Information about Prader-Willi Syndrome

Prader-Willi syndrome (PWS) is a complex neurobehavioural genetic disorder resulting from an abnormality of chromosome 15.  It is a rare condition, affecting an estimated 1500 – 2000 people in the UK.  This equates to a prevalence of one in 50,000 of the population living with PWS, occurring in one out of every 15,000 births.  PWS occurs in males and females and in all races. Definitive diagnosis is now based on genetic testing.

Those living with PWS need expert support from people who understand the condition and this includes support around the cognitive, behavioural and emotional challenges associated with PWS.  Symptoms can be apparent from birth or present between the ages of two and five. Early diagnosis is vital so parents can understand and manage their child’s individualised needs and support them through childhood and into adulthood in the setting that best meets their needs.

The most commonly publicised consequence of PWS is a tendency by some to overeat.

Some individuals with PWS are prone to challenging behaviours, temper outbursts and obsessive or compulsive traits as well as other complex behaviours. A significant number of individuals with PWS also have autism, additional learning disabilities and complex needs.

Other factors that may cause difficulties include negative reactions to medications, high pain tolerance, gastro-intestinal and respiratory issues, lack of vomiting, and unstable temperature. Severe medical complications can develop rapidly in individuals with PWS.

Consensus Gretton are passionate about raising awareness of Prader-Willi Syndrome among the medical profession in particular, as an early diagnosis, an understanding of how symptoms present and associated medical intervention is vital.   We have worked collaboratively with the NHS, A&E and the Ambulance services to broaden understanding on how to support people living with PWS.

Our Medical Alerts guide below was written by medical specialists in PWS and is designed to support those living with PWS, their families and carers and medical professionals. It outlines the key medical alerts to be aware of and is a crucial companion:

  • For families and support workers supporting people daily with PWS to recognise symptoms and refer to at medical appointments
  • For medical and healthcare professionals to understand why and how symptoms are presenting and act quickly in routine or emergency situation

 

“Holland House is a fantastic place that saved my daughter’s life.  It is well organised, kind and gives residents love and stability.”

Relative of supported individual – September 2017