The experience with elexacaftor/tezacaftor/ivacaftor + ivacaftor in adult patients with cystic fibrosis in the Omsk region

Elexacaftor/tezacaftor/ivacaftor + ivacaftor, a drug modulating the cystic fibrosis transmembrane conductance regulator protein, is currently revolutionizing the management of patients with cystic fibrosis, particularly those with at least one F508del variant. CFTR modulators are characterized by individual pharmacokinetic variability and a tendency to drug-drug interactions, which accounts for their variable effects in different patients. This article reports the experience of using the targeted therapy of cystic fibrosis, elexacaftor/tezacaftor/ivacaftor + ivacaftor, for the treatment of adult patients with cystic fibrosis for a year. The aim was to study the efficacy and side effects of the drug elexacaftor/tezacaftor/ivacaftor + ivacaftor during 12 months of treatment of adult patients with cystic fibrosis in the Omsk region. Methods. The article presents the experience of using elexacaftor/tezacaftor/ivacaftor + ivacaftor for targeted therapy of adult patients with CF for 12 months. Results. The positive effect, good tolerability and safety of the combined drug elexacaftor/tezacaftor/ivacaftor + ivacaftor for the treatment of adult patients with CF were demonstrated. Conclusion. Timely initiation of targeted therapy for CF in childhood is likely to prevent the development of severe manifestations of the disease, but monitoring of patients with CF receiving targeted therapy should be continued.