How Common Is Sickle Cell Disease?
Millions of people around the world live with sickle cell disease (SCD). It is most common in Africa, the Middle East, and India. The exact number of people with SCD in the United States is unknown, but the estimate is around 100,000.1
Experts predict this number will increase over the next few decades. In the United States, health outcomes for people with SCD have improved greatly. However, complications from SCD limit opportunities and worsen quality of life for many people. Expanding access to care and reducing treatment costs can improve the lives of people with SCD.
Sickle cell disease around the world
There is no reliable estimate of global SCD cases. However, about 300,000 babies are born every year with sickle cell anemia. Most of these cases occur in Nigeria, the Democratic Republic of the Congo, and India.2
Experts predict this number will pass 400,000 by 2050. This is because low-income and middle-income countries are showing reduced infant mortality through better SCD diagnosing and treatments.2
The World Health Organization and United Nations recognize sickle cell disease as a global health issue. Many advances made in the treatment of sickle cell disease have not yet reached countries with high poverty levels. For example:3-7
- As high as 40 percent of people in some African countries have sickle cell trait
- In countries with poor resources, more than 90 percent of children with SCD do not survive to adulthood
- About 1,000 children in Africa are born with SCD every day, and more than half will die before they reach 5 years old
- About 20 percent of children with SCD in India die by 2 years old
- By 2050, the number of people with SCD will increase by 30 percent
SCD in the United States
SCD is more common in Americans whose ancestors lived in Africa, South and Central America, and India. The U.S. Centers for Disease Control and Prevention (CDC) projects that:1
- About 100,000 Americans have SCD
- About 1 in every 13 Blacks have sickle cell trait
- About 1 in every 365 Blacks have SCD
- About 1 in every 16,300 Hispanics have SCD
Health outcomes for people with SCD in the United States have improved because of better diagnosis and treatments. For example:8
- In the 1970s, life expectancy was less than 20 years old. Now, most people live past 50 years old.
- Until the 1990s, up to 30 percent of children with sickle cell anemia died from infections. Early diagnosis, antibiotics, and education have reduced this to below 3 percent.
- More than 90 percent of Americans with SCD live into adulthood
Despite these improvements, many people with SCD suffer from severe pain, depression, discrimination, and stigma. As health outcomes continue to improve, the number of Americans with SCD will increase. It is important to keep expanding access to care and reducing treatment costs.2
Race and ethnicity
The global distribution of sickle hemoglobin (HbS) overlaps mostly with areas affected by malaria. Malaria is an infectious disease spread by mosquitoes in tropical climates. Scientists first realized this in the 1950s. Data now proves that sickle cell trait (HbAS) protects people against severe malaria. In fact, people with sickle cell trait are 90 percent less likely to experience severe malaria.2,9
Researchers do not know exactly why sickle hemoglobin protects people from severe malaria. However, it explains why SCD is most common in the tropical climates of Africa, the Middle East, and India. Population movements have caused SCD to be found around the world, but it is most common in people whose ancestors lived in areas affected by malaria.2,9
How can we improve disease management?
There have been great improvements in SCD health outcomes.
However, there are many things that impact access to high-quality care, including:8,10-15
- Hydroxyurea improves SCD outcomes and reduces healthcare costs, however more than 75 percent of adults with SCD with frequent pain crises cannot get it
- Follow-up care after diagnosis in newborns is not performed for about 30 percent of people
- On average, total healthcare costs for people with SCD reach $1 million by age 45, with annual costs around $10,000 for children and $30,000 for adults
- People with SCD seeking care in the emergency room experience longer delays in treatment because of discrimination and a lack of specialized doctors, despite having higher levels of pain
- Only 33 percent of children with SCD are monitored for stroke risk
- 25 percent of children with SCD do not receive the pneumococcal vaccination
- Only 20 percent of family doctors feel comfortable treating SCD
- The transition from childhood to adult care has the greatest risk for hospital admissions and medical problems.