Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist

BackgroundLung infections are among the most consequential manifestations of cystic fibrosis (CF) and are associated with reduced lung function and shortened survival. Drugs called CF transmembrane conductance regulator (CFTR) modulators improve activity of dysfunctional CFTR channels, which is the physiological defect causing CF. However, it is unclear how improved CFTR activity affects CF lung infections.MethodsWe performed a prospective, multicenter, observational study to measure the effect of the newest and most effective CFTR modulator, elexacaftor/tezacaftor/ivacaftor (ETI), on CF lung infections. We studied sputum from 236 people with CF during their first 6 months of ETI using bacterial cultures, PCR,…

• CF
  Cystic Fibrosis Foundation

  Award: PROMISE-MICRO18K1
• GS
  Gilead Sciences
• VP
  Vertex Pharmaceuticals
• NI
  National Institutes of Health

  Awards: R01HL148274, P30DK089507
• NO
  NIH Office of the Director

  Award: 5R01HL148274-03