This guide is for providers of Supported Independent Living (SIL) services (managers, supervisors and direct care staff) who can assist people with Prader-Willi syndrome in domestic settings. This includes SIL workers in specialist disability accommodation (SDA), short-term (respite) accommodation or other residential settings.

What is Prader-Willi Syndrome?

Prader-Willi Syndrome (PWS) is a complex multistage genetic disorder affecting multiple systems in the body. It significantly impacts on behaviour, mental and physical health. People with PWS require cognitive, social and learning 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 PWS. Learn more about PWS from the international organization www.ipwso.org.

What impact does a person with Prader-Willi Syndrome have on your role as a SIL worker?

Preparing for the complexity of PWS

• A suitable option for residential care and support will vary according to the needs of each person living with PWS. But regardless of the type of setting that is selected, the person with PWS will require substantive, usually 24-hour 7 days a week supervision and support for the rest of their lives. Independent living is not known to have been achieved successfully.
• Staff who support the person with PWS have to be highly organized, creative, intuitive, consistent, persistent, agile thinkers, and be positive. This will reduce anxiety in the participant. It will also help the participant to engage in life, to be more motivated and to develop behaviours that are socially appropriate and less dangerous to themselves and those who support them.
• To maintain a highly skilled staff, managers must provide support for and ongoing professional development. High turnover of staff is a major problem when trying to provide a consistent routine and house rules/values to support to people who live with PWS.
• It is often difficult to provide the preferred configuration of living for a PWS specific environment in existing housing stock . A purpose-built home or modification or alterations are needed to ensure a PWS safe environment
• SDA houses/units or townhouse developments in either PWS specific or mixed ability accommodation should incorporate robust features into the plans, required for a PWS safe environment.
• The appropriate level of support for people living with PWS will differ from the concepts of what constitutes appropriate levels for any other resident with equivalent intellectual or behaviour disorders. Mixed ability settings can be challenging for people living with PWS as the needs of all the residents have to be considered.
• People with PWS need to live with Restrictive Practices particularly in regards to food accessibility which will impact on other residents. The complex needs of people with PWS will need to be reflected in the staffing ratios for the residential setting. NDIS Plans need to hold an appropriate SIL budget for this.
• It is important that the guidance here is followed, otherwise the person living with PWS is likely to feel extreme anxiety and behaviours of concern will quickly emerge. Poor preparation leads to a situation where the resident and the staff have been set up to fail.

Transition to alternative accommodation

• People with PWS will need support to be able to make appropriate life changing decisions; The risk of harm is too high on their own. The parents/Nominee/guardian or someone who knows the person well should be involved in all levels of the planning and implementation of the transition to out-of-home care or to another supported residence.
• In PWS, change of routine, expectations and relationships may trigger periods of acute anxiety and resultant behaviour changes. This transition must be carefully planned and implemented with clear channels of communication between all participants.

How will you provide optimum services to a person with PWS in the residential setting?

The supports conducive to promoting safety and personal growth should be in place prior to the person moving in. These will involve a variety of practical and human resource activities listed below:

• PWS specific training: All staff, including casual staff should participate in generic PWS because of the commonality of characteristics. Training in the areas of dietary and behaviour management/support, effective communication strategies and health and wellbeing are relevant. Then the staff must learn about the needs of the individual in addition to the baseline requirements. Training must be ongoing and made available to all new staff, prior to their commencement of employment in the house. The use of casual staff should be minimised, and if used get the PWS training.
• Audit of the SDA or other Residential Setting: The residential setting must be as PWS friendly as possible. This may require some alterations to the physical environment to ensure it is robust and makes restrictive practices, where required, as inconspicuous as possible.

An appropriate environment includes the audit items in Appendix 1.

• Information Gathering: Gather detailed Information to identify how the agreed supports will be put in place by the residential support team. This includes supports for daily living, in coordination with supports for capacity building and community access. Various allied health providers will give plans for the resident, which need to be implemented by the SIL workers. Data gathering for reporting to the NDIA and allied health providers will be ongoing.

Schedule meetings with the parents/carers or someone who knows the adult well. This should be both with and without the participant present. Make sure that information supplied by the person living with PWS upon which decisions are being made is corroborated by another party.

It is important to establish links with the family/informal supports/Nominee/guardian for ongoing information sharing. This will help to ensure consistent supports and an ongoing positive supportive relationship.

• Establish links with the participant’s disability support workers. These may include the NDIS Support Coordinator, day program provider, supported /mainstream workplace, community access support providers, sporting groups or other social group.
  • Initiate or participate in communication networks between the different disability support workers
  • Identify any transport and /or timetable difficulties. Travel training program may need to be initiated or alternative transport may need to be organized.
  • This linking process will extend to key medical professionals involved in the care of the person with PWS especially the endocrinologist, dietitian, other lifestyle professionals and GP.
• Familiarization with their new neighbourhood: Organize times for the person with PWS to visit their new home prior to moving in, to meet other residents and their key worker. Help them to become familiar with the layout of their new home and neighbouring surrounds.
• Inform neighbours and nearby shop staff of the strict dietary needs of the person with PWS to discourage them from providing extra food/beverages if the person with PWS visits.
• Ensure that the staff have the capabilities to effectively support the participant living with PWS:
• A shared understanding of best practice management strategies, often contained in a Behaviour Support Plan
  • Communicating and implementing the Code of Conduct for the residential setting
  • Listening, mood monitoring and delivery of pre-emptive responses skills

See Appendix 1 for capabilities to be in place prior to the participant moving into the residence.

