Psychosocial functioning in Barth syndrome: Assessment of individual and parental adjustment

Marni L. Jacob*, Carly Johnco, Brittney F. Dane, Amanda Collier, Eric A. Storch

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

7 Citations (Scopus)


Barth syndrome is a rare, x-linked genetic disorder. Few studies have examined psychosocial functioning in this population. The current study examined the psychosocial adjustment in individuals with Barth syndrome, as well as their parents. A secondary aim was to examine demographic as well as psychological correlates and predictors of poorer adjustment in order to identify relevant areas for intervention. Individuals with Barth syndrome completed measures of psychosocial functioning, health-related quality of life, and attitudes toward their illness. Parents completed measures relating to their child’s psychosocial functioning and reported on their own psychological functioning and coping. Results indicated that the majority of individuals and parents reported normative levels of psychological functioning. Younger age was associated with poorer health-related quality of life in some domains. Increased levels of internalizing symptoms were associated with poorer psychosocial functioning in individuals with Barth syndrome. Having a child with externalizing symptoms was associated with increased emotional symptoms for parents, as were certain maladaptive coping strategies. Clinical implications are discussed.

Original languageEnglish
Pages (from-to)66-92
Number of pages27
JournalChildren's Health Care
Issue number1
Publication statusPublished - 2 Jan 2017
Externally publishedYes


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