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Objectives: Appropriate management of chronic rhinosinusitis (CRS) among patients with cystic fibrosis (CF) is important in improving quality of life. Otolaryngologists play a critical role in reducing CRS symptom burden. This study seeks to evaluate the role of patient-reported quality-of-life measures in guiding interventions for CF-related sinus disease. Methods: We performed a prospective, cross-sectional study of 105 patients presenting to a CF-accredited clinic between July and September 2018. Demographic data and sinus surgery history were collected, in addition to Sino-Nasal Outcome Test (SNOT-22) and Questionnaire of Olfactory Disorders (QOD-NS) scores. Statistical analysis was conducted using correlation and non-parametric Mann-Whitney U tests. Results: Baseline well-care visits accounted for 71.4% of all clinical evaluations. Prior otolaryngology intervention was noted in 69 (66%) patients, where the majority of these patients (63/69; 91%) underwent endoscopic sinus surgery (ESS). Patients with a history of otolaryngology intervention had an average SNOT-22 score of 33.2 (SD = 20.6) compared to 24.9 (SD = 18.5) for patients without prior intervention (P =.048). The average QOD-NS score was 5.5 (SD = 6.4) among patients referred to otolaryngologists and 3.1 (SD = 5.7) for non-referred patients (P =.012). SNOT-22 and QOD-NS scores were modestly correlated (R of.43). Conclusion: CF patients with symptoms resulting in worse quality-of-life assessments were more likely to have established coordinated care with an otolaryngologist. Further validation of the utility of SNOT-22 and QOD-NS questionnaires as care coordination metrics is necessary in the CF population. [ABSTRACT FROM AUTHOR]

• Adults with CF report significant sinonasal symptoms • Elexacaftor-tezacaftor-ivacaftor improves sinonasal symptoms as measured by SNOT-22 • Patients taking tez/iva had more sinonasal symptoms than those not taking modulators 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. [ABSTRACT FROM AUTHOR]

Background: In cystic fibrosis (CF), the relationship between chronic rhinosinusitis (CRS) and pulmonary disease is poorly understood. The purpose of this study was to evaluate the relationship between scores on the 22‐item Sino‐Nasal Outcome Test (SNOT‐22) and CF Questionnaire―revised for adolescents and adults over 14 (CFQ‐R 14+), and pulmonary function tests in 2 cohorts of CF patients: those at their baseline health and those with a pulmonary exacerbation. Methods: Patients >18 years old seen in a Cystic Fibrosis Foundation‒accredited clinic completed the SNOT‐22 and CFQ‐R 14+ instruments. Patients presenting for routine care represented the baseline cohort. Patients diagnosed with a pulmonary exacerbation represented the exacerbation cohort. Average SNOT‐22 and CFQ‐R 14+ scores for both groups were compared using a 2‐sample t test, and correlation coefficient was calculated. Results: One hundred three patients were enrolled over 3 months (30 exacerbations and 73 baseline). Patients' mean age was 32 years (56% female and 44% male). Average SNOT‐22 and CFQ‐R 14+ scores were significantly worse for exacerbation patients (p = 0.001 and p = 0.0003, respectively). Percent predicted forced expiratory volume in 1 second and forced vital capacity were both higher for baseline patients (p = 0.002 and p = 0.001, respectively). Average SNOT‐22 score for all patients was worse than the average score for non‐CF, non‐CRS patients. Conclusion: CF patients with pulmonary exacerbations have worse SNOT‐22 and CFQ‐R 14+ scores than CF patients at their baseline health. This finding suggests a temporal relationship between sinonasal and pulmonary quality of life, and that worsening of both is associated with reduced pulmonary function. [ABSTRACT FROM AUTHOR]

Background: Elexacaftor/tezacaftor/ivacaftor is a highly effective modulator that improves function of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, resulting in improved pulmonary function in patients with cystic fibrosis (CF). We hypothesize that improvements in lung function are associated with improvements in health‐related quality of life and sinonasal health. The aim of this study is to measure the effect of elexacaftor/tezacaftor/ivacaftor on patient‐reported sinonasal and overall quality of life, and to determine the relationship between changes in these 2 outcome measures. Methods: A prospective cohort study was conducted at an accredited adult CF care center. Participants completed the 22‐item Sino‐Nasal Outcome Test (SNOT‐22) and the Cystic Fibrosis Questionnaire–Revised (CFQ‐R), a validated patient‐reported outcome metric for CF patients, at baseline and at 3 months after initiation of elexacaftor/tezacaftor/ivacaftor. Results: Forty‐three individuals completed the study. There was significant improvement in nearly all domains of the SNOT‐22 and CFQ‐R after 3 months of therapy. SNOT‐22 improved from 34.8 to 24.4 (p = 0.000003). Mean baseline FEV‐1 improved from 65% to 76% predicted (p = 0.0000005). The greatest effect was seen in those participants previously taking modulator therapy. Linear regression between the change in SNOT‐22 individual domains and the CFQ‐R respiratory domain revealed the strongest correlation between the extranasal domain score and the respiratory domain of the CFQ‐R (R2 = 0.24). Conclusion: CF patients taking elexacaftor/tezacaftor/ivacaftor experience a significant improvement in both sinonasal and health‐related quality of life. [ABSTRACT FROM AUTHOR]