Prader-Willi Syndrome

The Arc of Alachua has successfully served people with Prader-Willi Syndrome since 1989. The Arc has served over 85 people with the syndrome and is currently serving sixty-one individuals. The improvement in these individuals' lives has been striking. We have shown that we can manage weight loss for these individuals in a much less restrictive environment than anyone in the field ever thought possible. The improvements in the quality of life for these individuals have been remarkable. These improvements include the resolution of many serious health issues including Type 2 diabetes, sleep apnea, and congestive heart conditions.

Hypothalamic dysfunction is thought to be the cause of the disordered appetite/satiety function characteristic of PWS. Compulsive eating and obsession with food usually begin before age 6. The urge to eat is physiological and overwhelming; it is difficult to control and requires constant vigilance.

Infants and young children with PWS are typically happy and loving and exhibit few behavior problems. Most older children and adults with PWS, however, do have many difficulties with behavior regulation manifested as difficulties with transitions and unanticipated changes. Onset of behavioral symptoms usually coincides with onset of hyperphagia (although not all problem behaviors are food-related) and difficulties peak in adolescence or early adulthood. Daily routines and structure, firm rules and limits, "time out," and positive rewards work best for behavior management.

The following areas are the three components to the Arc Treatment program.

Weight Management

A dietician sees each individual we serve who has PWS at least once each year. The dietician monitors nutritional intake, establishes the Ideal Body Mass (based on age, gender, and height), and prescribes the individual's allotted caloric intake each day. The individual him or herself then tracks their caloric intake each day on a specially designed calorie sheet. Prior to each meal the individual sits down with their calorie sheet and indicates in writing what they wish to eat. The caloric value of the food they chose is then deducted from their base calories and a new total is generated. Once the total number of calories remaining reaches zero the individual is done eating for the day. Our goal is to have individuals lose 1-2 pounds each week until their IBM is achieved and maintained.

Exercise Program

Exercise programs are established for each individual we serve who has PWS according to their physical ability and their doctor's recommendations. The exercise program is divided into "hard" and "easy" days. Hard days are done on a piece of exercise equipment and easy days are any form of continuous aerobic exercise. We start by identifying the individual's target heart rate based on age and gender. Our goal is to have individual's exercise 85% of available opportunities or more. Once the target heart rate is established we focus on perfecting the individual's exercise program. We do not want to start off too intense for fear of individuals refusing to participate. We start off with a program that the individual can complete that will burn some calories and elevate the individual's heart rate. Based on the individual's heart rate and respiration we fix the end point for their exercise program. Once we have established an achievable hard and easy day requirement we will gradually increase or decrease this criteria based on the individual's level of fitness, their participation, and their weight loss.

Behavior Modification

In addition to establishing healthy eating habits and some level of physical fitness we also use Applied Behavior Analysis to modify individual's behaviors. We deal with issues such as: excessive eating, skin picking, tantrums, elopement, and aggressive or destructive behavior. We also teach people to: do their laundry, balance a checkbook, shop for groceries, cook their own meals, and safely access the community. To accomplish this we must establish and maintain a predominantly positive social environment. We train staff to give clear contingencies and reinforce appropriate behavior while ignoring or redirecting inappropriate behavior. Staff are monitored monthly and reinforced for meeting competency criteria.

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