Special Needs of People with Prader-Willi Syndrome

Some points for Care Quality Commissioner Inspectors

Introduction
People with Prader-Willi Syndrome (PWS) have a set of special needs which do not fit readily into the usual requirements for people with learning and/or physical disabilities. These needs constitute a duty of care and reflect the person's best interest. Recommendations to accommodate these needs have been formulated across the PWS community worldwide, and operate regardless of usual social norms and practices in any country. These include:

  • Restricted access to food - for some this may mean locked kitchens and food cupboards and supervision when out and about
  • (Usually) A lower daily calorie intake than other people
  • Emphasis on healthy foods - very few treats and snacks
  • Structured meals and eating times
  • Restricted locations as to where eating is allowed
  • (Often) Restricted access to money

In addition, many people with PWS appear more able than they actually are. Some may test outside the learning disability intellectual spectrum but, because of immature emotional and social skills are unable to function in everyday life at their intellectual level. There are similarities with people with autism spectrum disorders.

Characteristics of PWS
PWS is a rare and complex genetic syndrome which affects people throughout their life. There are probably no more than 2000 people with the syndrome throughout the UK. It is very rare for an adult with PWS to live totally independently; most still live with parents or relatives, or are in residential care or supported living. The range and severity of symptoms differs between individuals, but all will have the following characteristics in some degree:

  • Hyperphagia (overeating) - still not fully understood, but appears to stem from a dysfunction in the hypothalamus which means that the person does not know when they have had enough to eat - hence they feel hungry most of the time and act as someone who is starving.
  • Hypotonia (low muscle tone) - this means the person is less active and needs fewer calories to maintain a reasonable weight.
  • Short stature (if the person has not received growth hormone treatment)
  • Obesity - the combination of hyperphagia and hypotonia (exacerbated by short stature) means that the person with PWS will become morbidly obese if external controls are not in place to prevent them accessing food. The PWSA (UK) is aware of cases of a 6 year old weighing 10 stone and a man in his mid-20s weighing 37 stone. Early death as a result of morbid obesity is not uncommon. However, with restricted access to food, the person can live well into middle age and beyond. The oldest known person with PWS died at age 74 - she had lived in an institution for the greater part of her life.
  • Intellectual abilities - range from 50-100. The majority are in the 60-65 range.
  • Immature or delayed social and emotional skills and challenging behaviour. Most people with PWS operate at the social and emotional level of a young child, although they can appear very able on first meeting.
  • Various cognitive deficits, eg:
       - problems with attention-switching, resulting in oppositional behaviour and resistance to change;
       - problems with auditory processing - find it difficult to carry out instructions given verbally
       - perseverative speech

Mental Capacity
Although a person with PWS may have the capacity to make decisions about many things, their capacity around food, money and health can questioned, and a two part argument for lack of capacity can be put forward:

Q: Does the person with PWS have impairment of brain or mind?
A: Yes, even if they do not have an intellectual learning disability. Brain scans done under research conditions show definite impairment in the brain of people with PWS - ie messages from the stomach to the brain that they have had enough to eat are not getting through. A diagnosis of PWS automatically allows this argument to be put forward.

Q: Can the person make a reasoned decision about whether or not to eat?
A: No, the drive to eat overwhelms the rational decision-making process. Also the more "chaotic" the environment is, the more capacity to make decisions is decreased in PWS (ie they find it difficult to switch attention from one option or stimulus to another).

For more information about Mental Capacity and PWS see here.

Money
Many people with PWS are very good with money and enjoy shopping. However, access to money without supervision or restrictions may not work in their best interests. Money is likely to be spent on food, hence increasing weight, as well as not being used to purchase other necessities or pay bills.

Further information
For training and further information about PWS, contact:

PWSA (UK)
125a London Road
Derby
DE1 2QQ
Tel 01332 365676
Fax 01332 360401
email: admin@pwsa.co.uk