Ejiofor LCK, Mathiesen IHM, Jensen‐Fangel S, et al. - The safety and efficacy outcomes for Lumacaftor/Ivacaftor therapy have been examined in several studies. Researchers here examined how Lumacaftor/Ivacaftor affects lung function, physical performance, and health‐related quality of life (HRQOL) in a subpopulation of Danish people with cystic fibrosis (PWCF) with advanced pulmonary disease who would not fulfill inclusion criteria for these studies. In this follow‐up study, they included 21 patients homozygous for the F508del mutation and a median ppFEV1 of 38.7. After 6 months of treatment, there were significant improvements in ppFEV1, ppFEV1 slope, and saturation at cardiopulmonary exercise testing initiation and termination. Finally, significant improvement was observed in HRQOL in all Cystic Fibrosis Questionnaire–Revised domains except Emotion and Treat. Per these findings, Lumacaftor/Ivacaftor decreases lung function decline, improves lung function, physical performance, and HRQOL to a greater extent in PWCF with severe lung disease than previously identified.