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Who is this guide for?

Guide for PlannersThis guide is for anyone helping to prepare an NDIS plan for, or interviewing a person with Prader-Willi Syndrome (PWS). It is also relevant for those implementing a plan such as an NDIS Local Area Coordinator or Support Coordinator.

What is Prader-Willi Syndrome?

PWS is a complex, multistage genetic disorder which affects multiple systems in the body. It significantly impacts on behaviour, mental and physical health. People with PWS require 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.

Learn more about PWS from the international organisation www.ipwso.org.

What impact does a participant with PWS have on your role?

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

Before a meeting, the Planner must be informed about the particular characteristics of PWS. Talk to the person’s guardian or carer privately, 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 they 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, putting themselves at risk (confabulation)
  • Cannot report reliably on their state of health
  • Have cognitive deficits, particularly in the executive functions of the brain, meaning they are poor at understanding abstract concepts, assessing reality, making decisions and recognising consequences
  • Are impulsive and unrealistic in understanding their own abilities and limitations
  • Have autistic–like characteristics, and can become fatigued, anxious and emotional in new situations, especially when difficult topics arise.

They have a 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 and 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 in an effort to understand and assimilate the information, and 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 (eg 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, guardian or someone 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 which 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 this hope is not fulfilled or challenged with appropriate discussion, can lead to disappointment, distress and potential behavioural breakdown.
  • People 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 supporter/nominee/parent/guardian/carer for review.

Tips

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

Video: Can’t Stop Eating – UK documentary
https://www.youtube.com/watch?v=dKCL2O0NfXg

Video: My Deadly Appetite: Prader-Willi Syndrome – US documentary
https://www.youtube.com/watch?v=ltCFFINalBc

Further information

PWS Awareness for Meetings with Professionals (IPWSO)
http://media.wix.com/ugd/a71d4c_ed96afec72db454bb700f769bbcba01d.pdf

The International ‘Standards of Care & Best Practice Guidelines for Prader-Willi Syndrome’
http://www.ipwso.org/#!best-practice-guidelines-for-pws-care/c1jg

Confabulation in Prader-Willi Syndrome (IPWSO)
http://media.wix.com/ugd/a71d4c_a1faf165b293412d8620e5fe87450467.docx?dn=Confabulation%20in%20PWS.docx

Prader-Willi Syndrome Association of Australia
http://www.pws.org.au

Funded by the National Disability Insurance Scheme