What is Prader-Willi Syndrome
Prader-Willi syndrome is caused by a genetic abnormality, which is non- hereditary in the majority of cases. 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 often needs tube feeding in the first month. The second stage starts after age 1-3 years 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 put into 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. Most persons with PWS are mildly mentally retarded, although some variation is seen. Motor development is delayed. 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 1:15,000, could suggest some 350,000 or more individuals with PWS world-wide. 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 nowadays. Until recently, diagnosis was commonly not made before the child reached a stage where lack of pubertal development became obvious, weight gain was extreme and challenging behavior (usually around food) had set in.
Today diagnosis can be made in the first month by DNA testing (available in many countries) and an early diagnosis can give parents and families a real chance of establishing a good management pattern for their child. Food management has to be maintained for life and this is something the PWS persons themselves are not capable of doing. 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.
You will find information about Prader-Willi-Syndrome in different languages on the homepage of IPWSO = International Prader-Willi-Syndrome Oranization www.ipwso.org
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