Sickle Cell Anemia
What is Sickle Cell Disease?
Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin* S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels.
When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that doesn't receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There is currently no universal cure for sickle cell disease.
Hemoglobin is the main substance of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body. Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Normal red blood cells are soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live for about 120 days before new ones replace them.
Medical Problems
Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones.
The sickle cells also block the flow of blood through vessels resulting in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest, and abdomen, stroke and priapism. It also causes damage to mort organs including spleen, kidneys, and liver. Damage to the spleen makes sickle cell disease patients especially young children , easily overwhelmed by certain bacterial infections.
Treatment
Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis vaccination against pneumococcus bacteria and folic acid supplementation.
Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusions, and surgery all backed by psychological support. Like all patients with chronic disease patients are best managed in a comprehensive multi-disciplinary program of care.
Blood transfusions help benefit sickle cell disease patients by reducing recurrent pain crisis, risk of stroke and other complications. Because red blood cells contain iron, and there is no natural way for the body to eliminate it, patients who receive repeated blood r=transfusions can accumulate iron in the blood until it reaches toxic levels. It is important to remove excess iron from the body, because it can gather iron in the heart, liver, and other organs and may lead to organ damage. Treatments are available to eliminate iron overload.
AN ESTIMATED 100,000 PEOPLE LIVE WITH SICKLE CELL DISEASE IN THE U.S. AND MILLIONS AFFECTED GLOBALLY.
Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin* S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels.
When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that doesn't receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There is currently no universal cure for sickle cell disease.
Hemoglobin is the main substance of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body. Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Normal red blood cells are soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live for about 120 days before new ones replace them.
Medical Problems
Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones.
The sickle cells also block the flow of blood through vessels resulting in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest, and abdomen, stroke and priapism. It also causes damage to mort organs including spleen, kidneys, and liver. Damage to the spleen makes sickle cell disease patients especially young children , easily overwhelmed by certain bacterial infections.
Treatment
Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis vaccination against pneumococcus bacteria and folic acid supplementation.
Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusions, and surgery all backed by psychological support. Like all patients with chronic disease patients are best managed in a comprehensive multi-disciplinary program of care.
Blood transfusions help benefit sickle cell disease patients by reducing recurrent pain crisis, risk of stroke and other complications. Because red blood cells contain iron, and there is no natural way for the body to eliminate it, patients who receive repeated blood r=transfusions can accumulate iron in the blood until it reaches toxic levels. It is important to remove excess iron from the body, because it can gather iron in the heart, liver, and other organs and may lead to organ damage. Treatments are available to eliminate iron overload.
AN ESTIMATED 100,000 PEOPLE LIVE WITH SICKLE CELL DISEASE IN THE U.S. AND MILLIONS AFFECTED GLOBALLY.