, they have to be on a strict diet, for life. If their food intake isn’t controlled, they could suffer from life-threatening obesity. 

So Christmas can be difficult. The Western world loads its trolleys up with treats and gets ready to give that regular belt notch a week off. We show our love for our family and friends by feeding them, treating them, and passing round the Belgian chocs (if you’re posh), or Ferrero Rocher (if you’ve got no class, like me).

Except that when you have a person with PWS in your family, you have to twerk. I’m sorry, I didn’t mean bend over and shake your behind provocatively, although I might decide to do that after a couple of Baileys. I meant to say tweak. You have to tweak.

If someone’s hungry all the time, but has to have a limited diet, you need rules. Meals need to be at set times, and they have to be consistent. My daughter knows that she always has a main meal, low-fat pudding and fruit for dinner. She knows when her snack times are. When she was younger, screaming tantrums would ensue if the clock ticked past the allotted serving time. Now, her response to tardiness is more prosecutorial: I am in the dock, and she’s the terrier-like chair of the Parliamentary Select Committee on Parental Failings, and I had better have a remarkably good explanation, or I’ll NEVER hear the end of it.

So chuck Christmas into the mix, with people popping round at odd times for a mince pie, with a table heaving with traditionally rich foods, with choccies going round like rationing has just been lifted, and the season to be jolly could be a fa la la la flipping nightmare.

So what do we do? Well, after 16 years of PWS planning we do what we always do: adapt, distract, substitute, and be sneaky.

Our advent calendar is a cloth one with pockets we fill up ourselves with no-sugar sweets (popped in one day at a time, so there’s no temptation for Josie to chomp through the lot). 

Christmas parties with buffets need preparation and vigilance. I ring up to find out about the menu in advance, we sit away from the food, and we pack my handbag with healthy back-ups in case the fare is lighter on carrot sticks than expected. I make sure the host gives me the nod on the ‘grub up’ announcement, so I’m always first in the queue, even if I have to elbow an elderly aunt out of the way.

Christmas dinner itself is served at its special-occasion-negotiated time of 1pm At The Very Latest (apart from that year we had a flood when my sink got blocked and started firing potato peelings at me).

Josie gets a Christmas dinner like the rest of us, but she has one roastie and two boiled potatoes; her turkey skin’s binned; the Cranberry sauce is eked, not heaped; it’s Brussels heavy (increasing subsequent trumping levels); and her Christmas pud is a low-fat version. 

We also keep back a few spoonfuls so if there’s a dropped spoon disaster and three peas go on the floor, then three peas are replaced. This is fair, and you HAVE to be fair with Josie’s food: there are limits to what she can have, so she is blummin’ well going to have every last scrap of it.

Ironically, Daniel, our six year old son, is a fussy eater, and has to be cajoled, persuaded, and occasionally threatened to eat his meals. Josie’s polishes her plate clean at one end of the table and he pushes the chicken round his plate at the other, and I sometimes dream, wistfully, of a mythical middle ground. He understands Josie has a special diet, and she understands that he has different ‘versions’ of food to her. So he’ll have chocolate spread on toast and she’ll have a smidge of honey, and they’re both happy with that. And he’ll sometimes sneak into the kitchen with me and ask for a biscuit, and I’ll tell him it’s OK, as long as he eats it away from his sister so she doesn’t get jealous. He’s kind of small for the special forces, but he’s signed up.

We do have to be sneaky. At Christmas, all our usual tricks are in place: pile up the meal on a small dish, so it looks fuller. Keep empty boxes of raisins and fill them up with half the number they’d normally have - then she can have an ‘extra’ box. Cut things in half and tell her it’s twice as much (so sue me). Clear food away sharpish, and get the board games out. Talk all through the Queen’s Speech to deliberately wind Nanna up - that’s good for half an hour’s distraction at least. This is a bit mean, considering Nanna not only makes a beautifully iced Christmas cake for us, but she also makes a delicious no-sugar fruit cake alternative, so her grand-daughter doesn’t feel like she’s missing out.

There’s one thing I’ve forgotten to mention: how much we ENJOY Christmas. Forget the planning - it’s second nature after all these years. It’s all worth it when I see my daughter’s thrilled expression as she catches sight of the glitter tracks in the garden from Santa’s sleigh. (I can’t think how they got there, and yes, Josie still believes). 

We’re lucky. Our daughter is currently a healthy weight and is relatively accepting of Operation Christmas rules. We know from others’ experience that food issues often escalate dramatically as PWS children get older and struggle with semi-independence. Some young people and adults with Prader-Willi will sneak through bins or raid the freezer and even eat frozen food if they can get their hands on it. Their families and carers will have their own military ops centre and battle tactics, and whatever works best for them is the right thing to do. 

I do worry hugely about how Josie’s days will be filled after school and college finishes. Will she have the structure, routines, stimulation, and distraction in her life that are vital to successfully controlling her food issues and ensuring her happiness and health? Is the right living environment out there? Will professionals understand her complex needs? What balance needs to be struck between her right to independence and how allowing her more choices could have a dangerous effect on her health? 

Oh, and will my son ever eat a Brussels Sprout?

Video is The Killers - Smile Like You Mean It