Prader-Willi Syndrome (PWS) is a multifaceted and enduring disorder that poses significant challenges, including low muscle tone, sensory integration dysfunction, and cognitive and learning difficulties. However, the primary symptoms of PWS are emotional instability and hyperphagia - an uncontrollable and excessive appetite.

Individuals with PWS experience a constant and overpowering physiological urge to eat, coupled with a slower metabolism that leads to rapid weight gain. Due to weakened stomach muscles and a limited ability to vomit, even a single episode of overeating can result in life-threatening complications such as gastric rupture or tissue death. It is crucial to adhere to the Principles of Food Security* and restrict access to food at all times.

Despite the challenges, individuals with Prader-Willi syndrome generally possess a positive outlook on life and a fondness for people. However, they often struggle with managing their emotions and behavior when feeling frustrated or anxious. To assist them in better regulating their feelings and actions, it is important to be familiar with some general do's and don'ts when interacting with individuals with PWS.

DO's:

1. Keep all food items inaccessible except during designated mealtimes. Recognize that individuals with PWS cannot resist their physiological drive to obtain food. Secure all food sources as their quick hands can easily outpace your vigilance.

2. Display a daily schedule of activities and a set of rules. Review and discuss them at the beginning of each day to ensure clear understanding.

3. Provide transition prompts, such as 5, 3, and 1-minute warnings, before transitioning to the next activity. Individuals with PWS benefit from prior notice of even minor changes.

4. Remember that individuals with PWS respond exceptionally well to praise, encouragement, positive reinforcement, smiles, and hugs. Maintain a positive and uplifting demeanor, regardless of your own internal feelings.

5. Always speak to individuals with PWS in a calm manner. Raised, critical, or angry voices can heighten their already prevalent sense of anxiety. Ignore inappropriate behavior that may be an attempt to seek attention. Respond to raised voices or temper outbursts with soothing words like, "I understand you want xyz and feel frustrated. I'm sorry you feel that way."

6. Be specific about what you would like the individual to do, rather than focusing on what they should not do. For instance, say, "Please put all the items back in the box and close the lid. Thank you!"

7. If there is a disagreement, consider creating written contracts collaboratively to establish a shared understanding.

8. Consult the written schedule or contract to prevent power struggles or misunderstandings.

9. Recognize that logical reasoning may not be effective when an individual with PWS becomes upset or fixated on an idea. Demonstrating empathy by reflecting their feelings or desires back to them can help them move past their fixation.

10. Avoid ambiguity in your instructions. Instead of saying "In a while" or "Later," restate the timeframe as "After lunch, around 2:00."

11. Be attentive and listen when an individual with PWS needs to talk. Taking the time to genuinely listen and express empathy is invaluable. If they repeatedly ask the same question, inquire about their own thoughts on the matter, confirm it, and calmly inform them that the question has been addressed. Allow them to have the final say.

12. Understand that individuals with PWS often exhibit oppositional behavior due to the nature of their condition. Instead of asking a question when you should make a statement, provide clear instructions after offering 2-3 prior prompts. For example, say, "Okay, it's time to go now. Thank you for getting ready for the next adventure!"

13. Allow ample time for individuals with PWS to process the information you provide.

14. Recognize that individuals with PWS tend to be self-centered and focused on their own needs.

15. Safeguard your personal belongings by keeping them securely locked, as stealing, lying, and fabricating stories are common behaviors in some individuals with PWS.

16. Treat each individual with PWS with utmost respect and dignity.

By implementing these strategies, we can create a supportive environment that promotes positive behavior and enhances the overall well-being of individuals with Prader-Willi Syndrome.

Let us continue to strive towards understanding and providing the necessary care for those with PWS, ensuring they lead fulfilling lives.

Join Our Team of Compassionate Caregivers