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What is Sickle Cell Anaemia? All types of sickle cell disease are caused by a genetic change in the hemoglobin portion of the red blood cell. Hemoglobin is the oxygen-carrying protein inside the red blood cell. Normal red blood cells are oval and flexible. Red blood cells in sickle cell disease have a tendency to reshape themselves into rod-like structures that resemble the curved blade of a sickle; thus, the term sickle cell. Sickle cells have a shorter life span than normal red blood cells. This results in chronic anemia and reduced oxygen to the tissues of the body. In addition, sickle cells are less flexible than normal red blood cells. This presents a problem since they can be trapped in the small blood vessels preventing blood flow to the body's tissues. This compromise in the delivery of oxygen to the tissues results in pain and potential damage to the associated tissues and organs. |
Sickle
cell anemia is an inherited blood disorder of the hemoglobin (HbS).
Its main symptoms are long-term (chronic) anemia and episodes of
pain. In the disorder, hemoglobin molecules in the red blood cells,
which carry oxygen throughout the body, are defective.
These defective molecules cause some of the red blood cells to
change and form a sickle shape. These abnormally shaped blood cells
are the source of the anemia and pain. There are three types of
sickle cell anemia but with each their intensity.
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· Sickle cell anemia (HbSS) · Hemoglobin SA · Sickle cell beta thalassaemia (Hb SB thalassaemia) |
The worst type is sickle cell anemia (HbSS). Sickle cell anemia is
an inherited blood disorder where the red blood cells, normally
round in shape, are sickle, hence the name. This because a type of
haemoglobin called haemoglobin S. Normal haemoglobin that is
haemoglobin A causes sickle cell, is a protein in the red blood
cells that carries oxygen. Haemoglobin S however, reduces the
amount of oxygen within the cells and destroys their normal shape.
Consequently, the blood cells therefore act abnormal and this can
cause blood clots and may lead to painful joints and bones that can
be severe. Therefore causing damage to lungs, kidneys, liver, bones
and other organs and tissues.
People who inherit sickle cell gene from one parent and a normal gene from the other parent have a condition called sickle cell trait. Sickle cell trait is different from sickle cell anemia. People who have sickle cell trait don`t have the disease, but they have one of the genes that cause it. Like people who have sickle cell anemia, people who have sickle cell trait can pass the gene to their children.
