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Sickle cell anemia is an inherited blood disease that causes red blood cells
to become deformed (sickle-shaped). The red blood cells deform because they
contain an abnormal type of hemoglobin,
called hemoglobin S, instead of the normal hemoglobin, called hemoglobin A.
A person with sickle cell anemia can have sickle cell trait or sickle cell disease. In the United States, sickle cell anemia is
found mainly in African Americans and in people of Hispanic descent from the
Caribbean and Central America.
Each person inherits two sets of chromosomes (one from each parent). As a result, a
person may have:
Two sets of chromosomes that produce
normal hemoglobin (hemoglobin A). These people have normal red blood cells
(unless they have some other disease).
One set of chromosomes that produces
hemoglobin A and one set that produces hemoglobin S. These people carry the sickle cell trait
(and so are called “carriers” of sickle cell anemia) but they
do not have sickle
cell disease. Sickle cell trait is usually a harmless condition.
Two sets of chromosomes that produce
hemoglobin S. These people have sickle cell disease. A person may have sickle
cell disease if both parents have sickle cell trait. Red blood cells that
become sickled often cause recurring health problems (called sickle cell crises).
One set
of chromosomes that produces
hemoglobin S and one set that produces some other abnormal type of hemoglobin.
Depending on the other type of abnormal hemoglobin, these people can have
mild or severe sickle cell disease.
Sickle cell test
The sickle cell test determines
whether red blood cells in a blood sample become sickle-shaped after adding
a certain chemical solution.
During a sickle cell test, a blood
sample is mixed with a solution that causes red blood cells containing hemoglobin
S to become sickle-shaped. If sickled cells are found, a second test (called
hemoglobin electrophoresis) is needed to diagnose the exact blood disease
and to measure the amount of each type of hemoglobin.
This test is done on a blood sample
taken from a vein.
Sickle Cell Test Results
Normal:
No sickle-shaped red
blood cells are present or develop during the test.
Abnormal:
Sickle-shaped red blood cells are present. The amount of hemoglobin
S in each red blood cell determines how quickly and completely the cells turn
sickle-shaped. The majority of red blood cells from a person who has sickle cell disease
are sickled. Less than half of the red blood cells from a person with sickle cell trait
are sickled.