Experience of microbiological monitoring of the respiratory tract microflora in patients with cystic fibrosis during therapy with the combination drug elexacaftor/tezacaftor/ivacaftor + ivacaftor

Abstract

Bacterial complications of the respiratory tract are negative prognostic factors leading to a decrease in pulmonary function, duration, and quality of life of patients with cystic fibrosis. To date, a sufficient number of studies have not been accumulated to reliably prove or reject microbiological improvement during targeted therapy.

The aim of the study was to collect continuous microbiological data from patients with cystic fibrosis in the Samara region receiving treatment with elexacaftor/tezacaftor/ivacaftor + ivacaftor from November-December 2021. The article presents eight clinical observations. At the time of therapy initiation, seven patients had chronic respiratory tract infection associated with Burkholderia cenocepacia ST 208, combined with chronic Pseudomonas aeruginosa infection in two of them. One patient had a chronic P. aeruginosa infection in association with Aspergillus fumigatus.

Conclusion. An expansion of the species composition in the respiratory samples was demonstrated in all cases. The majority of patients showed microbiological improvement. Thus, biochemical and, as a result, microecological changes in the lungs against the normalization of chlorine channel function allow suggesting that key pathogens may be eradicated during the therapy with elexacaftor/tezacaftor/ ivacaftor + ivacaftor.