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Sickle cell disease
refers to a group of inherited blood diseases which can cause severe
pain, damage to vital organs and for some,
death in childhood or
early adulthood. Sickle cell disease affects the oxygen-carrying
protein inside the red blood cells called hemoglobin.
Sickle cell disease
occurs when an individual inherits two sickle hemoglobin genes from
both parents or a combination of one sickle
hemoglobin gene and
another abnormal hemoglobin gene from the parents.
In sickle
cell disease, the red blood cells which are normally round in shape change to a
distorted, rigid, sickled shape when oxygen is
released from the red blood cell. Sickled red blood cells cannot
pass easily through the blood vessels and therefore block the normal
blood
flow. This blockage of blood flow causes severe pain and damage to
organs and tissues.
Although there is currently no cure for sickle cell disease, a few effective treatments
and appropriate medical care have greatly improved
survival and the quality of life for persons with sickle cell disease.
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