Committee Secretariat Joint Standing Committee for the National Disability Insurance Scheme Committee Office | Department of the Senate Parliament House, Canberra https://www.aph.gov.au/Parliamentary_Business/Committees/Joint/National_Disability_Insurance_ Scheme/GeneralIssues

5th September 2021

Dear Sir or Madam,

Re: Prader-Willi Syndrome Association of Victoria’s submission to NDIA’s Consultation on Policy for Home and Living- An ordinary life at home including the “PWSA Position Paper: Enabling personal choice and control in an environment of least restrictive practices in the support of people living with Prader-Willi syndrome (PWS”) which has been included as an appendix.

Please find attached a copy of a submission which we have submitted to the NDIA’s Consultation on Policy for Home and Living- An ordinary life at home.

The reason we are submitting a copy to your committee is because we want to alert you to our grave concerns about some of the themes in the Paper. The policy direction being taken by the NDIA has many gaps, and in its current form will result in welfare risks for people who have Prader-Willi Syndrome.

We know Prader-Willi Syndrome presents the NDIS with a real conundrum - It is a complex disability to manage and do so successfully requires substantive support for the person throughout their life. There are no short cuts to establishing best practice PWS management.

With PWS there is not the luxury of trial and error when it comes to home or living choices. Nor is there a lot of leeway when it comes to what constitutes a PWS friendly environment, if the person with PWS is to have the opportunity to live a healthy and safe life of any longevity. This concern is backed up by data that shows that the average life expectancy of someone who has PWS in Australia, is still only 37.8 years, with most deaths caused by the comorbidities of obesity.

PWS research worldwide tells us that the best practice PWS management involves consistent food security/access and daily exercise in a supportive PWS safe environment. People with PWS find it difficult to sustain life in the absence of significant and ongoing specialized support practices. We acknowledge that the specifics of that ongoing specialized support will vary from individual to individual.

However, we have serious concerns as to how the individual support needs and requirements of the more complex disabilities, such as PWS, will be accommodated in this model put forward in the Home and Living Policy.

We would like the committee to consider our comments in the submission in conjunction with the information presented in the PWSA position paper on Choice and Control which is included as Appendix.

Prader-Willi Syndrome Association of Victoria (PWSA Vic) generally supports the NDIA goal to strengthen the NDIS to make it simpler, more consistent, flexible, and conducive to NDIS participants having more choice and control over where they live and who they live with.

However, the welfare of the cognitively impaired participant with PWS must be paramount, as the likelihood of them making decisions that are not in their own best interests is high. A guaranteed quick and sturdy safety net must be in place. The concept of dignity of risk must have defined caveats when applied to PWS participants.

As it stands we cannot endorse the NDIS “Home and Living Policy “Consultation Paper, but we hope the recommendations we make will increase the understandings about the particular support and accommodation needs of people with Prader-Willi Syndrome.

We look forward to your comment and feedback. Our common goal is, I believe, to protect and promote the wellbeing of people living with PWS, so they can live their best life, in a safe PWS environment, using the best, least restrictive methods possible.

If you would like more information about PWS, there is an important resource that the NDIA commissioned through DANA, who selected several disabilities and invited the various support groups to participate in the project. Together with DANA we produced a video and an information summary of PWS which was to be made available to all NDIS Planners and LACs on the NDIA intranet with the intended view to releasing this resource more widely. I would be interested to know if your team was able to access this resource.

I approve that this submission be made public.

Regards

Kate de Josselin -PWSA Vic Training Coordinator

On behalf of the PWSA Vic Board

17th August 2021

Consultation Manager National Disability Insurance Agency Geelong, Victoria, 3220

Dear Sir/Madam,

Re: Consultation on Policy for Home and Living- An ordinary life at home

We represent people across Victoria who have Prader-Willi Syndrome (PWS), their families and their support networks. As such, this response to the NDIA consultation paper highlights the needs of people with this very complex disability.

Prader-Willi Syndrome (PWS) is a rare, complex multistage, genetic disorder affecting multiple systems in the body. It significantly impacts behaviour, mental and physical health. People with PWS require cognitive, social and learning support throughout their lives.1

PWS is a neuro-behavioural disorder that includes altered intellectual functioning and centrally driven maladaptive behaviors. People with PWS are highly stress sensitive, have difficulty managing anxiety levels and, at times, exhibit challenging behaviours throughout their lives2. i They are also at a much higher risk of developing psychiatric illnesses than the average population.

Whilst they have variable developmental delay, they all have significant cognitive and functional impairments typically including deficits in executive brain function. This can include lack of ability to assess, judge and foresee adverse consequences of poor, life changing decisions. They can also lack insight about their level of capability.

Due to impaired hypothalamic and endocrine regulatory systems, people with PWS lack satiety, therefore always feel hungry and readily gain weight. There is often abnormal food seeking behaviour and they require a strictly controlled diet.3 Weight gain can be very rapid, leading to obesity, health complications and early death.4 Obesity and its complications are major causes of morbidity and mortality for people

1 International Prader-Willi syndrome organization www.ipwso.org 2 M.A. Angulo, M. G. Butler, and M. E. Cataletto. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings, ‘Journal of Endocrinological Investigation’. 2015; 38: 1249–1263. Published online 2015 Jun 11. doi: 10.1007/s40618-015-0312-9 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4630255/ 3 PWS Medical Alert Booklet (Australian Edition) https://pws.org.au/medalert/ 4 Allen, K., “Managing Prader-Willi Syndrome in families: an embodied exploration”, Social Science & Medicine, 72, Issue 4, 2011, pp. 460-468. https://doi.org/10.1016/j.socsimed.2010.11.032.

Prader-Willi Syndrome Association of Victoria Inc.

with PWS. Adults with PWS do not have the capacity to monitor or manage their own health and wellbeing. This necessitates careful monitoring and intervention by those who support them, particularly around diet and activity, which requires strict food security4. A recent study from the United States showed those with PWS have a significantly shorter life expectancy than those without PWS.5 The main causes of death in those with PWS are related to issues with food security.

PWS is a spectrum disorder. That is, people may be more or less affected by its various characteristics. Nevertheless, even the highest functioning individuals typically have severe to extreme functional impairment of critical dimensions of their lives (e.g. food security) and need a very significant level of support to avoid an untimely death. They have impairments across all six activity domains:

self-care
mobility
learning
communication
social interaction
self-management

Due to the complexity of PWS, there is a need for substantive, consistent support, in an environment that is PWS safe, throughout the person’s life. With PWS there is not the luxury of trial and error when it comes to home or living choices. Nor is there a lot of leeway when it comes to what constitutes a PWS friendly environment, if the person with PWS is to have the opportunity to live a healthy and safe life of any longevity.

Prader-Willi Syndrome Association of Victoria (PWSA Vic) generally supports the NDIA goal to strengthen the NDIS to make it simpler, more consistent, flexible and conducive to NDIS participants having more choice and control over where they live and who they live with. However, irrespective of the details, process and increased ‘independence’, the policy must assure that when considering options, harm to the participant will not be probable. A guaranteed quick and sturdy safety net must be in place. The welfare of the cognitively impaired participant with PWS must be paramount, as the likelihood of them making decisions that are not in their own best interests is high. The concept of dignity of risk must have defined caveats when applied to PWS participants.

In this submission we would like to voice PWSA Vic’s serious concerns about several of the premises underpinning the Home and Living consultation paper.

5 Butler, M., Manzardo, A., Heinemann, J. et al. Causes of death in Prader-Willi syndrome: Prader-Willi Syndrome Association (USA) 40-year mortality survey. Genet Med 19, 635–642 (2017). https://doi.org/10.1038/gim.2016.178 Prader-Willi Syndrome Association of Victoria Inc.

