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- Title
Impact of elexacaftor/tezacaftor/ivacaftor combination therapy on body plethysmography in adults with cystic fibrosis: Beyond FEV<sub>1</sub>.
- Authors
Wollsching-Strobel, Maximilian; Dieninghoff, Doris; Mathes, Tim; Stanzel, Sarah B.; Criée, Carl-Peter; Windisch, Wolfram; Berger, Melanie P.
- Abstract
Introduction: The treatment approach involving a combination of three cystic fibrosis (CF) transmembrane conductance regulatory (CFTR) protein modulators (elexacaftor, tezacaftor, ivacaftor) in the treatment of CF patients has provided beneficial results on FEV1. Methods: The aim of the retrospective single-center observational study was to show the impact of elexacaftor/tezacaftor/ivacaftor with special emphasis on full body plethysmography, exercise capacity and quality of life. Adult cystic fibrosis patients were evaluated after the initiation of triple CFTR therapy between July 2020 and March 2021. A two-tailed t-test for dependent samples with Bonferroni correction was used to compare outcomes between the two visits. Results: A total of 38 adult CF-patients (20 females), aged 37.2 ± 11.6 years were included in the study. The analysis shows significant improvements in FEV1 and vital capacity (VC). The Tiffeneau-Index (FEV1/VC) only showed a minor increase from 59 ± 14% to 62 ± 13%. Hyperinflation (RV) and effective specific airway resistance (sRAWeff) significantly improved such as exercise capacity (6MWD) and CFQ-R. sRAWeff showed a strong significant association with an improvement in exercise capacity (Pearson's r=0.593; 95% CI: 0.296–0.786, p<0.001) and FEV1 (Pearson's r=0.560; 95% CI: 0.292–0.746, p<0.001) while all other parameters did not show significant correlations. Conclusions: Triple CFTR modulator therapy improves not only FEV1 but also airway resistance and pulmonary hyperinflation. This is most reliably assessed by measuring sRAWeff, which is associated with exercise capacity, representing the work of breathing. Futures studies should incorporate body plethysmography assessments especially in patients with limited ability to perform spirometry or with non-reproducible spirometry results.
- Subjects
AEROBIC capacity; PULMONARY function tests; AIRWAY resistance (Respiration); CYSTIC fibrosis; VITAL capacity (Respiration)
- Publication
Pneumon, 2024, Vol 37, Issue 2, p1
- ISSN
1105-848X
- Publication type
Article
- DOI
10.18332/pne/186913