Basic Facts And Questions About PWS
Basic Facts And Questions About PWS
What is Prader- Willi Syndrome?
Prader -Willi Syndrome is caused by a genetic abnormality. Males and females are equally affected and so far PWS has been seen in all countries and all races. Recognized as a "two-stage" syndrome, the first stage is "failure to thrive". The child is weak and floppy from birth and sometimes needs tube feeding. The second stage usually starts around 3 years of age, when an abnormal interest in food and eating is seen. This leads to the onset of obesity from early age, if management structures are not in place. An environment where food is controlled and where persons with PWS feel secure and respected is essential- otherwise rapid weight gain and associated problems can drastically shorten the life span. In persons with PWS, Hypogonadism and low adult height are also part of the syndrome.
The lack of normal satiety is due to an abnormality in the satiety center in the brain, and until now, no medication has been found to treat this problem. Because it is essential that food intake is controlled, people with PWS are functionally disabled and will most often require 24 hour care, particularly when the IQ level is below average.
The incidence rate of PWS, usually given as 1: 12,000, could suggest some 350,000 or more individuals with PWS worldwide. Not so many years ago a baby born with PWS would have had very little chance of survival, as the equipment and procedures available in special care units were not so advanced as today. Until recently, diagnosis was commonly not made before the child reached a stage where a lack of pubertal development became obvious, weight gain was extreme and challenging behavior (usually around food) had begun.
Today diagnosis can be made in the first month by DNA testing (available in most countries), and an early diagnosis can give parents and families a real chance of establishing a good management pattern for their children. Food management has to be maintained for life and this is something the PWS persons themselves are not capable of doing. A lifetime of support is vital. Weight can be gained very easily and is extremely difficult to "exercise" off. This is due to the special body composition in PWS: muscles are small, muscle tone is low and the motor activity level usually below normal. This altogether means a lower than normal need for calories. To secure nutrition without becoming overweight, persons with PWS profit from dietary advice given life long.
- Thanks to PWSA (USA) for the above information.-
Do Diet Medications Work On PWS ?
What Causes The Appetite And Obesity Problems?