We also take to task the submission questions that accompanied the consultation paper. Given that the Home and Living policy is still in the draft stage, we believe that it is premature to talk about website content, resources, information gathering and networking for different support options before the policy is drafted. We cannot help but get the sense that this consultation is not sincere as the questions posed presuppose the adoption of the Home and Living approach even before the consultation process has concluded.

The consultation document does note that the “solutions available to live an ordinary life are not one size fits all” and that “they are tailored to your situation and circumstances”. However, we have serious concerns as to how the individual support needs and requirements of the more complex disabilities, such as PWS, will be accommodated in this model.

PWSA Vic requests that our concerns with the intended Home and Living Policy as outlined in the consultation paper be noted and addressed.

Changing the conversation and supporting you to be an informed consumer

We agree that many of the goals, ideas and imagery cited in the Home and Living consultation paper are broad and are necessary for improving the housing and living options for participants with PWS. However, they are only a part of the answer to the complex task of finding suitable housing and support for someone who has PWS. This proposed Home and Living consultation paper presents a simplistic roadmap to a very complex issue for our cohort of participants.

The Home and Living consultation paper is built on the premise that, at present, participants and their support networks do not seek out information about the best Home and Living options for a person with PWS. This is an erroneous assumption. In the case of the PWS community, the conundrum of Home and Living options dominates discussions both formally and informally once the person with PWS reaches adulthood.
It is not the lack of consumer information or conversation that is the problem. It is the small stock of robust build, PWS suitable housing (e.g., premises with the capacity to provide and maintain food security) that is available in Victoria that is the main problem.

The PWSA Vic is concerned about the NDIA intention to “Introduce automatic business system alerts for early identification of participants who may need a new home and living solution in the future.” Alerts could easily become intrusive. There needs to be consultation with the disability sector about how and when such alerts would be applied. There needs to be agreed criteria and a clear process for ensuring that

Prader-Willi Syndrome Association of Victoria Inc.

people who live in SDA and/or with SIL services do not have their welfare jeopardised or are left homeless.

Expanding support for decision making

The Home and Living consultation paper also appears to be built on the premise that NDIS participants are not included in a supported decision-making process at present.

We challenge this assumption - Due to the excellent work done through advocacy bodies such as VALID, NDIS support coordinators, disability providers and parent training, there is good understanding in the PWS community about choice and control and supported decision making. Future data collection, we believe, would show that the vast majority of our PWS adults are already included in discussions and supported decision-making processes. The importance of informed consent and supported decision-making is a well understood concept in the PWS community. Indeed, we suggest it is the process most parents use to manage the person with PWS’s lack of satiety from when they are toddlers.

The Home and Living consultation paper presents a very simplistic view that, by expanding the scope of the information and increasing the support to explore their home and living options, NDIS participants will be able to exercise choice and control over their living options.

Given the complexity of PWS and the importance of living in a specialised, PWS- safe environment, many factors must be taken into consideration before best fit home and living options can be determined. These include:

Need for a robust structure build
Compatibility with others, given a PWS predisposition to anxiety and dysfunctional social behaviours
Proximity to family and support networks
Proximity to workplace /volunteer or community activity base
Proximity to medical care
Geographical positioning so they are not near to, or can easily access food outlets
Lack of insight about actual capability, and adverse consequences
Confabulation which means Supported Independent Living (SIL) and other staff must be specially trained in PWS management techniques
Ongoing food security and cash management, to a greater or lesser degree
Evidence that people with PWS can be articulate and mislead support staff about their level of functional capacity and decision-making capacity, leading to inadequate support and poor welfare outcomes

With all the above competing and often conflicting considerations, we question whether it is realistic or ethical for this Home and Living approach to be championed as a viable option for many of the participants with more complex disabilities. As presented, this Home and Living approach does not capture the logistical challenges and presents unrealistic expectations that the person with PWS, even with the

Prader-Willi Syndrome Association of Victoria Inc.

assistance of their support network, will indeed have any choice and control over where and how they live, when so many of these factors are beyond their control.

Another concern is that the Home and Living consultation paper appears to be based on the premise that all participants have an active and engaged support network to guide their supported decision making. For many of our PWS adults, their parents are either elderly or no longer engaged actively with their support.

There is also a percentage of our PWS adults who do not have the cognitive function to understand the complex discussions and decision making involved in choosing home and living options without that support.

“In some instances, people with PWS may lack capacity. Capacity refers to a functional concept, determined by the person’s ability to understand, retain, and weigh up information relevant to the decision to arrive at a choice, and then to communicate that choice. Approaches to decision- making in adults who lack capacity include anticipatory decisions made through advance health care statements or decisions by proxy based on ‘best interests’ or ’substituted judgement.”6

Without the benefit of considerable input from a support network, including family, medical and psychological professionals, it is unclear how the supported decision-making progress can be validated.

The paper refers to ‘peer support’ for decision making. PWSA Vic believes this will put people with PWS at undue risk. PWS is a rare condition. Even professionals have difficulty delivering an appropriate service to people with PWS. It would be dangerous for naïve peers to be expected to play an influential role supporting a person with PWS. Decision support is a skilled activity, highly individualised7 and contextual, and made more complex by PWS characteristics.

In cases where the person with PWS has no evidence of any support network does this responsibility fall to the Office of the Public Advocate (OPA) or does NDIS propose a process to fill this void? None of these issues are addressed in the Home and Living information provided by NDIA.

While PWSA Vic supports the idea of greater budget flexibility, it does cause concern when the Home and Living consultation discussion paper states:

“You will have the independence to use your budget how you want and choose the supports that give you the best outcome to live an ordinary life.

6 Wong J, Clare I., Gunn M., Holland A, (1999), “Capacity to make health care decisions: its importance in clinical practice”, Psychological Medicine, 29(2), 437-446. https://www.ncbi.nlm.nih.gov/pubmed/10218935 7 A process of decision-making support: Exploring supported decision-making practice in Canada https://www.tandfonline.com/doi/full/10.3109/13668250.2020.1789269 Prader-Willi Syndrome Association of Victoria Inc.

You can also decide how to use your budget to design a solution that complements the other supports in your life, instead of replacing these natural supports (such as informal support provided by family and friends or supports from other government services).”

“The NDIA recognises this way of budgeting and using your NDIS funds will be new. We will support you to take reasonable risks, to pursue your goals in the planning and delivery of your supports. You will have the independence to decide what level of risk you are comfortable with.”

PWSA Vic would like to know more detail about the checks and balances that would be in place. Assurance is needed that the budget provided is, firstly, generated by the Participant’s own evidence of individual needs, and then used responsibly to meet the day to day needs of the person with PWS without compromising their health, wellbeing or safety.

Another area of concern in this Home and Living consultation is the premise that all participants will be able to work towards independent living and that once capacity is built supports are no longer required. In the case of PWS, it cannot be assumed that once the capacity of a person has been built, the supports can be withdrawn. Once established, a baseline level of maintenance support will always be needed for people with this life-threatening disability. They lack the capacity to make significant decisions in their own best interests.

People with PWS often lack volition, initiative and executive brain function. Therefore, the skills that a person with PWS might have, are often dependant on the monitoring and prompting of the support worker to put the skills to functional use. If that support is not guaranteed, there is a serious risk that the base competencies to build sustainable capacity will ever be of any practical use. Nor can it be assumed as stated in the Home and Living Consultation paper that all participants are able to live independently.

