Ivacaftor has been shown to increase CFTR activity and lung function in people with Cystic Fibrosis and the G551D mutation, but until a recent study, scientists were not sure if ivacaftor changed the level of bacteria in the lungs.

The results of a deeper study on ivacaftor have been published, showing that mucus plugging and inflammatory measures in sputum continue to improve over two years of treatment, but the declines in Pseudomonas Aeruginosa density do not persist after the first year.

The study looked at 12 people with CF and G551D on ivacaftor over two years, and measured bacterial density and inflammatory markers in their sputum, as well as mucus plugging via radiographic imagery.


  • Ivacaftor reduced the density of Pseudomonas Aeruginosa within 48-hours of beginning treatment and throughout the first year.
  • Total bacterial concentrations in sputum decreased, and P. Aeruginosa had the most decrease.
  • After one year, P. Aeruginosa densities began to rebound.
  • All patients continued to have P. Aeruginosa infections.
  • Sputum inflammatory measures decreased significantly in the first week of treatment and continued to decline over two years.
  • CT scans done before treatment started and one year after ivacaftor treatment revealed decreased mucus plugging.

The study was published in The American Journal of Respiratory and Critical Care Medicine on February 21, 2017, “Restoring CFTR Function Reduces Airway Bacteria and Inflammation in People With Cystic Fibrosis and Chronic Lung Infections.”


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