Adherence and psychopathology in children and adolescents with cystic fibrosis

Background The striking increase in the lifespan of individuals with cystic fibrosis (CF) has largely been attributed to the dramatic improvements in treatment regimens. These treatments are time intensive and may interfere with healthy development and family function. The objective of this study was to investigate the association between psychopathology and treatment adherence in children and adolescents with CF. Methods Structured psychiatric interviews were performed on 52 patients with CF. Additional information on family function and youth behaviors were also collected. Youth and parent reports of adherence to the CF treatments were obtained and compared with the CF teams' records. Results The mean overall adherence to the CF teams' recommendations was 77-81% for the child and parent reports, respectively. Children with anxiety disorders and families who were more cohesive showed significantly higher rates of adherence to the CF treatments. In addition, children in families with a balance of structure and flexibility also report higher levels of adherence to the CF treatments. Conclusions Anxiety disorders in children with CF may be associated with increased adherence to the numerous CF treatment regimens. In addition, family patterns that are cohesive and balanced are better able to incorporate the CF treatments into family life.