Healthy red blood cells are necessary to distribute oxygen. Sickle cell anemia, an inherited condition, makes the red blood cells firm and malformed into a sickle shap. Normally flexible and round, a patient who suffers from sickle cell anemia will have red blood cells that are shaped in the form of a crescent moon. Due to this change in the shape of the cell, the life cycle of the red blood cells in the person with sickle cell anemia is also shortened. The cell shape reduces the ability of the cells to carry oxygen and creates problems when they are unable to pass each other in the small capillaries. This results in an occlusive crisis.
A cure for sickle cell anemia has not been found. Treatment is aimed at alleviating the pain associated with the disease and reducing any future problems. Although difficult and rare to find a donor match, a bone marrow transplant will effect a cure for the disease. However, if it is not successful, it can also result in the death of the patient.
Other treatments for sickle cell anemia by medications, blood transfusions, and oxygen administration. Some of the medications used in the treatment of sickle cell anemia are antibiotics, hydroxyurea, and pain relievers. The antibiotics will be taken prophylactically in children to prevent infection during the growing years when it is most critical in the prevention of childhood illnesses. It is also used in adults for the same reason.
A cancer medication, known as hydroxyurea, can help in severe cases. It will decrease the need for blood transfusions. This medication should be used with discretion, as it is linked to tumor production.
Over-the-counter pain relievers may also be beneficial in alleviating pain from the symptoms of sickle cell anemia. Blood transfusions are effective in the treatment of sickle cell anemia due to the increased supply of needed red blood cells to the body. This will help to relieve the symptoms of anemia. The iron containing properties in a blood transfusion, however, is a cause for concern. When iron is excessive, damage to other internal organs is a possibility. Iron levels must be monitored and reduced with medication if need be. Treatment with oxygen can also be helping in the person with sickle cell anemia allowing them to breathe without causing their body any more strain or fatigue.
A bone marrow transplant offers the most relief for sickle cell anemia. Attempts to find a suitable donor are often lengthy. When found, the victims own bone marrow is eliminated first and replaced with the new. This procedure requires staying in the hospital for quite a few days and the new marrow does not always take and can be rejected. Medications are taken to help prevent this, but they do not always work.
Some possible new treatments that are being evaluated are: medications, research in gene study, and the use of nitric oxide. Because sickle cell is caused by a defective gene, scientists are experimenting with insertion of a normal gene into the bone marrow to increase the amount of normal red blood cells that are produced and potentially eliminate the sickle cells. Nitric oxide is a gas normally found in the body and helps to increase the flexibility of the blood vessels and reduce the stickiness of the red blood cells.
Patients can also help to reduce the potential for crisis by remaining well hydrated and drinking at least 8 glasses of water each day. Taking a multi-vitamin, getting enough sunlight for adequate amounts of vitamin D, getting 8 hours of sleep each night and remaining up to date on all immunizations will help to reduce the potential for infection, which can trigger a crisis.
Physicians may also recommend ultrasounds of the head every 2 years beginning at age 2 to evaluate blood flow in the brain. This is done to help prevent a stroke from sickling of the red blood cells and occlusive crisis in the brain.
RESOURCES
National Heart Lung and Blood Institute: How is Sickle Cell Treated
Dayton Children\’s: Sickle Cell
Center for Disease Control and Prevention: Complications and Treatment
The Children\’s Hospital of Philadelphia: Overview of Sickle Cell Disease
University of Maryland Medical Center: Sickle Cell Disease