Sickle Cell Disease

Sickle Cell Disease is a hereditary blood disorder that affects the oxygen-carrying part of blood, the red blood cell. In other words, it is visible by diseased red blood cells that have a sickle shape. Red blood cells have proteins called hemoglobin. Hemoglobin transports oxygen from your lungs to every part of your body. When a normal red blood cell (with normal hemoglobin) releases its oxygen, it maintains its disc shape. However, when a diseased red blood cell containing sickle hemoglobin releases its oxygen, the appearance of the cell can changes from disc shaped to sickle-shaped (crescent shape). Sickle hemoglobin within a particular red blood cell causes the sickle cell shape. Possible health problems may include fatigue, breathlessness, rapid heartbeat, delayed growth, vulnerability to infections, skin ulcers, and vision problems.

While sickle hemoglobin and normal hemoglobin carry the same amount of oxygen, two major differences exist between the two kinds of cells. First, they differ in the way they flow through the blood vessels. Normal hemoglobin is found in disc-shaped red blood cells that are soft (like a bag of jelly), which allows them to easily flow through small blood vessels. Diseased red blood cells, which are sickle-shaped and are hard (like pieces of wood), often get stuck in small blood vessels and stop the flow of blood.

The second difference between the two types of cells is their Life span. Sickle cells do not live as long as normal cells. Normal, healthy cells can survive for about 120 days, while the more fragile sickle cells can only survive for about 60 days or less. Unfortunately, the body cannot make new red blood cells as fast as it loses sickle blood cells. As a result, a sickle cell patient has fewer red blood cells and less hemoglobin than normal red blood cells. This decreased hemoglobin (called anemia), results in less oxygen being available for use by the cells of the body.

The only way you can get sickle cell disease is by genetic inheritance. You cannot get sickle cell disease by contact or by blood transfusions. People with sickle cell disease either have sickle cell trait or sickle cell anemia. The difference between sickle cell trait and sickle cell anemia is in the inheritance pattern of the sickle cell gene. As sickle cell clogged blood capillaries can lead to different types of problems, depending upon where the blockage occurs. The outcome of this blockage may lead to problems such as kidney infections; intense pain in the chest, arms and legs, death and decay