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

Christine M Liu; Ethan J Han; Jakob L Fischer; Jess C Mace; Jose L Mattos; Karolin Markarian; Jeremiah A Alt; Todd E Bodner; Naweed I Chowdhury; Patricia H Eshaghian; Anne E Getz; Peter H Hwang; Ashoke Khanwalkar; Adam J Kimple; Jivianne T Lee; Douglas A Li; Meghan Norris; Jayakar V Nayak; Cameran Owens; Zara M Patel; Katie Poch; Rodney J Schlosser; Kristine A Smith; Timothy L Smith; Zachary M Soler; Jeffrey D Suh; Grant A Turner; Marilene B Wang; Jennifer L Taylor-Cousar; Milene T Saavedra; Daniel M Beswick

doi:10.1002/alr.23332

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

Int Forum Allergy Rhinol. 2024 Feb 11. doi: 10.1002/alr.23332. Online ahead of print.

Authors

Christine M Liu¹, Ethan J Han¹, Jakob L Fischer¹, Jess C Mace², Jose L Mattos³, Karolin Markarian⁴, Jeremiah A Alt⁵, Todd E Bodner⁶, Naweed I Chowdhury⁷, Patricia H Eshaghian⁸, Anne E Getz⁹, Peter H Hwang¹⁰, Ashoke Khanwalkar⁹, Adam J Kimple¹¹, Jivianne T Lee¹, Douglas A Li⁸, Meghan Norris¹¹, Jayakar V Nayak¹⁰, Cameran Owens⁹, Zara M Patel¹⁰, Katie Poch¹², Rodney J Schlosser¹³, Kristine A Smith⁵, Timothy L Smith², Zachary M Soler¹³, Jeffrey D Suh¹, Grant A Turner⁸, Marilene B Wang¹, Jennifer L Taylor-Cousar^{12

14}, Milene T Saavedra¹², Daniel M Beswick¹

Affiliations

¹ Department of Head and Neck Surgery, University of California, Los Angeles, California, USA.
² Department of Otolaryngology-Head and Neck Surgery, Oregon Health Sciences University, Portland, Oregon, USA.
³ Department of Otolaryngology-Head and Neck Surgery, Division of Rhinology and Sinus Surgery, University of Virginia, Charlottesville, Virginia, USA.
⁴ CTSI, David Geffen School of Medicine, University of California, Los Angeles, California, USA.
⁵ Department of Otolaryngology-Head and Neck Surgery, University of Utah, Salt Lake City, Utah, USA.
⁶ Department of Psychology, Portland State University, Portland, Oregon, USA.
⁷ Department of Otolaryngology-Head and Neck Surgery, Vanderbilt Health, Nashville, Tennessee, USA.
⁸ Department of Pulmonary Medicine, University of California, Los Angeles, California, USA.
⁹ Department of Otolaryngology-Head and Neck Surgery, University of Colorado, Aurora, Colorado, USA.
¹⁰ Department of Otolaryngology-Head and Neck Surgery, Stanford University, Stanford, California, USA.
¹¹ Department of Otolaryngology-Head and Neck Surgery, University of North Carolina, Chapel Hill, North Carolina, USA.
¹² Department of Medicine, National Jewish Health, Denver, Colorado, USA.
¹³ Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, South Carolina, USA.
¹⁴ Department of Pediatrics, National Jewish Health, Denver, Colorado, USA.

PMID: 38343143
DOI: 10.1002/alr.23332

Abstract

Background: 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.

Methods: 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.

Results: 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).

Conclusions: 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.

Keywords: chronic rhinosinusitis; cystic fibrosis; endoscopic sinus surgery; highly effective modulator therapy; patient priorities; patient symptoms; symptom importance.

Grants and funding

R25DC020151/DC/NIDCD NIH HHS/United States