1. Sickle Cell Disease By Brent Durschmidt

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

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

4. 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

5. The Stats

6. …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

7. 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

8. 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

9. 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

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

12. 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…

13. Prodigy has it too… word

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

16. Sources ► SCDAA. “What is Sickle Cell Disease?” 2005. 15 April 2005. http://www.sicklecelldisease.org/about_scd/. ► Wikipedia. “Sickle Cell Disease.” 2001. 4 June 2004. http://en.wikipedia.org/wiki/Sickle-cell_disease ► KidsHealth. “Sickle Cell Disease.” 1995. June 2007. http://kidshealth.org/parent/medical/heart/sickle_cell_anemia.html. ► DHS/NIH. “What is Sickle Cell Anemia.” 1998. August 2008. http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html. ► Genetics Home Reference. “Sickle Cell Disease.” 1993. February 2007. http://ghr.nlm.nih.gov/condition=sicklecelldisease. ► Prof. Ishita Mukerji. “Sickle Cell Disease.” 2004. 5 March 2004. http://www.sicklecellinfo.net/index.htm. ► Bryan Bledsoe. Essentials of Paramedic Care 2 nd Ed. Pg 1398-1399. Copyright 2007. ► Marshall Lichtman. Williams Hematology 7 th Ed. Pg 678-690. Copyright 2006.