Sickle Cell Disease in the United States
Sickle cell disease (SCD) is one of the most common inherited blood disorders in the United States. While sickle cell disease disproportionately affects African Americans, nearly every ethnic population is affected. Compared with other inherited conditions, however, awareness of the disease remains low.1-4
In the United States, African Americans comprise over 90% of the population with sickle cell disease. People of Hispanic, South Asian, Southern European, and Middle Eastern descent are also affected.2,3,6
The highest concentrations of sickle cell disease occur in southern and eastern states. More than 50% of the population with sickle cell disease resides in just 10 states.2
High Prevalence, Low Awareness
~100,000 people in the United States are affected by sickle cell disease, more than 3 times the prevalence of some other rare inherited disorders.5
Despite its prevalence, awareness of and funding for sickle cell disease are lower than for less common genetic diseases. For example, funding per individual for cystic fibrosis was 7.6 to 11.4 times higher than for sickle cell disease in 2011.4,11
Cost of Care
Sickle cell disease is associated with frequent hospital admissions and high health care costs.
References: 1. Brousseau DC, Panepinto JA, Nimmer M, Hoffmann RG. The number of people with sickle-cell disease in the United States: national and state estimates. Am J Hematol. 2010;85(1):77-78. 2. Hassell KL. Population estimates of sickle cell disease in the US. Am J Prev Med. 2010;38(4 Suppl):S512-S521. 3. Ashley-Koch A, Yang Q, Olney RS. Sickle hemoglobin (HbS) allele and sickle cell disease: a HuGE review. Am J Epidemiol. 2000;151(9):839-845. 4. Adams-Graves P, Bronte-Jordan L. Recent treatment guidelines for managing adult patients with sickle cell disease: challenges in access to care, social issues, and adherence. Expert Rev Hematol. 2016;9(6):541-542. 5. Centers for Disease Control and Prevention. Data & Statistics: Sickle Cell Disease. https://www.cdc.gov/ncbddd/sicklecell/data.html. Accessed July 6, 2018. 6. Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood. 2010;115(22):4331-4336. 7. NORD (National Organization for Rare Disorders). Huntington’s Disease. https://rarediseases.org/rare-diseases/huntingtons-disease/. Accessed September 10, 2018. 8. Cystic Fibrosis Foundation. Patient Registry 2016 Annual Data Report. https://www.cff.org/Search.aspx?srchtext=2016%20Patient%20Registry. 9. Orphanet. Becker muscular dystrophy. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=98895. Accessed September 10, 2018. 10. Centers for Disease Control and Prevention. Hemophilia: Data & Statistics. https://www.cdc.gov/ncbddd/hemophilia/data.html. Accessed August 26, 2018. 11. Strouse JJ, Lobner K, Lanzkron S, Haywood C. NIH and National Foundation expenditures for sickle cell disease and cystic fibrosis are associated with PubMed publications and FDA approvals. Blood. 2013;122(21):1739. 12. Lentz MB, Kautz DD. Acute vaso-occlusive crisis in patients with sickle cell disease. Nursing 2018. 2017;41(1):67-68. 13. Lanzkron S, Carroll CP, Haywood C. The burden of emergency department use for sickle cell disease: an analysis of the national emergency department sample database. Am J Hematol. 2010;85(10):797-799. 14. American Society of Hematology. State of Sickle Cell Disease: 2016 Report. Washington, DC: 2016. Available at: http://www.scdcoalition.org/report.html.