SICKLE CELL ANAEMIA

What Is Sickle Cell Disease?

It is an inherited blood disease which can cause bouts of pain or crisis, damage to vital organs, and for some, death in childhood or early adulthood. Sickle cell disease affects a protein inside the red blood cells called haemoglobin. It occurs when a person inherits two sickle cell genes or a combination of one sickle cell gene plus any one of several other abnormal haemoglobin genes that affect the red blood cells.

Sickle-Cell, a sickle-shaped blood-cell, esp. as found in a type of severe hereditary anaemia.

Haemoglobin in the red blood cells carries oxygen from the lungs and takes it to every part of the body. A change in the oxygen- carrying haemoglobin inside red blood cells causes the symptoms of sickle cell disease. Red blood cells are normally round and flexible. But when oxygen is released by the red blood cells in people with sickle cell disease, the cells become distorted, forming a rigid banana or sickle shape that can clog blood vessels.

Sickle cells tend to become trapped and destroyed in the liver and in the spleen. This results in a shortage of red blood cells called anaemia which, when severe, can cause the patient to be pale, short of breath and easily tired. Certain conditions such as infections may worsen a patient's anaemia by speeding up destruction of red blood cells or reducing red blood cell production.

Two of the most common forms of sickle cell disease are sickle cell anaemia (SS disease) and sickle "C" disease. Sickle beta thalassemia is a less common form of sickle cell disease. The effects of sickle cell disease vary greatly from one person to the next. Some affected people rarely see their doctors for sickle cell-related complaints; others may be hospitalised frequently.