Top cystic fibrosis updates from 2019: Triple therapy approved, invasive mechanical ventilation reduces mortality and more

The Healio Pulmonology Editors have compiled a list of updates in cystic fibrosis that Healio reported on in 2019.

See below for news on a new triple therapy approved by the FDA for patients with the most common cystic fibrosis mutation and other new research published this year.

Triple therapy approved to treat cystic fibrosis

The FDA announced it has approved elexacaftor/ivacaftor/tezacaftor (Trikafta, Vertex Pharmaceuticals), the first triple combination therapy for patients with the most common cystic fibrosis mutation. Read more

Mortality rates decreasing in cystic fibrosis patients treated with invasive mechanical ventilation

Mortality rates in adults with cystic fibrosis treated with invasive mechanical ventilation have decreased over time, suggesting mechanical ventilation in these patients is not futile. Read more

Azithromycin treatment outcomes may differ based on antibiotic type

Patients with cystic fibrosis saw a greater increase in FEV₁ when given oral azithromycin in combination with certain IV antibiotics for the treatment of Pseudomonas aeruginosa, as well as an increased overall improvement of FEV₁, according to a study published in Annals of the American Thoracic Society. Read more

Ivacaftor may reduce respiratory infections in cystic fibrosis

Among patients with cystic fibrosis, the use of ivacaftor was associated with a 32% reduction in the rate of Pseudomonas aeruginosa infection and a 15% reduction in the rate of Staphylococcus aureus infection over time, according to data published in the Annals of the American Thoracic Society. Read more

Q&A: Success of triple therapy in cystic fibrosis

Two studies — one published in The New England Journal of Medicine and one published in The Lancet — have shown the promise of combination therapy with elexacaftor, tezacaftor and ivacaftor in certain patients with cystic fibrosis. Although the treatment was studied in two different patient populations, the findings from both studies highlight the therapeutic benefits of this triple therapy. This Q&A focuses on the new findings and other important issues related to caring for patients with cystic fibrosis. Read more