Introduction Types of sickle cell Causes of sickle cell Symptoms Exams and Tests Treatment References
Sickle cell disease refers to a genetic illness Sickle cell anemia is one type of anemia. Anemia is a condition in which your blood has a lower than normal number of red blood cells. This condition also can occur if your red blood cells dont contain enough hemoglobin It occurs when an individual inherit two Hb S genes from both parents
Sickle cell anemia in which red blood cells form an abnormal crescent shape. (Red blood cells are normally shaped like a disc.) Sickle cells have a shorter-than-normal life span, which leads to anemia (low RBC count). A normal red blood cell lives for about 120 days in circulation, whereas a sickle cell lives for only 10 to 20 days.
there are many different types of sickle cell disease, but Hemoglobin SS, Hemoglobin SC, and Sickle Beta Thalassemia are the most common types of sickle cell disease
Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen. Hemoglobin S changes the shape of red blood cells, especially when the cells are exposed to low oxygen levels. The red blood cells become shaped like crescents or sickles. The fragile, sickle-shaped cells deliver less oxygen to the bodys tissues. They can also get stuck more easily in small blood vessels, and break into pieces that interupt healthy blood flow.
Symptoms usually dont occur until after age 4 months. Common symptoms include:1) Attacks of abdominal pain2) Bone pain3) Breathlessness4) Delayed growth and puberty5) Fatigue6) Fever7) Paleness8) Rapid heart rate9) Ulcers on the lower legs (in adolescents and adults)10) Yellowing of the eyes and skin (jaundice)
Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:1) Complete blood count (CBC)2) Hemoglobin electrophoresis3) Sickle cell test Other tests may include:1) Bilirubin2) Blood oxygen
Treatment for a sickle cell crisis includes:1) Blood transfusions (may also be given regularly to prevent stroke)2) Pain medicines3) Plenty of fluids
Other treatments for sickle cell anemia may include:1) Hydroxyurea (Hydrea), a medicine that may help reduce the number of pain episodes (including chest pain and difficulty breathing) in some people2) Antibiotics to prevent bacterial infections, which are common in children with sickle cell disease
Lee MT, Piomelli S, Granger S, et al. Stroke prevention trial in sickle cell anemia (STOP): extended follow-up and final results. Blood. 2006;108:847-852. Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. 2008;148:932-938. Geller AK, OConnor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320-323.
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