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01-12-2007 | Original Research

Psychosocial Issues in Families Affected by Maple Syrup Urine Disease

Authors: Wendy Packman, Shelly L. Henderson, Indira Mehta, Rama Ronen, Dean Danner, Beth Chesterman, Seymour Packman

Published in: Journal of Genetic Counseling | Issue 6/2007

Abstract

The primary aim of this study was to ascertain the psychosocial issues faced by families affected by maple syrup urine disease (MSUD). The psychosocial adjustment and quality of life of children with MSUD were also described. Participants included 55 families and their children (ages 5 to 18 years) and teachers. Measures included a MSUD Family Survey, the Behavior Assessment System for Children (BASC) and the Pediatric Quality of Life Inventory (PedsQL). Parents reported via the MSUD Family Survey that the greatest sources of stress were financial and emotional. Many parents reported difficulty interacting with the medical staff and with schools. On the BASC, half of the children fell within the average range in psychosocial adjustment, although there were elevations in scales measuring attention, hyperactivity, and learning problems. On the PedsQL, the mean quality of life scores were closer to children with cancer than to a healthy sample. Despite the emotional and financial burden, parents reported that MSUD has also had a positive influence on their lives, leading to a world-view that is more compassionate and caring.

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Title: Psychosocial Issues in Families Affected by Maple Syrup Urine Disease
Authors: Wendy Packman
Shelly L. Henderson
Indira Mehta
Rama Ronen
Dean Danner
Beth Chesterman
Seymour Packman
Publication date: 01-12-2007
Publisher: Springer US
Published in: Journal of Genetic Counseling / Issue 6/2007
Print ISSN: 1059-7700
Electronic ISSN: 1573-3599
DOI: https://doi.org/10.1007/s10897-007-9114-3

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Psychosocial Issues in Families Affected by Maple Syrup Urine Disease

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