What is Prader-Willi Syndrome?

Prader-Willi syndrome (PWS) is a non-hereditary birth defect that results from an abnormality of the 15th chromosome. Physical characteristics of those with the syndrome include short stature and low muscle tone.

One of the most dangerous aspects of PWS is a constant feeling of hunger paired with a metabolism that uses far fewer calories than normal. This combination can lead to life-threatening obesity. Learning disabilities always accompany PWS. The majority of individuals with the syndrome function intellectually in the range from borderline to low average IQ. The overall average IQ is about 70.

Symptoms Of Prader-Willi Syndrome

The occurrence of Prader-Willi syndrome is linked to missing information on chromosome 15. There are 46 chromosomes found in the normal human body. Twenty-three are contributed by each parent during conception.

As it happens, chromosome 15 utilises something called "genomic imprinting." This means that part of the DNA—in this case, the mother's contribution—is "turned off." Trouble arises when the remaining DNA—the father's contribution—is missing or damaged. Without the programming provided by the paternal DNA, the normal development and functioning of the body is altered, and Prader-Willi syndrome is the result. PWS occurs in about 1 in 15,000 births, although estimates vary widely. Currently, there are about 2,000 PWS sufferers in the UK.

Babies with PWS often suffer with a "failure to thrive" condition. The low muscle tone and general physical weakness that accompanies this syndrome make it difficult for infants to take in food. However, during the ages of two to four, children with PWS are likely to develop an appetite that cannot be satisfied. The appetite leads to compulsive eating, which is made much worse by easy weight gain. Unfortunately, temper tantrums and stubbornness also tend to accompany this condition.

Without constant monitoring, eating can lead to dangerous levels of obesity. But the behaviour problems make monitoring a difficult and exhausting chore. Several internet sites now offer support in this area, including nutritional guidelines specifically created for Prader-Willi syndrome.

Learning Disabilities And PWS

Learning disabilities are nearly universal among those with Prader-Willi syndrome. Children with PWS tend to have what many experts call a unusual cognitive profile. They often have very strong visual organisation and perception skills. They are also very high functioning in the areas of reading and vocabulary. However, spoken language is very difficult for them and will be much poorer than their level of comprehension.

Arithmetic and writing skills are very poor and they have difficulty processing auditory information. In a strange twist, people with PWS are often highly skilled in completing jigsaw puzzles.

This condition received some attention in the UK recently when Channel 4 aired "Can't Stop Eating." The program followed the lives of Joe and Tamara, sufferers of PWS. The show was criticised by many PWS advocates for not realistically depicting the challenges faced by those with this condition.

The learning disabilities associated with PWS do not normally prevent inclusion in mainstream schools. Difficulties in school most often relate to children's challenging behaviour and quest for food. Effective management of these behaviours based on better understanding of PWS can create an environment that allows children with PWS to reach their full academic potential. GCSEs have been obtained by many people with Prader-Willi syndrome.

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