What is Prader-Willi Syndrome?

Prader-Willi Syndrome (PWS) is a complex genetic disorder that occurs due to an abnormality on chromosome 15. It affects various aspects of an individual’s physical, cognitive, and behavioral development.

Prader-Willi Syndrome is a rare, complex genetic disorder that affects both males and females from birth.

It causes low muscle tone with consequent motor developmental delays, mild to moderate learning difficulty, and emotional and social immaturity, which can lead to challenging behaviors.

During childhood, an overwhelming and insatiable chronic appetite usually develops which can lead to food seeking, stealing, and life-threatening obesity. This can be well managed with rigorous food management and exercise regimes. Basically, PWS means my brain tells the individual’s brain they are still hungry all of the time. The drive to eat overwhelms rational decision-making.

Here are some key points to help people understand PWS:

1. Genetic Cause: Prader-Willi Syndrome is typically caused by a deletion of a small piece of chromosome 15 from the father or by other genetic anomalies that result in the loss of specific genes on the chromosome.
2. Excessive Hunger: One of the hallmark features of PWS is hyperphagia, an insatiable appetite, and an overwhelming drive to eat. Individuals with PWS have a constant feeling of hunger, which can lead to uncontrollable eating and significant weight gain.
3. Obesity and Related Health Issues: If the eating behaviors are not managed, individuals with PWS can develop obesity and related health problems, such as type 2 diabetes, cardiovascular issues, and respiratory difficulties.
4. Cognitive and Behavioral Challenges: People with PWS may have mild to moderate intellectual disabilities, learning difficulties, and behavioral challenges. Behavioral features can include stubbornness, repetitive behaviors, temper tantrums, and obsessive-compulsive tendencies.

Why do people with Prader-Willi Syndrome need to control their food intake and follow strict diets?

One of the most prominent symptoms of Prader-Willi Syndrome is hyperphagia, an insatiable appetite, and an overwhelming drive to eat. Individuals with PWS have a constant feeling of hunger, which can lead to uncontrollable eating and significant weight gain. If an individual with PWS does not follow a strict meal resume they are at risk of significant weight gain causing various health conditions such as obesity and diabetes. Following a meal plan ensures the individual is living a healthy and happy life while maintaining a reasonable weight.

Why is it important to follow a strict meal plan and exercise regularly?

Following a meal plan ensures the individual is living a healthy and happy life while maintaining a reasonable weight. Individuals with Prader-Willi Syndrome gain weight very easily which affects their quality of life and health and well-being. It is important to keep a consistent routine for someone with PWS to help mitigate their food-related behaviors. If the eating behaviors are not managed, individuals with PWS can develop obesity and related health problems, such as type 2 diabetes, cardiovascular issues, and respiratory difficulties.

What can we do to improve their health and wellbeing?

To improve not only the health and well-being of an individual with Prader-Willi Syndrome but also their quality of life, we can implement some of the following practices:

• Dietitian / Meal plans: By engaging with a dietician, they can create a custom- and tailor-made meal plan to suit the dietary requirements of a PWS individual. Meal plans can detail the number of foods allowed, as well as approved and non-approved foods to ensure optimal health.
• Positive Behavioral Therapy (PBS): By engaging in positive behavior supports a clinician is able to provide resources and strategies to families and carers of individuals with PWS to help manage and mitigate behaviors such as stubbornness, repetitive behaviors, temper tantrums, and obsessive-compulsive tendencies.
• Restrictive practice’: Restrictive practice’ means any practice or intervention that has the effect of restricting the rights or freedom of movement of a person with a disability. A behavioral therapist is able to come and complete an assessment to determine if a restrictive practice is suitable. This may include locking fridges or cupboards to avoid overeating. PBS also monitors to see if fade-out plans are suitable to remove the restriction if needed. This needs to be approved by NDIS.
• Support Staff Training: Therapist care teams can organize a “CTM” Care team meeting to provide training to family and staff on strategies to assist a PWS individual.
• Regular Exercise: Regular exercise is very important to maintain a healthy body weight and healthy lifestyle. GPs and Exercise Physiologists can help make a tailor-made exercise plan that can be followed by family and staff.
• Occupational Therapy: By engaging with an Occupational Therapist they can assist in achieving goals relating to increasing independence as well as helping Prader-Willi Syndrome individuals find ways to function more effectively and confidently and identify strengths and difficulties Prader-Willi Syndrome individuals face in everyday life.
• Speech Therapy: By engaging with a speech therapist, individuals with PWS can improve skills and develop strategies to communicate their wants and needs as well as help to improve the clarity of speech and language.

How can a Support Coordinator assist an individual with Prader-Willi Syndrome?

By engaging with a Support coordinator, they are there to assist you by linking you with services such as Dietitians, Occupational Therapy, Behavioral Therapy, etc.  It is their role to monitor progress and work alongside allied health supports to identify what supports and services an individual with Prader-Willi Syndrome require, and how agencies like this NDIS can help. Here at iDeal Care Services, we have vast experience assisting individuals with PWS to live a safe and healthy life while remaining independent. By linking to support services such as Supported Independent Living (SIL) it enables individuals with PWS to live independently while following allied health recommendations, this also mitigates carers burnout on families of PWS individuals. We advocate for the needs of our participants to ensure they receive the support they need to achieve their goals.

How can a Support Worker assist an individual with Prader-Willi Syndrome?

Support Workers play a vital role in supporting individuals with Prader-Willi Syndrome (PWS). They can get assistance to increase their social and community access to help build independence while being supported to stay safe, happy, and healthy. Support workers can build up individuals’ confidence and self-esteem by gently encouraging and motivating them. Support workers can help individuals try new things they may not have done before. Support workers provide the physical and emotional support that someone might need to achieve their goals that their family is unable to provide.

How can Supported Independent Living (SIL) assist an individual with Prader-Willi Syndrome?

Supported Independent Living (SIL) has many benefits for individuals with Prader-Willi Syndrome (PWS). These benefits include:

• Becoming independent by moving into their own space and out of the family home.
• Making new friends and building new relationships.
• Immediate access to support when needed.
• More opportunities.
• Peace of mind to family/carers knowing that PWS individuals are living life in a safe and healthy

Individuals with Prader-Willi Syndrome are supported to live independently while learning new ways to build skills to complete daily tasks that they may not be able to do if they live unsupported. SIL also provides peace of mind to families of those with PWS knowing that they are supported to live their lives while looking out for their health. SIL also gives individuals a sense of community and belonging, Supported Independent Living is often 1:3, meaning that individuals do not live alone, they are matched with a housemate to ensure they are building and maintaining new friendships with like-minded people.

Contact Our team to get help for Prader-Willi Syndrome  Call Us at 03 8820 8474.