Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis.

ABSTRACT:

Background

Many individuals with cystic fibrosis (CF) have chronic rhinosinusitis resulting in nasal obstruction, sinus infections, and repeated surgeries. Elexacaftor-tezacaftor-ivacaftor is a highly effective modulator therapy approved for individuals aged 6 years or older with CF who have at least one F508del allele or other responsive mutation. The current study tests the hypothesis that ELX/TEZ/IVA improves sinonasal disease in CF.

Methods

The study was a pre/post, observational cohort study conducted at two sites. Participants underwent a study visit prior to starting ELX/TEZ/IVA and a second visit at a median of 9 months on therapy. Each visit included sinus CT scan, rigid nasal endoscopy, and sweat chloride measurement. Symptoms were measured with the 22 item Sinonas

SUBMITTER: Stapleton AL 

PROVIDER: S-EPMC9470769 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature