SICKLE CELL DISEASE -Monica

Sickle Cell is inherited, and it affects the shape of red blood cells. Genetic: Caused by a Hemoglobin S (sickle) molecule. Hemoglobin F (fetal): What we start with. Hemoglobin A (adult): What we end up with. (Some people never replace all of the HGB F)

HGB S is only 1 odd protein out of 300, but it causes severe defects. Affected RBCs undergo polymerization and lose most of the water in the cell. -They become crescent shaped, sticky, and inflexible. -Hard to pass through capillaries.

Sickled cells

Lifespan: days -Compared to our days -Destroyed by the spleen. -Sometimes the body can’t make new RBCs fast enough. -Resulting in anemia.

Inheritance: -Most common in African Americans. -2 recessive traits = the disease. -One parents with the disease and one parent with the recessive trait have a 1 in 2 chance of having an affected child.

Signs and Symptoms -Anemia, infections, and pain. -Acute Chest Syndrome -Hand-Foot Syndrome -Splenic Crises -Generalized body pain -Possible stroke, or MI

Treatment -Daily Penicillin -Bone marrow transplants -meds to prevent water from leaving the cells (Clotrimazole)

New Experimental Treatment Gene Replacement

Prehospital Treatment Mainly treatment of symptoms. -Pain management -Fluid replacement

Sources -Nelson D.L. (2005). Lehninger Principles of Biochemistry:4 th ed. New York,NY: WH Freeman and Co. -Sickle Cell Disease Association of America, Inc SCDAA Homepage Sickle cell Society