Sickle Cell Disease Awareness

Sickle cell disease is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has Sickle cell Disease, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”. The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such infection, acute chest syndrome and stroke. 

Sickle cell disease is the most common genetic disease in the world and is most common in people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.

In the United States, about 1 in every 500 African Americans are born with the disease. Hispanic Americans also are affected at a rate of about 1 in 1,000 to 1,400.

​Specifically, Sickle cell disease is characterized by defective hemoglobin (a protein in red blood cells that carries oxygen to the tissues of the body).

Sickle cell disease inhibits the ability of hemoglobin in red blood cells to carry oxygen. Cells containing normal hemoglobin are smooth, disk-shaped, and flexible, like doughnuts without holes, so they can move through the vessels in our bodies easily. Cells containing sickle cell hemoglobin are stiff and sticky and form into the shape of a sickle or crescent, like the letter C, when they lose their oxygen. These sickle cells tend to cluster together and cannot easily move through the blood vessels. The cluster causes a blockage in small arteries or capillaries and stops the movement of healthy, normal oxygen-carrying blood. This blockage is what causes the painful and damaging complications of sickle cell disease.

Sickle cells only live for about 10 to 20 days, while normal red blood cells can live up to 120 days. Also, sickle cells risk being destroyed by the spleen because of their shape and stiffness. The spleen is an organ that helps filter the blood of infections. Sickle cells get stuck in this filter and die. Due to the decreased number of healthy red blood cells circulating in the body, a person with sickle cell disease is chronically anemic. The spleen also suffers damage from the sickle cells blocking healthy oxygen carrying cells and typically infarcts in the first few years of life. Without a normal functioning spleen, these individuals are more at risk for infections. Infants and young children are at risk for life-threatening infections.