Family functioning and health-related quality of life in children and young adults with Marfan syndrome.

Abstract

Purpose: Evaluate family functioning (FF) and associations with quality of life (QOL) in a large, multicenter cohort of children and young adults with Marfan syndrome (MFS) who participated in the Pediatric Heart Network (PHN) Marfan Trial.

Methods: Of the 608 patients enrolled in the PHN Marfan Trial, 359 families completed one or more of the following: the General Functioning subscale of the Family Assessment Device (FAD), the Condition Management Ability module of the Family Management Measure (FaMM), and the Pediatric Quality of Life Inventory (PedsQL) at the final trial visit at three years. The correlations between FAD and FaMM scores and patient-related factors were examined. Linear regression was used to determine the relationship between FAD, FaMM, patient-related factors, and PedsQL.

Results: 25% of families by self-report and 9% of families by parent-report had impaired functioning (FAD score > 2). FAD and FaMM were inversely correlated (R=-0.41, p < 0.001). Higher (worse) FAD scores were correlated with more patient-reported symptoms (R = 0.17, p = 0.003) and with lower QOL (R=-0.28, p < 0.001), independent of other factors typically related to QOL.

Conclusions: In patients with MFS, family dysfunction and more symptoms were independently associated with worse QOL. This suggests that interventions addressing family dysfunction and symptoms may positively impact QOL for patients and families living with MFS.