Sickle-cell disease in Malawian children is associated with restrictive spirometry: a cross-sectional survey

Abstract

A proportion of children with sickle-cell disease (SCD) demonstrate clinical findings consistent with the diagnosis of asthma. These children are at increased risk of complications, including acute chest syndrome. To assess lung function and symptoms of asthma in children with SCD in Blantyre, Malawi. Twenty-five children aged 7-16 years with electrophoretically confirmed SCD were recruited to undergo spirometry and questionnaire screening of asthma symptoms. Forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC) and FEV1/FVC ratio were compared with local and international reference ranges. Symptoms were assessed using the International Study of Asthma and Allergies in Childhood questionnaire. Mean spirometric indices, represented as Z-scores derived from international reference ranges, were low: FEV1 -1.64 (95%CI -2.04 to -1.23), FVC -1.49 (95%CI -1.90 to -1.09), FEV1/FVC -0.39 (95%CI -0.76 to -0.03). Comparison with local reference ranges, represented as percentage of predicted value, revealed similar impairments: FEV1 86.9 (95%CI 81.1 to 92.7), FVC 89.0 (95%CI 83.5 to 94.4), FEV1/FVC ratio 97.7 (95%CI 95.4 to 99.9). The prevalence of wheeze was 16.7%. We present spirometric abnormalities suggestive of restrictive lung disease with no evidence of obstructive defects or increased prevalence of wheeze.