BACKGROUND: The management of cystic fibrosis (CF) includes adherence to a home management program (airway clearance, medication, nutritional advice, and exercise). This has led to an increase in life expectancy, although the benefits depend greatly on a patient's level of adherence to daily treatments at home. To date, no systematic review has established adherence rates to all World Health Organization guidelines in the home setting; hence, this review was undertaken. METHODS: The review comprised 3 phases. A methodological assessment of databases (Embase, CINAHL, PsychINFO, PEDro, PubMed, Cochrane Central Register of Controlled Trials) identified potentially relevant papers. These papers were screened for inclusion criteria by 2 independent reviewers, data were extracted, and the internal validity was rated using a valid and reliable scale. Results were categorized into 4 themes: medication, nutrition, airway clearance techniques, and exercise. RESULTS: The search generated a total of 26 papers, 24 of which were rated as being poor quality. Adherence to a treatment program for CF patients is generally low (from 22% for nutritional guidelines to 130% for oral antibiotics), and it varies greatly depending on the type of treatment and the method of assessment employed (objective tool vs self-reported questionnaires). CONCLUSIONS: Consensus on how to measure adherence is lacking, and the quality of studies addressing adherence in this population is generally poor. Overall, studies using self-reported measures yielded higher adherence scores than those that used objective measures, suggesting that current efforts to improve methods of adherence are appropriate. The prevalence of non-adherence remains unclear due to these limitations.

No abstract