1. SICKLE CELL DISEASE -Monica

2. 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)

3. 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.

4. Sickled cells

5. Lifespan: 10-20 days -Compared to our 90-120 days -Destroyed by the spleen. -Sometimes the body can’t make new RBCs fast enough. -Resulting in anemia.

6. 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.

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

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

11. New Experimental Treatment Gene Replacement

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

13. 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. 2005. SCDAA Homepage. http://sicklecelldisease.org http://sicklecelldisease.org -Sickle cell Society