Sickle Cell Disease
Description
A disorder that causes abnormal red blood cells, and these cells can clog blood vessels. Sickle cell disease is the most common of the inherited blood disorders, and occurs almost exclusively among black Americans and black Africans. A sickle cell crisis causes pain because blood vessels become blocked and the defective red blood cells can damage organs in the body. The most commonly affected organs include: lungs, liver, bone, muscles, brain, spleen, penis, eyes, and kidneys. Patients also experience chronic anemia.
Symptoms
Abdominal pain, chest pain, flank pain, bone pain, breathlessness, delayed growth and puberty, fatigue, fever, jaundice, rapid heart rate, susceptibility to infections, ulcers on the lower legs (in adolescents and adults), anemia.
Tests
A history and physical exam will be performed. Tests will be performed to diagnose and monitor the disease.
- CBC
- Chem 12
- UA
Treatment
The goal of therapy is to control symptoms, and to limit the frequency of crises. Hydroxyurea (Hydrea) is used to reduce the acute episodes of pain but is not effective in all patients. An acute episode is treated with the following: intravenous fluids, supplemental oxygen, pain medications, antibiotics (if infection is found), and/or blood transfusions. For additional information contact the National Heart, Lung and Blood Institute Health Information Center at: http://www.nhlbi.nih.gov or call (301)592-8573. For more information contact: Sickle Cell Disease Association of America: (800)421-8453
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