Abstract

Background: Pulmonary complications are a major cause of morbidity and mortality in sickle cell disease (SCD). The relationship of asthma with SCD and acute chest syndrome (ACS) remains uncertain. A study was undertaken to test the hypotheses that asthma and bronchial hyperreactivity (BHR) are more common in children with SCD than in ethnic matched controls and that SCD children with atopic asthma are more likely to have recurrent episodes of ACS.

Methods: A modified International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire was administered and skin prick tests undertaken in 80 children with SCD and 80 ethnic matched controls aged 5–10 years. BHR was assessed by measurement of forced expiratory volume in 1 second before and after a bronchodilator (albuterol 200 μg) or an exercise challenge.

Results: Asthma (48% v 22%, p = 0.002) and BHR (p = 0.02) but not atopy were more common in children with SCD than in controls. Atopy (66.6% v 29%, p = 0.007) and asthma (80% v 40%, p = 0.005), particularly atopic asthma (53% v 12%, p<0.001), were more common in children with SCD who had suffered recurrent episodes of ACS than in those who had suffered a single or no episode.

Conclusions: Asthma and BHR are more common in children with SCD than in ethnic matched controls, and atopic asthma appears to be associated with recurrent ACS. Early and effective anti-asthma therapy might reduce the pulmonary morbidity associated with SCD.