Helping kids who don’t ‘get’ full
It’s not unusual for a kid to sneak an extra cookie when their parents aren’t looking.
Given the chance, Cindy Thomson’s three-year old daughter Megan will sneak the whole box, the container of noodles next to it, maybe everything in the cupboard, then on to the garbage container under the sink, even the squashed spider on the front stoop.
Megan has Prader-Willi syndrome, a genetic disorder that turns off the reflex that tells us our stomachs are full. It’s the number one genetic cause of childhood obesity. It randomly affects one of every 12,000 births.
Thomson knew something wasn’t right with Megan when she drooped like a rag doll after she was born. Poor muscle strength and tone is another manifestation of PWS. Doctors confirmed their suspicion of PWS with a series of tests, a CT scan, spinal tap, blood work.
“I had heard of it, but I didn’t know what it was,” says Thomson of getting the diagnosis.
Already challenged by her own struggles with Multiple Sclerosis, Thomson tasked her husband Glen to do more research on the Internet.
“I couldn’t bring myself to do it,” says Thomson. “It was just too hard. Everyone has dreams for their child when they’re born and knowing they have any kind of condition, you have to let those dreams die.”
They tapped into the resources and support available from the British Columbia Prader Willi Syndrome Association, meeting other families facing the same struggle. They were referred to genetic counsellors at BC Women’s Hospital. They connected with physical therapists.
For the first weeks of her life Megan had to be fed by a tube through her nose. When she progressed to a bottle, her diet had to be supplemented with formula. She never cried, never made any sound to indicate she was full, or hungry.
As Megan transitioned to solid food, her parents had a new worry; PWS kids have a different sensitivity to food and its textures so they puréed everything to ensure she didn’t bite off more than she could safely chew and swallow.
Children with Prader-Willi face other hurdles; many are developmentally delayed, they have an abnormally high tolerance to pain so they’ll pick at their skin without realizing the damage they’re doing, and they have cognition and learning disabilities.
Thomson monitors and measures everything Megan eats, as well as her activity levels; she’s on a strict 1,000-calorie-a-day regime that she’ll have to maintain the rest of her life. The kitchen cupboards and refrigerator are locked; they’ve heard the horror stories of PWS kids raiding the pantry in the middle of the night, eating everything they can reach while their parents slept.
Even at family dinner time, Thomson has to take a hands-on role with Megan’s meals.
“She never feels full. She’ll say ‘I’m hungry’ even though there’s food right in front of her,” says Thomson. “We have to remind her she’s got food in her tummy.”
The challenge of controlling Megan’s food intake will be compounded when she starts attending pre-school, and then eventually regular school, independent of mom’s vigilance.
“Some kids forage garbage, some steal food or lie to get food,” says Thomson. “You don’t see (PWS). They look normal.”
Building awareness in the community about PWS will help ease that struggle. Families with PWS recently used social media to campaign for an audience a the White House with Michelle Obama, who’s made it her mission to fight childhood obesity.
On Aug. 26, Thomson will be hosting the third annual One SMALL Step walk for Prader-Willi Syndrome at Burnaby’s Foreshore Park. All the money raised goes towards the PWS research plan, a collaborative effort by 65 doctors and scientists around the world to advance the understanding and treatment of the disorder.
When Thomson organized her first walk, there were only a few such efforts around the world. This year walks are being held across Canada and the United States, Belgium, France, Germany and the United Kingdom, with the goal of raising $1 million. Thomson hopes Burnaby’s efforts will contribute $20,000.
“It’s a fun day,” says Thomson as PWS families get to meet each other, share stories and educate others about what they’re going through. “They don’t feel so alone. There’s a lot more PWS people out there than we’re aware of.”
This year’s One SMALL Step walk for Prader-Willi Syndrome begins at 10 a.m., Aug. 26, at Fraser Foreshore Park. For more information, to register or to pledge support to a participant, go to www.onesmallstep.fpwr.org.