Multimorbidity in the modulator era: challenges of managing an ageing cystic fibrosis population.

Abstract

Introduction: Cystic Fibrosis (CF) is an autosomal recessive disorder caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene, leading to defective chloride ion transport and multisystem disease. The introduction of CFTR modulators, particularly elexacaftor/tezacaftor/ivacaftor (ETI), has significantly improved life expectancy and quality of life for people with CF (pwCF). As survival increases, an aging CF population faces different new challenges.

Areas covered: This review aims to highlight emerging challenges and comorbidities in an aging CF population to ensure sustained benefits from recent therapeutic advancements. The review includes the discussion of the changes in lung function, nutrition, cardiometabolic diseases, malignancy risk and psychosocial health. A structured literature search was conducted using PubMed, focusing on articles published between 2000 and 2025, including recent clinical trials and international guidelines. National registry data has also been reviewed.

Expert opinion: With CFTR modulators progress rapidly, the future focus should shift to refining and optimizing CF care. Standard practice should involve a multidisciplinary, proactive and preventative strategy to manage co-morbidities, ensuring the ultimate goal moving from simply extending pwCF's life to ensuring a high quality of life throughout their lifespan.