Background. Using data from the Cooperative Study of Sickle Cell Disease which ran from 1978 -19**, published figures for median life expectancy for men with sickle cell disease is 42 years and for women 46 years. Recent data from Texas show a declining childhood mortality rate, compared to the CSSCD data, in a cohort who have been followed from infancy. However, despite the strides made in reducing childhood mortality and improving length and quality of life in some adults, there is still very significant morbidity and mortality from cumulative damage to organs, especially the lungs and the kidneys.

Aims. The purpose of this investigation is to analyze the numbers and causes of death in individuals with sickle cell disease over the past several years in North Carolina. The information gathered may suggest areas in which health services to people with sickle cell disease may be improved.

Methods. The North Carolina State Center for Health Statistics provided the following data for the years 1997–2003. Total number of SCD deaths for each of those years, and the age, county of residence, place of death, the ICD codes for the underlying and contributing cause of death as listed on the death certificates of the decedents.

Results. There were 277 deaths over the 7 year period. Fifty-three percent of these deaths occurred during the earlier half of this period. Overall, 49% of the deaths occurred in people from age 30 to age 49, and this proportion remained quite stable during the 7 years. Deaths of people 20 years and younger made up 14% of total deaths; there were fewer in the second half of the time period than in the first half—40% of the total deaths in this age group occurred from late 2000 through 2003 vs 60% from 1997 through early 2000. Deaths in the youngest age group (0–5) were only 4.3% of the total deaths for the period. For the individuals who died between birth and 20 years of age, the underlying cause of death from the death certificates were grouped as “sickle cell disease with or without complications” in 50%, congenital heart disease in 11%, bacterial infection in 17%, gastroenteritis in 9.5%, UTI in 6% and other in 4%. For those who died between 30 and 40 years of age the underlying cause of death as listed on the death certificates were grouped as: sickle cell anemia, with and without crisis—58%, pulmonary complications—12%, cardiovascular problems—10%, CNS problems—5%, hepatic disorders and infections—each <5%, and other—10%.

Conclusions. Mortality in the youngest children appears to have lessened in comparison with historical data, and this age group no longer leads in mortality rates. Deaths in the young adult age range of 30–49 years have showed no downward trend over 7 years. The cause of death from death certificates do not match well with the clinical problems typically seen in people in this age group. The lack of precise diagnoses on death certificates may reflect a lack of understanding of sickle cell complications among doctors in the community.