People with Prader-Willi Syndrome (PWS) can present with many behavioural issues, most of which require consistent management. Understanding the complex inter-relationship between health, wellbeing and behaviour is essential to achieving the best possible outcomes when providing support for the person with PWS.
Prader-Willi Syndrome presents a common “neurobehavioral profile“, this typically includes intellectual disability, cognitive dysfunction and a range of maladaptive behaviours. The adults can present as overly stubborn, argumentative, demanding, moody uncooperative, noncompliant, aggressive and destructive. Full-scale meltdowns and temper tantrums are also noted by parents and support staff.
During the teenage years and into adulthood, other behaviours such as plausible lying (confabulation) and exaggerating increases, as does manipulation. In some cases, the incidents of stealing and hoarding also increases. OCD behaviours are also commonly reported in adults. Autistic like behaviour is not uncommon in a person with PWS
People with PWS also have a very rigid thinking pattern. They perceive their world in black and white with no grey areas. They are very reliant on a routine and do not cope with change easily.
It is essential to get the right NDIS Plan in place, with the help of the National Disability Insurance Scheme. Learn more about preparing for the NDIS on the Prader-Willi Syndrome Australia website. Most Planners will not have heard of PWS, and you will need to educate them on the complexities and advocate heavily to get adequate supports.
PWS behaviour management is all about management of anxiety levels
A person with Prader-Willi syndrome (PWS) typically feel high levels of anxiety – all the time. Maladaptive, unwanted behaviours are often attempting to reduce the level of anxiety the individual with PWS is feeling. They also have problems with prediction and control. Both are very important in the management of anxiety.
“If we can’t control events, they are more stressful. If we can’t predict events, they are more stressful.”John Ford (2008)
Many adults with Prader Willi Syndrome have difficulty controlling their emotions in situations of sudden change, not getting what they want (food) or during a temper outburst it is usually impossible to reason with them. This aspect of their disability can cause rejection by others and social isolation.
Behavioural problems may be accompanied by psychiatric illness, which may develop for the first time in the teenage years or early adult life.
Studies show that there is an increased frequency of mental illness in PWS adults compared to the general population, and a particular susceptibility to psychotic illness in those with PWS.
It is important that families and support personal to monitor behaviour carefully and look for any changes to behaviour patterns.
Be alert for the indicators of the onset of mental illness which causes psychosocial impairments. If a person exhibits behaviours outside their usual patterns, mental health issues should be considered and referred for assessment.
- The deterioration of existing problem behaviour
- Withdrawn and tearful
- Temper outbursts
- Talking excessively
- Over activity
- Excessive irritability
- Severe mood swings
- Changes to eating habits
- Changes to self-care /grooming
- Changes to sleeping patterns
If these changes in usual behaviour are not related to physical illness and have continued for more than 1-2 weeks, medical advice should be sort and a psychiatric assessment requested as required
Mental health and behavioural problems are a significant challenge for many individuals with PWS. Such problems can have a significant impact on quality of life and independence for both the person with PWS and their family.
Psychiatric and behaviour management should be provided by medical professionals who have an understanding of the complexities of PWS. Psychoactive medication in addition to behaviour management plans may be introduced.
An excellent fact sheet outlining the risks for specific mental illnesses, presentation, course and treatment is available at the International Prader-Willi Syndrome Organisation website.
Support from service providers
“Long lasting change in behaviour is achieved not solely by restriction but through positive and consistent support”.
It is vital that any staff supporting a person disabled by PWS, in any setting, have appropriate skills. Holistic support, through collaboration between providers delivers the best outcomes. Guidance is available to staff in a variety of settings in the Service Provider Guides applicable to all States and Territories in Australia on the Prader-Willi Syndrome Australia website.
Supporters and staff must build up an understanding of the triggers for challenging behaviours. Invariably the triggers will fall into one of the following categories:
- Food Access &Dietary Management issues
- Relationship problems
- Change /Deviation from set routines or expectations
- Unresolved pain /illness
The starting point may be to change routines, systems and environments as a means of changing behaviour.
We know that having an adult son or daughter living at home can sometimes be difficult. For living arrangements to work successfully, there often has to be many compromises made by parents and other family members. The support of extended family members and friends is very valuable to the well-being of the person with PWS and his or her parents.
For more information about managing PWS in a home environment, visit the Famcare project page on the International Prader-Willi Syndrome Organisation website which includes helpful information on the following topics:
- A guide to Confabulation
- A guide to Coping with Change
- A guide for what to say when your child says “I want the same”
- A Guide to managing a Meltdown
- A guide to PWS awareness for Adults
- A guide to managing Skin picking
- A guide to setting the Boundaries
- A guide to your Duty of Care
- A guide to Exercise
- A guide to Personal care
- A guide to promoting Positive Behaviour
Note – Family Care (Famcare) is a project of IPWSO’s designed to support families whose adult son or daughter lives at home.
Understanding PWS supports in the Australian context, including brochures for service providers in Victoria
How does a person With Prader-Willi Syndrome Think”
Positive Behaviour Strategies “Tips for Working with People who have Prader-Willi Syndrome”
Prader-Willi Syndrome Australia
Video: Food security and the TRAIN model – Dr Janice Forster, Conference presentation, 2015