In the case of PWS, successful, sustained, independent living has not yet been demonstrated anywhere worldwide.

“The person with PWS will always need food security and behavioural support. The proper goal for someone with PWS is maximal functioning with supports, not independent functioning”.8

Reforming the funding model and Improving choice through flexible budgets

8 Forster. J & L. Gourash, Prader-Willi Syndrome: The Behavioural Challenge – A Brief Summary for Professional Pittsburg Partnership. pittsburghpartnership.com/handouts/The%20Behavioral%20Challenge%20for%20Profes sionals.pdf

Prader-Willi Syndrome Association of Victoria Inc.

The discussion in the Home and Living Policy paper appears to be very biased against group settings and presents this as the least preferred home and living option.

The point of view expressed in the Home and Living discussion paper is only one side of the coin.

Research findings that group living produces quality of life outcomes that are vastly inferior to Individual living options isn’t conclusive. As an example, we refer to research completed by Oliver and Gosden-Kay whose findings were as follows:

“Individualized housing was positively associated with human rights (i.e., self- determination, choice and autonomy) outcomes. Individualized housing also demonstrated favourable outcomes in regard to domestic tasks, social relationships, challenging behaviour and mood. However, outcomes regarding adaptive behaviour, self-care, scheduled activities and safety showed no difference, or less favourable results, when compared to group homes”.9

If an NDIS participant prefers to stay in a group home because it is better suited to their needs, there needs to be a guarantee the NDIA would continue to provide appropriate SIL and SDA funding to enable them to do so. A person with PWS may wish to stay in the robust built group home, that supports the person with PWS to stick to their diet plan with food security arrangements. It is also likely to have permanent staff who have the specialized skills to manage the complex demands of PWS and established routines that allow people with PWS to live their best lives, in the least restrictive manner.

PWSA Vic has seen a major failing in NDIA policy around home and living in that it fails to recognize the complexity of support required and to fund adequately. That is, to date PWSA Vic has seen numerous cases of people with PWS and their supporters having to fight ‘tooth and nail’ to get (a) an adequate Plan budget and (b) an appropriate combination of willing SDA/SIL (home and living) providers in place, without success.

The NDIA has not delivered on its most basic promise to ensure availability of an integrated accommodation Provider of Last Resort (PoLR). The policy must be explicit in enabling Participants to choose group living, in SDA, if they choose. And ensuring people with PWS have housing stability and do not become homeless

It is concerning when the Home and Living Policy consultation paper states that the NDIA will “target participants in traditional housing models to explore and design other options where that is their choice. “

What form will this targeting take? Will it be subtle pressure by NDIS and their paid emissaries (Planners, LACs, ILO providers), or will it be death by 1000 funding cuts that

9 Stacey Oliver, Emily Z. Gosden-Kaye, Dianne Winkler & Jacinta M. Douglas (2020) The outcomes of individualized housing for people with disability and complex needs: a scoping review, Disability and Rehabilitation, DOI: 10.1080/09638288.2020.1785023

Prader-Willi Syndrome Association of Victoria Inc.

make it unviable for disability support providers to support the more complex care participants such as those who have PWS. This scenario is heralded in the paper: “…the NDIA is already looking to no longer financially supporting institutional or large group homes (five or more people…)”.

The discussion paper also suggests that the proposed changes to the funding model will see the allocated budget determine housing options and support options. The unspoken assumption is that budget allocations will ultimately predetermine the parameters of home and living options, not the participants exercising their individual choice and control. The participants’ needs should drive the size of the budget. Then the Participant can choose appropriate accommodation and domestic support.

Who are the providers of last resort when it becomes uneconomical for the disability providers to support those NDIS participants with more complex needs? This issue isn’t addressed in this discussion paper.

The paper includes in its vision that “An ordinary life at home means a sense of belonging, safety and security”. It is disappointing that the paper does not then include a section in Appendix D about ‘belonging, safety and security’ and how the market should be supplying such services in the Participant’s living/domestic environment. This policy is an ideal opportunity to guide SIL (or similar domestic providers) in the kind of minimum service model that should apply, for example, Active Support. Considering the severe cognitive impairment of people with PWS and the lack of knowledge of their informal support network about what constitutes a good service model, the NDIA should be setting a benchmark.

Also, the concept of ‘buyer beware’ is not appropriate in the context of buyers with cognitive impairments like PWS because they do not have the capacity to be adequately cautious of, and alert to potential dangers or risks. To be beware needs capacity to foresee consequences, weigh up and compare options and assess and judge pros and cons, plus monitor results. For many with PWS this premise just does not hold true.

In summary, PWSA Vic cannot endorse this paper as it stands because it is too short on detail. In its current form, it does not demonstrate how the new policy will enable people with PWS to live a life of “belonging, safety and security”.

Thank you for the opportunity to contribute to this consultation. The question of Home and Living options for people with PWS is one of the most important decisions in their adult life. We are happy to discuss this submission in more detail if that would assist.

Yours sincerely

Renee Di Genova (President of Prader-Willi Syndrome Association of Victoria)

On behalf of the PWSA Vic Board

Prader-Willi Syndrome Association of Victoria Inc.

Prader-Willi Syndrome Australia Ltd

ABN 12 625 483 909

Significant Individual Support Needs – Supportive Practices and Modified Environments for people living with Prader-Willi Syndrome

Position Paper (Draft)

Prader-Willi Syndrome Australia, July 2020

Position Paper (Draft)

Prader-Willi Syndrome Australia, July 2020

EXECUTIVE SUMMARY

This paper is written in support of the Prader-Willi Syndrome Australia (PWSA) statement that:

People with PWS find it difficult to sustain life in the absence of significant and ongoing specialized support practices. What constitutes ‘ongoing specialized support’ will vary from individual to individual. Each case has to be taken on its own merits, depending on the ability of the person to make specific decisions about their own welfare, especially eating.

Practices that deliver food security enable a person with PWS to live a more ordinary life. Personal freedom, in line with United Nations Convention on the Rights of Persons with Disabilities, should be pursued at all times. However, practices which modify the support provision environment to enable ‘due care and diligence’ could potentially have an impact on ‘personal choice and control.

These statements and this position paper will be revisited and updated as part of an international Personal Choice and Control review being undertaken by the International Prader-Willi Syndrome Organization (IPWSO)i, its 100+ member countries and associated support organisations. The review is aimed at drawing up World Best Practice Guidelines. Such guidelines will encourage least-restrictive practices, while recognizing that some elements of food security and ongoing support practices are required to sustain life, and enable people living with PWS to reach their full potential.

KEY FACTS

Prader-Willi syndrome (PWS) is a complex multi-system, multi-stage genetic disorder which has only ever been seen to result in early death if appropriate supports are unavailable or withdrawn.
Personal choice and control can be maximized when appropriate and informed supports are in place.
Support when around food is essential to maintain life for a person with PWS, due to their genetically altered satiety regulation and changed brain structure and function.

i The International Prader-Willi Syndrome Organization (IPWSO), website www.ipwso.org

Prader-Willi Syndrome Australia Ltd

Supports around food access must be delivered in a highly sophisticated manner; This is essential to avoid exacerbating other aspects of the PWS condition (such as anxiety- related behaviours of concern), whilst optimizing personal choice and control.
‘Reasonable and necessary’ supports enabled by adequate public/community funding1 will be the most significant contributing factors to increased life-expectancy for the community of people with PWS.

