LOCATION: Centennial Park, Etobicoke (TTC #48 Rathburn Bus from Royal York station) [MAP]
TIME: Early registration is 9:30 AM; 2.5 km and 5 km walk begins at 11 AM. Family events throughout the day.
It is recognized less for its proper name than it is for its most notorious and dangerous characteristic: insatiable hunger. Prader-Willi syndrome is a genetic disorder that can result in poor muscle-tone, learning deficits, a high pain threshold, as well as delayed motor and linguistic development. People with PWS also lack the signal that normally tells us we are full and have a constant, uncontrollable impulse to eat.
This Sunday, One Small Step is hosting a walkathon to benefit Prader-Willi research and raise awareness for the syndrome and families affected by it. The event will include a silent auction, appearances by Farley Flex, Hazel McCallion, Elmo and the Raptors mascot, and prizes from sports tickets to a five-star Punta Cana vacation. More on that in a moment, but first, what is this unique disorder that affects one in every 12,000 Canadians?
PWS is caused by the deletion or partial-deletion of genes on chromosome 15. The first symptoms are often noticed immediately after birth when the child may exhibit a poor sucking reflex, respiratory problems and weak muscle tone that causes the baby to seem overly “floppy.” Parents need to be ensure that the infant gets adequate calories and since crawling, walking and speech can be somewhat delayed, they must prepare for the child’s future development needs. Otherwise, most PWS babies are happy and look pretty much like any of their little peers.
As the child grows, the challenges become significant. Prader-Willi children achieve the usual motor and speech milestones, albeit more slowly, but can encounter other roadblocks such as short-term memory, intellectual impairment, and compulsive behaviour. Their relatively normal appearance may be a reason why some children with PWS to go undiagnosed, but there are classic characteristics that include almond-shaped eyes, a down-turned mouth, light skin and hair, and narrow temples. People with PWS are often short with small hands and feet, with low-muscle tone and excess fat, predominantly in their midsection.
Childhood also brings forth the most famous and troublesome hallmark of the syndrome, which is an unregulated appetite. Food intake is not typically controllable as the brain sends the signal that the body is continually starving, triggering a host of very significant, life-threatening issues. Since individuals with the disorder can’t regulate their appetites, they must be closely monitored at all times and access to food must be prevented, causing much anxiety for people with PWS and their families. Group residences are a common option as the child grows-up, and Prader-Willi homes usually have locks on cupboard doors and refrigerators.
Obesity is by far the most crucial risk factor as the child approaches adulthood, bringing with it the usual obstacles common to morbidly obese individuals like diabetes, heart disease, and social ostracism. Prader-Willi can cause a lack of sexual development, sterility, and stature, but Human Growth Hormone (HGH) treatment has been found to be effective in better regulating height, muscle mass, and weight distribution. Special food plans have been developed to more easily monitor caloric intake and weight management.
Families touched by Prader-Willi syndrome must radically alter their lifestyles and routines, as the syndrome has no cure, it needs to be supervised 24-7, and it lasts for a lifetime. Further anxiety for families comes when interacting with the public who may not be able to identify an individual with PWS or how to support its unique characteristics. Emotional, behavioural, and cognitive challenges can incite much anxiety for all parties.
That being said, people with PWS can today live happy, productive, and relatively normal lives. If it wasn’t for their frenzied appetites, they would likely have the luxury of independence. Prader-Willi Individuals can hold down jobs and enjoy relationships (though not bear children), and scientific research is promising better treatment for the future.
On Sunday, the One Small Step walkathon aims to raise $100,000 for Prader-Willi research, as well as general awareness for those affected by PWS. If you aren’t able to attend, tax-deductible donations are graciously accepted as well. The event is a family affair, with prizes and activities for adults and children alike. Pledgers get chances to win prizes like game tickets, electronics, and the grand prize of a Dominican Republic trip for two at a five-star resort.
Mississauga mayor Hazel McCallion will be present, and Canadian Idol’s Farley Flex will be delivering a motivational speech. Silent auction items include a one-week getaway, year-long Toronto Zoo membership, a $1000 RESP, and a Blackberry, among other items.
Read some real-world stories about those with PWS here and here. More links and resources about the syndrome can be found at the Ontario Prader-Willi Syndrome Association website. If you wish participate in or donate to the walkathon, full event information can be found at the One Small Step website.