1 / 28

Behavior Characteristics of Prader-Willi Syndrome Cindy Stolp

Behavior Characteristics of Prader-Willi Syndrome Cindy Stolp. Prader-Willi syndrome (PWS). PWS is a chromosomal disorder from a deletion or disruption of genes in the proximal arm of chromosome 15. Deletion 70%. Chromosome 15. Imprinting defect – 5% (pwsausa.org). Maternal Disomy – 25%.

pearly
Download Presentation

Behavior Characteristics of Prader-Willi Syndrome Cindy Stolp

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Behavior Characteristics ofPrader-WilliSyndromeCindy Stolp

  2. Prader-Willi syndrome (PWS) PWS is a chromosomal disorder from a deletion or disruption of genes in the proximal arm of chromosome 15 Deletion 70%

  3. Chromosome 15 Imprinting defect – 5% (pwsausa.org) Maternal Disomy – 25%

  4. 1 in 12-15,ooo Rare disorder But PWS is the most common genetic cause of obesity All races Both sexes (pwsausa.org) Prader-Willi Syndrome

  5. Diagnosis often delayed until early childhood. Genetic testing detects nearly 100% of Prader-Willi Syndrome Diagnosis

  6. Difficulty feeding Poor sucking reflex Failure to thrive Hypotonia Early developmental milestones are delayed Newborns

  7. Difficulty with feeding transitions into hyperphagia After the 1st year Victor 9 months Victor 24 months

  8. What doesn’t work Appetite suppressants What has worked Extremely low-calorie diet (their entire life) Limited access to food Treatment

  9. Prader-Willi Food Pyramid • Typical person – 1600 – 2800 calories per day • Person with PWS – 800 – 1200 calories per day • Vegetables make up the bulk of the recommended diet

  10. Almond Shaped Eyes Eye Abnormalities (Esotropia & myopia) Thin upper lip Narrowing of the temples Common Facial Characteristics

  11. Short stature Relatively small hands Relatively small feet Between 1-6 progress into development of morbid obesity Common Characteristics

  12. Primary care physician Ophthalmologist Pediatric endocrinologist OT PT Speech Therapist ECSE & Special Education Recommended Team Members

  13. Temper tantrums Stubbornness High pain threshold Sleep disturbances Obsessive compulsive behaviors Repetitive thoughts Verbalizations Collecting & hoarding possessions Picking at skin Behavior Problems

  14. Garbage Leftovers from cafeteria trays Scraps off the floor Frozen food Stealing to obtain food Dangerous High threshold for pain Decreased ability to vomit Need to Seek Food

  15. Need for Routine & Consistency Strategies • Visual schedules or lists • Discuss changes in schedules or staff • Provide praise for flexibility • Breakdown procedures into concise steps

  16. Obsessive Thinking Strategies • Less is best – give smaller amount of work at one time • Don’t give more info than necessary • Use”strategic timing” – schedule an activity they have difficulty ending right before snack or lunch

  17. Emotional Control • Strategies • Over stimulation in crowded hallways – enter & exit school in a less populated area • At the beginning of the day, review the daily schedule to help decrease anxiety • Encourage communication • Listen carefully • Teach, reteach & practice frustration coping strategies • Don’t try to reason with them when out of control

  18. Food Cravings • Educate all people working with the student • They will lie to get food, “I didn’t have breakfast this morning” – check with parents • Avoid letting them have money – • money buys food • Don’t delay snacks • Limit the visibility of food • Be aware of smells – popcorn • Avoid using food as a reward or incentive

  19. Poor Stamina • Strategies • Send them on an errand – take a walk • Schedule high energy activity after lunch • Deep breathing activities – balloons • Schedule rest times or quiet activities

  20. Scratching & Picking Skin • Strategies • Keep hands busy – playdoh, hand held games • Keep nails short • Apply lotion liberally – keeps the skin moist and slippery

  21. Baby monitors Security system with alarms Motion detectors 160 degree mirrors (see what’s going on behind you) Security

  22. Recent research • The brain’s pleasure center • 3x as much neurotransmitter gammaaminobutyric acid in their blood • Neurotransmitters inhibit dopamine

  23. I’m still hungry

  24. 1999 U.S. Supreme Court • “Developmentally disabled people must be given the choice to live in the community if they can be supported there.” • www.pwsaa.org

  25. Learning disabilities are always present Ranging from Low normal intelligence To mental retardation Global Delays

  26. Mainstreamed into classroom Need additional physical activity periods

  27. Appear to be Lazy & lack motivation Manipulative, Lying & stealing Lack of empathy & Lack of conscience Stubborn Temperamental Controlling Perspective on Behaviors(Teresa Kellerman)

More Related