Prader-Willi syndrome

A genetic condition where the sense of fullness never arrives. Food security is a safeguarding matter, not a diet.

What it is

Prader-Willi syndrome is caused by the loss of function of genes on chromosome 15. It affects around 1 in 15,000 births.

Babies are floppy and feed poorly. Then, usually between two and six, something changes: an insatiable appetite develops and never resolves. The hypothalamus does not register fullness, so the person is genuinely, permanently hungry.

This is not a lack of willpower and it cannot be addressed by education about healthy eating. Without controlled access to food, people with Prader-Willi eat to the point of stomach rupture, choking and death.

Signs you might notice

Low muscle tone and poor feeding as a baby.

Then relentless food seeking: taking food, hiding it, eating from bins, eating frozen or raw food.

Learning disability, usually mild to moderate.

Short stature, small hands and feet, and characteristic facial features.

Temper outbursts, rigidity and difficulty with change, with an autism-like profile in many.

A high pain threshold and reduced vomiting reflex, both of which hide serious illness.

How it can affect day-to-day life

Food security is the whole of daily practice: locked kitchens, supervised meals, and no unsupervised access to money or shops. That sounds restrictive because it is, and it is also the least restrictive option available when the alternative is death.

Getting it wrong in a care setting is a recognised cause of preventable death.

The high pain threshold and reduced vomiting reflex mean serious illness presents late and quietly. Behaviour change should always be checked physically first.

Supporting someone well

Make food security absolute and consistent across every setting. Inconsistency causes distress, because uncertainty about food is what drives the anxiety.

Give a visual food routine so the person knows what and when, which reduces preoccupation considerably.

Never use food as a reward or a sanction.

Take any physical symptom seriously and early. Stomach pain in Prader-Willi is an emergency until proved otherwise, because gastric rupture presents with little pain and little vomiting.

Keep activity high, and support growth hormone treatment where prescribed.

Prepare for transitions carefully and warn in advance.

Fiducia Together links the dietary register to meal planning and the clinical record, so the food plan is the same on every shift, and Fiducia Life gives the person a visual my day, which is what turns an imposed rule into a predictable routine.

Where to get help

The Prader-Willi Syndrome Association UK provide specialist advice, and their guidance for care providers is essential reading before anybody supports a person with PWS.

Ask for referral to an endocrinologist and a dietitian with PWS experience.

Annual learning disability health check from 14, as for any learning disability.