Who is this guide for?

This guide is for anyone helping to prepare a NDIS plan for, or interviewing a person living with Prader-Willi syndrome (PWS). It is also relevant for those implementing a plan such as a NDIS Local Area Coordinator (LAC), Support Coordinator or other intermediary.

What is Prader-Willi Syndrome?

PWS is a complex, multistage genetic disorder which affects multiple systems in the body. It significantly impacts behaviour, mental and physical health. While there is a range of abilities, all people living with PWS will require a degree of cognitive, social, learning and health support throughout their lives. A person with PWS can live a healthy, fulfilling life when they have ongoing, consistent support from people who understand the intricacies of the syndrome.

What impact might a participant living with PWS have on your role?

There is a high risk of underestimating the difficulties experienced by someone living with PWS. This can lead to inadequate service support, a decline in the participant’s health, safety and wellbeing, and a possible increased risk to public safety.

Before a meeting, the Planner must be informed about the particular characteristics of PWS. Talk privately to the person’s guardian, informal support or carer before the meeting date. Vigilance is required to avoid misunderstandings. The person with PWS may be able to speak to you clearly, with good expressive language. However, many can have a relatively poorer level of comprehension and typically:

• Lie and exaggerate – embellishing stories about themselves and their circumstances, to the point of telling very plausible lies (confabulation), putting themselves at risk.
• Usually cannot report reliably on their state of health and safety.
• Are likely to have cognitive deficits, particularly in the executive functions of the brain, meaning they are often poor at understanding abstract concepts, assessing reality, making decisions and recognising consequences.
• Are likely to be impulsive and unrealistic in understanding their own abilities and limitations.
• May have autistic–like characteristics, and can become fatigued, anxious and emotional in new situations, especially when difficult topics arise.

Most experience poor short-term memory and auditory processing difficulties, so they find it hard to take in lots of information, especially in conversation. They can think through things slowly, ask questions and understand small amounts of information at a time.

How will you provide optimum service to a person with PWS?

• Speak in short sentences; allow enough time for the person to think and respond. Look for body cues that the participant is not telling the truth such as avoiding eye contact more than previously.
• Allow more time for the meeting, as the person may want to revisit some of the discussion to understand and assimilate the information. This will alleviate their anxiety.
• People with PWS often give answers that attempt to please others. Use a variety of questioning techniques to try to get their own, real views.
• The person may have some mobility difficulties (e.g., low muscle tone or obesity), tire easily and need ready access to a toilet.
• Children should have a parent present. For adults, include a parent/informal support or guardian who knows the person well, in the planning process. This should be both with and without the participant present, allowing for a reality check, without upsetting the participant.
• Make sure that any information supplied by the person with PWS that will be used to make decisions is corroborated by another party.
• As a Planner you will be perceived as a person with significant authority and with the ability to facilitate change. It is vital that your responses are considered, and options are qualified, as unqualified options can lead to rapid health and wellbeing deterioration, with life threatening consequences. It is common for a person with PWS to build unrealistic hope based on their perception and, if their expectations are not fulfilled or challenged with appropriate discussion, can lead to disappointment, distress and potential behavioural breakdown.
• Most people living with PWS have a minimal sense of time. So, realistic planning for a day, week, month, year or the future will be difficult for them to conceive of or act upon, despite the words they say to you.
• If a change of accommodation is planned, see the guide specifically about accommodation selection.
• Provide a draft of the NDIS plan to the informal support/nominee/parent/guardian for review.

Further information

Video See examples of behaviours, food seeking and social / health consequences.

‘My Deadly Appetite: Prader-Willi Syndrome’ – US documentary My Deadly Appetite Part 1 of 3 (youtube.com)

‘PWS Awareness for Meetings with Professionals’ (IPWSO)

Meeting with Professionals – IPWSO

The International ‘Standards of Care & Best Practice Guidelines for Prader-Willi Syndrome’

Best Practice Guidelines for Residential Care – IPWSO

Confabulation in Prader-Willi Syndrome (IPWSO)

Confabulation – IPWSO

Prader-Willi Syndrome Australia www.pws.org.au