Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
Emily DiMangoDavid A Gudis

Abstract

Elexacaftor-tezacaftor-ivacaftor is a highly effective modulator for cystic fibrosis (CF) patients homozygous or heterozygous for F508del. Effects of the drug on sinonasal symptoms have not been studied. Adult participants were prospectively evaluated at baseline and after three months of treatment using validated questionnaires assessing sinonasal symptoms (SNOT-22) and CF-related quality of life (CFQ-R). Forty-three participants completed the study; 23 were taking other CF transmembrane conductance (CFTR) modulators at the time of study participation. There was a significant improvement in mean SNOT-22 from 34.8 (29.4-40, 95% confidence interval) to 24.4 (19.9-29.0) (p = 0.000003) and in the Respiratory domain of the CFQR from 60.6 (57.1-64.1) to 83.3 (79.4-87.2) (p = 0.0000002), both achieving a minimal clinically important difference. Patients previously taking CFTR modulators experienced a greater benefit in sinonasal and respiratory symptoms. Elexacaftor-tezacaftor-ivacaftor is associated with significant improvement in sinonasal symptoms; previous use of CFTR modulators is associated with greater benefit.

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Citations

Sep 29, 2020·International Forum of Allergy & Rhinology·Emily DiMangoDavid A Gudis
Oct 1, 2020·International Forum of Allergy & Rhinology·Daniel M Beswick, Milene T Saavedra
Dec 1, 2020·Expert Review of Respiratory Medicine·Peter G Middleton, Jennifer L Taylor-Cousar
Jul 3, 2021·International Journal of Molecular Sciences·Christelle Bergeron, André M Cantin
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Aug 28, 2021·Journal of Personalized Medicine·Bente L AalbersHarry G M Heijerman

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