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J Cyst Fibros. 2019 May 21. pii: S1569-1993(19)30113-4. doi: 10.1016/j.jcf.2019.05.009. [Epub ahead of print]

Correspondence between lung function and symptom measures from the Cystic Fibrosis Respiratory Symptom Diary-Chronic Respiratory Infection Symptom Score (CFRSD-CRISS).

Author information

1
Department of Radiology, University of Washington, Seattle, Washington, United States. Electronic address: goldl@uw.edu.
2
Department of Health Services, University of Washington, Seattle, Washington, United States. Electronic address: donald@uw.edu.
3
School of Pharmacy, University of Washington, Seattle, Washington, United States. Electronic address: rhansen@uw.edu.
4
Departments of Medicine and Pediatrics, University of Washington, Seattle, Washington, United States. Electronic address: goss@uw.edu.
5
Department of Health Services, University of Washington, Seattle, Washington, United States. Electronic address: esslerl@uw.edu.

Abstract

BACKGROUND:

Pulmonary exacerbations (PEx) in cystic fibrosis (CF) patients reduce quality of life. Lung function, measured by the percent predicted forced expiratory volume in 1 s (ppFEV1), is widely used to evaluate PEx treatments. We analyzed the correspondence of ppFEV1 with 8 patient-reported symptom-based questions from the Cystic Fibrosis Respiratory Symptom Diary-Chronic Respiratory Infection Symptom Score (CFRSD-CRISS).

METHODS:

Data were derived from the observational Standardized Treatment of Pulmonary Exacerbations (STOP) study. CF patients who had CFRSD-CRISS and ppFEV1 measurements on ≥2 timepoints were included: 1) day of initial PEx, 2) 7 days later, and/or 3) end of PEx. We calculated age-stratified Spearman correlation coefficients and 95% confidence intervals (95% CIs) between the change in ppFEV1 and change in CFRSD-CRISS items from index to day 7 and from index to the end of PEx treatment.

RESULTS:

Lung function and symptom scores improved by the end of treatment; however, correlations between ppFEV1 and the specific CFRSD-CRISS measures were mostly weak to moderate. An exception was that among patients <18, we observed moderately strong correlations between changes in ppFEV1 and cough severity (r = -0.58 (95% CI: -0.80, -0.21)), mucus quantity (r = -0.51 (-0.77, -0.11)), and wheezing (r = -0.53 (-0.78, -0.14)) from index until end of treatment.

CONCLUSIONS:

As novel treatments are developed for PEx, it is important to ensure that improvement is measured meaningfully. The generally weak associations between patient-reported symptoms and ppFEV1 that we found suggest that these measures capture different aspects of the disease and both metrics are important when evaluating new treatments.

KEYWORDS:

Cystic Fibrosis Respiratory Symptom Diary - Chronic Respiratory Infection Symptom Score; Cystic fibrosis; Lung function; Pulmonary exacerbations

PMID:
31126901
DOI:
10.1016/j.jcf.2019.05.009

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