Sickle Cell Disease is a genetic blood disorder that occurs in one of every 500 African Americans, the predominant population impacted by it in the United States. To the naked eye a drop of blood is just one red mass. Viewed under the microscope however, one sees a lot of red bodies called cells. Though normally the red cells are round, in some individuals some of the red cells are shaped like a farmer’s sickle. They’re called sickle cells and the disease produced by them is known as Sickle Cell Anemia.
In order to understand Sickle Cell Anemia you need to know that all red blood cells contain a red substance called hemoglobin. Hemoglobin carries oxygen from the lungs to all parts of the body. The normal red cell contains normal hemoglobin; when it releases the oxygen it carries its shape remains round. But if the cell contains what is known as sickle hemoglobin, when it releases its oxygen the red cell’s shape can change to a sickle. In other words red cells become sickle shaped because they contain sickle hemoglobin which is different from normal hemoglobin.
You might ask now what is wrong with having red cells that are shaped like a sickle? Well, although sickle hemoglobin carries just as much oxygen as normal hemoglobin there are two important differences between sickled cells and the normal round cells. First, sickled cells flow differently in blood vessels. Round red cells are soft like a bag of jelly so their shape can be changed readily permitting them to flow through small blood vessels very easily. But sickle cells are hard like pieces of wood and tend to jam up in small blood vessels. The flow of blood then stops.
The second problem with sickle cells is that they do not last as long as normal red blood cells. Normal round red cells live about 120 days. Sickled red cells are more fragile than normal red cells and live for less than 60 days. The body cannot make red cells as fast as the sickle cells are being broken down. As a result the body has fewer red cells and less hemoglobin than normal, and this we call anemia. All of the medical problems in Sickle Cell Anemia are the result of either the plugging of blood vessels with sickle cells or the anemia.
Individuals have who have Sickle Cell Anemia have pain that might occur at any time in any part of the body. The pain might last for only a few minutes or for as long as a week or two. The pain might be so mild that aspirin relieves it or it might be severe enough to require hospital treatment. The pain occurs when sickle cells plug blood vessels and stop the flow of blood. Because of the anemia they usually tire easily. Some individuals have slow healing sores or ulcers on their legs. Some have gallstones and some have strokes. In addition to these and other problems, the life span of some individuals with Sickle Cell Anemia is shortened.
Although they tend to tire easily, in between the pain attacks, called sickle cell pain crises, children can play (they should not try to play on school teams) and go to school (their intelligence is not affected). Adults are able to work if the job is not too strenuous.
Unfortunately, no cure has been discovered for Sickle Cell Anemia. You are born with it and you have it all of your life. Primarily, to treat it doctors give medicine for the pain and blood transfusions are given when the number of red cells and quantity of hemoglobin in the body is too low.
It is important for you to know that everyone who has sickle hemoglobin in their red blood cells does not have Sickle Cell Anemia. Some are just carriers of the disease and the condition they have is called Sickle Cell Trait.
Incidence of Some Important Childhood Diseases
|Sickle Cell Anemia||1:500|
Thalassemia – 2 million with trait in US
Estimated 35,000 Greeks and Italians have trait in Chicago area