Tips

• The person living with PWS may feel reluctant about, or even strongly resistant to moving into a residential setting like Specialist Disability Accommodation (SDA), thinking they will get less food access. Therefore, it is better to emphasise that it’s a home where residents have healthy options and choices.
• Include the person living with PWS in discussions where possible, but be aware that they take time to absorb information. They find it hard to shift their thinking to a new idea. At times they will feel over-whelmed with information and may be better off getting the concise version of your information, and only when there is concrete information available and choices to be made. Otherwise, anxiety may rise.
• Food security and barriers is not just about calories. Consistent support is also about forming habits and longer term, constructive expectations. Setting good habits will help to minimise challenging behaviours. A reduction in Incidents will be due to good staff practices. The person with PWS continues to remain susceptible to challenging behaviours if the scaffold of appropriate support reduces or is removed with a false assumption that the participant is ‘cured’.

Further information

‘How does a person living with Prader-Willi Syndrome Think’ https://www.pwsausa.org/wp-content/uploads/2015/12/How-a-Person-With-PWS-Thinks-GA-543.pdf

Confabulating behaviour and how to respond: Lying-and-Confabulating.pdf (pwcf.org)

Positive Behaviour Strategies:

• Managing behaviour – USA https://www.pwsausa.org/managing-behavior/
• Behaviour management strategies toolkit – USA https://www.pwsausa.org/wp-content/uploads/2016/02/Behavior-Tool-Kit-from-Prader-Willi-California-Foundation.pdf

Prader-Willi Syndrome Association UK – Residential care: Support for those living with Prader-Willi Syndrome – for providers

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

‘Supporting Adults with Prader-Willi Syndrome in Residential settings’ B. Goff. Order via Prader-Willi Syndrome USA   Goff Book Flyer.pub (pwcf.org)

‘Prader-Willi Syndrome Residential Staff Training’ – Purchase from Prader-Willi Syndrome California Foundation Training – Prader-Willi California Foundation (pwcf.org)

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

Appendix 1 -Accommodation Audit

This PWS safety audit should include both the physical environment and the staff capabilities:

Physical

Staff capabilities

A shared understanding of the complexities of PWS particularly:

• People living with PWS tend to talk a lot and will often repeat themselves. While it is important to readily engage with the person as they really appreciate this social activity it is also important to have strategies to end discussions in a sensitive and respectful manner.
• People living with PWS can be very manipulative and the staff should be aware of the ploys they use. The response to allegations needs to be considered only after hearing both sides of the story.
• People living with PWS tend to overestimate their abilities. They may confabulate (lie) about their skills and planning abilities and may inadvertently place themselves at risk. Staff need to be very wary about allowing the participant into an environment where they cannot manage the risk, despite verbal assurances that they can.

• A shared understanding of best management strategies:
• Consistent dietary, exercise and behaviour management/support is key to successful outcomes
• Use team meetings to discuss / establish a consensus on how the staff will support and manage the following:
  • Food security and/or barriers during the preparation, serving, and storage of food,
  • Exercise and recreation activity schedules within the home,
  • Access to money. Staff should be informed about the reasons why food/money must not be accessible
• Ensure that the importance of good communication skills is understood by all the staff
• Have a clear behaviour management action plan when things go wrong /behavioural outbursts
• Avoid getting into arguments. Limit excessive questioning
• Be aware of confabulation (lying)
• Provision and use of a time out space in a private area
• SIL staff need to be reliable: Don’t promise what will not be delivered or the person living with PWS will lose trust and behaviours may increase as home won’t feel safe.
• Code of Conduct and/or house values (rules) are in place; The Service Agreement must be detailed, and not depend solely on item numbers to explain the Provider’s offering. This will enable expectations of the Service Agreement are clearly and concisely communicated to all stakeholders. For example:
• Allocate a key worker who the person with PWS can go to if there are any problems. The role of the key worker must be clearly explained to the person living with PWS
• Establish a protocol of communication between the key support worker and the house manager. Establish a process for resolving issues in a timely fashion.
• The key support worker role will include:
  • Revisiting the house expectations and code of conduct/values/rules when needed and confirm that the person living with PWS does in fact understand what it means for them.
  • Making sure the person living with PWS knows how to use the visual display boards to get information about daily routines, activity schedules, medical appointments and staff changes
  • Introducing the dietary support and exercise routines
  • Taking the time to help the person living with PWS to get to know the new neighbourhood facilities they will be using such as the swimming pool, gym, and library.
  • Providing extra support and prompts if needed until the new routines and schedules are bedded down (which may not be fully realised due to executive brain dysfunction and problems with planning, organising, follow through initiation and volition).
  • A description of the responsibilities.

• Active listening, pre-emptive responses and mood monitoring skills:
• Monitor mood and behaviour carefully and pre-empt any triggers in the environment that cause increased anxiety. Listen to concerns, likes and dislikes respectfully and tweak the routines and structures accordingly.
• Positive plans will be used with verbal and sometimes tangible rewards help to foster participation, engagement and task completion. Support and guidance will be needed to assist the person living with PWS achieve personal growth and development in their daily living skills, which will promote increased independence and an improved self- image.
• Provides opportunities for the person living with PWS to establish and maintain relationships with their peers and family members. This can be strengthened through positive social skills training and facilitation, as well as verbal support and guidance.
• Celebrates group achievements through positive peer praise and recognition.
• Fosters a homely environment that is respectful, harmonious, safe, caring and fun.