Monitoring of Structure and Function in Early Cystic Fibrosis Lung Disease

Abstract

Concurrent with improving survival in cystic fibrosis (CF), lung function has also steadily improved. Forced expiratory volume in one second (FEV 1), the measure of airway obstruction most widely monitored in patients with CF, often does not fall below the normal range until late adolescence. Clearly, FEV1 is losing relevance for clinicians monitoring lung disease in children with CF, as it is frequently normal, declines slowly, and cannot be measured by conventional techniques in children <6 years of age. As clinicians increasingly aim to identify and treat early CF lung disease before irreversible structural airway damage occurs, there is a compelling need for better tools with which to mo..