What is Sickle cell anemia? Sickle cell anemia (a common form of sickle cell disease) is a genetic blood disorder that affects the red blood cells in the body. Instead of being shaped like soft, spongy discs like normal red blood cells, blood cells affected by sickle cell anemia are curved like a sickle or crescent. This causes the blood cells to be sticky and less flexible than normal blood cells so that they may block blood flow through the blood vessels.
This blockage is dangerous because it causes not only acute pain (known as sickle crisis), but can also result in infection and organ damage. These blood cells also don’t live as long as normal blood cells (usually 120 days) and die after about 10 to 20 days. The body can’t produce enough red blood cells to keep up with the dying sickle cells, resulting in anemia. Other common complications of sickle cell anemia include increased risks of stroke, acute chest syndrome, pulmonary hypertension, blindness, skin ulcers, gallstones, and priapism.
What causes sickle cell anemia?
Sickle cell anemia is a genetic disorder and a person must be born with the disease in order to be affected by it. The sickle cell gene is passed down by the parents and both parents must carry the gene in order for a child to be born with the sickle cell disease. If only one parent passes down the gene, the child has what is called the sickle cell trait, and although they will not have the disease, they will pass the gene down to their children.
Red blood cells affected by the sickle cell disease contain abnormal hemoglobin (hemoglobin S) which cause the blood cells to mutate and become rigid, sticky and form the sickle or crescent shape.
Common treatments for sickle cell anemia
Sickle cell anemia is a lifelong disease with no commonly available cure. A small number of people may find a cure in blood and bone marrow stem cell transplants if they meet the requirements. Although not every person affected with sickle cell anemia will qualify for stem cell transplants, there are treatments available to help manage and even avoid certain complications of the disease. Antibiotics are prescribed to children until age 5 to help prevent life-threatening infections.
Pain medication is a common treatment for pain brought on by sickle crisis and may be bought over the counter or prescribed by a doctor depending on the severity of the sickle crisis. Blood transfusions increase the number of red blood cells in order to relieve anemia and to decrease the risk of stroke. Breaking supplemental oxygen helps add oxygen to the blood and relieves symptoms of acute chest syndrome and sickle cell crisis. Experimental treatments of sickle cell anemia include gene therapy, nitric oxide, and drugs to boost fetal hemoglobin production in the womb.