Chapter 7: Information Sources
Section 3: Specific Disabilities – Information Sources
Subsection: Sickle Cell Disease
National resource for information. Sickle cell anemia 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 back carbon dioxide to the lungs.
The abnormal hemoglobin molecules tend to cluster together and from long, rod like structures. These structures cause some of the red blood cells to become stiff and to assume a sickle shape.
In sickle cell anemia, 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 and to assume a sickle shape. Unlike normal red cells, which are usually smooth and donut-shaped, the sickled red cells cannot squeeze through small blood vessels. Instead, they stack up and cause blockages that deprive the organs and tissue of oxygen-carrying blood. This process produces the periodic episodes of pain and ultimately can damage the tissues and vital organs and lead to other serious medical problems.
National resource for information.