TY - JOUR T1 - Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0112-2019 VL - 29 IS - 155 SP - 190112 AU - Michal Shteinberg AU - Jennifer L. Taylor-Cousar Y1 - 2020/03/31 UR - http://err.ersjournals.com/content/29/155/190112.abstract N2 - Drug compounds that augment the production and activity of the cystic fibrosis (CF) transmembrane regulator (CFTR) have revolutionised CF care. Many adults and some children with CF suffer advanced and severe lung disease or await lung transplantation. While the hope is that these drug compounds will prevent lung damage when started early in life, there is an ongoing need to care for people with advanced lung disease. The focus of this review is the accumulating data from clinical trials and case series regarding the benefits of CFTR modulator therapy in people with advanced pulmonary disease. We address the impact of treatment with ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor and elexacaftor/tezacaftor/ivacaftor on lung function, pulmonary exacerbations, nutrition and quality of life. Adverse events of the different CFTR modulators, as well as the potential for drug–drug interactions, are discussed.CFTR modulators are efficacious in improving lung function and exacerbations, even in advanced CF. However, adverse drug reactions and drug interactions are frequent. http://bit.ly/37aN81E ER -