The Aging Patient with Cystic Fibrosis.

Abstract

Cystic fibrosis (CF) is an inherited condition that leads to multiorgan dysfunction, especially in the respiratory, gastrointestinal, and reproductive tracts, with associated conditions including persistent pulmonary infection, liver disease, pancreatic insufficiency, and infertility. Historically, people with CF (pwCF) suffered a shortened lifespan due to complications of the condition, namely respiratory. The emphasis on center-based, multidisciplinary care and the widespread introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy has resulted in pwCF living longer and healthier lives. Now they may encounter some of the health and social issues associated with growing older, which previously were not a typical experience for this population. In this article, we review relevant health issues for the aging CF population, including complications that arise from the condition itself, issues encountered due to treatment, and general conditions associated with aging that may manifest earlier or differently in pwCF. We discuss the recommendations for screening and treatment of relevant conditions, and considerations for the integration of healthcare professionals across disciplines into the care of this population.