Working to Have a Normal Life With Cystic Fibrosis in an Adherence-Driven Health Care System.

BACKGROUND: Adults with cystic fibrosis (CF) must continuously manage their condition, while working for a living, and want a normal life. Adherence rates to treatments/medications are less than optimal. Existing theory offers little to explain adherence rates. The purpose of this study was to develop a theory to further the understanding of how people with CF manage their condition in an adherence-driven health care system. METHODS: Constructivist Grounded Theory methodology was used to conduct 27 semistructured interviews with adults with CF, family members, and health care providers. Data collection and analysis were simultaneous, using constant comparative methods, initial and focused coding, and category identification and reduction to develop a theory. RESULTS: Doing what works to balance life and CF is the theory generated from this study. The main concern of participants was to be seen as normal. The theory depicts what participants with CF and their family members do about their concerns and involves 4 interrelated processes: working overtime, receiving support, passing as normal, and facing disease progression. CONCLUSION: Participants did not relate to the term nonadherent; rather they described working overtime to manage CF, to work, and to have a normal life. Health care provider and researcher perspectives on adherence differ from those of people with CF. Engaging adults with CF and health care providers in a dialogue in which expectations are shared may lead to individualized treatment regimens that work, because adults with CF will do what works.

Modeling cystic fibrosis disease progression in patients with the rare CFTR mutation P67L.

BACKGROUND: The progression of cystic fibrosis (CF) in patients with the rare mutation P67L was examined to determine if it induced a milder form of CF compared to the common severe ΔF508 mutation. METHODS: Parameters of lung function, level of bacterial infection, nutritional status and hospitalization were used to represent CF progression. Age at diagnosis and pancreatic status were used to assess CF presentation. Analysis of data from the CF Canada Registry collected over a 15-year period included 266 ΔF508/ΔF508 homozygote patients from CF clinics in Atlantic Canada and 26 compound heterozygote patients with the rare P67L mutation from clinics across Canada. RESULTS: Late age at diagnosis, high incidence of pancreatic sufficiency, maintained Body Mass Index (BMI) with age, delayed life-threatening bacterial infection, and fewer days in hospital were observed for P67L heterozygote patients included in this study. Although the decline of lung function did not differ from ΔF508 homozygotes, the fact that a greater proportion of P67L heterozygotes live to an older age suggests that lung function is not the primary factor determining CF progression for P67L heterozygote patients. CONCLUSION: The P67L mutation is associated with a mild disease, even when combined with the severe ΔF508 mutation.