Sickle Cell Disease. Quick Definition ► A genetic disorder of red blood cell (hemoglobin) production that gives red blood cells a sickle shape.

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Presentation transcript:

Sickle Cell Disease

Quick Definition ► A genetic disorder of red blood cell (hemoglobin) production that gives red blood cells a sickle shape

History of Sickle Cell  The origin of the gene mutation that led to sickle-cell occurred in the Arabian peninsula. It spread to Asia and Africa.  Malaria was the selective pressure.  This mutation occurred up to 6,000 generations ago, approximately 150,000 years back.

Who has it?  Mostly descendants of:  Tropical parts of Africa  Southeast Asia

The Stats

How it Affects the Body ► Red Blood Cells  Die Early  16 days rather than 120 ► Blood Flow  Sickle shape can clog ► Body Parts/Organs  Oxygen Debt (Ischemia) ► Immune System  Functions Poorly

Symptoms ► Vasoocclusive Crises ( Blood Vessel Blockage)  Necrosis (dead tissue) ► Ulcers (open wounds)  Pain ► Muscles, Skeleton, Chest, Abdomen  Lung Tissue Damage  Heart Ischemia (lack of blood)  Renal (kidney) Failure  Stroke  Swelling ► Hematological Problems (Blood)  Fall in hemoglobin level  Organ damage ► Spleen - from absorption of sickle shaped RBC’s ► Kidneys ► Liver  Bone marrow function suffers ► Anemia

Symptoms (cont.)

► Infectious Crises  Immunosuppression (reduced immune response) from loss of splene function ► Life expectancy: 42 – 48 ► Pregnancy – miscarriages ► Clots Symptoms (cont.)

Statistics and the facts ► 70,000 – 100,000 Americans suffer from the disease ► 1 in 500 African Americans have the disease  1 in 12 are Carriers ► 1 in 1200 Hispanic Americans ► Sickle cell disease still prevents Malaria

Has It TLC Singer T-Boz Tionne T-Boz Watkins As well as – Rapper Prodigy, Pro Football Player Tiki Barber, Actor Larenz Tate, Trumpeter Miles Davis and many, many others!

What about a future? ► Stem Cell research ► Gene therapy

Sources ► SCDAA. “What is Sickle Cell Disease?” April ► Wikipedia. “Sickle Cell Disease.” June ► KidsHealth. “Sickle Cell Disease.” June ► DHS/NIH. “What is Sickle Cell Anemia.” August ► Genetics Home Reference. “Sickle Cell Disease.” February ► Prof. Ishita Mukerji. “Sickle Cell Disease.” March ► Bryan Bledsoe. Essentials of Paramedic Care 2 nd Ed. Pg Copyright ► Marshall Lichtman. Williams Hematology 7 th Ed. Pg Copyright 2006.