What is Sickle Cell

Sickle cell is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle and break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickle-shaped red blood cells, which are stiff and inflexible, get stuck in small blood vessels. 

 

These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen and brain. A particularly serious complications of sickle cell anaemia is stroke & high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of people with sickle cell anaemia and can lead to heart failure.

 

A genetic problem causes sickle cell anaemia. People with the disease are born with two sickle cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait. About 1 in 7 Ugandans  carry the sickle cell trait, 1 in 125 have the disease. 

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Hemoglobin SS

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.

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Hemoglobin SC

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.

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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.

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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.

People with sickle cell trait don't have sickle cell disease or exhibit any signs of the disorder, but they can pass the gene for the disease to their children. With each pregnancy, two people with sickle cell traits have:

 

•  A 25 percent chance of having an unaffected child with normal hemoglobin

•  A 50 percent chance of having a child who also is a carrier

•  A 25 percent chance of having a child with sickle cell anemia

 

A blood test can show if you have the trait or anaemia.

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