Adherence to therapies in cystic fibrosis: a targeted literature review.

INTRODUCTION: Cystic fibrosis (CF) is a life-shortening condition with no cure. Available therapies relieving the symptoms of CF are complex and time-consuming. A comprehensive review assessing adherence to different CF therapies, association of adherence with outcomes, and factors influencing adherence could inform optimal patient management strategies. Areas covered: A targeted literature review of studies published from 2010-2016 assessed adherence to CF therapies. Nineteen studies qualified for inclusion. Adherence to CF therapies was sub-optimal, and varied by treatment, mode of treatment administration, age, season, time and method of adherence measurement. Adherence to ivacaftor and inhaled antibiotics were reported higher than dornase alfa or hypertonic saline, oral pancreatic enzyme and vitamin supplements, and airway clearance therapy. Several patient, healthcare provider and treatment related factors influenced adherence. Sub-optimal adherence was shown to impact clinical and economic burden of the disease. Expert commentary: Higher adherence to CF therapies can lower disease burden, and improve patient outcomes. Healthcare providers and policy makers should devise patient-centered and caregiver-enabled interventions to improve adherence. Research on long-term adherence and outcomes associated with promising oral treatments such as CFTR modulators is needed. Identifying ways to overcome key barriers to adherence can positively affect outcomes associated with CF.