sickle cell disease

Sickle Cell Disease is an inherited blood disorder, characterized primarily by chronic anemia and periodic episodes of pain.  The underlying problem involves hemoglobin, a component of the red cells in the blood.  The hemoglobin molecules in each red blood cell carry oxygen from the lungs to the body organs and tissues and bring carbon dioxide back to the lungs.

In Sickle Cell Disease the hemoglobin is defective.  After the hemoglobin molecules give up their oxygen, some of them may cluster together and form long rod-like structures.  These structures cause the red blood cells to become stiff, which are normally soft and donut shaped, creating difficulty for the sickled red blood cells to squeeze through small blood vessels.  This causes them to stack up and cause blockages that deprive the organs and tissue of oxygen carrying blood.  This process produces periodic episodes of pain and can ultimately damage the tissues and vital organs and lead to other serious medical problems.

Sickle Cell Disease is a global disease affecting approximately 80,000 people in the United States with those of African descent being affected in the greatest numbers. In addition people of Mediterranean, Hispanic, Asian, Sicilian, Greek, Italian, Indian and Turkish ancestry are also affected, but in smaller percentages.

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