Health maintenance for individuals with sickle cell disease begins with early diagnosis preferably as a newborn. It is mandated in most states that each newborn be tested for sickle cell. Health care for newborns with sickle cell should include penicillin prophylaxis, required vaccination against pneumococcus bacteria and any nutritional supplements necessary. Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusion and surgery. Individuals with sickle cell disease are best managed in a comprehensive multi-disciplinary program of care. All persons affected with Sickle Cell Disease should be followed by a hematologist. There should always be collarboration between the hematologist and any specialty service involved with the patient. It is also suggested that ophthalmology care should be initiated by five years of age as retinal detachment or hemorrhage have been detected early in life. Make certain that all medical caretakers are aware of the diagnosis of Sickle Cell Disease.
Mild pain episodes usually can be treated with pain medication, water and bed rest. While treating mild pain, the patient should increase his or her daily water intake by at least 50%, and should be sure that his or her environment is well ventilated. The pain intensity should be monitored because mild pain can evolve into severe pain. However, all pain is not necessarily sickle cell related. Any prolonged or frequent pain in any part of the body should be brought to the attention of a health care provider. Severe pain episodes, also known as pain crises, may require treatment in a medical emergency facility. Treatments for severe pain usually include intravenous (administered directly into the vein) hydration, deep breathing and relaxation therapies together with pain medication. Oxygen therapies may be used if there are lung complications.
Dehydration occurs when the body contains low levels of fluid. When this happens blood can thicken and create blockages in narrow blood vessels of individuals with sickle cell disease. Muscles affected by blocked blood vessels can signal pain. That is why sickle cell patients are given fluids intravenously to rapidly hydrate them.
Normal red blood cells (hemoglobin A) have a life span of about three to four months. Sickle red blood cells (hemoglobin S) have a life span of about four to six weeks. Anemia is the result of the short life span of the sickle cells because new red blood cells produced by the bone marrow cannot keep up with the rate of the destroyed sickle cells. Individuals with sickle cell disease who overexert themselves may experience exhaustion rapidly because of the anemia. Overexertion will cause shortness of breath and will decrease oxygen supply for the body. All persons affected with sickle cell disease should learn about their laboratory results. They should request copies of the results and compare results to get to know their normal values.
Anemia is the normal state of individuals with sickle cell anemia. They should monitor their blood count levels to know what level is "normal" for them. This will avoid unnecessary blood transfusions. Individuals with severe anemia are sometimes treated with blood transfusions. Donor blood cells are added directly into the patient's blood stream. Some patients report renewed energy, and sometimes less pain, after receiving a blood transfusion.
Hydroxyurea is another medication that is currently used to treat chronic sickle cell patients. It increases the amount of fetal hemoglobin (hemoglobin that is resistant to sickling) in the blood. Hydroxyurea was found to reduce the frequency of severe pain, acute chest syndrome and the need for blood transfusions in adult individuals with sickle cell. Although the Food Drug Administration (FDA) approved the drug for use in adults in 1998, it can be toxic to the bone marrow and may cause very serious complications such as leukemia. For some, however, the benefits outweigh the danger. Its effectiveness varies from patient to patient. Studies are being conducted to determine the proper dosage for children.