Bronchiectasis is an extremely heterogeneous airways disease, making it difficult to study. For years, the noncystic fibrosis (CF) bronchiectasis community has been trying to organize to provide better care for more than half a million adults with bronchiectasis in the United States. Internationally, the Europeans created the European Bronchiectasis Registry, which has been a powerful tool including nearly 20,000 patients, to answer important epidemiologic and management questions. We must do more for the bronchiectasis community.
Clinicaltrials.gov indicates that there are 8 international phase 3 or 4 clinical trials that are currently enrolling; 3 of those have enrollment sites in the United States. One such study from University of North Carolina at Chapel Hill is looking at the use of nebulized hypertonic saline in patients with non-CF bronchiectasis to understand the effect it has on mucociliary clearance. Emory University is looking at the use of elexacaftor/tezacaftor/ivacaftor (Trikafta) in patients with non-CF bronchiectasis; these patients have only 1 targetable mutation and a phenotype that resembles CF. This 8-week, open-label, single-center study aims to measure both clinical and biomarker outcomes after treatment with Trikafta. Finally, a phase 3 trial out of Florida, the ICoN-1 study, is examining the efficacy and safety of inhaled clofazimine in the treatment of nontuberculous mycobacteria (NTM). This double-blind, randomized trial will look at culture conversion and quality of life measures. Additionally, the COPD Foundation has created the Bronchiectasis and NTM Research Registry, an American cohort containing more than 5,000 patients and data from 22 different sites, to answer some of the most important questions for clinicians and patients.
We have made significant progress in bronchiectasis research; however, there is still much to learn. Together, we must make a concerted effort to enroll patients in clinical trials. Doing so will allow us to define our epidemiologic profile more precisely and explore new treatments and airway clearance techniques.