Center for Prader-Willi Syndrome

Originally Released: September, 1999
Patient / Consumer Inquiries: 1-800-492-5538
Media Contact: 410-328-8919

UNIVERSITY OF MARYLAND HOSPITAL FOR CHILDREN OPENS CENTER TO TREAT RARE GENETIC EATING DISORDER

The University of Maryland Hospital for Children in Baltimore will open the only center in the Mid-Atlantic region to provide comprehensive care for children and adults with a genetic disorder known as Prader-Willi Syndrome. The center, which opens on September 22, will also serve as a source for research and community education.

Prader-Willi Syndrome (PWS) was first identified by Swiss doctors A. Prader, A. Labhart, and H. Willi, in 1956. It affects about 1 person in every 15,000 and is caused by a genetic defect in chromosome 15.

The most serious and life threatening characteristic of PWS is hyperphagia, or an inability to control one's appetite. "These children generally do not eat much when they are infants. Then around age two or three, they develop a ravenous appetite and a compulsive obsession with food. They never feel full and will literally do anything to get more food," says Debra R. Counts, M.D., director of Pediatric Endocrinology at the University of Maryland Medical Center and assistant professor of pediatrics at the University of Maryland School of Medicine.

Obesity is a problem for PWS patients, because of their insatiable appetite. They also lack the muscle tone to break down fat stored in their bodies. As a result, PSW patients often suffer from diabetes, heart disease, and other health problems associated with obesity. PSW sufferers require life-long supervision and counseling in order to control their eating.

Children with PWS also have a distinctive appearance characterized by narrow facial features, almond-shaped eyes, small appearing mouth, and down turned lips. Later in life, many PSW sufferers experience problems with hypogonadism or delayed puberty, osteoporosis, congenital dislocated hips, and mild to moderate mental retardation.

To treat these physical and developmental problems, patients at the new center will receive multidisciplinary care from specialists in endocrinology, behavioral psychology, orthopedics, nutrition, and genetics. In addition to providing clinical care, doctors at the Prader-Willi Syndrome Center will be conducting research on the use of growth hormones to treat the disorder.

The center will also work with the Prader-Willi Syndrome Association of Maryland to assist with outreach programs and community education.

"This syndrome is extremely difficult for families to cope with and manage," says Dr. Counts. "We will be working closely with parents, local schools and daycare centers to help caregivers understand the special needs of these children. We will also be a resource for referring physicians," says Dr. Counts.

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This page was last updated on: March 21, 2008.