Sickle cell disease is an inherited disease in which red blood cells contain abnormal hemoglobin (the substance that carries oxygen to body tissues). This makes the red cells fragile, rigid, and sickle shaped.
The abnormal red cells may be destroyed prematurely, leading to anemia.
They may also become lodged in small blood vessels, obstructing blood flow and depriving organs of oxygen. This may lead to episodes of severe pain (sickle cell crises) and eventual organ damage. The condition is most common in people of African or Afro-Caribbean origin and is caused by an abnormal gene.
If a child inherits two abnormal genes (one from each parent), he or she will develop sickle cell disease.
However, if a child inherits only one abnormal gene, a condition known as sickle cell trait results, which does not usually produce symptoms. Screening for sickle cell disease may be offered in pregnancy or shortly after birth.
Treatment is aimed at preventing and treating the crises with medication, rehydration, or blood transfusions. In some cases, a bone marrow transplant may be an option.