Support Workers/Carers

People with Prader-Willi Syndrome are friendly, social, empathetic eager to please. Providing a safe and healthy environment for someone with Prader-Willi Syndrome means providing a consistent approach to effectively support the complex mix of behavioural, health and social challenges that are characteristic of PWS.

Prader-Willi Syndrome (PWS) is a rare, complex, unique, multistage genetic disorder. It is a spectrum disorder and symptoms vary in severity and occurrence among individuals. PWS affects the part of their brain called the hypothalamus, which is responsible for the regulation of all the systems in the body and the resultant regulation of hunger, thirst, temperature, emotional regulation, pain and stimulation hormone production amongst others. People with Prader-Willi Syndrome experience a constant preoccupation with food accompanied by an unrelenting, overwhelming physiological drive to eat called hyperphagia.

It is important for Service Providers to understand the complexities of Prader-Willi Syndrome and be diligent in a variety of ways and be aware of the many Prader-Willi Syndrome traits that can impact daily life and affect community access.

Food Seeking behaviour

People with Prader-Willi Syndrome follow a strict diet from a very early age. This is driven by those who care for them and is usually supported by a dietician or nutritionist.

The International Prader-Willi Syndrome Organisation notes the following on their website with regards to dietary management:

Starvation Syndrome

To understand the importance of this hunger drive, try to look at Prader-Willi Syndrome as a ‘starvation’ syndrome rather than an over-eating one. Because of the dysfunction in the hypothalamus, there is no on/off mechanism that tells the brain, “I’ve eaten enough”. What happens instead is that the brain keeps telling the stomach, “you’re starving, you need food”, and the drive to find food overrides everything else.

Unfortunately, food seeking behaviour is very challenging for those with Prader-Willi Syndrome and their carers. Firstly, what we need to remember is their drive doesn’t come from a natural hunger, but something more consistent with a need to survive.

Typically, our recommendation, and that of Prader-Willi Syndrome professionals around the world, is that Food Security is essential at all times, in all settings. In a school setting, we recommend that children’s lunch box’s are kept in a separate room (locked) and are handed to students at mealtimes, much the same as what would happen on a school excursion. To have the food unsecured (in the same room) only makes the person with Prader-Willi Syndrome anxious that the food is there, and the anxiety will escalate into a behavioural outburst.

It is also important to understand that a people with Prader-Willi Syndrome have can be highly anxious and can become overly excited. It is not uncommon to experience uncooperative behaviour if there is an unexpected change in routine or change in plans. Their social sensitivity is reduced so they can become loud and disruptive as a result of negative or positive responses to their feelings or perceptions.

Many of the Challenging behaviours associated with Prader-Willi Syndrome are anxiety related, therefore if you can reduce or eliminate the cause of the anxiety, you’ll reduce or eliminate the unwanted behaviour.

To assist with providing a safe and healthy environment for someone with PWS

To ensure best outcomes, we suggest the best start is to begin by implementing the following strategies. Once someone with Prader-Willi Syndrome can put their anxiety aside, there is an underlying drive to want to please you.

