Key points
- Sickle cell disease (SCD) affects about 100,000 people in the United States; more than 90% are non-Hispanic Black or African American, and an estimated 3%–9% are Hispanic or Latino.
- The estimated life expectancy of those with SCD in the United States is more than 20 years shorter than the average expected.
- Many people with SCD do not receive the recommended healthcare screenings and treatments.
Prevalence
Sickle cell disease (SCD) affects millions of people throughout the world and is particularly common among those whose ancestors came from parts of the world where malaria is or was common:
- Sub-Saharan Africa.
- Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America).
- Saudi Arabia.
- India.
- Mediterranean countries such as Turkey, Greece, and Italy.
- The exact number of people living with SCD in the United States is unknown.
- Two studies published in 2010 estimated that SCD affects approximately 100,000 people in the United States.
- More than 90% of people in the United States with SCD are non-Hispanic Black or African American (Black), and an estimated 3%–9% are Hispanic or Latino.
- SCD occurs in about 1 out of every 365 Black or African American births and about 1 out of every 16,300 Hispanic American births.
- About 1 in 13 Black or African American babies is born with sickle cell trait (SCT, inheritance of a sickle cell gene from only one parent).
Complications and Mortality
- In people with SCD, red blood cells become rigid and deform into a crescent or sickle shape. Sickled cells die early and often become lodged in small blood vessels, restricting blood flow, which can lead to serious health problems throughout the body.
- SCD-associated complications include anemia, acute and chronic pain, infections, pneumonia and acute chest syndrome, stroke, and kidney, liver, and heart disease.
- Estimated life expectancy of those with SCD in the United States is more than 20 years shorter than the average expected. Quality-adjusted life expectancyA is more than 30 years shorter.
Healthcare access
- Despite their extensive healthcare needs, many persons with SCD have difficulty accessing appropriate care and report feeling stigmatized and having their symptoms dismissed when they do seek care.
- In 2014, an expert panel convened by the National Institutes of Health's National Heart, Lung, and Blood Institute (NHLBI) developed recommendations to prevent or reduce complications of SCD. Two of the recommendations were specific to children and adolescents with sickle cell anemia (SCA, the type of SCD that is typically most severe):
- Given that SCA is a common cause of childhood stroke, the panel recommended that children and adolescents aged 2–16 years with SCA be screened annually with transcranial Doppler (TCD) ultrasound to identify those at increased risk for stroke.
- The panel also recommended that children and adolescents aged 9 months or older with SCA be offered treatment with hydroxyurea, a medication that has been shown to prevent or reduce severe pain episodes, acute chest syndrome, and other SCA-associated complications and to increase patient survival.
- Given that SCA is a common cause of childhood stroke, the panel recommended that children and adolescents aged 2–16 years with SCA be screened annually with transcranial Doppler (TCD) ultrasound to identify those at increased risk for stroke.
- A CDC study of children and adolescents enrolled in Medicaid found that in 2019 less than half of children and adolescents received the recommended stroke screening.
- Additionally, less than half of children aged 2–9 years of age were using hydroxyurea and approximately one half of those aged 10–16 years used hydroxyurea.
- These findings highlight the ongoing gaps in health care for people with SCD and speak to the urgent need to address barriers to care.
- Additionally, less than half of children aged 2–9 years of age were using hydroxyurea and approximately one half of those aged 10–16 years used hydroxyurea.
- Statistical estimate of the average life expectancy for a population of people that considers not only the expected length of life but also the likely quality of life as people age.
- National Academies of Sciences, Engineering, and Medicine. 2020. Addressing sickle cell disease: A strategic plan and blueprint for action. Washington, DC: The National Academies Press.
- Hassell KL. Population estimates of sickle cell disease in the U.S. Am J Prev Med. 2010;38(Suppl):S512-21.
- Brosseau 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 Jan;85(1):77-8.
- Ojodu J, Hulihan MM, Pope SN, Grant AM; Centers for Disease Control and Prevention (CDC). Incidence of sickle cell trait—United States, 2010. MMWR Morb Mortal Wkly Rep. 2014 Dec 12;63(49):1155-8. PMID: 25503918; PMCID: PMC4584538.
- Lubeck D, Agodoa I, Bhakta N, et al. Estimated life expectancy and income of patients with sickle cell disease compared with those without sickle cell disease. JAMA Netw Open. 2019;2(11):e1915374.
- National Heart Lung and Blood Institute, National Institutes of Health. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014.
- Schieve LA, Simmons GM, Payne AB, Abe K, Hsu LL, Hulihan M, Pope S, Rhie S, Dupervil B, Hooper WC. Vital Signs: Use of Recommended Health Care Measures to Prevent Selected Complications of Sickle Cell Anemia in Children and Adolescents — Selected U.S. States, 2019. MMWR 2022 Sep 30;71(39):1241-1246.