Why is maintaining diet for children with Prader-Willi syndrome important?

One of the main symptoms of Prader-Willi syndrome is excessive hunger, known as hyperphagia. Children with Prader-Willi tend to have a rapid increase in their interest with food, especially at around 3 - 8 years old. If excessive eating is not strictly monitored, it can cause significant weight gain and obesity. Obesity has numerous long term health implications including cardiovascular disease, type 2 diabetes mellitus and sleep apnea.

Maintaining a well-balanced calorie controlled diet for children with Prader-Willi is critical. The important thing to know about hyperphagia is that people with Prader-Willi have no control over their feeling of hunger. Support and help from friends and family can be vital in helping the person with Prader-Willi maintain their health and enjoy a full and rewarding social life.

Why do people with Prader-Willi have hyperphagia?

Hyperphagia in Prader-Willi is still not fully understood, but one cause implicated is an abnormal imbalance of the hormones oxytocin and ghrelin.

Ghrelin is known as the 'hunger hormone'. When your stomach is empty, it triggers the secretion of ghrelin which sends signals to the brain to feel hunger. This hormone tends to increase appetite rapidly right before eating, and then gradually decreases during and after consumption. People with Prader-Willi have been shown to have high ghrelin levels, which may be a contributor to hyperphagia. In addition to this, studies have shown that people with Prader-Willi can have low levels of oxytocin in the body. Oxytocin is widely known as the 'happy hormone' and is linked with appetite suppression. The combination of these factors may contribute to hyperphagia.

But it's just one snack - how can that hurt?

People with Prader-Willi generally have a lower caloric need, sometimes by 20 - 40% less calories than someone without Prader-Willi. There are a number of reasons for this including lower lean muscle mass and differences in physical activity of people with Prader-Willi.

Healthcare professionals are generally involved in understanding an individuals' caloric need and recommending daily nutritional intake. This means that even occasional small snacks or treats can have a significant impact.

Still wanting to give a treat? There's lots of alternatives to food - think about books, small toys, or a chance to spend time doing something the person enjoys.

How can I prepare if someone with Prader-Willi is visiting my house?

Understand that the parent, family or carer live with Prader-Willi every day. Their requests, suggestions and advice around food is based on sound medical advice.
Research and understand Prader-Willi syndrome and the symptoms. We recommend starting at praderwilli.org.au , fpwr.org and pws.org.au
Make sure food or snacks are put away out of view (e.g. in cupboards). This can make it easier for the person with Prader-Willi to avoid temptation or being distracted.
Understand that Prader-Willi syndrome evolves as the child grows older, and this can also be affected due to medications administered. Their needs may change over time, so asking for an update if you haven't seen the child for awhile is the best way to make sure you've got everything covered.