Sickle Cell Disease with Special Emphasis to African Americans: An Overview

Abstract

September is sickle cell awareness month which dictated us to write an overview. The term Sickle Cell Disease (SCD) describes a group of inherited red blood cell (erythrocytes) disorders. People with SCD have abnormal haemoglobin, called haemoglobin S or sickle haemoglobin, in their red blood cells. SCD is a serious blood disorder that affects the red blood cells, which use a protein called haemoglobin to transport oxygen from the lungs to the rest of the body [1]. Mutations in the haemoglobin gene cause sickle cell disease. SCD is the most common genetic disorder in the United States with higher frequency in African Americans when compared to other populations. About one in 500 African American babies are born with sickle cell anemia and one in 12 African American people carry the gene for this trait or disease. About 100,000 Americans are thought to be living with SCD, and every year another 1,000 babies are born with the disease. Approximately 3 million Americans including 10% of African American population carry one gene for SCD (i.e. sickle cell trait). SCD has several recognized forms including sickle cell anaemia, sickle cell haemoglobin C disease and sickle cell thalassemia disease. The red blood cells of patients with SCD don’t live as long as healthy red blood cells. So people with this disorder often have low red blood cell counts (anemia), which is why this disease is commonly referred to as sickle cell anaemia. Affected patients characteristically are asymptomatic until approximately 4 to 6 months of age. The median age at death is approximately 42 years for men and 48 years for women [2,3].