Mortality from sickle cell disease: Geographic and ethnic variation within the United States

Background: Sickle Cell Disease Sickle (SCD), an inherited hemoglobinopathy characterized by a chronic hemolytic anemia and frequent painful vaso-occlusive episodes, is a major health problem in the United States (US) that affects primarily minority populations. It yields significantly decreased life expectancy, with a devastating burden of disease on the patient and family. SCD is the most common genetic disorder identified at birth by newborn screening in the US. Little is known about geographic and ethnic variations in US mortality due to SCD. Objective: Describe mortality from SCD among blacks according to Hispanic ethnicity and state for the years 1999-2006. Methods: Mortality data was from the Centers for Disease Control and Prevention Compressed Mortality File available on the Wonder web site. Results: US mortality from SCD among black Hispanics (0.2 per 100,000, 95% confidence interval [CI] 0.13-0.29) was 6.5 times lower than that among non-Hispanic blacks (1.3 per 100,000, 95% CI 1.27-1.36). Among 28 states with reliable mortality rates, there was a two-fold ethnic difference between the lowest rate (0.9 per 100,000 in Connecticut, Maryland, and Massachusetts) and the highest rate (1.8 per 100,000 in South Carolina and Wisconsin). Variations in mortality were slight across US regions, and there was no correlation between mortality from SCD with and without crisis. Conclusions: Further study is required to determine whether observed variations in SCD mortality among blacks by ethnicity and state is due to artifact, variations in disease severity, or variations in access and utilization of health care.