Pulmonary function abnormalities in children with sickle cell disease

Adults with sickle cell disease (SCD) have restrictive lung function abnormalities which are thought to result from repeated lung damage caused by episodes of pulmonary vaso-occlusion; such episodes start in childhood. A study was therefore undertaken to determine whether children with SCD have restrictive lung function abnormalities and whether the severity of such abnormalities increases with age.

Sixty four children with SCD aged 5-16 years and 64 ethnic matched controls were recruited. Weight and sitting and standing height were measured, and lung function was assessed by measurement of lung volumes and forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and peak expiratory flow (PEF) before and after bronchodilator.

Compared with the control subjects, the children with SCD had lower mean (SD) sitting height (69 (6.3) cm v 73 (7.7) cm; p=0.004), sitting:standing height ratio (0.50 (0.02) v 0.51 (0.01); p<0.0001), weight (33 (10.9) kg v 41 (14.9) kg; p=0.001), functional residual capacity measured by a helium gas dilution technique (1.2 (0.3) l v 1.3 (0.4) l; p=0.04), FEV1 (1.5 (0.5) l v 1.9 (0.7) l; p=0.0008), FVC (1.7 (0.6) l v 2.1 (0.8) l; p=0.001), and PEF (3.9 (1.3) l/s v 4.8 (1.5) l/s; p=0.0004). The effect of age on lung function differed significantly between the children with SCD and the controls for total lung capacity and vital capacity measured by plethysmography and functional residual capacity measured by helium gas dilution.

Lung function differs significantly in children with SCD compared with ethnic matched controls of a similar age. Our results suggest that restrictive abnormalities may become more prominent with increasing age.