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An analysis of a prospective cohort of individuals with sickle cell anemia (SCA), enrolled from birth through adulthood, was conducted to determine if asthma is a risk factor for death in SCA. All-cause mortality was determined for participants after adjusting for known risk factors for death in SCA. The study included 1,963 individuals who were followed for 18,495 patient-years. After controlling for established risk factors, individuals with SCA and asthma had a more than two-fold higher risk of mortality (hazard ratio 2.36, 95% CI 1.21 to 4.62, p=0.01). To summarize, asthma is a risk factor for death in SCA.