Use of a CFTR-modulator in an elderly patient with cystic fibrosis: a case report

Cystic fibrosis is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which is expressed on the apical surface of epithelial cells. Advances in the management of cystic fibrosis have significantly improved patient survival. In recent years, CFTR modulators - drugs that target the underlying CFTR defect - have become available, leading to marked clinical improvement. However, data on their efficacy and safety in patients aged 65 years and older are limited.

The aim of this study was to demonstrate the effectiveness of CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor) in an elderly patient with cystic fibrosis.

Conclusion. Treatment with elexacaftor/tezacaftor/ivacaftor over 14 months in an elderly patient led to significant positive clinical, functional, and radiological changes. No significant adverse reactions or worsening of comorbid conditions were observed during the entire observation period.

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