Prader-Willi Syndrome

Posted on by Kylie Muccilli

girl with PWS smiling standing in field at hot air balloon festival

What causes Prader-Willi syndrome?

Most individuals with Prader-Willi syndrome (PWS) have a deletion of several genes on chromosome 15. The deletion occurs in the chromosome 15 contributed by the father. If an individual does not have a deletion, he or she may have two chromosome 15s, both of which have been contributed by the mother. PWS occurs in about 1 in 15,000 births. The diagnosis occurs equally in males and females and occurs across all ethnic groups. 

What are the effects of PWS?

While everyone with PWS is unique, individuals may share certain characteristics. They may include:

  • At birth, hypotonia (low muscle tone) and failure to thrive. 
  • As infants, delays in motor, cognitive skills, and speech. 
  • In early childhood, a compulsive eating and obsession with food may develop. This feature usually begins before age 6. The drive to eat is physiological and overwhelming and is difficult to control. 
  • Obesity is common though not universal.
  • Behavior issues such as repetitive behaviors, anxiety, rigidity to schedules and routines, and temper tantrums are common. 
  • Skills in solving jigsaw puzzles and word searches. 
  • Especially nurturing and loving towards animals and babies.  
  • Great perseverance in finishing tasks that can seem monotonous or routine to others. 

Can PWS be treated?

While there is currently no cure for Prader-Willi syndrome, individuals with PWS may benefit by receiving input from geneticists, primary care physicians, endocrinologists, nutritionists, psychologists, psychiatrists, special educators, speech-language therapists, occupational and physical therapists, families, support staff, and other care providers. 

Several treatments appear promising for better outcomes in weight management.

  • Early diagnosis and intervention
  • Growth hormone treatment
  • Healthy diet and daily exercise
  • Close supervision to prevent access to food

In terms of behavior and psychiatric issues, an early diagnosis, appropriate medications, and implementation of effective behavior management by family, staff, and professionals are key strategies for helping individuals reach their potential. Many of the strengths listed in the Effects section of this fact sheet can be maximized in vocational settings. 

With interventions in place and careful monitoring, quality of life can be greatly improved for persons with PWS and their families.

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[November 2013]