Sickle Cell Disease By Brent Durschmidt. Quick Definition ► It is a disorder of red blood cell production that gives red blood cells a sickle shape during.

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Presentation transcript:

Sickle Cell Disease By Brent Durschmidt

Quick Definition ► It is a disorder of red blood cell production that gives red blood cells a sickle shape during hypoxia

Lets go back in time…  James Herrick  Hahn and Gillespie 1927  Genetic Mutation  Inherited, not contagious  Malaria

So…who has it?  Mostly descendants of:  Tropical parts of Africa – E of the Niger River around North Gana, Benin, Central African Republic  Asia – Burma, Thailand, Laos,Cambodia,Malaysia,Indonesia,India  America – LA, Largely populated African American areas

The Stats

…and how does it affect them? ► Blood Cells  They die ► Blood Flow  Not so good ► Body Parts/Organs  Yeah, they’re affected ► Immune System  As open as Detroit’s offensive line

S/S – just to name a few ► Vasoocclusive Crises  Necrosis ► ulcers  Pain ► Musculoskeletal, chest, abd  Priapism  Lung Tissue Damage ► Acute Chest Syndrome  Heart ischemia/ RVF  Renal Crises ► Infarction, pain  Stroke  Swelling ► Hematological Crises  Fall in hemoglobin level  Organ damage ► Spleen from sequestration of RBC’s ► kidneys ► Liver  Bone marrow function – shunting? ► Anemia Priapism

but wait, there’s more ► Infectious Crises  Immunosuppression from loss of splenic function  Parvovirus B19 ► Life expectancy: 42 – 48 ► Pregnancy – spontaneous abortion, pre-eclampsia, growth problems ► Clots

Dang, how do I treat that? ► Mostly supportive ► VOMIT (ew) ► Fluid – isotonic crystalloid if possible ► Lots o’ analgesics ► Possibly acetaminophen in ped’s c temp ► Pts will have a hx of blood transfusions, vaccinations, iron tx, Hydroxyuria/ Droxia ► Bone marrow transplant, PCN, vitamins

What about a future? ► Stem Cell research ► Other medicines  Nicosan, Senicopac ► Gene therapy

Statistics and the facts ► 70,000 – 80,000 Americans suffer from the disease ► 1 in 500 African Americans have the disease  1 in 12 carry the trait ► 1 in 1000 – 1400 Hispanic Americans ► Sickle cell disease can stand up against Malaria and…

Prodigy has it too… word

That’s just wrong… ► Wikipedia says some dude named Vernon Mason coined the term “Sickle Cell Anemia.” ► Actually, it was James B. Herrick.

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.