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Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders that are typically inherited. [2] The most common type is known as sickle cell anemia. [2] Sickle cell anemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. [2]
The disease affects how oxygen is delivered to the red blood cells and is often diagnosed at a young age, discovered through a diagnosis of anemia. In a 2019 study on racial discrimination and dental health in the U.S. the authors found that the "emotional impact of racial discrimination" results in fewer visits to a dentist. [49]
This is unreliable evidence with the disease being present in many different groups as noted above with the trait also present in some Mediterranean European populations. Definitive diagnosis comes from examining the blood of the patient. In the US, screening for sickle cell anemia is done on all newborns regardless of race. [95]
Populations with West African ancestry, including among the African diaspora brought via the Trans-Atlantic slave trade, tend to have occurrences of sickle cell anemia and thalassemia. [42] The Sickle Hemoglobin S trait occurs in 8% of African Americans, and, generally, sickle cell anemia occurs in 0.02% of African Americans. [42]
This would be similar to the hemoglobin allele which is responsible for sickle-cell disease, but solely in people with two copies; those with just one copy of the allele have a sickle cell trait and gain partial immunity to malaria as a result. This effect is called heterozygote advantage. [27]
In 1987, Wethers acted as the chairwoman of a panel on sickle-cell screening commissioned by the National Institutes of Health. [5] The panel recommended that all newborn babies, regardless of ethnicity, be tested routinely for sickle-cell anemia; by 2006 this practice had been implemented in all fifty states. [1]
Hereditary blood disorders, such as sickle cell anemia and thalassemia, produce an effect on the development of hemoglobin, which, consequently, prevents the reproduction of malaria parasites within the erythrocyte. [15]
Red cell alloimmunisation is common in people with sickle cell disease who receive transfusions in Europe and North America. [4] This is because there are ethnic differences in the frequencies of blood group antigens. [4] Blood donors are usually Caucasian whereas the blood transfusion recipients usually have an African or Afro-Caribbean ancestry.