Adherence to therapies in cystic fibrosis: a targeted literature review

ABSTRACT

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.

KEYWORDS:

• Adherence
• outcomes
• barriers
• cystic fibrosis
• drivers
• therapies

Declaration of interest

S. Narayanan is a consultant for Abbott, AbbVie, Incyte, Johnson & Johnson, Novartis, Pfizer, Teva, and Vertex. JG. Mainz has received a research grant from Vertex for an investigator initiated trial and served in advisory boards for Vertex, PTC and Teva. JG. Mainz lectures for Pari, Vertex, Chiesi and Pharmaxis. S. Gala is a consultant for Abbott, AbbVie, Johnson & Johnson, Novartis, Incyte and Pfizer. H. Tabori has received Short-Term Grants (number: 57130097) of the German Academic Exchange Service (DAAD). D. Grossoehme has received research grants from the John Templeton Foundation, the Division of Pulmonary Medicine and the Department of Pastoral Care, Cincinnati Children’s Hospital Medical Center, and Vertex. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Additional information

Funding

This paper was not funded.