The Sickle Cell Disease Association of America (SCDAA) defines sickle cell disease as an inheritable disease that affects the shape of red blood cells. Instead of a normal round shape, the red blood cells are C- or sickle shaped. The shape of the cells causes different problems within the body. According to the SCDAA, the most common types of sickle cell disease are sickle cell anemia, sickle-hemoglobin C disease, sickle beta-plus thalassemia and sickle beta-zero thalassemia. The most well known is sickle cell anemia, where the body does not have enough healthy red blood cells to function the way it needs to. There is currently no definitive cure for sickle cell disease.
Red Blood Cell Destruction
The fact that the sickle-shaped red blood cells do not have normal hemoglobin means they do not live as long as normal blood cells. The National Heart Lung and Blood Institute reports that a normal blood cell lives for 120 days, while a sickle blood cell lives for 10 to 20 days. The short life span means the body is constantly destroying and making red blood cells. After a while, the body cannot keep up with the demand, and not enough red blood cells are made. This will lead to fatigue, rapid breathing and lack of endurance.
Clotting and Obstruction
The sickle cell is not only oddly shaped, but it can become rather rigid. The combination of these factors can cause the cells to become jammed or stuck in any blood vessel, usually near those branching off of arteries. This jam will not allow other blood cells to pass through and will start to damage the blood vessel locally. This clotting can lead to a number of complications, including acute lung syndrome, which causes difficulty breathing and is a medical emergency; pain episodes near the area and downstream of the block; stroke; priapism, a long-lasting painful erection; blindness from cells damaging the blood vessels of the eye; and damage to other organs such as the kidneys and spleen.
Infections
The spleen is a small organ located under the front of the left rib cage, near the stomach. It helps filter and destroy old or oddly shaped red blood cells, and helps the immune system by searching for germs. In sickle cell anemia, the spleen is overrun with destroyed red blood cells and starts to enlarge from not being able to remove all the waste products of blood destruction. It becomes harder for the spleen to recognize germs in the blood, thus leading to a widespread infection in the blood that normally would have been found. This is called septic infection and can be fatal. If the spleen becomes too overrun with destroyed red blood cells, it will enlarge to the point that it may become damaged, or it may erupt and have to be surgically removed.
Jaundice and Gallstones
Part of the destruction of red blood cells is the production of bilirubin. The gallbladder uses bilirubin and helps to remove it from the body. Too much bilirubin in the system will cause the skin and the whites of the eyes to turn yellow. High levels of bilirubin will also lead to the formation of gallstones. Gallstones can be very painful as they try to exit the gallbladder, and can cause a complete blockage of the bile duct. If this happens, the stones will need to be reduced in size or surgically removed. If there are more stones in the gallbladder, the whole organ may be removed.
