Sickle Cell Defined
Sickle Cell Disease is a genetically inherited blood disorder prevalent among people of African, Caribbean, Central/South American, East Indian, Middle Eastern, Mediterranean, Asian and Southeast Asian ancestry. Sickle cell trait and disease is indigenous to all these populations because of malaria. These genetic mutations developed over thousands of years as a result of the human body’s natural immune response against malaria.
The disease process results in severe anemia, oxygen deprivation, poor circulation and extreme pain. Painful episodes known as “crisis” are disabling and debilitating. Complications associated with sickle cell disease are:
- Enlargement and damage of the spleen (life threatening, especially in children).
- Dysfunction or acute failure of the liver or kidneys.
- Damage to tissue and bone due to diminished blood flow and loss of oxygen.Organs most commonly affected are the heart, lungs, brain, shoulders and hips bones as well as upper and lower extremities – resulting in the development of arm and leg ulcers.
- Stroke due to infarction of blood vessels.
In some cases, beginning as early as the age of 6 months, individuals with sickle cell disease can experience frequent, severe painful episodes that require multiple hospitalizations for treatment. It is common to spend weeks, sometimes months in the hospital for excruciating crisis episodes, especially when accompanied by other complications. Even in the case of adequate medical care, severe disability or death may result in childhood and early adult years.
Center for Disease Control
In the United States1
The exact number of people living with SCD in the U.S. is unknown. However, the CDC in collaboration with the National Institutes of Health and 7 states (California, Florida, Georgia, North Carolina, New York, Michigan and Pennsylvania) are coordinating the Registry and Surveillance System for Hemoglobinopathies(RuSH) project to learn about the number of people living with SCD to better understand how the disease impacts their health.
It is estimated that:
- SCD affects 90,000 to 100,000 Americans.
- SCD occurs among about 1 out of every 500 Black or African-American births.
- SCD occurs among about 1 out of every 36,000 Hispanic-American births.
- SCT occurs among about 1 in 12 Blacks or African Americans.
- People with SCD have less access to comprehensive team care than people with genetic disorders such as hemophilia and cystic fibrosis. [Read article]