Sickle cell anemia causes hard, sticky, crescent-shaped red blood cells that block blood vessels, leading to painful crises. Management includes blood transfusions, supplements, and bone marrow transplants, but all have associated risks. Bone marrow transplants are the only cure but are not available to everyone.
A genetic condition, in sickle cell anemia, the patient becomes anemic due to a decrease in functional red blood cells in the circulation. Because of the disease, many red blood cells become hard, sticky, and twist into a crescent shape. This deformity prevents cells from traveling easily through blood vessels, causing painful blockages called crises. Management of sickle cell disease includes blood transfusions, supplements intended to encourage red blood cell production, and bone marrow transplants.
Red blood cell transfusions can be an important component of sickle cell management. Transfusions are given to patients as needed and may be periodic or routine depending on the severity of their disease. Transfusions replenish functional red blood cells and can help prevent seizures 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 transfused blood produces antibodies against the patient.
Supplements given to patients in the management of sickle cell disease include folic acid, hydroxyurea, and erythropoietin. Folic acid is needed during red blood cell production, so supplementation can help increase production. This subsequently increases the rate at which the patient replenishes functional red blood cells and helps prevent the patient from becoming anemic. Patients are typically prescribed one milligram of folic acid daily for life.
Hydroxyurea is given to patients with moderate to severe sickle cell disease. The compound is released in the management of sickle cell disease because it increases the production of hemoglobin F, a functional form of hemoglobin. This increase in hemoglobin can suppress the production of HbS and reduce both the number and severity of pain crises. Hydroxyurea was once used as a chemotherapy drug, so it has 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 times of anemia, but giving patients injections of the hormone while managing sickle cell disease helps provide them with an extra boost. Because it occurs naturally in the bone marrow, there are very few associated side effects.
Finally, bone marrow transplantation is the only form of sickle cell disease treatment that serves as a cure. This form of treatment is typically given to younger patients who are extremely ill. Due to the need for and the difficulty of finding a suitable donor, bone marrow transplants are not available to everyone who needs them. There are also associated risks, including graft-versus-host disease, where transplanted cells attack the patient, infection and bleeding. If successful, the patient’s life expectancy increases significantly.
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