Health-related quality of life and family functioning in parents of children with Barth syndrome: an application of the Double ABCX model

Abstract

Background: Living with children with disabilities has a significant impact on parental health-related quality of life (HRQoL) and family functioning. Barth syndrome (BTHS) is a rare, X-linked disorder that primarily affects males, presenting symptoms such as cardiomyopathy, neutropenia, muscle weakness, and growth delays. In this study, we investigated how a child’s functional performance, family cohesion, and satisfaction with healthcare affect parents of children with BTHS. Methods: Thirty-three parents of children with BTHS and 31 parents of age-matched unaffected children participated in this study. The parents completed a series of questionnaires. The Double ABCX model was applied to select measurement variables for this study. An independent samples t-test was used to compare HRQoL and family functioning between the two groups. Regression analysis was conducted to determine how a child’s functional performance, family cohesion, and satisfaction with healthcare affect HRQoL and family functioning of parents of children with BTHS. Results: The HRQoL and family functioning of parents of children with BTHS were significantly lower than those of unaffected children (p