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Updated: Nov 28, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Lung clearance index in children with sickle cell disease
Evans M Machogu1, Monica Khurana2, Jennifer Kaericher2
1Section of Pediatric Pulmonology, Allergy, and Sleep Medicine, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Insights
The lung clearance index (LCI) did not show differences between children with sickle cell disease (SCD) and healthy children. Total lung capacity (TLC) may be a more useful measure for monitoring lung health in pediatric SCD.
Area of Science:
- Pulmonary Medicine
- Pediatric Hematology
- Respiratory Physiology
Background:
- The lung clearance index (LCI) is a sensitive measure for early lung disease detection in children with cystic fibrosis and asthma.
- Its utility in children with sickle cell disease (SCD) has not been previously investigated.
Purpose of the Study:
- To investigate the utility of LCI in detecting lung disease in children with SCD.
- To compare LCI values in children with SCD to those in healthy controls.
Main Methods:
- A single-center study involving children with SCD (ages 6-18) and healthy controls.
- Pulmonary function tests including multiple breath washout (MBW) for LCI, spirometry, and plethysmography were performed.
- Serum markers were analyzed, and results were compared between groups.
Main Results:
- No significant difference in LCI was found between children with SCD and healthy controls (p=0.514).
- Healthy controls had significantly higher total lung capacity (TLC) z-scores compared to children with SCD (p=0.005).
- Differences in forced expiratory volume in 1-second z-scores approached significance (p=0.055).
Conclusions:
- LCI did not differentiate children with SCD from healthy controls at baseline health.
- TLC may serve as a more important pulmonary function measure for longitudinal monitoring in pediatric SCD.
Introduction:
The lung clearance index (LCI) derived from the multiple breath washout test (MBW), is both feasible and sensitive to early lung disease detection in young children with cystic fibrosis and asthma. The utility of LCI has not been studied in children with sickle cell disease (SCD). We hypothesized that children with SCD, with or without asthma or airway hyperreactivity (AHR), would have an elevated LCI compared to healthy controls.
Methods:
Children with SCD from a single center between the ages of 6 and 18 years were studied at baseline health and completed MBW, spirometry, plethysmography and blood was drawn for serum markers. Results were compared to healthy controls of similar race, age, and gender.
Results:
Healthy controls (n = 35) had a significantly higher daytime oxygen saturation level, weight and body mass index but not height compared to participants with SCD (n = 34). Total lung capacity (TLC) z-scores were significantly higher in the healthy controls compared to those with SCD (0.87 [1.13] vs. 0.02 [1.27]; p = .005) while differences in forced expiratory volume in 1 s z-scores approached significance (0.26 [0.97] vs. -0.22 [1.09]; p = .055). There was no significant difference in LCI between the healthy controls compared to participants with SCD (7.29 [0.72] vs. 7.40 [0.69]; p = .514).
Conclusion:
LCI did not differentiate SCD from healthy controls in children between the ages of 6 and 18 years at baseline health. TLC may be an important pulmonary function measure to follow longitudinally in the pediatric SCD population.
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