Sickle-Cell Disease Guidelines
- Sickle-cell disease is inherited from both parents and is common in people of Mediterranean or African descent. When one parent has normal hemoglobin, which is a component of red-blood cells, and one parent has abnormal hemoglobin, or sickle-cell anemia, their child will have what is referred to as sickle-cell trait. In sickle-cell trait, the person makes both normal and abnormal hemoglobin. The child suffers none of the symptoms of sickle-cell anemia, but can pass the disorder onto their children if her partner also suffers from sickle cell anemia.
- Symptoms normally occur after the age of 4 months and include swelling of the hands and feet, fatigue, jaundice, infections, vision problems and delayed growth. Other symptoms may include fever, abdominal swelling, pale skin or nails, excessive thirst, chest pain and frequent urination.
- Sickle-cell disease can cause severe pain episodes sometimes called pain crises. These episodes can last hours or days. Some people have an episode a few times a year; others may only have one every few years. The disease also can lead to stroke, pulmonary hypertension, organ failure, blindness and gallstones. Most of these conditions arise from blocked blood vessels caused by the sickle cells.
- A blood test will reveal if the abnormal hemoglobin is present. When the blood test is positive, blood will be studied under a microscope to check for large quantities of sickle cells.
- Medications are used to relieve pain episodes and control symptoms. Antibiotics are used to fight infections that could lead to pneumonia. Droxia is a prescription medication that when taken daily reduces the number of pain episodes. A transcranial Doppler ultrasound machine is a device used to measure blood flow through the vessels of the brain and can reveal if you or your child is at a high risk for stroke. Blood transfusions relieve anemia by increasing the number of normal red-blood cells circulating in your body. When receiving regular blood transfusions, you will need to take a medication to reduce iron buildup in your body. Bone-marrow transplants may cure sickle-cell disease, but the procedure carries with the risk of your body rejecting the donated marrow. Your doctor will be able to advise you on the best form of treatment for you.