Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor

In people with CF (PWCF), CFTR dysfunction in the lower airways results in dehydrated airway secretions and a severe impairment of mucociliary clearance, which results in a cycle of chronic infection and inflammation. Ivacaftor, a first-in-class molecule, enhances CFTR function in class III (gating) and class IV (conductance) mutations [1,2] by increasing the probability of CFTR-channel opening. The G551D-CFTR mutation displays a severe phenotype and is the most commonly detected class III mutation with a worldwide prevalence of 4-5% [3].