Lecture Presenters: Barbara Dorn, RN, BSW and Paul White, MS

1. Issues in Developmental DisabilitiesPrader Willi SyndromePart 2: Support Service & Care TakingQuestions & Answers Lecture Presenters: Barbara Dorn, RN, BSW and Paul White, MS

2. Video

3. Least Restrictive Living Environments • The meaning of “least restrictive” for person with PWS • Promoting choice, independence, and self-correction • For people with PWS, self-corrective services may not be an option • May need to set more limits, provide more structure, and use more intensive behavioral support • Caregivers may need to re-educate themselves around these issues

4. Residential Services • Pros and cons in provision of residential services • Mixed versus all persons with PWS • Issues pertaining to food access • Number of people living together • Need for sameness • Two-person model • Group living - 6 to 8 may be more manageable • Individualized programs

5. Behavioral Response and Emotional Support • Persons with PWS – control over temper and emotions • People with PWS may require vigilance • Tenuous emotional control • Effect of changes in hypothalamus regulation • Once emotional control is lost, may be difficult to come around • May lack knowledge and skills in handling frustration, anxiety and anger

6. Food Intake • Choice between limiting food or giving in and avoiding temper outburst • Caregiver needs to be proactive • All food issues should be planned for • Issues with new staff • Idea is to neutralize food

7. Intervention and Support Consultation for Families and Support Workers • Frequent situations for behavior consultants • Number one issue – lack of understanding • Anger management • Self-injurious behavior • Increase supervision • Teach replacement behaviors • Loosening/tightening of structure • Education of community partners

8. Behavior Management • Use of consequences for behavior management • Yes, if harm to self, others or property • Need to be immediate • Reason shouldn’t be punitive • Community outings can be motivating • Alternative methods to handling anger and frustration • Example of stealing food • Need to consider sequencing, processing deficit

9. Primary Advice • Advice regarding behavior to parent, caregiver, teacher or support person • Develop a positive relationship • Involves a balance between compassion/support and setting limits • Always be a step ahead • Food issues and structuring routine and predictability • Use of visuals, writing things down to accommodate short-term memory • Teaching and use of repetition and obsessive compulsive tendencies

10. An Ethical Issue • Should person be allowed to eat as much as they want? Do we have right to limit food? • Opinion – should be limiting access to food • Comparison/contrast with alcoholics – biological component • Analogy of person with severe anorexia • Situations where person with PWS in escalated state • Use of diversion, avoid reasoning, limit talking, avoid confrontation, and neutralize situation

11. Mental Health, Aging, and Illness • People with PWS and higher incidence of mental illness • Research shows more significant mental health issues and psychosis in people with PWS due to maternal uniparental disomy • May be part of PWS, but inconclusive evidence • Weight issues and normal life span

12. Comments Related to Focus in Research • New areas in research • Genetics – subdivision of people with deletions • Behavior and medications – SSRI (Prozac, Luvox, Celexa), and 5-HT (precursor to serotonin) • Obesity – research should revolve around hypothalamus • Gastric bypass surgery for morbid obesity • Ghrelin hormone levels