Patient perspectives on chronic rhinosinusitis in cystic fibrosis: Symptom prioritization in the era of highly effective modulator therapy.

Abstract

Chronic rhinosinusitis (CRS) is common in people with cystic fibrosis (PwCF). Rhinologic symptom prioritization and areas that influence CRS treatment choices, including pursuing endoscopic sinus surgery (ESS), remain understudied. Adult PwCF+CRS were enrolled at eight centers into a prospective, observational study (2019-2023). Participants were administered the 22-SinoNasal Outcome Test (SNOT-22) survey and a modified SNOT-22 instrument examining symptom importance. We determined importance rankings for individual symptoms and SNOT-22 symptom importance subdomains in two sets of subgroups-those pursuing ESS versus continuing medical management (CMT), and those on elexacaftor/tezacaftor/ivacaftor (ETI) versus not on ETI. Among 69 participants, the highest priorities were nasal congestion (n=48, 69.6% important), post-nasal discharge (32, 46.4%), facial pain (29, 43.3%), waking up tired (27, 39.1%), and fatigue (26, 37.7%). Those electing surgery (n=23) prioritized sleep and psychological dysfunction symptoms compared to those pursuing CMT (n=49) (sleep median score=19.0 [interquartile range: 12.0, 25.0] vs. 4.5 [0.0, 12.8]; p<0.0001; psychological=17.0 [7.0, 26.0] vs. 7.0 [0.0, 15.8]; p=0.002). ETI users had comparable SNOT-22 total symptom importance scores to non-ETI users (p=0.14). Non-ETI users (n=34) showed a trend toward prioritizing sleep symptoms compared to ETI users (n=35) (13.0 [2.8, 22.3] vs. 6.0 [2.0, 17.0]; p=0.055). Nasal congestion and post-nasal discharge were top priorities reported by PwCF+CRS. Those electing surgery prioritized sleep and psychological symptoms, highlighting their importance in pre-operative discussions. Non-ETI users' prioritization of sleep improvement may highlight their unique disease impact and therapeutic needs; however, additional investigation is required.