The Blood Disorder Sickle Cell Disease

Sickle cell is an inherited blood disorder affecting the red blood cells (RBCs) in our bodies. What is different about the red blood cells of those with sickle cell disease is that their red blood...

Sickle cell is an inherited blood disorder affecting the red blood cells (RBCs) in our bodies. What is different about the red blood cells of those with sickle cell disease is that their red blood cells contain mostly hemoglobin S, which is an abnormal type of hemoglobin. The red blood cells are usually a sickle cell shape or crescent shape, which makes it difficult for them to pass through small blood vessels of the body. Because they can’t pass through these small blood vessels they stack up, blocking the vessel. When a blockage occurs, blood can’t flow the way it is suppose to in that part of the body. Tissue that does not receive blood flow lacks oxygen and other nutrients carried by the blood and can be damaged.

There is no current cure for sickle cell disease.

There are several types of sickle cell disease including sickle cell anemia (SS), sickle-hemoglobin C disease (SC), sickle beta-plus thalassemia, and sickle beta-zero thalassemia.

There is also something called a sickle cell trait. Sickle cell trait (AS) is also an inherited condition in which hemoglobin A and S are produced in the red blood cells, with more of A than S. The sickle cell trait is not a type of sickle cell disease like those listed above because those with sickle cell trait are healthy individuals. Hemoglobin genes are inherited in two sets, one set from each parent.

Sickle cell disease is diagnosed at birth through a blood test.

Medical problems can result from a person having sickle cell disease such as anemia, gallstones, jaundice, lung tissue damage, pain episodes in the abdomen, arms, chest, and legs. A person can also have a stroke as a complication of sickle cell disease.

As soon as an infant tests positive for sickle cell disease penicillin prophylaxis is started along with vaccination against pneumococcus bacteria and also folic acid supplementation. When complications arise from sickle cell anemia other treatments may include continued antibiotics, blood transfusions, intravenous fluids, pain management, and possible surgery.

Individuals can find out what trait they have by having a hemoglobin electrophoresis performed. This laboratory technique will determine what type of hemoglobin is in your red blood cells. Normal hemoblobin is (A), Sickle cell hemoglobin is (S), and other types of hemoglobin can be (C), (D), (E).

Individuals with Sickle cell disease can have anemia, jaundice or can form gallstones when sickle cells block the vessels leading to the lungs, lung tissue damage can result, or when they block other blood vessels chest pain may happen, or pain in the extremities, chest and abdomen can also occur. It can also cause priapism, or stroke and damage to the spleen, kidneys and liver.

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