BACKGROUND

Sickle cell disease is an inherited blood disorder characterized primarily by chronic anemia and periodic episodes of pain. In sickle cell anemia, the hemoglobin is defec-tive. After hemoglobin molecules give up their oxygen, some may cluster together and form long, rod-like structures. These structures cause red blood cells to become stiff and assume a “C” or sickle shape.

Unlike normal red cells, which are usually smooth and donut-shaped, sickled red cells cannot squeeze through small blood vessels; but they instead stack up and cause blockages that deprive organs and tissues of oxygen-carrying blood. This pro-cess produces periodic episodes of pain and ultimately can damage tissues and vital organs and lead to other serious medical problems. Normal red blood cells live about 120 days in the bloodstream, but sickled red cells die after about 10 to 20 days. Because they cannot be replaced fast enough, the blood is chronically short of red blood cells, resulting in anemia.

Sickle cell is carried by genes that control the manufacture of hemoglobin, the pro-tein that carries oxygen within the red blood cell. A child must receive the gene from both parents in order for the condition to be expressed; a single copy of the gene is latent. In the U.S., about 2 million people are carriers of the trait, amounting to 1 in 12 African Americans. Sickle cell occurs in about one in every 500 African American births.1 Overall, SCD affects an estimated 70,000 to 100,000 Americans.2

A study reported in The American Journal of Hematology in 2009 showed that an av-erage patient with sickle cell disease, by age 45, had total undiscounted health care costs in excess of $950,000. The same study further estimated the cost of medical care for the roughly 70,000 individuals with sickle cell disease in the U.S. at more than $1.1 billion.3

Sickle Cell Disease and SC Medicaid Recipients SFY 2010 Fact Sheet

1 www.ornl.gov/sci/techresources/Human_Genome/posters/chromosome/sca.shtml 2 www.cdc.gov/NCBDDD/sicklecell/data.html3 onlinelibrary.wiley.com/doi/10.1002/ajh.21443/pdf

SC Medicaid Population Demographics Sickle Cell Disease–2010

Ages Race Gender Geographic Residence

18 and Under 1,497 Black 2,313 Female 1,777 Rural 947

19-64 1,427 White 75 Male 1,170 Urban 1,979

65+ 23 Hispanic 18 Unclassified 21

Other 541

Expenditure and mapped prevalence data for Medicaid recipients in South Carolina is provided on the reverse side.

SC FISCAL YEAR 2010

A total of 2,947 Medicaid recipients had paid claims associated with a primary diag-nosis of sickle cell disease or 0.31 percent of the total Medicaid recipient population. Total medical expenditures for this population were $43,563,083, accounting for 0.84 percent of the state Medicaid expenditures.

Developed by the Division of Policy and Research on Medicaid and Medicare at the University of South Carolina’s Institute for Families in Society under contract to the SC Department of Health and Human Services. This factsheet and important information about its use and data limitations can be found at ifs.sc.edu/PRMM/Factsheets. Suggested citation: Lòpez-De Fede, A., Mayfield-Smith, K., Stewart, J., Sudarshan, N., Rodgers, M., & Sudduth, D. (2012). Sickle cell disease and SC Medicaid recipients: SFY 2010 factsheet. Columbia, SC: Institute for Families in Society, University of South Carolina.