To enable people living with PWS to live as close to an ordinary life as possible (optimum health and wellbeing), and experience maximized personal choice and control (personal independence), Prader-Willi Syndrome Australia (PWSA) recommends:

Recommendation 1: Specialized and ongoing training and intensive support provision for challenging behaviors must be implemented, to enable maximized personal choice and control

Recommendation 2: Each person should be assessed to gauge their functional impairments caused by over-eating and behaviours of concern, with suitable, least restrictive supports implemented to enable good health and wellbeing

Recommendation 3: A holistic and collaborative approach must be taken to support a person living with PWS

This multi-disciplinary approach is needed because food is available in most settings in life and managing only the domestic environment will not be enough support for the person with PWS. A holistic approach requires a ‘lead’ provider or nominee to be responsible. Much ‘behind the scenes’ supporting of daily life is needed to keep anxiety-producing occurrences to a minimum and address any adverse consequences of poor collaboration. Common examples are consistent practices to support dietary planning, or contingency arrangements to overcome daily challenges, like transport.

PURPOSE

This Paper describes the current position of PWSA in relation to significant supportive practices that promote personal choice and control, which may be deemed too restrictive by some jurisdictions.

The necessity for this Position Paper has been brought about by:

The adoption of the United Nations Convention on the Rights of Persons with Disabilities (UN CRPD) by Australian legislators and by PWSA.
The advent of the National Disability Insurance Scheme (NDIS).
The subsequent and ongoing entry of many new service providers into the disability sector.

The need for a consistent and specialist approach to supporting persons with PWS has become possible under the NDIS. NDIS Plans will need to include adequate funding to ensure that service providers can deliver appropriate outcomes. The nature of this rare eating disorder and accompanying behaviours of concern, requires significant and highly skilled supportive practices.

Prader-Willi Syndrome Australia Ltd

This Paper will help service-providers and regulators to understand the continuum of significant supportive practices that are needed, to ensure that people with PWS can live a full and ordinary life, and exercise choice, control and their human rights to the greatest extent possible. Some people may deem the practices described as being restrictive, but they are in fact life-saving for a person with PWS.

SCOPE

This position paper is primarily designed to inform disability service providers supporting persons with PWS who are in residential specialist disability accommodation or receiving other services from disability providers in the community.

BACKGROUND

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.

PWS presents at birth and continues, with significant intensity, throughout life. PWS occurs equally in males and females and affects all races. Hyperphagiaii, food-seeking and behaviours of concern are compounded by a typically low muscle to fat ratio and low energy requirements, resulting in rapid weight gain. “Consequentially, without careful monitoring and intervention around diet and activity, weight gain can be very rapid, leading to obesity, health complications and early death.”2 “Obesity and its complications are major causes of morbidity and mortality for people with PWS.”3 People with PWS are also at a much higher risk of developing psychiatric illnesses.

PWS is recognised as the most common genetic cause of life-threatening childhood obesity4, a condition that invariably carries over to adulthood. People with PWS have a flaw in the part of the brain called the hypothalamus. This part of the brain is an important supervisory centre and hormone regulator. The hypothalamus, when functioning normally, registers feelings of hunger and satiety (fullness). For individuals with PWS this does not occur, and most individuals with PWS will never feel full. They have a continuous urge to eat. This part of the brain also controls metabolism of fats and carbohydrates.

In Australia, the general population can expect to live for an average 82 years, however, people with PWS have a much lower life expectancy, with the average life expectancy being less than 38 years of age. This is mainly due to the lack of knowledge about PWS, combined with maladaptive behaviours that cause fatal ill health or accidental death. The risk of early death can be significantly reduced when appropriate, reasonable and necessary supports, environmental modifications, and PWS-specific supportive practices are put in place, including access to food (food security). This is the current accepted international best practice for people with PWS.

Characteristics relevant to significant supportive practice

ii Hyperphagia (noun used in medicine): an abnormally great desire for food; excessive eating

Prader-Willi Syndrome Australia Ltd

Most people with PWS have a mild or moderate intellectual disability with additional cognitive problems, including poor executive brain function.5
There may also be some physical, sensory and receptive/expressive language impairment.
They have malfunctioning satietyiii. They therefore present as always hungry, will constantly food-seek, suffer from hyperphagia and readily gain weight. They usually display genetically related abnormal food seeking behaviour. They have a reduced resting metabolism related to an altered body composition (muscle to fat ratio), leading to an abnormally lower rate of energy expenditure. Best practice support would include a low energy diet coupled with regular physical activity, necessitated by these physiological characteristics.
People with PWS experience extreme limitations in their ability to self-regulate in relation to food consumption. Their resulting obesity is not their own choice. International research6 on the brains of people with PWS has shown that their physiological response to food is different to the neuro-typical person.
Although there may be variation in the intensity of the food-seeking drive, people with PWS have a preoccupation with food, along with the overriding desire to eat. Therefore, the energy intake of every person with PWS must be regulated by responsible support persons, or they will die prematurely from obesity related, or other, co-morbidities.
People with PWS have chronic behaviour disturbance. There is “a typical neurobehavioral profile that includes altered intellectual functioning and centrally driven maladaptive behaviours”.7 People with PWS have difficulty managing anxiety levels and are very stress sensitive. Oppositional behaviour may quickly escalate to major temper outbursts, if not supported appropriately by persons with a good understanding of PWS.
It cannot be assumed that once the capacity of a person with PWS has been built, the supports can be withdrawn. Successful independent living has not yet been demonstrated anywhere worldwide.
People with PWS find it extremely difficult to extrapolate from one situation to another. Their ongoing, genetic impairment affects executive brain function, satiety and regulation of anxiety and emotions. The risk of early death from overeating or accidental death may never be totally eliminated and supports of some type must always be in place to manage food security (and money), and guard against their increased risk of accidental death.

CORE PRINCIPLES

In preparing this discussion paper, PWSA has identified the following core principles:

PWSA endorses the United Nations Convention on the Rights of Persons with Disabilities and support all individuals in their pursuit of maximised personal choice and control.

Quinn8 coined the “sword and shield” concept to describe the core role of the United Nations Convention on the Rights of Persons with Disabilities. The Convention offers a ‘shield’ to defend against intrusion (restrictions) on rights by the State or others, as well as an assertive ‘weapon’ to demand resources (supports) needed to exercise those rights.

iii Satiety: the feeling of having eaten sufficient food

Prader-Willi Syndrome Australia Ltd

PWSA believes that Quinn’s analogy justifies the case for seeking ‘reasonable and necessary’ NDIS support funding to enable improved personal choice and control by the person with PWS, leading to a more ‘ordinary life’, when living within complex functional impairments.

In maximising personal freedom, we recognise the need to safeguard the competing rights of individuals to practice choice and control in an environment that is least restrictive.

We identify that an ethical “tension exists between a genetic deterministic perspective and that of individual choice “9.

“If the person is found to lack capacity, the common-law principles of acting in the person’s ‘best interests’ using the ‘least restrictive alternatives’ may be helpful. Allowing serious weight gain, in the absence of careful consideration of these issues is an abdication of responsibility”.10

We identify that PWS is a spectrum disorder and there are subtle differences in the genetic variation causing PWS. Not everyone affected by PWS will have all the characteristics. The degree to which they are affected may also vary. We recognize that there is no one solution to fit all individuals who have PWS:

” Adoption of the capacity approach implies that each case has to be taken on its own merits depending on the ability of the person to make specific decisions about eating. There may be some people who never have the capacity to make decisions about their eating while others may be deemed capable, especially if they have been educated about diet, and strategies about reducing weight such as exercise. In those lacking capacity, preventing access to food by locking the kitchen would be justified under the ‘best interest’ principle.” 11

It must be recognized that the degree of capacity will span a spectrum, and this range of capacity to make decisions about their eating must be taken into consideration when making decision about the type of support required on individual merit.

