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Sickle Cell Crisis
Sickle Cell Crisis Overview
Sickle cell disease is the most common of the hereditary blood disorders. It occurs almost exclusively among black Americans and black Africans.
Sickle cell disease in black Americans occurs in 3 of every 1,000 (or about 1 in 375) live births. Estimates indicate that the severe form of sickle cell disease affects more than 50,000 black Americans.
Understanding Anemia -- the Basics
Anemia is a condition that develops when your blood lacks enough healthy red blood cells or hemoglobin. Hemoglobin is a main part of red blood cells and binds oxygen. If you have too few or abnormal red blood cells, or your hemoglobin is abnormal or low, the cells in your body will not get enough oxygen. Symptoms of anemia -- like fatigue -- occur because organs aren't getting what they need to function properly. Anemia is the most common blood condition...
Read the Understanding Anemia -- the Basics article > >
The first account of what was then called sickle cell anemia in the medical literature was in 1910. James B. Herrick, a Chicago physician, described the symptoms of a 20-year-old black male student from the West Indies. The man had reported "shortness of breath, palpitations, and episodes of icterus [yellow eyes]. He had an anemia." Dr. Herrick described the patient's blood smear as showing "thin, sickle-shaped and crescent-shaped red cells."
Red blood cells deliver oxygen to working or active tissues. In the lungs, hemoglobin (the molecule in the red blood cell) takes on oxygen and, at the same time, releases carbon dioxide. This process is called oxygenation. At the tissue level, this activity is reversed. The same hemoglobin molecule releases oxygen and takes on carbon dioxide. This process is called deoxygenation.
In sickle cell disease, certain red blood cells become crescent-shaped (the sickle cell Dr. Herrick described). These abnormal red blood cells, carrying an abnormal hemoglobin known as hemoglobin S, are fragile. A person who has sickle cell disease can become more likely to get infections because the damaged cells eventually clog the spleen. A severe attack, known as sickle cell crisis, can cause pain because blood vessels can become blocked or the defective red blood cells can damage organs in the body.
Sickle Cell Crisis Causes
Sickle cell disease results from mutation, or change, of certain types of hemoglobin chains in red blood cells (the beta hemoglobin chains).
The changes in the building of normal hemoglobin result in the abnormal hemoglobin of sickle cell disease. These mutated molecules do not have the smooth motion needed for oxygenation and deoxygenation. When the oxygen concentration in the blood is reduced, the red blood cell assumes the characteristic sickle shape. This causes the red blood cell to be stiff and rigid, and stops the smooth passage of the red blood cells through the narrow blood vessels.
It does not take much imagination to see sharp-end "sickled" red cells stacking up in narrow blood vessels known as capillaries. When this happens, red blood cells are not able to carry oxygen to tissues, and tissue cell injury or death occurs. Someone with sickle cell disease would be experiencing pain with this process—the sickle cell crisis.
Sickle Cell Crisis Symptoms
The sites most often affected by the blocking or stacking action of sickled cells are found in the lungs, liver, bone, muscles, brain, spleen, penis, eyes, and kidneys.
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