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Sickle Cell Vasculopathy


Sickle cell disease affects the red blood cells and is one of the most common genetic (inherited) diseases. It affects 70 000 people in the United States. The normal red blood cell carries oxygen and delivers it to the tissues. Sickle-shaped red blood cells have difficulty traveling through the blood vessels to deliver oxygen to the tissues. These sickle cells are more stiff and can get stuck inside the blood vessels. People with sickle cell disease often have problems caused by blockages of these sickle cells within their blood vessels. Compared with normal red blood cells, sickle cells are more fragile and are often broken up within the blood vessels. When red blood cells are broken, the affected person becomes anemic (low blood count) and may develop a yellow discoloration in the whites of the eyes called jaundice. Pain is a major consequence of red blood cells sickling within the blood vessels. When pain is severe, it is called a sickle cell pain crisis. The contents of the sickle cell released during its breakage can damage the blood vessel wall, resulting in vasculopathy (disorder of the blood vessel wall that further restricts blood circulation to tissues or organs). SIGNS AND SYMPTOMS Pain in the affected area of the body Fever Acute chest syndrome—pain in the chest associated with cough, shortness of breath, and fever resembling pneumonia Jaundice Lower leg ulcers that are often slow to heal Stroke when a blood vessel in the brain is affected, resulting in lost or reduced function in the affected part of the body Priapism—painful erection that can last more than 4 hours when the blood vessels in the penis are affected Pulmonary hypertension—elevated blood pressure in the artery that carries the blood from the heart to the lungs TREATMENT Medication to relieve pain Intravenous fluid hydration Oxygen therapy Wound care for ulcers Blood transfusions Hydroxyurea—a drug used to decrease the frequency of pain crises and need for blood transfusions Medications for pulmonary hypertension PREVENTION Individuals with sickle cell disease should Drink plenty of fluids Avoid extreme exertion Avoid extremes of temperature Avoid high altitudes Get appropriate vaccinations as recommended by your physician, including an annual influenza vaccine and the pneumococcal vaccine Discuss with your doctor whether hydroxyurea treatment is appropriate for prevention of sickle cell complications FOR MORE INFORMATION Centers for Disease Control and Prevention http://www.cdc.gov/features/sicklecell Sickle Cell Disease Association of America http://www.sicklecelldisease.org Source: JAMA. 2008;300(22):2690.