In some instances, people with PWS may lack capacity. “Capacity refers to a functional concept, determined by the person’s ability to understand, retain, and weigh up information relevant to the decision in order to arrive at a choice, and then to communicate that choice. Approaches to decision-making in adults who lack capacity include anticipatory decisions made through advance health care statements or decisions by proxy based on ‘best interests’ or ’substituted judgement”.12

A paper from the UK by Holland addresses the difficulties in assessing capacity in PWS:

“It will often be that families and care professionals have very useful anecdotal evidence that begins to explore the difficulties that people with PWS face when applying these executive functions to decisions about food. You do not have to be certain that such deficits exist - rather your judgement about capacity is made on the balance of probabilities. Evidence is crucial. For instance, keeping weight records can show that a person is gaining weight even though they may say they are not. Keep very detailed records and clear evidence that a serious problem exists.”13

Prader-Willi Syndrome Australia Ltd

We identify that PWS is a complex multi-system, multi-stage genetic disorder.14

Research has found that there may be up to 9 distinct phases over the lifetime of a person with PWS, each with different support needs. Therefore, it follows that the management strategies may need to change over time. Strategies used by the person(s) supporting the individual with PWS surrounding food security15, and associated behaviours, will need to undergo regular review and assessment throughout the person’s life. What was relevant food security and behaviour support strategies at 16 years old may not be best practice at 30.
Those who are supporting the person with PWS have a common law ‘duty of care’16 to manage the environment to optimize food security according to the needs of the individual.

“ The person with PWS will always need food security and behavioural support. The proper goal for someone with PWS is maximal functioning with supports, not independent functioning.”17

Unquestionably food is a major source of stress for someone with PWS:

“Families and professionals [support staff] often mistakenly believe that the patient cannot be happy unless he has as much food as he demands. Because efforts to limit food, if attempted without establishing food security cause increased stress and behaviour problems.

Successful Behaviour management of PWS means that uncertainty about food must be eliminated as much as possible.”18

An essential element of successful behaviour management is the skill of the staff to deliver the dietician’s diet plan and not defer to the manipulative elements of PWS behaviour which lead to an escalation of syndrome-driven behaviours. A total staff commitment to the consistent management of a dietary plan, as detailed by the dietitian, is critical to the success of individual wellbeing. The success of the implementation is directly related to the level of understanding of PWS and level of skill of the staff in providing support to avoid complex PWS behaviours. This in turn correlates directly with all staff having ongoing PWS training in best practice dietary management and behaviour support.

The achievement of consistent dietary management and behaviour support allows the person with PWS to minimize anxiety /stress levels and to get on with their life in a productive, ordinary and meaningful way.

HOW CAN LIVING AN ORDINARY LIFE WITH PWS BE ACHIEVED?

Behaviour support of PWS in residential settings includes physical environment modification to optimize food security, according to the needs of the individual.

“Intervention [in the form of support around minimising exposure to excess calories] is justified on the grounds that it puts people with PWS on more of a level playing field with people who do not face the challenges that they have, as a result of genetic difference.”19

Prader-Willi Syndrome Australia Ltd

It is the policy of PWSA to promote residential practices that pursue maximised personal choice and control. We are also aware that these freedoms will look different for each individual, depending on their capacity for self-determination.

A healthy life in the case of people living with PWS will always involve elements of ‘food security’ and/or ‘food control’.

We support the arguments of by Piers Gooding and Tony Holland in the context of the UN CRPD:

“Supported decision-making” does not appear in the CRPD but generally refers to efforts to boost the decision-making of persons with disabilities, offering them resources for making choices from ‘good’ options about how to live.”20

Professor Tony Holland (pers. com.) has said:

Support [to food control/security] improves capacity and therefore improves engagement. This is in line with what the UN CRPD wants for people with disability. I think we see this in people with PWS - those living in food unrestricted environments are not able to engage for many reasons and may also have limited capacity to understand the risks and to balance those risks in deciding what to do. With the ‘support’ of food security it can be very different - in that context their understanding may also improve to the extent that they have sufficient understanding of the risks [and] they are willing to consent to the restrictions that a food secure environment implies.21

See Appendix 2 for a table of accepted techniques to support food security and/or control.

A consideration: How does food security differ from food control?

Food Security: Pittsburgh Partnership defines food security for people with PWS as psychological security in relation to a clear plan or understanding for all food consumed including non-calorie food and beverages. There is variation across the syndrome cohort. However, most individuals must have no doubts (or concerns) about what they will be receiving at each meal/snack, and no expectations (or hopes) of obtaining food outside their food plan, which will result in no disappointment that can lead to behaviours of concern. In the case of people with PWS, food security therefore exists when individuals have certainty regarding what they would eat, when they would eat it, and how much they would eat. This reduces stress, anxiety and adverse behaviours, enabling a productive and healthy life. Ensuring food security can involve significant and ongoing individual support and capacity building.
Food Control: Due to the changed brain development of people with PWS, which results in hyperphagia (overeating), as a result of an uncontrollable urge to eat, individuals are rarely able to resist consuming excess calories. If not managed, this consistent over consumption of energy results in obesity and related co-morbidities, eventually reducing life expectancy. In many cases, food control is needed, involving the establishment of physical mechanisms or barriers to prevent additional food access, such as locked kitchens

Prader-Willi Syndrome Australia Ltd

or pantries. This process is a restrictive practice and should be closely monitored to asses if any restrictions can be eased or removed over time.

See Appendices 1 and 2 for detailed strategies to support food security and food control.

CONCLUSION

Best practice PWS support involves a multifaceted and holistic approach that utilizes a wide range of positive behaviour support and support strategies, and organisation and communication strategies. It involves the input of professional advice and constant assessment and refining of programs and strategies.

All of this is driven by the needs and capacity of the person who has PWS. If the best professional advice from the Support Team deems that locks/physical barriers are required in certain cases, then this should be allowed as this is a proven method to deliver the best practice support for healthy calorie consumption and reducing behaviours that otherwise lead to social isolation.

Other considerations such as prudent planning of new housing builds/modifications is also important. These would factor in design features such as butler pantries and open plan kitchen, so staff can support the healthy eating plan implementation in a robust, PWS friendly environment that enables sufficient personal space.

These support practices around diet may be required in other settings, such as educational or day program environments, together with community access activities.

Food security and food control are a continuum of practice, implemented to a greater or lesser degree, depending on the individual. Restrictive practices should never be viewed as the beginning and end of food security or behaviour support practices. It is too simplistic to talk about management of food security for PWS in terms of just locks and barriers.

NDIS PLANNING

The NDIS offers great opportunities for developing individual capacity in the area of personal choice and control for people living with PWS. However, enabling maximised personal choice and control is complex due to the multi-system, multi-stage nature of the syndrome. The syndrome presents a collection of functional impairments.

A holistic approach to duty of care is required of providers, to support the collection of PWS impairments and enable a balance. A lack of balance leads to a disruption of the person’s ordinary life. Therefore, the NDIS will need to provide considerable 1:1, or small group support funding to enable increased freedom and minimise other practices that may be deemed restrictive. The degree to which the individual can participate in self-determination should be assessed on a case by case basis, with the understanding that there will always be at least an element of support needed for healthy food choices.

Since independent living by people with PWS has not been successfully achieved, the planning process will need to address food security and/or food control arrangements in the supported residential setting.

