Prader-Willi Syndrome

WHAT IS PRADER-WILLI SYNDROME?

Prader-Willi Syndrome (‘PWS’) is a rare genetic disorder causing weak muscle tone, incomplete sexual development, short stature, learning difficulties, behavioural problems and an insatiable hunger.

Insatiable hunger often does not appear until a child is about three years old but babies with PWS often experience problems with feeding. Weak muscle tone means that babies have difficulty sucking and therefore special feeding techniques or tube feeding will need to be used for the first few weeks or months of a child’s life. 

The insatiable hunger often appears as the child’s muscle tone improves and they become more mobile, at around six months to a year old.   Children then develop a strong, often obsessive interest in food and can gain weight very quickly.

This insatiable appetite is caused by a defect in the hypothalamus, the area of the brain that registers whether we are full or not.  This means that children with PWS are always hungry, irrelevant of how much they eat. This can drive them to eating almost anything for example, soil or paper.  Children often need a strict, calorie controlled diet and families with a child who has PWS sometimes have to lock up their kitchen cupboards to prevent their child from getting to the food.

At about the same time as this food problem develops, children with PWS can start to develop behavioural problems.  They often have obsessive/compulsive behaviours such as collecting and hoarding possessions and developing repetitive thoughts and habits.  Changes to daily routine can also cause unpredictable rages.

Most children with PWS also have learning difficulties, and social and emotional skills are also often underdeveloped.

TREATMENT OF PRADER-WILLI SYNDROME

There is no straightforward treatment of  PWS, but many of the characteristics of PWS can be lessened by often introducing simple changes and a structured environment.  Controlling a child’s diet is essential and most people with PWS will need their diet controlled throughout their whole life. 

There are some group homes specifically designed for people with PWS that adults can live in.  This gives them freedom and allows them to leave home but their diets can still be controlled because food access is restricted.  Exercise programmes and therapy can also help to improve muscle tone.

Also, people with PWS often have a short stature but the use of a growth hormone can help reduce this.  It can also help with weight control as the body develops more in proportion to itself and physical strength is also improved.  Underdeveloped genital organs can also respond to hormonal treatments. 

So far, no drug has successfully consistently controlled the appetite of a child or person with PWS although there have been some that have worked for short periods of time.

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Although the information we have provided here is meant to be helpful to you, Douglas Silas Solicitors cannot be held responsible for any damage or loss caused by any inaccuracy or reliance placed upon it. If you have any concerns about your child, you should seek professional educational or healthcare advice as soon as possible.