Journal of Pediatric Psychology Advance Access originally published online on February 23, 2008
Journal of Pediatric Psychology 2008 33(7):728-738; doi:10.1093/jpepsy/jsn017
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This article appears in the following Journal of Pediatric Psychology issue: Series on Methodology Article [View the issue table of contents]
Psychosocial Functioning in Youth with Glycogen Storage Disease Type I
1Department of Psychiatry and Department of Pediatrics, University of Florida, 2Department of Psychiatry, University of Florida, 3Department of Psychology, University of Georgia, and 4Department of Pediatrics, University of Florida
All correspondence concerning this article should be addressed to Dr Eric Storch, Department of Psychiatry, University of Florida, Box 100234, Gainesville, FL 32610. E-mail: estorch{at}ufl.edu
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Abstract |
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Objective To assess the quality of life and psychosocial functioning among pediatric patients with Glycogen Storage Disease (GSD) types Ia and Ib. Methods Thirty-one youth with GSD types Ia and Ib and 42 healthy controls participated. Quality of life ratings from the GSD types Ia and Ib sample were compared with a previously reported clinical comparison sample. Children completed measures of quality of life, loneliness, family functioning, and sibling relationship quality (e.g., warmth, conflict). Parents completed measures of parental distress, parenting stress, child adaptive behavior, and child emotional and behavioral functioning. Results Quality of life was generally lower in youth with GSD relative to healthy controls but similar to those with a chronic illness. Children with GSD were rated as having more internalizing symptoms, social problems, and lower independent functioning relative to healthy controls. Parents reported greater distress and parenting stress relative to healthy controls. Conclusions The presence of GSD types Ia and Ib are associated with reduced quality of life and independent functioning, and elevated levels of internalizing distress and parental stress relative to healthy peers. Relative to their children, parents generally reported that their child was more impaired, which suggests the need for multiple informants during assessment and active parental involvement during psychological treatment. These points should be kept in mind when assessing and treating youth with this disease and their families as psychological interventions that target areas of concern (e.g., adherence, coping with having a chronic disease) may be helpful for improving child and family outcomes.
Key words: adjustment; children; glycogen storage disease; quality of life.
Received September 21, 2007; revision received February 5, 2008; accepted February 11, 2008