Food security is essential, this is not something that can be ignored.
Clear, concise and consistent communication of routine and strategies for communicating changes of routines are absolutely essential.
Someone with Prader-Willi Syndrome whose mind is kept busy on a task, is not focusing on food or their hunger,
People with Prader-Willi Syndrome have a processing delay and often get stuck in the moment and need prompting to move onto the next task or idea.
Allow extra processing time, use clear and unambiguous language, avoid lengthy instructions and check understanding
Due to poor auditory processing and memory, allow extra processing time, use clear and unambiguous language, avoid lengthy instructions and check understanding
Poor working memory – avoid multi-tasking and assist with taking notes as some people with Prader-Willi Syndrome exhibit auditory and visual processing difficulties
People with Prader-Willi Syndrome have Good long term, but poor short-term memory therefore use repetition
When someone with Prader-Willi Syndrome is stuck in the moment and exhibiting a behaviour that is not acceptable, distraction often helps.
Often a person with Prader-Willi Syndrome needs help in finding a solution to a problem or the correct way of executing a task,
Subtly model appropriate social interactions to help a person with Prader-Willi Syndrome to learn how to act in a social situation
Due to impaired executive functioning we strongly recommend the use visual schedules, checklists. Always break tasks into smaller steps, minimise distraction and plan ahead. Always prepare for changes in task/activity
Find a subtle way to join the person with Prader-Willi Syndrome in ‘double-checking’ that they have everything. This is particularly important if there are several activities on one day.
People with Prader-Willi Syndrome exhibit Low muscle tone and motor difficulties / dyspraxia. It is advisable to use assistive technology, allow for fatigue, consider sensorimotor issues and plan for development of gross motor skills
All people with Prader-Willi Syndrome will require ongoing support to help develop appropriate social skills i.e. conflict resolution, maintaining friendships and cooperating with others. It is important to provide support in group activities and facilitate social connection. People with Prader-Willi Syndrome are sociable but have poor social skills

It is important to remember that Prader-Willi Syndrome is All About Anxiety

“Persons with Prader-Willi Syndrome typically feel high levels of anxiety – all the time.

Maladaptive, unwanted behaviours are often attempts to reduce the level of anxiety the individual with Prader-Willi Syndrome is feeling.

High anxiety puts them in the fight or flight zone

Examples of this behaviour are repeated questions, excessive talking, and controlling, oppositional, argumentative or aggressive behaviour. Running away or hiding are also other examples of behaviour resultant from heightened anxiety levels.

Your attitude is important

The attitudes of others that surround and care for people with Prader-Willi Syndrome is very important.

Never argue with a person who has Prader-Willi Syndrome. This will only create further angst and escalate into a behaviour. People with Prader-Willi Syndrome are unable to control their emotions.
Always exhibit a firm but loving and caring demeanour towards someone with Prader-Willi Syndrome
If you notice someone with Prader-Willi Syndrome begin to show signs of an emotional outburst or behaviour, it is best to use the TI “Tactically Ignore” strategy. Let it go if it is not important, or respond once, let it go and ignore it.
Use humour but ALWAYS ensure you are laughing with the person with Prader-Willi Syndrome, NEVER at them
People with Prader-Willi Syndrome are eager to please, so where possible, praise positive behaviour, this will help build a wonderful relationship.
NEVER take a behavioural outburst personally. We understand that when someone has wronged you in some way, anger is a natural reaction. It is important to understand that people with Prader-Willi Syndrome, whilst unable to regulate their emotions are unable to identify personally how that have wronged you in that moment. You may notice that someone with Prader-Willi Syndrome may exhibit an emotional outburst and half an hour later, it as though it never happened.

We recommend that anyone who cares for someone with Prader-Willi Syndrome read through as much information within this website as possible, to ensure a thorough understanding of the requirements for safety and adequate support.

To request a detailed list of things to be aware of, please contact us at info@pwsavic.org.au

Prader-Willi Syndrome Association of Vic On-site Training and Education

The Prader-Willi Syndrome Association of Victoria Inc. (PWSA Victoria) is a volunteer led ACNC registered Australian Charity committed to working with service providers who care for people with Prader-Willi Syndrome. We can work with you and any support persons required to help create an environment in which people Prader-Willi Syndrome can safely and enjoyably access the community.

During the consultation process, we work closely with you to understand your requirements and develop a training session tailored to your needs. We offer several training modules including, but not limited to:

Understanding Prader-Willi Syndrome
Dietary Management and Prader-Willi Syndrome
Health and Wellbeing
Understanding and Managing Behaviour

For a consultation, please contact the Prader-Willi Syndrome Association of Victoria on 0451 797 284 or email us at info@pwsavic.org.au

Janice Forster – Food Train Concept