Prader-Willi Syndrome Australia Ltd

Support and guidance from the care team/allied health/medical professionals to prepare the evidence of functional impairment(s) needs to be sought, documented and submitted during the planning process. The team’s support will also be essential in the initial stages of transition to the residential house and after the move is complete. The support person(s) should have regular input and guidance including, but not limited to, the following:

Dietician
Endocrinologist
Psychologist /Psychiatrist
Respiratory Specialist
Exercise physiologist
Parents/Support workers

Their professional opinions should be stated in any NDIS planning documents, to provide clear and transparent guidance to the Support Coordinator about the behavioural practice and physical supports needed.

The planning request should also include “Investigating Housing Solution” funding. This will enable the Participant to work with a Specialist Support Coordinator to search for appropriate housing. It also covers completion of a housing plan and funding of various allied health assessments to determine the supports the person with PWS would need when they transition from the family home to the supported residential setting, or transfer from one supported setting to another.

ONGOING REVIEW OF PERSONAL CHOICE AND CONTROL BY PWSA AND OTHERS

PWSA is currently taking a lead role in a world-wide review of personal choice and control (restrictive practices) in relation to PWS. Participating in the review are:

Individuals living with PWS and their families
The International Prader-Willi Syndrome Organisation (IPWSO)
Numerous national person and family Associations
Major support providers that provide specialist disability accommodation (SDA) to over 600 people living with PWS
Psychiatrists/psychologists, professors, clinicians and lawyers from various universities and institutions

The purpose of this review is to establish a set of more detailed world best practice principles that enable maximised personal choice and control, thereby minimising, and where possible, eliminating, restrictive practices. Australia has come a long way when one compares practices on an international basis, however, we recognise that more needs to be done to promote personal freedoms in line with the United Nations Convention on the Rights of Persons with Disabilities.

Prader-Willi Syndrome Australia Ltd

This review will take some time to complete, before international best practice principles can be progressed. In the interim, PWSA has undertaken its own independent review, and will continue to refine our position in relation to supportive practices that are designed to maximise personal choice and control and empower a healthy and full life for people living with PWS across Australia.

Prader-Willi Syndrome Australia Ltd

Description of different strategies to be used – a global perspective

Best practice should support a healthy, ordinary and fulfilling life. Supporting a person living with PWS currently demands of the support workers a comprehensive and sophisticated response. It is their responsibility to support people with PWS, while recognising and upholding the person’s statutory and common law rights, including a right to receive ‘due care and diligence’ in the delivery of support by the team.

In order to deliver the standard of support required, the staff and support workers must have a sound understanding of PWS. They must have the confidence and fortitude to manage the multi-system, multi-stage spectrum of PWS behaviours. In addition to PWS specific training, the support worker must have a manageable workload. That enables them to consistently implement the organisational aspect of communication, behaviour support and food secutity [corrected to: security] measures that PWS support demands.

The provision of consistent food security is as complex as it is demanding. It requires a well- trained team of support staff, to provide the environment that ensures there is minimal opportunity for excess calorie consumption, and minimised behavioural outbursts that arise from inconsistency.

Healthy diet: All individuals require education and training to understand the complexities of a healthy diet, including people with PWS and their support team. No exception. Unsupported access to inappropriate food will endanger the lives of people with PWS.
All staff, including casual support workers, should participate in specific PWS training in the areas of dietary and positive behaviour support planning, effective communication strategies and health/wellbeing support strategies.
Advanced planning of quality, low calorie meal choices, as recommended by a dietitian.
Shopping for the house reflects the dietary advice received – this does not involve bulk buying of highly processed food. It does include thoughtful purchases, such as limits to pre-packaged processed foods, purchase of fresh fruit & vegetables in the quantities stipulated by dietitian. Consideration of portion sized when shopping can reduce the likelihood of over consumption.
Storage of food reflects advice from the dietitian – allowed snacks, allocated daily and accessible (within the parameters of diabetic intake patterns, if applicable). In instances where the person is deemed to lack capacity in relation to making appropriate decisions about their eating, physical barriers (eg. Locks) in the kitchen area may be justified under the ‘best interest’ principle.
Meals served by staff should reflect portion sizes as advised by dietician.
Before leaving the home to eat out, careful planning assists in identifying which foods are compatible with their diet, and thus managing expectations about choice during the outing.
Scheduled daily activities with the place and time of meals clearly identified helps the person with PWS to engage in a meaningful and purposeful daily program. They are then less likely to be focused solely on food.

Prader-Willi Syndrome Australia Ltd

Consistent supervision at meal preparation, eating and after meal ‘food clearing’ to safeguards against consumption of food being taken from others, or accessed from food storage or scrap storage places. Subject to the individual’s hyperphagia drive, all food will most likely require secure stored at all other times.
Exercise should become an integral part of daily routine.
Access to money may need to be supervised and receipts kept to support healthy living in line with their dietician’s advice.

The support team needs to encourage and monitor food access arrangements at all times. The goal is to obtain the agreement of the person with PWS to the arrangements. A contract based on informed consent is sometimes appropriate. For example:

“I (the person) do not like being overweight and want to return to a normal weight range/X kilo. You (support person,) can support me in managing my diet in a food secure environment, thereby assisting me in reducing the stressors associated with food in my life. I will try to abide by my diet and the agreed food security routine and measures, which are (list measures)”.

Such an agreement must be consistently applied and the person with PWS must be supported emotionally and socially in all settings, to maintain their consent, in the face of many temptations.

The provision of appetising, well presented, quality food, in small serving portions, at regular meal and snack times, has been shown to assist emotional wellbeing for the person living with PWS.

Adherence to consistent food routines can reduce friction in most cases. What these food security measures look like, will vary from individual to individual and will need to be established, maintained and reviewed, with the support of the individual’s Care Team. ‘Food security’ is a psychological state of mind. When food security is not achieved, other management strategies will have to be explored including the use of locks.

It is essential that this support is implemented with appropriate ‘duty of care’.

The restriction of choice of foods (i.e. low fat, low calorie foods) as recommended by a dietitian, has many precedents in everyday practice in the community. Disability support houses/schools/day care centres regularly restrict food choices for people who have coeliac or peanuts/seafood or other allergies. Therefore, the restriction of food choices to people with PWS should not be a contentious issue. Many schools/residential support houses/day- care places enforce rules prohibiting the bringing of certain foods, based on medical/dietician’s advice due to the fact someone in that house or school has a severe allergy to certain foods. Excess .food (callories) of any type is, over time, life-threatening to those with PWS.

The PWS low-calorie diet is comparable to the needs of others with specific requirements in everyday practice in the community. If this diet is not adhered to, obesity and related co- morbidities will risk the health and wellbeing of the person with PWS. This is similar to an anaphylaxis reaction risk for the person with the allergies; albeit one type is fast acting (anaphylaxis) and the other slow (morbid obesity).

Prader-Willi Syndrome Australia Ltd

While PWSA does not necessarily promote a specific method of enabling food security, numerous different practices and devices exist across the globe. Four alternative practices are outlined below.

It should be noted that some of these programs will necessitate considerable increased support provision and capacity building. Existing practices, if working successfully for the wellbeing of the person with PWS, should be maintained until new programs for food security are fully developed. Transition to a new support practice should only occur once residents have been consulted, capacity of the residents has been developed, the program has been fully researched and developed, support workers have been fully trained and the support organisation is satisfied that they have the resources to implement the new program in an effective and sustainable manner.

The primary aim of implementing an alternative food security practice, such as those cited below, would be to enable a least restrictive practice, while maintaining good health and wellbeing. Choosing the most appropriate option will rely heavily on individual capacity and organisational capacity.

