Information for professional support providers and caregivers

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Information for professional support providers and caregivers

Jan 25, 2024

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Each person is an individual. Prader-Willi syndrome (PWS) is a spectrum disorder where the needs of the individual will change over time. People living with have their own unique likes and dislikes, while many experience complex behaviours and delayed learning. They need your support to live an ordinary life.

 

Best practice support guidelines

Best Practice Guidelines for the support of people living with PWS have been developed by the International Prader Willi Syndrome Organisation (IPWSO). It consulted with care providers across the world to prepare consensus. The full Guidelines can be found here.

You can read more about learning challenges for people living with PWS in our article ‘Speech and Language for Adults’. Behavioural challenges are explained in ‘Understanding and Supporting Mental Wellbeing’.

All guidelines should consider the assessment of the individual to assure health and safety to promote quality of life. Below is a summary of the key elements of the Guidelines.

Highlights from the PWS Best practice Guidelines include:

From ‘Environmental structure of living’:

Across cultural lines, all participants agreed that for an individual with PWS to succeed and live a healthy productive quality of life, a baseline level of supports conducive to promoting safety and personal growth must be in place prior to providing care. It was determined that environmental supports were non-negotiable, including but not limited to:

  • food security
  • daily schedules
  • personal growth and development plans
  • trained caregivers
  • a continuum of care throughout their day.

It was agreed that it is imperative that care providers continue to explore a cohesive collaboration of efforts through on-going education, knowledge sharing, training, and awareness.

From ‘Communicating with persons with PWS’:

Caregivers should be trained in active and diagnostic listening. This means they must discern the person’s ability to communicate and to assess their emotional state. They should be aware that there can be a large discrepancy between a PWS client’s apparent understanding and their actual grasp of the big picture, or long-term goals.

From ‘Neuropsychocognitive’:

When people with PWS have food security they can pursue other interests for a meaningful life.  People with PWS have unique neuropsychological strengths and vulnerabilities.  Strengths include good long-term memory, visual processing, shape discrimination, attention and perseverance for preferred activities.  The environment must consider that people with PWS can experience short-term memory deficits, deficits in dichotic listening, fine motor deficits, sensory perception and integration problems, deficits in simultaneous and sequential processing, poor time sense, cognitive inflexibility and rigidity, impulsivity and they have poor coping strategies.

From ‘Fitness, sports and motivation’:

The importance of exercise in the life of an individual with PWS was universally endorsed.  The difficulty in motivating individuals with PWS to begin or maintain effective exercise as a matter of routine lifestyle was also identified by all group members. All clients with PWS will require motivation to do something that is new and may require some effort. But once the activity is a routine habit and consistent, the ongoing motivating factor can be that of good health and success.

From ‘Training the Teachers’:

Families of children with PWS find it quite disheartening to see their children enter a school system that is ill-prepared to teach them.  Students with PWS can be quite challenging in an educational environment; yet those challenges can be addressed when there is a shared knowledge and understanding of PWS and how it is manifested by a particular student.

From ‘Nutrition’:

People with PWS need a special, lifelong diet. The calorie needs are based upon their body composition, BMI, and their daily physical activity. Even though most adults with PWS need a low-calorie diet administered by others, there are considerable individual variations in calorie needs.

The importance of ‘food security’ must be stressed and ‘food barriers’ may be needed. An individualized diet and ongoing education of all people involved in the care of the person with PWS is required.

‘Food security planning is vital, however, each person is an individual. Food security will look slightly different for each person. It can sometimes be relaxed for some people by focusing on what they can do, not what they can’t do. We can sometimes say “you’ve got a football game, you can have a sausage or perhaps even two at the footy, but don’t expect a full meal when you get home”. It is important to develop trust (both ways), then flexibility can be introduced.’ Care worker.

See PWSA articles ‘Healthy Eating’ and ‘Environmental support’.

From ‘Crisis Intervention’:

While certain characteristics are attributed to persons with PWS, an in-depth knowledge of the individual being supported is of equal or greater importance in identifying specific triggers to potential crisis-inducing situations.  Personality style, coping skills, temperament, self-esteem, and past experiences vary from person to person and all contribute toward a person’s tendency to experience a crisis.

A crisis impacts the life of a person with PWS in significant ways. To begin with, a crisis calls into question whether or not their world is secure, and their caregivers are trustworthy.  (Under the NDIS it may have to be documented formally as an ‘Incident’, potentially reportable to the Quality and Safeguards Commission.) If those questions are not answered affirmatively through the effective resolution of a crisis, then the increased anxiety of the person with PWS will likely lead to even more crisis situations.  Conversely, if the crisis situation is handled appropriately and effectively, then proper crisis management can strengthen the bond between the person with PWS and their caregivers, leading to a more crisis-free environment in the future.

Some individuals may need a Behaviour Support Plan prepared by an NDIS Behaviour Support Practitioner. See the PWSA article ‘Positive Behavioural Support Planning’.

From ‘Communication between Support Workers/Caregivers and Families’:

The flow of communication between a support team and the family of the individual receiving care is a crucial component to creating synergy and stability in a person’s life.

From’ Structure of Work’:

“Every person has dreams and wishes for their future.  How do we manage so their (the persons living with PWS) dreams can come true?”

…a PWS supported work environment…must include safety and security, skill development, choice, an ability to promote positive self-image, and allow them the personal experience of being a productive member within their community.

