CFTR and cystic fibrosis: Over the next several years, 3 other combination CFTR protein modulators, called lumacaftor/ivacaftor (LUM/IVA), tezacaftor/ivacaftor (TEZ/IVA) and, most recently, elexacaftor/tezacaftor/ivacaftor (ETI) became available for the majority of people with CF with responsive CFTR mutations based on data demonstrating increased lung function, improved quality of life and decreased pulmonary exacerbations among other benefits [7,8,9,10,11].