Sickle cell disease is a genetic disease where red blood cells are abnormally shaped. Different names for this disease, all of which vary slightly from one another, are: hemoglobin SS disease, sickle cell anemia, hemoglobin S beta thalassemia. Some people may carry the sickle cell trait, without suffering from the disease but pass it on to their children who do suffer.
Sickle cell disease is usually diagnosed at birth when a routine blood test is done. If the baby tests positive, a second blood test is performed (a hemoglobin electrophoresis). More than 40 states in theUnited States require this test to be carried out at birth, as these children have an increased rate of sickness and infection.
The only known cure for sickle cell disease is a bone marrow transplant. These are complex and difficult operations and are only an option for acute sufferers. However, even without this, children can lead a relatively normal life. The risk of death has also been reduced by the use of penicillin (or a similar antibiotic, amoxicillin) and appropriate immunizations. Medicines are available to help manage the pain, as well as regular immunization and daily doses of penicillin.
No two people suffering from sickle disease may experience the same side effects. Many people with sickle cell disease have some degree of anemia and may suffer from some of the following symptoms:
- serious infections
- chronic anemia
- damaged body organs
- Acute chest syndrome:
- Hand-foot syndrome (also called dactylitis) (the inflammation of an entire digit, finger or toe
- Splenic sequestration crises (when sickled cells block the blood vessels leading out of the spleen)
- Painful crises
- Aplastic crisis (when the body stops making red blood cells, resulting in anaemia)
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