What is PWS > Treatment

Prader-Willi syndrome treatment

There is currently no cure for Prader-Willi syndrome, nor is there a drug that can be prescribed to alleviate all of the symptoms. As of this writing, there is no known substance that has been demonstrated to lessen the drive to eat, although much research is being conducted to find such a substance.

Treatment is primarily symptomatic and most importantly consists of:

  • Early diagnosis and intervention

  • Lifetime weight control through diet, environmental controls (lack of access to food), and exercise

  • Growth hormone replacement therapy, when available

  • Behavior management , with consistent limit-setting

  • Special education and sheltered or supported employment

By Suzanne Cassidy, MD

Early Diagnosis:

Early diagnosis of PWS gives parents an opportunity to manage their child‘s diet, prevent or treat infantile failure to thrive, avoid obesity from the start, and improve behavior. It may facilitate a family‘s access to critical early intervention services and help identify areas of need or risk. It allows families to know what to expect and institute appropriate preventative measures.

 

Weight Control:

People with PWS have a flaw in the part of their brain that determines hunger and satiety that gives them a continuous urge to eat following their period of poor eating in the early years. In addition, they need fewer calories to maintain an appropriate weight. This is believed to be due to decreased muscle tone and bulk and lower physical activity. It is important to provide the needed nutrients while restricting calories in order to maintain a healthy weight. Access to food must be limited and regular (preferably daily) exercise should begin as early as possible. Weight management should be individualized and include weekly weighing, complete control of food intake, environmental barriers to food access (such as locks on kitchens) and diets acknowledging that fewer calories are required.

 

Growth Hormone:

In recent years, numerous studies have demonstrated conclusively that growth hormone replacement therapy can very positively affect growth, body composition (the proportion of fat to muscle and bone), and activity level in people with PWS. It can also improve breathing and probably also speech and academic performance. Since it can improve body shape and physical abilities, it also improves self-image. However, growth hormone requires a daily subcutaneous injection, is expensive, and is not covered by all health programs in all countries..

 

Behavior Management:

Behavior management is crucial for both weight and behavior problems. Behavior management should be oriented towards intervention and prevention.  Consistent enforcement of limits by all caregivers (including parents, the wider family, teachers, and residential carers)  is very important. For best results, pre-schedule, pre-plan, avoid arguments and anticipate. 

 

Special Education and Sheltered  or supported Employment: 

Because of the developmental delay and intellectual disability combined with the behavioral problems, people with PWS require special assistance in school in order to learn as much as their abilities allow. Once they have completed school, they need assistance in finding employment that matches their abilities optimally, and supervision to control access to extra food and deal with behavioral outbursts when they occur. Adults with PWS who do not live with their family will require a residence that includes supportive supervision and accommodation to their dietary and behavior control issues while simultaneously allowing them as much success and social interaction as possible.

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Station Road
Cambridge CB1 2LA
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Registered as a charity in England & Wales, charity no. 1182873