PWS Blog

Telepresence Expands Reach for Prader-Willi Syndrome Services

Posted: Mar 28, 2017 by The Children's Institute


It’s snowing outside The Children’s Institute of Pittsburgh but, a few hundred miles away on the East Coast, where Amy McTighe, PhD, is beaming a group of school teachers a PowerPoint training deck on Prader-Willi syndrome (PWS), the temperature is 30 degrees warmer.

McTighe, though, isn’t concerned with the clouds. She has a job on her mind: getting teachers for a teenager with PWS, who was an inpatient at The Children’s Institute’s Center for Prader-Willi Syndrome, up to speed on treating the debilitating disorder and understanding how it dramatically impacts the young girl’s life.

“The hypothalamus, which PWS affects, is the area of the brain where you, as a typical, normal-functioning person feel full when you eat,” said McTighe, who directs the PWS program here in Pittsburgh. “The thing that you need to keep in mind with PWS is that it’s a life-threatening condition. People with PWS, among other things, can participate in what’s called a food binge and eat themselves to death, to the point where their stomachs rupture.” 
     
Three times this week, McTighe will sit down in a fourth-floor conference room lined with photos of children with special needs and lead distance- or tele-training for interested parties in PWS. It’s part of her mission; training modules also are available for purchase in The Children’s Institute’s Knowledge Center. The brunt of the institute’s work these days with PWS, however, revolves around therapy and treatment. The CARF-accredited facility, established in 1902, cares for more than 300 inpatients and averages 70,000 outpatient visits per year, and its Day School serves nearly 200 students from 67 school districts.

PWS, the disorder of the body’s 15th chromosome, in addition to food-related behaviors, brings with it cognitive rigidity, oppositional or defiant behavior, anxiety and insecurity, and picking of the skin and body.

Gone are the tactics of preparing patients with PWS for independent living; their lives are lived supervised and, when it comes to food, under lock and key.

“This is a very different syndrome than ones you’re accustomed to in the school setting,” McTighe told the teachers, as a tele-training camera panned the room. “The ultimate goal for kids with PWS is maximum functioning, not independent functioning. We are not aware of any person with PWS who’s living independently in the community. They’re always going to need food security, they’re always going to need food supervision.”

At The Children’s Institute, she told the teachers, that means a special low-calorie diet we dub Red Yellow Green. The “green” is food that is low in calories, not unlimited but okay for seconds. The “yellow” food is high in calories, rich in nutrients and consists of the basic foods for a well-balanced diet. These foods are eaten in specified amounts.

The “red” food is high in calories and limited in nutrients and very limited in the daily meal plan. The red foods are items that are offered maybe once a month or on a special occasion or holiday.

Patients with PWS also have a poor sense of temperature and difficulty retaining information they read and learn. McTighe and her staff of therapists, clinicians and support personnel are ready for the challenge.

Life expectancy for PWS used to linger in the 20s, but times have changed, and The Children’s Institute of Pittsburgh has treated patients aged 5 to 65. And all patients receive one-to-one care.

But training is just one part of a larger picture.

“Training about PWS is essential for professionals and staff working in group homes, schools and any location where persons with PWS are being served. It is wonderful that we offer this quality training and support,” McTighe said. “The state of the art equipment that we have here at The Children’s Institute allows us to educate more people across the country and the world, rather than just locally. It makes a difference for so many involved in the care of a person with PWS. Most importantly, it makes a difference for that child or adult with PWS.”

The Children’s Institute recently expanded our care and service delivery through extensive telepresence infrastructure by hosting the PWS conferences, remote Individualized Education Program (IEP) conferences with school districts, and parent support groups. Lead donors contributed nearly $500,000 for our telepresence program.

To learn more and view training modules through The Children’s Institute’s Knowledge Center, visit http://amazingkidslearning.org/ today!


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