Adolescents' Spirituality and Cystic Fibrosis Airway Clearance Treatment Adherence: Examining Mediators.

OBJECTIVE: Adolescent cystic fibrosis (CF) treatment adherence is a significant multidimensional issue. Using the Theory of Reasoned Action (TRA), this study examined the role of spiritual factors in adherence. METHODS: Forty-five 11-19-year-olds diagnosed with CF completed questionnaires concerning psychosocial, spiritual, and adherence-related constructs and Daily Phone Diaries to calculate treatment adherence. Exploratory Factor Analysis identified two spiritual factors used in subsequent analyses. The mediating roles of attitude toward the treatment's value (utility), subjective behavioral norms (the product of perceived behavioral norms and one's motivation to comply with them), self-efficacy for completing the treatments and treatment intentions in the relationship between spiritual factors and treatment adherence were tested with path analysis. RESULTS: Lower 'spiritual struggle' and greater 'engaged spirituality' predicted treatment attitude (utility) and subjective behavioral norms, which, together with self-efficacy, predicted treatment intentions. Finally, treatment intentions predicted airway clearance adherence. CONCLUSIONS: Findings were consistent with the TRA. Engaged spirituality supports pro-adherence determinants and behavior. Spiritual struggle's negative associations with outcomes warrant screening and intervention.

Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use.

OBJECTIVE: To compare lung function and nutritional outcomes in cystic fibrosis (CF) for 2 birth cohorts in our CF center. STUDY DESIGN: Patients with CF born between 1985 and 2000 treated in our CF center before age 5 years were included. The patients were divided into 2 equal birth cohorts for comparison: birth cohort 1 (born between 1985 and 1992) and birth cohort 2 (born between 1993 and 2000). To compare lung function, we used forced expiratory volume in the first second (FEV(1))% predicted and FEV(1)% predicted slope from age 6 to 12 years. We hypothesized that we would find significant improvements in lung function and nutritional outcomes in our patients with CF. RESULTS: The patients born between 1993 and 2000 (birth cohort 2) had better lung function, a slower rate of decline in lung function, and better nutritional outcomes compared with those born between 1985 and 1992 (birth cohort 1). Factors associated with a slower rate of decline in lung function in both groups were a higher baseline body mass index (BMI)%, a slower BMI% rate of decline, absence of chronic Pseudomonas aeruginosa respiratory infection, and initiation of dornase alfa (Pulmozyme) therapy before age 9 years. CONCLUSION: Our results demonstrate dramatically improved lung function and nutritional outcomes in the children with CF in our center. The improvements in lung function outcomes are associated with better nutrition, fewer chronic P aeruginosa infections, and dornase alfa therapy.