Perceived Quality of Life Among Children With Prader-Willi Syndrome in Comparison to Obese Children

Elizabeth Hayslett¹, Norma Terrazas¹, Diana Arceo¹, Stephanie Abrams¹, William Klish¹, Ann Scheimann^2,1

Division of Pediatric Gastroenterology and Nutrition, Texas Children’s Hospital, Houston, TX¹, Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Hospital, Baltimore, MD²

Introduction: Prader-Willi syndrome is a genetic disorder localized to chromosome 15 characterized by early onset of obesity, hyperphagia, hypotonia, decreased linear growth attributed to underlying growth hormone deficiency. A limited variety of treatment options are available including growth hormone therapy, dietary modification including restriction of access to food, and supplemental therapies. During adulthood, the majority of individuals with Prader-Willi syndrome require careful supervision of food and money. To the best of our knowledge, there is no published data on the perceived quality of life among children with Prader-Willi syndrome. Published data by Schwimmerⁱ, et al., note that the perceived quality of life scores for obese children are similar to those of pediatric oncology patients.

The objective of this study is to objectively characterize the perceived quality of life among children with Prader-Willi syndrome as reported by the children and their parents and compare the measured quality of life with BMI z-score matched obese control subjects among obese children followed in the obesity clinic at Texas Children’s Hospital.

ⁱSchwimmer JB, Burwinkle TM, Varni JW. Health-related Quality of Life Among Severely Obese Children and Adolescents. JAMA 2003;1813-9.