Alternative food security practices include:

Pittsburgh Partnership, Linda Gourash, MD; Janice Forster, MDA, suggest a food security support strategy:
- “No doubt” The person with PWS is able to relax and think less about food when they know the set times when meals arrive. Advanced planning provides them with expectations which will be reliably fulfilled.
- “No hope” of getting anything different from what is planned. This requires more measures to be put in place to ensure they are not successful in acquiring food. Chances to obtain food are stressful so therefore should be eliminated. This can require additional supports to be put in place to ensure the person is supported in successfully maintaining their diet, rather than successfully acquiring additional food calories. Implementation of appropriate supports will depend on the individual’s history and capability of unplanned food acquisition.
- “No disappointment” No false expectations. What you promise you deliver. This will reduce the number and intensity of angry outbursts.
Prader-Willi Syndrome Institute Germany (PWS-ID), Dr Norbert Hödebeck-Stuntebeck and Dr Hubert SoyerB promote a food security program that encourages individual food access training:
- “We say ‘Yes we can, yes we can, yes we can!’. We all have to deal with doubt, with hope and with disappointment because these are conditions of normal life. And we must prepare/help people with PWS to become competent for new and sudden changes, for visions and for situations of disappointments, so that they learn to develop coping strategies for such situations. If we do not give them the chance to come in contact with such situations, they have no chance to learn to manage them in a more and more proper way and become more and more independent from others.

A Founding Directors, Pittsburgh, Pennsylvania, USA www.pittsburghpartnership.com B Directors, www.pws-id.de

Prader-Willi Syndrome Australia Ltd

Over the years, we saw that people with PWS are much more self-controlled than professionals or parents think [is possible].” Norbert Hödebeck- Stuntebeck

Arc of Alachua County, Mark Swain and his associatesC at Ark have implemented a system of food dollars based around calorie content. Individuals are supported to manage their own daily calorie intake by trading food dollars for food. This method enables increased personal choice and control within a set daily calorie target.
Grankoglen, Carina Knudsen and Mai-Britt RosengreenD in Denmark:
- Danish experts believe that the person with PWS cannot be cured of their hyperphagia, so they should be relieved of the daily stress of worrying about food, thereby freeing them up for other, more fulfilling life pursuits.
- Grankoglen delivers ‘restaurant service’ as their preferred method of food security. Professional cooks are employed to undertake menu planning, food preparation, food serving and food service to the table.

Note: Some support providers in Australia may find this Danish concept challenging in relation to personal choice and control, however, it is worth noting that people living with PWS in Denmark live more than 50% longer on average than those living in Australia

Summary

These support practices around diet may be required in other settings, such as educational or day program environments, together with community access activities.

C Mark Swain, Executive Director; Mark Lister, Director Behavioural Services; Lynnette Ramos, Board Certified Behavioural Analyst and Clinical Director, Gainesville, Florida, USA www.arcalachua.org D Grankoglen, Grandbakke Ves 22B, 8961 Allingaobro, Denmark

Prader-Willi Syndrome Australia Ltd

Appendix 2

Food security management for PWS in mixed disability residential support houses

Tensions created by PWS food security/PWS daily exercise routine in mixed disability support houses	Suggested approach/solution	How can this be achieved	Extra resources required
PWS specific diet is difficult to implement in a house where other residents don’t have specific dietary requirements	Given that support workers have a duty of care to provide all residents with a healthy well balance diet – a Healthy Living approach for all residents should be a long-term goal. A good PWS diet is a healthy living diet with smaller servings. It takes time and skill to change poor dietary habits, particularly for a person with PWS. But a skilled support worker with the support of the dietician can coordinate a transition to, and maintenance of a healthier diet	Specific training for support workers on how to present and prepare a well-balanced diet that is appetizing. Healthy cooking methods should always be used in any SDA, i.e. fresh, without additives, managed calorie intake, minimal use of deep frying, few calorie rich sauces and condiments. Outings should not involve unhealthy food choices; a healthy range of options should be pre-identified and built in by the dietician.	Dietician should train all residents and support workers on site, about healthy cooking methods, food storage, serving sizes, including appropriate healthy snacks. Regular servings of fresh fruit and vegetables should be included in the diet. Support workers and residents may initially need the assistance of a dietitian to develop a file of dietitian approved recipes with attached shopping list for each recipe. Support workers must set aside time to refer to, and understand, an individual’s meal plan and then adhere to it consistently, e.g. cut lunches healthy options

Prader-Willi Syndrome Australia Ltd

General Issues around the Implementation and Performance of the NDIS

Submission 85

PWA Prader-Wills Syndrome Australia Support | Information | Advocacy

Tensions created by PWS food security/PWS daily exercise routine in mixed disability support houses | Suggested approach/solution| How can this be achieved| Extra resources required: —|:––––––––––:|:–––––––––––| Smaller plates can be used to make servings look more generous.| Support worker hours should be set aside for 1:1 food preparation, with no other residents in proximity -can lead to stress and outbursts for the person with PWS. involve the residents in the menu planning process utilising either/or choices to enable healthy options. Develop a weekly meal planner in consultation with the residents including prepared lunches & snacks Encourage residents to help prepare their meals on a roster according to capabilities and with support Weekly menus and dietary requirements of resident’s inform shopping Only foods from list should be purchased Where possible purchase items in specific small serving sizes Dietitian and House coordinator to work with staff to fine tune the purchasing of goods for house line with a healthy eating policy Staff ,support workers and residents develop clear understanding of food quality Healthy menus should start at point of purchase that determines the quality of the resident’s diet The goal of the support worker should be to purchase fresh quality food in quantities

General issues around the implementation and performance of the NDIS Submission 85

[Image not converted to Markdown – “PWSA Prader-Willi Syndrome Australia Support | Information | Advocacy” – check the source PDF page for the actual content]

Tensions created by PWS food security/PWS daily exercise routine in mixed disability support houses	Suggested approach/solution	How can this be achieved	Extra resources required
Shopping is done in a rush with no preplanning; pre-prepared items are bought in bulk to save time. Processed ‘treat’ foods/snacks are bought in high rotation. This can result in quantities of less healthy food being stored at the house and potential food wastage.	required for the weekly menu planner	sizes – yogurts, snack packs in 45 gm boxes	food labelling, energy content and ingredient lists.
Food purchased in bulk can often be stored in pantry/cupboards that are readily accessed by all residents. Additional food could be consumed by	Minimizing the quantity of food purchased per shop - focusing on quality of the food and correct storage of foods will reduce the amount stored. If the food is presented in an attractive and appetizing way, the healthy option will become	Allocating the number of snacks per day that are recommended by the dietitian Have fruit readily available in quantities recommended by the dietitian	Dietitian & house supervisor audit how food is stored in the house All staff identifies and records any breeches of food security for the person with PWS and problem solve strategies to rectify. Staff informed about, and prevent further food ‘leakage’ by being

Prader-Willi Syndrome Australia Ltd

General issues around the implementation and performance of the NDIS

Submission 85

[Image not converted to Markdown – “Image of page content” – check the source PDF page for the actual content]

PWS Australia (Prader-WillisiSyndrome) Support | Information | Advocacy

Tensions created by PWS food security / PWS daily exercise routine in shared disability support houses people with pws, with continuing repeatedly without being noticed by support workers.

Suggested approach/solution
Routine once the change to routine is bedded down. Change for anyone takes time, particularly if they have an intellectual disability. Realistic timelines and outcomes must be factored into any changes to food security routines.
in a case where the person is deemed not to have the capacity to make decisions about their food intake stress can be alleviated but not eliminated pantry and fridge are locked Note: Other residents can experience disadvantage due to this practice.

