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A genetic condition, in sickle cell anemia, the patient becomes anemic due to a decrease of functional red blood cells in circulation. As a result of the disease, many red blood cells become hard, sticky, and deformed into crescent shapes. This deformity prevents the cells from traveling easily through the blood vessels, resulting in painful blockages called crises. Management of sickle cell anemia includes blood transfusions, supplements intended to encourage red blood cell production, and bone marrow transplants.
Red blood cell transfusions can be a major component of the management of sickle cell anemia. Transfusions are given to patients as needed and may be periodic or routine depending on the severity of their disease. The transfusions replenish functional red cells and can help to prevent crises and suppress the production of sickle cell hemoglobin (HbS). There are a number of risks associated with this form of management, especially over time, including transfusion-transmitted infections and alloimmunization, where the transfused blood produces antibodies against the patient.
Supplements given to patients in management of sickle cell anemia include folic acid, hydroxyurea, and erythropoietin. Folic acid is required during the production of red blood cells, so supplementation can help to increase production. This subsequently increases the rate at which the patient replenishes functional red cells and helps to prevent the patient from becoming anemic. Patients are generally prescribed one milligram of folic acid per day for life.
Hydroxyurea is given to patients with moderate to severe sickle cell anemia. The compound is issued in the management of sickle cell anemia because it increases the production of hemoglobin F, a functional form of hemoglobin. This hemoglobin increase can suppress the production of HbS and reduces both the number and severity of the painful crises. Hydroxyurea was once used as a chemotherapy drug, so it does have a number of side effects including nausea, vomiting, and bone marrow suppression.
Erythropoietin is a natural hormone in the body that stimulates the production of red blood cells. It occurs naturally in the body and increases during periods of anemia, but giving patients injections of the hormone during management of sickle cell anemia helps to provide them with an extra boost. As it occurs naturally in the bone marrow, there are very few associated side effects.
Finally, bone marrow transplantation is the only form of management of sickle cell anemia that acts as a cure. This form of treatment is typically given to younger patients who are extremely ill. Due to the necessity and difficulty of finding a matching donor, bone marrow transplants are not available to all who need them. There are also associated risks, including graft-versus-host-disease, where the transplanted cells attack the patient, infection, and bleeding. If successful, the life expectancy of the patient is significantly increased.
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