Prader-Willi and not feasting at Christmas

The whole family Image copyright Other

Josie Drake has a condition that means she never feels full. Here, her mother Carolyn talks about the meticulous meal planning that goes into Christmas.

Our daughter Josie is 16 and has a rare medical condition called Prader-Willi Syndrome (PWS) which comes with learning difficulties and sometimes health complications.

Children with PWS have an insatiable appetite and never physically feel full. Some young people and adults will raid the fridge, go through bins or eat frozen food if they can get their hands on it.

Because people with the condition can't burn fat efficiently and have low muscle tone, they have to be on a strict and permanent calorie-controlled diet for fear of life-threatening obesity.

Families and carers will have their own tactics to combat this, and whatever works best for them is often the right thing to do. Problems with eating often escalate dramatically as children with the syndrome get older and struggle with semi-independence.

I worry about Josie's future but as things stand she's at a healthy weight and mostly accepts her diet. But at Christmas, with people popping round at odd times for a mince pie, a table heaving with rich foods, and chocolates being handed round (as though rationing has just been lifted), the festivities have the potential to become a nightmare.

This year we'll do what we always do to dodge a fattening foods disaster - adapt, distract, substitute, and be sneaky.

Josie's meals follow a strict timetable. When she was younger, screaming tantrums would begin if the clock ticked past the allotted serving time. Now she is 16 her response to tardiness is more prosecutorial - I'm in the dock, and she's the chair of the committee on parental failings.

Christmas dinner itself is served at 13:00 at the very latest. Josie gets a festive meal like the rest of us, but every ingredient is meticulously measured and the calories counted. She has one roast potato, two boiled potatoes, the turkey skins are binned, the cranberry sauce is measured. It's Brussels-heavy, and her Christmas pudding is a low-fat version.

One unusually calorific meal means that the rest of her intake that day is shaved down to compensate. Sometimes this means we have to be sneaky.

Image copyright Other

We always keep back a bit of her meal so that if there's a dropped spoon disaster, and three peas go on the floor, then three peas get replaced. For her this is fair, and it is important we are fair with Josie's food. She understands there are limits to what she's allowed to eat, and she is determined to have every last scrap of her allocated portion.

We pile up the meal on a small dish so it looks fuller. We cut food in half and tell her it's twice as much. We keep empty raisin boxes and fill them with half the number they'd normally contain so she can have an extra box.

Her six-year-old brother Daniel doesn't have PWS but knows 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.

What is Prader-Willi Syndrome?

Image copyright PWSA
  • It is a rare genetic condition that affects one in 15,000 children born in the UK
  • The cause of PWS is not known, it is thought to happen by chance
  • It causes a constant desire to eat, restricted growth, learning difficulties, reduced muscle tone and a lack of secondary sexual development
  • There is no cure for PWS so symptoms have to be managed

Source: NHS UK

Prader-Willi Syndrome Association

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.

Ironically, Daniel is a fussy eater and has to be cajoled, persuaded, and occasionally threatened to eat his meals. So while Josie polishes her plate clean at one end of the table, he pushes the chicken round his plate at the other. I dream of a middle ground.

We really enjoy Christmas. There's a lot of planning but that'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).

But until the big day arrives, we'll be opening our food-safe advent calendar every morning. It's a cloth one with pockets we fill up ourselves with sugar-free sweets, popped in one day at a time, so there's no temptation for Josie to chomp through the lot.

Read more from Carolyn's blog, A Drake's Progress, here.

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