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- What is Prader-willi syndrome?
- Is PWS hereditary?
- How common is PWS?
- Does the Hyperphagia start from birth?
- Why do people with PWS want to eat so much?
- Is there anything to control the eating?
- What is known about genetic defects?
- What behavioural problems are displayed in PWS sufferers?
- Does early diagnosis help?
- How can you get information about PWS?
What is Prader-willi syndrome?
PWS is a complex genetic disorder that includes learning difficulties, behavioural problems, mental retardation, low muscle tone, delayed or no puberty or sexual development, and an involuntary urge to eat and forage for food. This is called Hyperphagia and because of the poor muscle tone there is a reduced need for calories as they cannot burn them efficiently.
PWS is caused by an abnormality of chromosome 15, it is considered to be a spontaneous genetic birth defect, although it can very rarely be inherited. PWS can be found in both sexes and all races.
It affects around 1 in 15,000 people. It is in the top 10 most common genetic disorders.
Why do people with PWS want to eat so much?
PWS affects a part of the brain called the hypothalamus that controls hunger and satiety (fullness). PWS sufferers never feel full enough, so they feel constantly hungry. To combat this problem people with PWS need much fewer calories to maintain a healthy weight. The obesity that can result is the main cause of illness and early death in this disorder as with obesity in the general population it can cause high blood pressure, respiratory difficulties, diabetes and many other problems.
Does the Hyperphagia start from birth?
No, newborns are often described as ‘floppy’ and have a poor suck reflex due to poor muscle tone (hypertonia). The increased appetite usually started at around 2-5 years old, and if their diet is not strictly monitored they can rapidly gain weight.
Is there anything to control the eating?
Sadly, no appetite suppressant has worked with people with PWS. They must be on a strict low calorie diet all their lives and must be kept away from the temptation of food. For example having locks on fridges and cupboards.
What is known about genetic defects?
The occurrence of PWS is due to a few genes missing from a chromosome which affects the functioning of the hypothalamus, as well as other things. This is a very researched subject in laboratories worldwide, since understanding this defect will be helpful to pws sufferers as well as understanding obesity, mental retardation, and behaviour problems in non PWS people.
What behavioural problems are displayed in PWS sufferers?
As well as extreme and very clever ways to obtain food, people with PWS can have temper tantrums, stubbornness, rigidity, argumentativeness and repetitive thoughts and behaviour.
Early diagnosis helps parents to establish a diet for their child and avoid all the problems that come from PWS from the start. Intervention in infants can help to identify the areas of need or risk.
How can you get information about PWS?
PWSA( UK) is a great source of information and help. Visit their website: http://pwsa.co.uk