SICKLE CELL DISEASE

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SICKLE CELL DISEASE

• -Monica

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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)

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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.

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Sickled cells
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Lifespan 10-20 days

• -Compared to our 90-120 days
• -Destroyed by the spleen.
• -Sometimes the body cant make new RBCs fast
  enough.
• -Resulting in anemia.

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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.

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Signs and Symptoms

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

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Treatment

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

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New Experimental Treatment

• Gene Replacement

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Prehospital Treatment

• Mainly treatment of symptoms.
• -Pain management
• -Fluid replacement

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Sources

• -Nelson D.L. (2005). Lehninger Principles of
  Biochemistry4th ed. New York,NY WH Freeman and
  Co.
• -Sickle Cell Disease Association of America, Inc.
  2005. SCDAA Homepage. http//sicklecelldisease.org
  
• -Sickle cell Society lthttp//sicklecellsociety.org
  gt