Sickle cell disease is a blood disorder in which the body
produces an abnormal type of the oxygen-carrying substance hemoglobin in the
red blood cells. Normal hemoglobin is called hemoglobin A, but people with
sickle cell disease have only hemoglobin S, which turns normal, round red blood
cells into abnormally curved (sickle) shapes.
cells are destroyed by the body faster than normal blood cells, which can
result in an inadequate oxygen supply to the body (anemia). Most people with
sickle cell disease have at least mild symptoms of chronic anemia,
Sickle-shaped blood cells are also more likely to get stuck
in and block small blood vessels throughout the body. Reduced blood flow caused
by blocked blood vessels can damage certain organs, muscles, and bones. This
may cause repeated episodes of pain (called sickle cell crises) that may last
from hours to days. The pain most often occurs in the bones of the
spine, arms and legs, the chest, and the abdomen.
People who have
sickle cell disease need special medical care throughout their lives to treat
the variety of problems that can be caused by the illness.
October 1, 2012
E. Gregory Thompson, MD - Internal Medicine & Martin Steinberg, MD - Hematology
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