Doctor's Notes on Sickle Cell Disease
Sickle cell disease is the most common hereditary blood disorder, in which, red blood cells become crescent-shaped (the sickle cell). In healthy individuals, hemoglobin (a molecule in the red blood cell) takes on oxygen and releases carbon dioxide. In sickle cell disease, the red blood cells carry an abnormal hemoglobin known as hemoglobin S which reverses normal hemoglobin activity by releasing oxygen and taking on carbon dioxide (deoxygenation). Sickle cells are very fragile and people with the disease are more likely to get infections. Sickle cell disease occurs almost exclusively among black Americans and black Africans.
Symptoms of sickle cell disease include infections (such as flu viruses, pneumonia, and salmonella). In a acute sickle cell attack (sickle cell crises) severe pain is common, and conditions or symptoms that may contribute to the painful sickle cell crisis include dehydration, infection, fever, decrease in oxygen to body tissue (hypoxia), bleeding, cold exposure, drug and alcohol use, pregnancy, and stress. Other serious symptoms of sickle cell disease include blood clots and strokes, eye problems (retinopathy, retinal detachment, bleeding in the eye), kidney damage, prolonged penis erections (priapism), spleen damage, anemia (symptoms of anemia are shortness of breath, lightheadedness, and fatigue), heart attacks, and liver problems.
Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.