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Indian Prader-willi Syndrome Association

Indian Prader-willi Syndrome Association. Welcomes you all. What is prader-willi Syndrome?. A disorder of chromosome 15 Occurrence in poplulation 1:15000 (both sexes). Characteristics of Prader-willi Syndrome (PWS). low muscle tone Poor growth in early infancy

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Indian Prader-willi Syndrome Association

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  1. Indian Prader-willi Syndrome Association Welcomes you all

  2. What is prader-willi Syndrome? A disorder of chromosome 15 Occurrence in poplulation 1:15000 (both sexes)

  3. Characteristics of Prader-willi Syndrome (PWS) low muscle tone Poor growth in early infancy Small external sexual organs Excessive eating Learning difficulties Difficult behaviours Major medical concern : EXTREME OBESITY.

  4. Hypothalamus :command control • Appetite/satiety • Emotional regulation • Temperature regulation • Alertness and sleep • Hormones, puberty and sexual drive • Memory

  5. PWS requires … • WEIGHT control • CALORIE control • FOOD control

  6. WEIGHT MANAGEMENT Energy out Energy in

  7. FATS & SWEETS USE SPARINGLY MEAT,POULTRY FISH,DRY BEANS EGGS 1-2 SERVINGS DAILY 2 OZ. MILK,YOGURT CHEESE GROUP 2 SERVINGS DAILY BREAD,CEREAL FRUITGROUP RICE & PASTA 4SERVINGS 3-5 SERVINGS DAILY DAILY VEGETABLES GROUP 6-8 SERVINGS DAILY Prader-willi food pyramid

  8. EXERCISE • Daily or near daily routine (e.g.. 6 days a week) • Walking is safest; less effort dependent • “come exercise”, never “go exercise” • Schedule exercise period(s) before meal(s) • For weight loss walking 1 hour per day is desired. • Have a plan.

  9. Common … Asking for more Whining, crying for food. Sneaking food in home. Lying manipulating to get food. Others … stealing food outside of home/shoplifting/burglary. Phoning out for food. Stealing money to buy food. Breaking locks, stealing keys, dismantling doors. Running away to find food. Pushing, shoving destroying to get food. Food seeking behavior

  10. Man to man Close (1:1) supervision when child is outside the “ZONE”. Food control Zone defense • locks • Rules • Family • School • Child

  11. Food security Behavior and weight are managed by the same methods. lb.

  12. doubt anxiety stress hope disappointment

  13. STRESS BEHAVIOR PROBLEMS irritability Arguing Tantrums Self injury

  14. Food locked or inaccessible • Supervision in food accessible locations • No trips to grocery stores • Trips to restaurants are pre-planned • No participation in food preparation No falsehopes

  15. No serving bowls; plate prepared in kitchen • No seconds* • Careful planning for special occasions • NO giving in • Do not negotiate about food • No unplanned treats • Parental custody of money No falsehopes

  16. Opportunity • Time unsupervised • Locks unlocked • Food left out • Friends over • Parties • Birthdays • Holidays • Past history of making expectations. • “just this once” No falsehopes

  17. USING FOOD REWARDS • Sugarless gums (daily) • Crystal lite (daily) • Diet soda/”pop” (weekly) • Save “bags of veggies” for special occasions when other people are eating a lot

  18. “JUST THIS ONCE” • Avoid spontaneity; it looks like capriciousness • Plan exceptions; before your child thinks of it. • Tell your child about the exception • Be specific • Do not make the exception too momentous (don’t raise expectations) • Avoid buffets/ all you can eat*

  19. Food insecurity Punitive attitude Angry caretakers Changes in caretakers Life transitions Changes in family constellations Changes in schedule Holidays/ vacations SOURCES OF STRESS

  20. INFRASTRUCTURE Teams Rules Incentives Consequences Knowing what's what (Reminders) Yelling for help (Contingency plans)

  21. TRAIN YOUR TEAM :BASIC PHILOSOPHY • Never attribute behavior problem to “PWS” • Hold your child accountable for his/her own behavior. • Acknowledge that because of PWS he needs more help to control behavior and then provide that help.

  22. TRAIN YOUR TEAM :BASICS • Never threaten • Never bribe • Never argue • Never give in • Calm voice • Positive statements • Food security principles

  23. TRICKY RULES • Based on need for No Doubt and No Hope • Avoiding temptations • Continuous supervision Mantra : “Everyone has rules.” (Not: “Everyone has the same rules.”)

  24. INCENTIVES • Social • Praise, approval • Tangibles 1. Stickers 2. Tokens 3. Special attention 4. Food incentives

  25. NON-FOOD INCENTIVES:(USE EVERYTHING) • Computer time • TV time • Mummy time • Daddy time • Game time • Stickers and Star charts*

  26. TRICKY CONSEQUENCES • Natural (e.g. loss of privilege) • Planned (e.g. time out, ignoring) • Includes loss of incentives.

  27. TRICKY HOW TO USE CONSEQUENCES : • Do not threaten with consequences; remind child of the incentive before it is lost. • Loss of incentive is automatic, stated in matter-of fact tone with reminder next opportunity to earn scheduled incentive. • Expect tantrum. • Be emotionally supportive of the child in his disappointment but unyielding, unwavering.

  28. TRICKY Knowing what’s what : • Verbal reminders of schedule, rules and incentives (no threats). • Posted schedules or hand held schedules. • Posted menus • Star charts • Repetition and consistency are your friends.

  29. RESPONSE Most important goal of your response to a behavioral event is to do nothing to encourage the same behavior in the future. DO NOT expect your response to END the behavior.

  30. DURING Ignoring Redirecting Delaying decisions Managing your anger AFTER Consequences Apology Forgiveness Debriefing with problem solving. BASIC SKILLS AND TOOLS :

  31. THANK YOU.

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