Can't Stop Eating

First shown on Channel 4 in July 2007

Joe and Tamara are struggle with a never-ending desire to eat. They are amongst the 2,000 people in this country diagnosed with Prader-Willi Syndrome (PWS). There is as yet no cure for PWS and if their diet is not strictly controlled they are in serious danger of eating themselves to death.

Prader-Willi Syndrome

by Jackie Waters

Prader-Willi Syndrome | help and info

Imagine how it feels to be hungry all the time – no matter what or how much you eat. This is one of the main characteristics of Prader-Willi Syndrome (PWS).

© channel 4

Named after the Swiss doctors, Prader and Willi, who first described it in 1956, PWS is caused by a genetic abnormality that is present from birth, and affects the person throughout their whole life. In 95 per cent of cases it is not hereditary – hence it can happen in any family. Fortunately, it is quite rare, affecting about 2,000 people in the UK.

symptoms

It has a number of characteristic symptoms. These are:

• Floppiness at birth, with low muscle tone persisting throughout life



• Difficulty feeding in the first few weeks and months of life



• From around the age two onwards, the onset of a lifelong excessive appetite



• Learning disabilities in a majority of people with PWS



• Immature development of emotional and social skills



• Immature sexual development (only three women worldwide have had a child, and there are no records of a man fathering a child)



• Short stature (compared with other family members)

how does the excessive appetite affect the person?

Although a person with PWS may appear very overweight, they act in much the same way as a starving person would, because the message is not reaching their brain that they have had enough to eat. Hence they seek out food at every opportunity, and many will go to the lengths of stealing food or eating items which most of us would not consider either healthy or palatable such as, for example, frozen food and pet food.

The only treatment to maintain or reduce weight is sticking to a strict lower calorie diet and 24 hour supervision. The latter may often involve locking fridges, kitchens and other food areas, and accompanying the person whenever they go out – even though they may be perfectly capable of independent travel.

living with PWS

The combination of low muscle tone and excessive appetite means that a person with PWS needs fewer calories than the average person in order to maintain their weight at a reasonable level. The struggle to keep weight down is a real battle, and the vast majority of people with PWS are unable to do this without outside support from parents or carers who can manage their diet for them. For adults with PWS, this is sometimes very difficult to accept; others are happy that the temptation of food is taken away for them.

Many children with PWS now attend mainstream school, but sometimes placements break down because of the child's food-seeking and challenging behaviour. On the other hand, good management and understanding can provide a positive and structured environment for the child which means they can then reach their full potential educationally. Several people with PWS have obtained one or more GCSEs.

It would be easy to make the mistake that because some people with PWS appear superficially competent, they are able to make it alone in the outside world. Sadly, this very rarely happens. Left to their own devices, people with PWS can very rapidly become massively obese. Weight gains of a stone a week are not unusual. Inevitably this can lead to early death because of illnesses related to massive obesity, such as heart disease, diabetes and breathing problems. Sadly, obesity-related death has occurred on rare occasions in children as young as four years old, and death in their teens and twenties is very common in those whose diet has not been controlled. However, the oldest living person with PWS has been in care for most of her life and is 72 years old. So, having PWS does not automatically mean an early death.

Equally, neither does it mean a life full of negatives. As children they are very loving and make friends wherever they go – a quality which many carry through into their adult life. Some adults with the syndrome are able to manage some aspects of their diet successfully and are able to make valuable contributions to their local community through voluntary and paid work.

PWS is a difficult condition to manage within the usual learning disability settings, and thus specialist PWS residential homes or supported living situations are becoming increasingly common throughout the country. In these, weight is well managed and the person with PWS enjoys a full life of social, work and leisure opportunities.

is there a cure?

There is currently no cure for PWS itself, although some aspects of it can be treated or managed. Growth hormone treatment was approved by the National Institute for Clinical Excellence (NICE) a few years ago and is now used routinely in children with PWS to improve their height and muscle tone. Researchers are now looking into the benefits of this for adults. Sex hormone treatment is also sometimes used to improve sexual development.

So far, no cure for the excessive appetite has been found – conventional appetite suppressants do not work. This is partly due to the fact that the mechanism which is causing this dysfunction in the brain and/or digestive system has yet to be identified, although researchers are working hard to do so. Without knowing the cause, it is difficult to find the cure.

what to do if you think your child has Prader-Willi Syndrome

A good place to start with if you think your child has PWS, is to check out the Diagnostic Criteria on the website of the Prader-Willi Syndrome Association (USA). If your child scores highly on this checklist take it, along with information about PWS, to your GP and ask for referral to a geneticist or paediatrician. A genetic test, which involves taking blood samples from the child (and sometimes parents), will confirm PWS in 99 per cent of cases.

If your child or someone you work with has a diagnosis of PWS

As PWS is quite rare, there are not that many sources of information. The Prader-Willi Association (UK) has over 750 parent members and almost an equal number of professional members. It can offer parent to parent contact and provides links to specialist residential care providers. Full contact details for the Association are in help and information.

(July 2006)

Read on for details of relevant organisations, websites and reading.