Longitudinal assessment of lung function in children with sickle cell disease

Area of Science:

• Pediatric Pulmonology
• Hematology
• Respiratory Medicine

Background:

• Sickle cell disease (SCD) is a genetic blood disorder associated with various complications, including pulmonary issues.
• Longitudinal data on lung function decline in children with SCD is crucial for understanding disease progression and developing interventions.

Purpose of the Study:

• To prospectively evaluate the changes in lung function over time in children diagnosed with sickle cell disease (SCD).
• To compare lung function trajectories between children with SCD and healthy controls.
• To investigate the impact of acute chest syndrome (ACS) episodes on lung function decline in pediatric SCD patients.

Main Methods:

• Two prospective longitudinal cohorts of children with SCD and age/ethnically matched controls were recruited.
• Cohort One included 47 SCD patients and 26 controls (median age 8.8 years, 2-year follow-up).
• Cohort Two included 45 SCD patients and 26 controls (median age 10.2 years, 10-year follow-up).
• Key lung function parameters including FEV1, VC, FEF25-75, TLC, and RV were measured at two time points.

Main Results:

• Children with SCD showed a significant decline in lung function over time, unlike control groups.
• The rate of lung function decline was more pronounced in Cohort One (younger children) compared to Cohort Two.
• Acute chest syndrome (ACS) episodes were more frequent in Cohort One during the follow-up period.
• Restrictive lung abnormalities became more common in Cohort Two participants at follow-up.

Conclusions:

• Lung function progressively deteriorates with age in children with sickle cell disease (SCD).
• Younger SCD patients experiencing more frequent acute chest syndrome (ACS) episodes exhibit a faster rate of lung function decline.
• Findings highlight the significant pulmonary impact of SCD in childhood and the need for monitoring.