CFTR and respiratory failure: Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the gene encoding the CF conductance regulator (CFTR) anion channel protein.1 The absence of CFTR function in the airway leads to mucus stasis, inflammation and chronic infection, which often cause severe lung damage and eventual respiratory failure.2–4 The recent introduction of highly effective CFTR modulator therapy (HEMT) has significantly improved respiratory outcomes and increased life expectancy for many people with CF (pwCF).5,6 However, airway infection remains a major focus in CF treatment.