Charities leads in services for those with Prader-Willi

If you've never heard of Prader-Willi syndrome, that's not surprising: The genetic disorder affects only one in every 15,000 births worldwide, and the number of experts in caring for persons with PWS is so small that many of those medical professionals know one another.

What is surprising is that ten percent of those in New York State diagnosed with the disorder are served by Catholic Charities Disabilities Services of the Albany Diocese.

In July, several employees of Disabilities Services attended the National Prader-Willi syndrome conference in Grand Island, NY, hoping to improve their expertise in serving those with PWS. Instead, they were told, "There is little that we can teach you any more. You are now regarded as a premier service provider in this field."

Genesis

Catholic Charities' involvement with PWS patients began more than a decade ago. Disabilities Services has long served individuals with many developmental disabilities; when a parent whose child had PWS asked them to serve that population, they took on the challenge.

Prader-Willi syndrome causes low muscle tone, short stature, incomplete sexual development, cognitive disabilities and problem behaviors, but the most striking symptom of the disorder is that sufferers never feel full.

Their inability to control the urge to eat often results in morbid obesity and even death from perforated intestines.

Problems

The condition is not just difficult to manage, but also frustrating for family and other caregivers.

Anne Ogden, associate executive director for Disabilities Services, said Catholic Charities provides behavioral support for several families in dealing with their child's obsession with food, temper tantrums and aggression caused by PWS. Disabilities Services also provides respite care.

In 1995, Catholic Charities opened its first residence in Niskayuna for two individuals with PWS. Another two-person residence was added this summer, with a third in the planning stages.

Catholic Charities also has a residence in Clifton Park for persons with "atypical" PWS -- all the symptoms of the disorder, without the genetic markers.

Special needs

The residences are small because persons with PWS have such specialized needs.

"Their routine has to be highly structured," explained Jeff Covington, Disabilities Services' director of residential living. "They need to know what's coming up. The kitchens have to be locked; if an individual knows they can get at food, that will be their focus. It creates a great deal of anxiety, because they can't control it."

Structure and support comprise most of the services for those with PWS. The disorder cannot be cured, and treatments like appetite suppressants are ineffective. Individuals must be constantly monitored, keeping to a low-calorie diet, exercising five times a week and being accompanied by a staffer anytime they are out in the community.

Mr. Covington pointed out that any venture into the world is full of potential pitfalls for someone with PWS. Food is readily available at any workplace; even most gas stations are also convenience stores.

Caring staff

Catholic Charities staff who work with PWS patients must become very sensitive to food references, said Mrs. Ogden. For example, bringing a cup of coffee to work can make a person with PWS begin to obsess about the doughnuts associated with coffee.

"If they do get food," she added, "it's not about blaming the person, but looking at the system and how it failed."

About 25 staffers serve persons with PWS in the Albany Diocese. Success for those they serve, according to Mr. Covington, can often be measured in weight loss and ability to function more easily in society.

Successes

Mr. Covington gave the example of one woman who moved into a Catholic Charities residence weighing more than 200 pounds and using a wheelchair. Her goal was to be able to move from her bedroom to the kitchen.

"Now she's about 130 pounds and uses a walker," he stated.

Another woman was able to try several jobs because of Catholic Charities assistance, learning to be open about her condition so her co-workers wouldn't offer her food.

Tops in field

The Disabilities Services staff told The Evangelist that PWS conference organizers were "being kind" in saying Catholic Charities has nothing left to learn about the disorder.

"There isn't anyone else that has put time and energy into this," they noted, but there are still many goals the agency wants to meet.

First among them is a creating a day program for individuals with PWS. Mrs. Ogden said Catholic Charities also hopes to develop more connections with medical providers who can treat persons with PWS, which involves many specific health issues.

"We have put a lot of work into making ourselves experts," the staffers concluded; but "there's always something that we can learn."

(Catholic Charities Disabilities Services serves approximately 650 people across the Albany Diocese, including about two dozen persons with Prader-Willi syndrome. Call 783-1111. Learn more at www.pwsausa.org.)

Mother puts hopes in residence

Desise Famiano's teenage daughter has PWS. "It's affected the whole family in ways I couldn't begin to explain," she told Marianne Potter, host of the CW network's "District Issues" television program.

The most frustrating part of having a child with PWS, she noted, is that "there's not a lot of understanding out there." Wherever her daughter goes, "there's always a food issue."

Mrs. Famiano's daughter hopes to move into a Catholic Charities Disabilities Services residence for persons with PWS.

"Thanks to Catholic Charities," said her mother, "there will hopefully come a day that I can rest." (KB)

(12/7/06) [[In-content Ad]]