Prader-Willi Syndrome (PWS) is a complex genetic condition that affects every aspect of a person’s life. It is most commonly known for the fact that people with PWS become obsessed with food anytime from about 2yrs old onwards. Unless they are strictly managed, they will overeat (known as hyperphagia), become obese and will have a variety of associated medical problems.
However hyperphagia is not the only issue, people with PWS have weak muscles and poor co-ordination, learning difficulties, behavioural issues, OCD behaviours, such as skin picking, cognitive or intellectual impairment, emotional instability, lack of normal sexual development, lack of maturity and often mental health problems as adults.
PWS is caused as a result of a genetic abnormality. To explain very briefly, some information is missing from the paternal chromosome 15. In 99% of cases it is not an inherited disorder and it seems to occur completely randomly.
Babies are born extremely weak (low muscle tone) and are unable to feed. Most need to be tubefed for a while. They do not cry or react much to their environment… They are commonly referred to as “floppy”. They experience delayed development, most only sit at about a year and walk after the age of two. Speech is also delayed.
Early diagnosis gives the child a more positive start with early intervention and sensible eating plans. Growth hormone therapy helps. Apart from improving growth it helps improve muscle strength and allows them to get stronger and develop quicker. There are also great tried and tested eating plans that reduce the huge anxiety that PWS people have with food. As long as they know they are getting "x number" of meals in a day (no doubt) and as long as they know they are not going to get any other food (no hope) they will relax and stress less about food.
Every child is an individual and not every person affected by the syndrome will have all of the characteristics at the same time. They are also seen in varying degrees, as it is a spectrum disorder. They are extremely easy children to love as they are sweet, kind and thoughtful. They like to please and try hard to do their best in their daily activities. That is why our association refers to them as “People with Strength”. They survived such a tough start as babies and overcome so many challenges. They often surprise us with their achievements and positive attitude.