From ‘Aspects of Psychological Work’:

The psychologist/psychiatrist plays a primary role when providing direct supportive counselling or therapy to the person with PWS…a secondary role in providing emotional support and guidance to caregivers and family members.  PWSA also believes that the Behaviour Support Practitioner (sometimes qualified as a psychologist) can contribute to the monitoring, reduction and, where possible, elimination of restrictive practises, and to the training of support workers. The NDIS Support Coordinator plays a key role in facilitating communication among all stakeholders, including the psychologist and the person with PWS.

From ‘Interpersonal Relationships’:

Among persons with PWS, relationships of any sort are constrained by the “genetically and neurologically” determined incapacity for reciprocal relationships. The capacity for emotional understanding is further limited by a lack of opportunity and experience.  So, relationships on all levels need to be externally supported to teach new skills and reduce stress.

From ‘Behaviour Management’:

Every person who is involved with the care, management and support of individuals will eventually experience a crisis situation.  And each crisis experience offers an opportunity to learn how to better manage a crisis in the future.

  • First, caregivers must understand the crisis, the context and the trigger.
  • Second, caregivers must know what to do when a crisis occurs.
  • Third, after the crisis is resolved caregivers should take time to reflect with the client involved.
  • Fourth, caregivers should use information and insight gathered in the three previous steps to create improved support and prevention strategies.

The behaviour support plan should reflect the preferences, opinions and emotions of the individual. The individual should help create the plan, accept the final version of the plan, and perhaps sign the plan to demonstrate their agreement. The plan should be tailored to each individual’s mental level, and have clear rules and limits for appropriate behaviours, while offering structure in the form of limited choices. While focusing on prevention of behaviour crises, the appropriate consequences of maladaptive behaviours should be explained. It is important to consider that not all individuals with PWS will be able to comprehend the consequences of their behaviours.

Behaviour Support Planning in Australia

In Australia, it is extremely important that a behaviour support professional is engaged, preferably with PWS experience, to write a behaviour support plan (BSP). If the person is living in supported accommodation, they will require a behaviour support plan. If there are restrictive practices in place, and the person is a National Disability Insurance Scheme (NDIS) Participant, the BSP will also need to be registered with the NDIS Quality and Safety Commission. Training in PWS before the initial staff shift if vital. See the PWSA article on ‘Behavioural Support Planning’.

Professional Providers and Caregivers Conference

PWSA itself does not run any residential homes. But PWSA does sometimes hold a Professional Providers and Caregivers Conference. The International Prader Willi Syndrome Organisation (IPWSO) also holds regular PPCB conferences.

Find out about conferences at www.IPWSO.org or by emailing Social@pws.org.au

Professional support organisations and workers can receive notice of future events by becoming a member of PWSA.

Other resources

International Prader Willi Syndrome Organisation Best Practice Guidelines for Residential Care https://ipwso.org/wp-content/uploads/2020/10/PWS-Best_Practice_Guidelines.pdf

International Prader Willi Syndrome Organisation Caregiver ECHO video resources:

  1. IPWSO Caregivers ECHO video: Norbert Hödebeck-Stuntebeck ‘Basic philosophy of taking care of people with PWS’ https://www.youtube.com/watch?v=XvYpi5nacm0&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=1
  2. IPWSO Caregivers’ ECHO video: Larry Genstil: ‘Managing Behavioural Issues of the Child with PWS in School’ https://www.youtube.com/watch?v=3ej0GblJdIg&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=2
  3. IPWSO Caregivers’ ECHO video: Damien Jones: ‘Prader-Willi Syndrome (PWS) and Restrictive Practice’ https://www.youtube.com/watch?v=qZGqGXnCjyI&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=3
  4. IPWSO Caregivers’ ECHO video: Neil Gumley: ‘Transition for People with Prader-Willi Syndrome’ https://www.youtube.com/watch?v=bYoHU0gmsl8&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=4
  5. IPWSO Caregivers’ ECHO video: Norbert Hödebeck-Stuntebeck: ‘Young & clever: well educated people with PWS’. What are the consequences? https://www.youtube.com/watch?v=ES1HHRg3_Q8&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=5
  6. IPWSO Caregivers’ ECHO: Dr Constanze Lämmer: ‘utrition in PWS’ https://www.youtube.com/watch?v=-BtFDfPLonw&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=6
  7. IPWSO Caregivers’ ECHO video: Georgina Loughnan: ‘The Power of Exercise for People with PWS’ https://www.youtube.com/watch?v=W443iyp-7kM&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=7
  8. IPWSO Caregivers’ ECHO video: Larry Genstil: ‘Meaningful Employment for People with Prader-Willi Syndrome’ https://www.youtube.com/watch?v=5X9j98S1liY&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=8
  9. IPWSO Caregivers’ ECHO video: Lynn Garrick & Claire Poor-Harmon ‘How to Attract and Retain PWS Caregivers’ https://www.youtube.com/watch?v=t1DJOp-wwGs&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=9
  10. IPWSO Caregivers’ ECHO: Susanne Blichfeldt: ‘Managing medical checks and other requirements in PWS’ https://www.youtube.com/watch?v=2bomFl_JRRI&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=10
  11. IPWSO Caregivers’ ECHO: Brittni Kliment, Patrice Carroll: ‘Education, Behavior Appreciation…’ https://www.youtube.com/watch?v=vwroNNmhDlw&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=11
  12. IPWSO Caregivers ECHO: Norbert Hödebeck-Stuntebeck ‘The role of Diagnostic in supporting people w PWS’ https://www.youtube.com/watch?v=qgE8-69_bck&list=PL0hzNGbX1PrzoK7oMUkxY10Qp3cJwlvdW&index=12

Thank you ….

To the Director and representatives of IPWSO Professional Providers and Caregivers Board.

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