Question: What causes sickle cell anaemia

Cheryl Andisi answered on 26 Jan 2017:

@aawb279,
Hello,
Sickle cell anemia is a group of inherited blood disorders where there is a mutation on the gene coding for hemoglobin resulting in an abnormal hemoglobin molecule called hemoglobin S. An individual can inherit two copies of abnormal hemoglobin S, if both the parents are carriers ie parents have one copy of normal and one copy of abnormal hemoglobin.
Red blood cells that contain normal hemoglobin are disc shaped. This shape allows the cells to be flexible so that they can move through large and small blood vessels to deliver oxygen. Sickle hemoglobin is not like normal hemoglobin. It can form stiff rods within the red cell, changing it into a crescent, or sickle shape.
Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can’t reach nearby tissues. These cells are get destroyed more readily leading to anemia.

Currently, there are treatments that can help people suffering from sickle cell disorders to help them lead a normal life. These include treatment with hydroxyurea, blood transfusion in anemic cases and screening of people in populations where there is high frequency.

Sickle cell trait-carrier status has been shown to protect people from severe malaria. It is thought that this might be one of the reasons why there is high frequency of sickle cell disorders in malaria endemic regions.

If you have family-parents and or siblings who suffer from sickle cell disorders, it might be worth getting tested for sickle cell genes, and that of your partner in future, to avoid passing the genetic disorder to your offspring.