Trends in Pediatric Sickle Cell Disease-Related Mortality in the United States, 1983-2002

Objective

To analyze trends in sickle cell disease (SCD)-related mortality among black children during 1983-2002.

Study design

Using the multiple-cause mortality files compiled by the Centers for Disease Control and Prevention's National Center for Health Statistics, we analyzed deaths among children classified as black who were age 14 years and younger and had SCD identified on their death certificates.

Results

Relative to the rate for 1983-1986, the SCD mortality rate for 1999-2002 decreased by 68% (95% confidence interval [CI] = 58% to 75%) at age 0 to 3 years, by 39% (95% CI = 16% to 56%) at age 4 to 9 years, and by 24% (95% CI = −9% to 47%) at age 10 to 14 years. For the most recent period studied, a significant (42%) reduction in mortality at age 0 to 3 years was seen between 1995-1998 and 1999-2002, with essentially no reduction in SCD mortality at older ages.

Conclusions

Recent decreases in SCD mortality in black children under age 4 years coincided with the introduction of the 7-valent pneumococcal conjugate vaccine in 2000, although temporal association is not evidence of causation. The lack of significant recent reduction in SCD mortality in older children indicates the need for new treatment approaches.

Abbreviations: ACIP, Advisory Committee on Immunization Practices, CF, Cystic fibrosis, CI, Confidence interval, Hib, Haemophilus influenzae type b, ICD-9, International Classification of Diseases, 9th revision, ICD-10, International Classification of Diseases, 10th revision, IPD, Invasive pneumococcal disease, PCV7, Seven-valent pneumococcal conjugate vaccine, SCD, Sickle cell disease