Adherence to therapies in cystic fibrosis: a targeted literature review

Abstract

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.

Outcome evaluation of a pharmacy‐based therapy management program for patients with cystic fibrosis

“…In this research, the highest adherence level was for the new medication ivacaftor ( n = 17), and the 12% difference to the control cohort ( n = 15) was not significant likely due to the small matched count. High adherence to ivacaftor and low adherence to other CF treatments is consistent with findings from the recent literature review by Narayanan et al…”

“…However, both average length of stay and the proportion of patients with a hospitalization with IV antibiotics were similar between groups (both P > 0.10). Given the observation period of our study, we were able to assess adherence to ivacaftor but not the lumacaftor/ivacaftor combination therapy …”

“…Medication adherence was found to be higher for medications that are perceived to have more immediate health benefits (eg, digestive and respiratory drugs) than for more distal benefits (eg, physiotherapy and nutritional supplements) . Other factors associated with non‐adherence were age and seasonality . Although some studies did not find financial barriers to adherence, new, more expensive treatment options, such as ivacaftor, may increase issues of financial barriers .…”

Role of habit in treatment adherence among adults with cystic fibrosis

“…Effective adherence interventions are lacking,2 and reasons for low adherence are poorly understood. Studies of adherence determinants in CF have tended to focus on treatment burden and conscious motivational factors such as treatment beliefs 2.…”

“…Effective adherence interventions are lacking,2 and reasons for low adherence are poorly understood. Studies of adherence determinants in CF have tended to focus on treatment burden and conscious motivational factors such as treatment beliefs 2. However, behavioural theories such as the COM-B model (capability, opportunity, motivation and behaviour) propose a wider range of adherence predictors, including automatic processes such as habit 3.…”