Hungry for a cure -- If left to own devices, Alexandria child would eat himself to death
If Matthew Senstad had the chance, he would eat himself to death.
There are locks on his cupboards and locks on the refrigerator. If his parents, Al and Karin, or his sister, Marissa, forget to lock the locks, or accidentally leave food out, the 8-year-old boy shoves it in his mouth as fast as he can.
"It's a 24-hour-a-day, seven-days-a-week commitment," Karin said of Matt's constant need to be monitored.
Even a few weeks before Matt was born, Karin's doctors thought there may be something wrong.
"They were concerned about the lack of movement in me," Karin said.
When Matthew was born, he didn't cry, and he was "floppy."
At 3 weeks old, Matt was diagnosed with Prader Willi syndrome (PWS), a genetic defect that causes insatiable hunger, and mental and physical disabilities.
"They have an obsession with food and are never full," Al explained. "For the rest of their lives they will obsess on food. They will seek food. If they aren't watched, they will eat themselves to death."
Ironically, PWS causes failure to thrive for the first year because infants don't have enough muscle tone to suck, but once they start eating, obesity is a constant threat. To make matters worse, the lack of muscle tone and energy prevents them from burning the calories they do consume.
The Senstads have Matt on a strict diet of 1,200 calories a day. Although sometimes they forget, they have to keep all food locked up at all times. And they have to watch him all the time.
"I was on the phone this morning with a client and couldn't hang up," Al said. "He ran upstairs and got a bag of marshmallows and ate half of it before I could get upstairs."
Kids with PWS also have learning disabilities, obsessive-compulsive tendencies and behavioral problems. And they don't like departure from their normal routine.
"It's very important for him to know exactly what's going to happen next," said Al.
"Cuz when I know it's lunch or supper then I know it's like food time," Matt said, snuggling up on the couch next to his dad. "My tummy gets worried sometimes then I have to say, 'Don't worry.' I get worried again. Sometimes maybe that's the problem with my tummy."
A soon-to-be third grader at Voyager Elementary School in Alexandria, Matt attends a combination of special education and mainstream classes. Developmentally, he is at the level of a 4- or 5-year-old. He's just starting to recognize words and isn't able do math, except for basic counting.
Because of his disability, acceptance among his peers will always be a struggle.
"Some kids are absolutely wonderful," said Karin, an elementary school teacher. "But some kids at school don't understand why Matt is the way he is. It would be nice if all kids could be more accepting of kids with special needs. That's my biggest worry - that his peers and other adults don't accept him for his differences."
"I think both Karin and I fear for the future and hope that he's going to be accepted," Al agreed.
Matt's future is not going to be easy. Even as a teenager and an adult, he will have to be constantly watched and his food intake monitored. The Senstads think that eventually, he will be in an assisted living situation with constant supervision and meal planning, with no access to food.
And at an age when he should start being independent, he may not even be able to hop on his bike or hang out with friends - as he may go to great lengths to seek food, such as stealing from people's garbage cans, raiding gardens and begging for food door-to-door.
Although it wasn't the life they planned for their son, Matt has brought the Senstads more emotional fulfillment than they could have ever imagined.
"I adore this little monkey," Al said as he put his arm around his smiling son. "He has added so much to our lives."
"He's just a lovable, caring little boy who really enjoys life," Karin said. "Do I wish he didn't have it [PWS]? Of course. Could there be worse things? Absolutely! So we love him for who he is."
And that's exactly what the Senstads hope that everyone else can do when they encounter Matt, or anyone else with a disability.
"The real challenge is to make sure that other people realize that somebody with a disability is a person first, and the disability is just one minor aspect of the overall person," Al concluded. "Everybody has their own features that have an impact. And that doesn't change the fact that they are a person first and the disability is secondary."
ABOUT PRADER WILLI SYNDROME
PWS occurs when there is a defect or error in the paternal genes on chromosome 15. The genetic defect, which occurs once in every 10,000 to 15,000 births, disrupts the normal functions of the hypothalamus portion of the brain.
The hypothalamus controls hunger and thirst and releases hormones that prompt the release of other substances responsible for growth and sexual development. It results in uncontrollable hunger, stunted growth, sexual underdevelopment and other characteristics.
In infancy symptoms of PWS include: poor muscle tone; failure to thrive because of a poor sucking reflex; lack of eye coordination; poor responsiveness; intellectual delay; excessive sleeping.
In childhood symptoms include: speech delay; poor physical coordination; food craving and overeating; excessive weight gain; development of food-seeking behaviors, such as hoarding food or digging through garbage; poor growth and physical development; learning disabilities; delayed motor development; behavioral problems; obsessive-compulsive disorder; scoliosis; skin picking.
In adolescence PWS also causes obesity and delayed puberty, leading to infertility in adulthood.
PWS is the most common known genetic cause of life-threatening obesity in children.
PWS is a lifelong condition. There is no cure.
For information on PWS, to contribute to finding a cure or to be a volunteer, visit the website www.pwsausa.org.