A Young Man’s Inspirational Journey with Prader-Willi Syndrome
Advocacy It’s a rare, genetic disorder that leaves patients in the dark about whether they’ve had enough to eat or not. For some, this potentially life-threatening diagnosis means isolation — but not all.
Collectively, rare diseases are not so unusual. Nearly 30 million, or 1 in 10, Americans have one.
A rare appetite
Among these conditions is Prader-Willi syndrome (PWS), a complex genetic disorder that develops from an abnormality on the 15th chromosome and manifests most frequently with an insatiable appetite.
“There is no cure for PWS and it is the leading genetic cause of morbid obesity in children,” says Patrice Carroll, specialist at Latham Centers, a residential treatment program for children and adults with PWS, and other challenging special needs, on Cape Cod. “The only known treatment for the insatiable appetite of individuals with PWS is to provide a secure residential setting with supervised access to food 24/7.”
Identified in 1956, PWS occurs in about 1 in 15,000 births — and it is a reality that Brian Cass and his family know all too well. Brian graduated from Latham Centers’ school in 2015, before moving into one of Latham’s 11 supported homes for adults. Today, he is thriving despite the diagnosis.
Rise and thrive
“I have more freedom as an adult than I had as a child with PWS,” Brian shares, “but it makes it hard being around more food outside of Latham.”
PWS influences growth, metabolism, appetite, behavior and overall development. Patients characteristically experience stunted development, weaker muscles, short stature and a persistent feeling of hunger that, when paired with a metabolism that uses significantly fewer calories than normal, can lead to life-threatening obesity and related health problems, if the PWS is untreated.
“People with PWS can literally eat themselves to death. But with the right support system, they can live full and happy lives,” Carroll shares. “Without support, a person with PWS can experience high levels of anxiety that can lead to behavioral issues.”
“People with PWS can literally eat themselves to death. But with the right support system, they can live full and happy lives.”
Despite being genetically predisposed toward obesity, patients with PWS do not have to become obese. With proper diet management and regular exercise, a healthy weight can be maintained.
“I have learned to control myself,” Brian says. “I always try to be strong and remind myself to be that way.”
On the wing
Beyond providing a food-safe environment, Latham Centers also offers vocational training and last year made 46 job placements in the community for its adult residents, according to Maura Smith, Latham Vocational Services Director. Upon learning about his interest in aviation, Smith worked with the management team at Plymouth (MA) Municipal Airport to create a position that would serve both the airport’s needs and Brian’s interests and capabilities.
Today, Brian rides around the airport in a golf cart and records the planes’ tail numbers to satisfy the airport’s requirements to monitor the planes.
“I love my job,” he says. “The airport gave me a single prop airplane model that I keep in my room.” PWS is a lifelong condition with no known cause nor a cure, but this doesn’t keep Brian from living each day to the fullest. His advice to others facing a rare diagnosis? “That’s a tough one,” he says, pausing, then answer, “To stay strong.”