Role of Lung Clearance Index in the Early Detection of Pulmonary Changes in Children with Sickle Cell Disease

Abstract

Pulmonary complications including acute chest syndrome are leading causes of sickle cell disease related morbidity and mortality. Studies have shown that pulmonary changes can be detected during childhood. Spirometry is the current standard for measuring lung function. Growing evidence suggests that lung clearance index (LCI) is as sensitive as spirometry in identifying pulmonary changes in pediatric patients. Our cross-sectional study compared the sensitivity of LCI to spirometry in the detection of early pulmonary changes in children with sickle cell disease. Our results show that LCI significantly correlates to FEV1% predicted (Spearman’s coefficient -0.44, p = 0.003), FVC % predicted (Spearman’s coefficient -0.44, p = 0.006) and FEF25-75 (Spearman’s coefficient -0.49, p <0.001). Using receiver operating characteristic (ROC) curves, LCI was found to be more sensitive than spirometry, but less specific. The data support LCI’s use as a test to screen for pulmonary changes in children with sickle cell disease. Earlier monitoring of lung function will allow for preventative therapies and delayed progression of pulmonary dysfunction.