What is Sickle Cell?
Sickle Cell Disorders are a group of inherited blood conditions that affects the red blood cells. These individuals carry the sickle haemoglobin S gene mutation on at least one beta globin gene. Normally, healthy red blood cells are round and biconcave in shape and move around the body carrying oxygen. However, in SCD, the red blood cells resemble the shape of a farming tool known as the “sickle”. The lifespan of the sickle cells is greatly reduced from their normal counterparts. These cells can also get stuck as they travel through the small blood vessels, potentially causing painful episodes, acute chest syndrome and other complications.
Individuals that inherit two Sickle Cell genes “S”, one from each parent, are known to have this type of SCD. It is also commonly known as Sickle Cell Anaemia and is usually the most severe type of SCD. These individuals also tend to have anaemia.
Sickle Cell Trait
Sickle Cell Trait (HbAS) is diagnosed when an individual inherits one normal hemoglobin gene (A), and one abnormal sickle cell gene (S). People with Sickle Cell Trait essentially live normal lives. However, they can still pass on the Hb S gene to their children.
In HbSC disease, individuals inherit a Hb S gene from one parent, and a Hb C gene from another parent. This is the second most common type of sickle cell disease, with patients having some symptoms which may differ from those with Sickle Cell Anaemia.
Hemoglobin S-B Thalassemia
Individuals with HbS beta thalassemia inherited one “S” gene from one parent, and one gene for beta thalassemia from the other parent. Symptoms tend to range from mild to severe in these individuals; those with HbS beta +-thalassemia usually have the mild form of the disease, and those with HbS beta 0-thalassemia having the severe form of Sickle Cell Disease.