期刊
INTERNATIONAL FORUM OF ALLERGY & RHINOLOGY
卷 9, 期 3, 页码 292-297出版社
WILEY
DOI: 10.1002/alr.22251
关键词
chronic sinusitis; quality of life; cystic fibrosis; paranasal sinus disease; chronic disease
资金
- NIH (National Heart, Lung, and Blood Institute [NHLBI]) [1 R01 HL133006-03]
- NIH (National Institute of Diabetes and Digestive and Kidney Diseases [NIDDK]) [5 P30 DK072482]
Background: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that improves pulmonary function in cystic fibrosis (CF) patients with at least 1 copy of the G551D CFTR mutation. The purpose of this study is to evaluate the impact of ivacaftor on chronic rhinosinusitis (CRS) symptoms in this population. Methods: The G551D Observational (GOAL) study was a multicenter prospective cohort study enrolling CF patients 6 years with at least 1 G551D mutation. Subjects were provided 20-item Sino-Nasal Outcome Test (SNOT-20) questionnaires prior to ivacaftor therapy and at 1, 3, and 6 months afterward. The impact on rhinologic (R), psychological (P), sleep (S), and ear/facial (E) quality of life (QOL) domains was evaluated separately. Results: Of 153 subjects, 129 (84%) completed all questionnaires. Typical baseline symptom burden was low (75% with scores <1) and degree of improvement (ie, reduced scores) was greater with higher baseline scores. SNOT-20 decreased, reflecting improvement, at all follow-up intervals (1 month: [mean change +/- standard deviation] -0.25 +/- 0.53, p < 0.01; 3 months; -0.29 +/- 0.58, p < 0.01; 6 months: -0.21 +/- 0.58, p = 0.02), but less than the prespecified minimal clinically important difference (0.8). Significant improvement was observed at 1, 3, and 6 months in the R domain (1 month: -0.24, p < 0.01; 3 months: -0.34, p < 0.01; 6 months: -0.25, p < 0.01) and P domain (1 month: -0.25, p < 0.01; 3 months: -0.32, p < 0.01; 6 months: -0.26, p < 0.01), and 1 and 3 months in the S domain (1 months: -0.35, p < 0.01; 3 months: -0.32, p < 0.01; 6 months: -0.18, p = 0.07). There was no improvement in the E domain at any time point. Conclusion: Ivacaftor improves QOL in the R, P, and S domains in G551D CF patients, although QOL instruments validated for CRS may not translate well to CF CRS patients because symptom burden was surprisingly low. (C) 2018 ARS-AAOA, LLC.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据