CFTR and cystic fibrosis: Regular airway clearance and inhaled antibiotic therapy, in combination with the recent development of highly effective CFTR modulator therapies, have greatly extended the life expectancy of people living with CF, allowing these patients to live into, or indeed beyond, their fifth or sixth decade (Solomon et al., 2015; De Boeck and Amaral, 2016; Quon and Rowe, 2016; De Boeck and Davies, 2017; Burgener and Moss, 2018; McElvaney et al., 2018; Rubin, 2018; West and Flume, 2018).