| Sickle cell anemia is an inherited condition caused by having abnormal hemoglobin, the protein that carries oxygen in the blood. Sickle cell anemia is usually inherited from parents who are carriers, who have the sickle cell trait, or one abnormal hemoglobin. Persons with the sickle cell trait do not have any symptoms and it is supposed to protect you from infection with malaria. This is a very common finding, occurring in about 8% of African-Americans in the United States.
Unlike the sickle cell trait, children with sickle cell anemia (SS disease) produce two abnormal hemoglobin proteins (inheriting one from each parent), which makes the red blood cells of children with this condition easily destructible and gives them a sickle-like shape. SS affects mostly blacks and occurs in 1 out of every 400 African-American babies in the United States each year.
The common symptoms of SS disease include anemia, or low blood counts, vaso-occlusive pain crises, which are severe episodes of pain in the arms, legs or back, chest syndrome, with coughing, chest pain and fever, dactylitis, or hand-foot syndrome, with painful swelling of the bones in the hands or feet of young children, aplastic crises, during which the body stops making new red blood cells causing severe anemia usually following an infection with the Parovirus B19 which causes Fifth disease, priapism, a painful and prolonged erection, stroke, usually causing sudden weakness of one side of the body, and acute splenic sequestration, with pooling of blood causing a sudden enlargement of the spleen. Children with SS disease are also at increased risk of infection, especially from the Streptococcus pneumonia and H. influenzae bacteria, and they routinely receive prophylactic antibiotics for children under 5 years of age and appropriate vaccines.
Most children are diagnosed with SS disease by the newborn screen testing that is performed in the nursery when they are born. For children who weren't tested by the newborn screen, an electrophoresis test of the blood can detect the abnormal hemoglobin of SS disease.
Treatments are usually supportive and includes frequent monitoring of red blood counts, antibiotics for infections, intravenous fluids and pain medicines for pain crises, transfusions for aplastic crises and splenic sequestration if necessary, and antibiotics, oxygen and respiratory support for chest syndrome. Newer treatments are being studied and include hyroxyurea and bone marrow transplants.
Recommended Reading List:
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Resource List:
- American Sickle Cell Anemia Association: This site is intended to provide a variety of information as it relates to sickle cell anemia.
- Sickle Cell Society: The Sickle Cell Society was first set up as a registered charity in 1979. It was formed by a group of patients, parents and health professionals who were all concerned about the lack of understanding and the inadequacy of treatment for sufferers of sickle cell disorders.
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