What is Sickle Cell Disease?

Normal red blood cells are soft and round (A in image below) which allows them to pass through the blood vessels with ease. Someone who is affected by the sickle cell disorder has blood cells that are misshaped and sticky (B in image below).The cells are no longer round  and pliable (soft) and will clump to block the veins & arteries, making it difficult for blood to pass through.

As a result, less blood reaches the body which causes damage to tissues and organs. The resulting effects are seen in the complications of sickle cell disease.  Any part of the body can be damaged by poor blood flow and lack of blood that contains oxygen.  Hemoglobin (hee-muh-glow-bin) is the protein contained in red blood cells. Hemoglobin carries oxygen from the lungs to the rest of the body. Persons with Sickle Cell Disease have abnormal Hemoglobin that is unable to provide oxygenated blood to the tissues.

Blood with less than a normal amount of red blood cells is called anemia (a-nee-me- ah). Sickle Cell Anemia is one type .Sickle cells live less than 120 days in the bloodstream. Sickle Cells die in about 10-20 days. The bone marrow (spongy part inside of a bone ) is always making new red blood cells . The bone marrow can not make cells fast enough to keep up with the rapid death of the Sickle Cells.

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Image credit: National Heart Lung and Blood Institute