Family Functioning and Treatment Adherence in Children and Adolescents with Cystic Fibrosis

Abstract

Background: Cystic fibrosis (CF) is one of the most challenging pediatric illnesses for families to manage. There is, however, limited research that considers the associations between family functioning and treatment adherence in children and adolescents with CF. Methods: Nineteen children with CF (mean age = 12.42 years, mean forced expiratory volume in one second (FEV 1 ) = 90.9% predicted) and their families participated in the study. Caregiver and child participants completed interview-based assessments and were then videotaped during a family mealtime. Results: Mean scores on several domains of family functioning fell in the ''unhealthy'' range. Better family functioning was found among older children. Better family functioning was also associated with better adherence to antibiotic treatment and worse adherence to enzymes. Conclusions: Findings suggest that family functioning may be an important correlate of treatment adherence in children and adolescents with CF. Future research should replicate these findings in larger samples of children and adolescents with CF.

The lived experience of adults with cystic fibrosis: what they would tell their younger selves about the gut

“…The role of families in passing on information about diet and Creon was essential to their understanding and confidence and impacted on their Creon adherence. The link between family functioning and treatment adherence in CF has been identified previously , with families that find a balance between cohesion and flexibility reporting higher rates of adherence . In the present study, participants in supportive family units were, as children, confident to ask questions and seek the explanations they needed from the CF team; however, all participants urged children to be assertive, seek support and be actively involved in their dietary care.…”

Socioeconomic status and health outcomes: cystic fibrosis as a model

“…Children with CF are affected by the toll their disease takes on their parents/caregivers, leading to anxiety, depression, and impaired personal and family functioning [104][105][106][107]. A significant proportion of mothers of children with CF show depressive symptoms soon after the diagnosis of their child, independent of the degree of the child's illness [108], and many parents report significant psychological distress years afterward [109,110].…”

“…The mechanism of this relationship is likely multifactorial, including decreased adherence and self-management, which are affected negatively by depression [115]. Several studies underscore the role of family functioning [104,105]. Since family functioning has a major impact on child development [116], it likely exerts a powerful influence on the progression and severity of CF, affecting the child's clinical trajectory throughout life [117].…”

Parental Depression and Pancreatic Enzymes Adherence in Children With Cystic Fibrosis

“…Our study did not measure the processes by which parental depression may influence self-management, but it likely occurs through the dynamic interplay between self-management processes identified by the PSMF, including family functioning, parentchild communication, parental beliefs about treatment efficacy, family support, and parental supervision of treatments. 14,[17][18][19][20] There is evidence from 2 cross-sectional studies in pediatric patients with CF that the relationship between depression and treatment adherence may depend on 6…”

“…All of these processes have been linked to treatment adherence in CF. 14,[17][18][19][20] Given the elevated rates of depression documented in parents of children with CF and the theorized impact of parental depression on treatment adherence, the goal of the present article was to test whether parental depressive symptoms were predictive of adherence to enzyme regimens and whether that impact also affected short-term health outcomes. In accordance with the PSMF, we hypothesized that parental depressive symptoms would contribute to worse adherence, which in turn, would lead to worse weight outcomes.…”