For children diagnosed with Prader-Willi Syndrom (PWS) and their families, it can be difficult to deal with the effects of the condition.
PWS is a rare genetic disorder that causes an insatiable appetite and subsequent weight gain due to chronic overeating. The symptoms include intellectual disabilities, co-morbidities like breathing issues and diabetes, and behavioral issues related to food-seeking.
One of the first steps in treating children with PWS is managing their weight and encouraging weight loss. Jean Park, MS, MBA, RD, LD, Director of Nutritional Services and Monica LaBrie, Ph.D., BCBA, LBA, Behavioral Health Services Program Director, who help children in the Changes Health & Wellness Program at Nexus Children’s Hospital, discussed their recommendations for supporting weight management in children with PWS.
Perhaps the most important aspect of weight management for children with PWS is a healthy, nutritious diet. “Every child has individual needs, so my job is to work closely with the medical team and make changes to their meal plans based on their weight loss progress,” said Jean, who is a registered dietitian. “I help plan their meals and work in the kitchen coordinating food preparation.” Dietitians for school-age children with PWS recommend restricting calories from 800-1,000 per day for children struggling with obesity and including protein, complex carbohydrates, and healthy fats while eliminating sugar and processed foods as much as possible. Diet restrictions aid in weight loss by limiting the number of calories the children consume each day, while a balanced food plan gives them the nutrients their bodies need to stay healthy. Learning more about the food they eat and how it affects their weight at Nexus Children’s Hospital helps children and their families build a framework for continued meal planning success at home after leaving the program.
Exercise and physical activity are a big part of the Changes Health & Wellness Program at Nexus Children’s Hospital. “It’s important for our patients to exercise to support their weight loss at our hospital and when they discharge,” Jean said. “They learn basic exercise programs and participate in physical activities multiple times a day.” Even the slowest kinds of exercises such as walking burn calories, so frequent exercise is recommended for children with PWS as a critical component of their weight management plan. “Losing the weight through exercise paired with healthy eating habits helps reduce comorbidities like breathing issues and diabetes,” Jean added.
Meal & Snack Schedule
“At Nexus Children’s Hospital, we implement a strict, structured schedule around food,” said Dr. LaBrie. “Meals and snacks are always at the same time every day. That predictable schedule allows the kids to always know the next time they’re going to eat, which in turn helps control their aggressive food-seeking behaviors.” A planned meal schedule helps control a PWS patient’s overeating, and as children get used to a regimented food schedule, it becomes progressively easier to manage their behavior as they learn to expect their next meal or snack at certain times. As one of the only facilities in the U.S. that address both the medical and behavioral challenges that children with PWS face, Nexus Children’s Hospital works to set their patients up for success after discharging by treating the disruptive mealtime behaviors that are common in children with PWS.
Rewarding children with non-food incentives for not engaging in aggressive behavior has proven to be an effective way to encourage them to reach their goals. “We have a prize box that is filled with things the kids enjoy, and we let them pick out a prize when they’re behaving well and meeting their goals,” explained Dr. LaBrie. “Some kids may need to be rewarded with prizes more frequently – our positive reinforcement model is individualized for each kid, just like their diets.”
“My primary suggestion for families of children with PWS would be to set up a structure for home,” remarked Dr. LaBrie. “It’s easy for the kids to follow their meal schedules here at the hospital, but parents need to put in the work to maintain that schedule at home.”
Outside of a structured setting, parents may have a hard time maintaining dieting and keeping food away from their children and are often more willing to give in to a child’s aggressive behaviors. “The kids at Nexus gain confidence in their abilities while they’re here because they often haven’t been successful with weight loss at home,” added Jean. “That confidence is motivational, and it can help parents implement a home program once they discharge.”
Jean and Dr. LaBrie know the work they do in the Changes Health & Wellness Program is important to bettering the lives of the children they work with. “PWS is a difficult thing for these families to deal with,” said Jean. “We really hope that the parents of the children in our program have a moment to reset while their kids are here so that they can go home with a fresh regimen that will make their lives easier.”
To learn more about Nexus’ Changes Health & Wellness Program, click here.