The Residents can experience disadvantage enjoying related celebrations. every resident regardless whether has right socialise participate community events
Support rehearse how they can make when go out Prader-Willi Syndrome Australia Ltd

General issues around the implementation and performance of the NDIS Submission 85

[Image not converted to Markdown – “PWSA logo” – check the source PDF page for the actual content]

Tensions created by PWS food security/PWS daily exercise routine in mixed disability support houses	Suggested approach/solution	How can this be achieved	Extra resources required
eating out due to the dietary requirements of the person with PWS			at a celebration and calculate what has to be cut back over the next few days in exchange. They must inform staff on following shifts of the temporary arrangements, so it is reliably applied. Support staff apply a visual representation of the trading arrangements on the wall to help them understand what’s being negotiated, and stick to the trade once commenced
When the person with PWS has nothing to do they actively seek food	The daily planner for a person with PWS should be fully allocated with engaging and purposeful tasks - limited downtime should be a primary goal of any support/daily activity plan	Household duties/activities should be included to minimize and to redirect away from food seeking - get the mail, set the table, gardening tasks	House coordinator to set up a list of tasks for each resident Interesting and engaging activities that involve community participation & don’t involve food should be emphasized
The person with PWS keeps asking the other residents for food or takes food from other residents’ bag or rooms	This is a code of conduct issue. If the House Code of Conduct states very clearly – no sharing of food & no entering other residents’ bedrooms without	Support workers to have a range of management and support strategies to distract/redirect to avoid food seeking	Team meeting to communicate any issues and revise strategies. If behaviours continue, seek the advice of psychologist

Prader-Willi Syndrome Australia Ltd

General issues around the implementation and performance of the NDIS

Submission 85

PWS Australia Prader–Willi Syndrome Support | Information | Advocacy

Tensons created by FWS food security; PWS daily exercise routine in mixed disability support houses / kitchen and this causes tension within the house.

Suggested approach/solution
Permission: Then this becomes an expectation that this behaviour is not accepted All residents of the house should be fully aware of the House Code of Conduct and what it means for them in a day to day application.
Staff know: Staff knows and apply particular consequences relevant to the situation, without escalating the person with PWS concerns.
The residents are not interested in any exercise favouring a sedentary lifestyle such as watch TV and play on iPad.Support workers to model good habits: Residents to be given opportunity to participate in range of activities involving exercise both formally and informally Walking encouraged rather than being driven everywhere to gradually increase there exercise pattern.
All the worker supports consistently respond to incidents with appropriate consequence discussed prior have been discussd before these incident . If the incidence escalate living may require review arrangements
Skilled staff can trained skilled staffs to apply the supports or interventions. Local government sport participation officers assist introduction regular exercises into home routines Person supported access activity at local gym Skillled Support Workers Can Model Habits For Persons With PWs
Highly trained and skillfull staffed to apply support intervention if incidences escalates they will need live arrangeents.
Exercise physiologists
Local goverment sports participations officer assists introduce routine Personal suppoted community acess activitiy local Gym Skilful Supports Worker Models Habit For persons Wih pws
The

General issues around the implementation and performance of the NDIS

Submission 85

P WSA Prader-Wills Syndrome Australia Support | Information | Advocacy

Tensons created by FWS food security/PWS daily exercise routine in mixed disability support houses |

Suggested approach/solution	How can this be achieved	Extra resources required:
Membership of local gym should be encouraged and supported		that, for example, they regularly participate in a daily exercise routine before dinnertime.
Staff should exercise with the person, modelling and appreciating the fitness activity

Residents can store and eat food in their rooms. The person with pws can take food and consume more than dietitians’ recommendation without being observed and overeating goes un noticed.	Discourage food being kept in rooms on health & cleanliness rationale In some case This willbe included in the house Code of Conduct where it clearly states what can’t be consumed in rooms eg drinks can be consumed in rooms but foods	Support worker to encourage residents to take pride in their room and keep it clean and free of food Staff to educate resident about arrangements

The persons weight is going up despite appropriate access in home Note: Excess food can	Support workers to monitor individuals monitoring weight recording regular basis Key worker analyse gain pattern to support the	Support worker monitors client behaviour upon returning from community Evidence additional consumption
Staff liaise other providers (e.g day program) understand what food accessed and negotiate new security practices at location

General issues around the implementation and performance of the NDIS

Submission 85

P WSA Prader-Willi Syndrome Australia Support | Information | Advocacy

Tensons created by FWS food security/PWS daily exercise routine in mixed disability support houses cause bowel blockages, stomach rupture and death.

Suggested approach/solution
Person in avoiding rapid weight increase with subsequent health and mobility deterioration; identifying potential food sources
Introduction of the use of assistive technology and/or additional active night supports or physical barriers such as locking the kitchen at night
Person with PWS wakes up at night wanders to kitchen and takes food, or non-food items to eat (e.g., rubbish).
Person with PWS requests that the kitchen cupboards be locked for their own choice

The suggested solution is:

How can this achieved? noted and monitored e.g., food scraps packaging foods spills on clothes Introduce assistive technology such as light motion sensors or mats to alert staff about night time wandering Note: Support may have to upgrade roster to actively achieve benefits of this technology If problem persists it may be necessary to lock the kitchen fridge and pantry at night. Extra resources required Records can be maintained to monitors patterns Additional supports should be initiated in community access locations if additional food is accessed Use Assistive Technology Roster Active Night Staff To support person away from Food Seeking Team meeting to communicate any issues and revise strategies If behaviours continue seek advice psychologist and OT As needed Highly trained staff apply interventions Seek Advice Psychologist And Ot – Prader Willi Syndrome Australia Ltd

General issues around the implementation and performance of the NDIS Submission 85

PWSA Prader-Willi Syndrome Australia Support | Information | Advocacy

Tensions created by PWS food security/PWS daily exercise routine in mixed disability support houses	Suggested approach/solution	How can this be achieved	Extra resources required
safety and peace of mind Note: Other residents may not want this			This may require a review of living arrangements Seek OT and Specialist Support Coordinator reports
Support workers use of junk food or snacks as reward for compliant behaviour	Discontinue the practice of any food rewards Use intrinsic and more suitable extrinsic rewards	Ongoing training for staff in the importance of not using any food as management tool House coordinator support the support workers to use more suitable rewards	Seek dietitian advice Review staff training about appropriate positive behaviour support strategies Organise upskilling of staff and ensure better practices are applied

Prader-Willi Syndrome Australia Ltd

Support needs for people living with Prader-Willi Syndrome

Changing the conversation and supporting you to be an informed consumer

Expanding support for decision making

Reforming the funding model and Improving choice through flexible budgets

Significant Individual Support Needs – Supportive Practices and Modified Environments for people living with Prader-Willi Syndrome

EXECUTIVE SUMMARY

KEY FACTS

PURPOSE

SCOPE

BACKGROUND

Characteristics relevant to significant supportive practice

CORE PRINCIPLES

HOW CAN LIVING AN ORDINARY LIFE WITH PWS BE ACHIEVED?

A consideration: How does food security differ from food control?

CONCLUSION

NDIS PLANNING

ONGOING REVIEW OF PERSONAL CHOICE AND CONTROL BY PWSA AND OTHERS

SUGGESTED FURTHER READING

Description of different strategies to be used – a global perspective

Summary

Appendix 2

Food security management for PWS in mixed disability residential support houses

General Issues around the Implementation and Performance of the NDIS

General issues around the implementation and performance of the NDIS

General issues around the implementation and performance of the NDIS

General issues around the implementation and performance of the NDIS

General issues around the implementation and performance of the NDIS