Quality of life and parental adjustment in pediatric pulmonary hypertension.

Background: This study examines the impact of pulmonary hypertension (PH) on the quality of life (QoL) of affected youth, as well as the relationships among PH disease severity, parental adjustment variables, and family relational functioning.

Methods: Subjects were 47 eligible parents of children with PH aged 2 to 18 years who were evaluated at Boston Children's Hospital. Measures of patient QoL and of parental stress, coping, and adjustment were administered to the caregivers. Clinicians rated illness severity and family relational functioning.

Results: Youth with PH scored significantly lower than healthy norms on a measure of parent-reported QoL (total, psychosocial, and physical QoL, each P < .0001). The parents reported encountering stressful events more frequently than published norms of parents of children with cancer (P < .0001). Thirty-four percent of parents of children with PH met criteria for presumed psychiatric diagnosis, and they reported using psychologic coping strategies significantly more often than a normative sample. A summary parental stress measure correlated inversely with child QoL, an effect that held true even after controlling for disease severity (P = .03).

Conclusions: PH takes a major toll on children and their families. Decreased QoL among youth with PH was significantly associated with high levels of parental stress, over and above the effect of illness severity. These findings suggest that interventions to better support the caretakers of affected children and adolescents should accompany medical treatment advances so as to improve QoL for patients facing pediatric PH.