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  • 1. Sickle Cell Crises Steven Elsbecker DO Peds ICU Presentation November 15th 2013
  • 2. What We Will Discuss • Prevalence and epidemiology of Sickle Cell Disease • Various Sickle Cell Disease complications • The Sickle Cell Crises • The diagnosis, management and disposition of Sickle Cell Crises
  • 3. What We Won’t Discuss • Chronic outpatient management • Biochemical and molecular details • Genetic inheritance details
  • 4. Epidemiology • Hgb SS affects 1:5000 Sub-Saharan African Americans • 1:36000 Hispanic-Americans • 44 States currently perform neonatal screening for sickle cell trait and disease • 1:12 African Americans are carries of the sickle cell trait
  • 5. Sickle Cell Complications
  • 6. Skin • Stasis Ulcers
  • 7. Neuro • CVA 2/2 vaso-occlusion
  • 8. Eye • Retinopathy 2/2 vaso-occlusion
  • 9. Pulmonary • Embolism, Infarct, Infection
  • 10. Vascular • Vaso-occlusion causing downstream ischemia anywhere in the body
  • 11. Liver • Hepatitis 2/2 blood transfusion cholestasis, hepatic sequestration
  • 12. Gall Bladder • Cholelithiasis/Cholecystitis
  • 13. Spleen • Splenic Sequestration
  • 14. Genitourinary • Hematuria, decreased fertility, impotence, priapism
  • 15. Bone • Infarct / Aseptic Necrosis osteomyelitis, Aplastic Crisis
  • 16. Reproductive • Decreased fertility, placental insufficiency
  • 17. Immunologic • Relative immunodeficiency
  • 18. Erythrocytes • Hemolysis
  • 19. The Sickle Cell Crises
  • 20. The Crises • These 4 general categories cause the most morbidity and mortality • Splenic Sequestration Crisis • Hemolytic Crisis • Aplastic Crisis • Vaso-occlusive Crisis
  • 21. Splenic Sequestration Crisis • Splenic engorgement • Rapid drop in Hgb • Circulatory collapse • 3-24 hours • Transfusion/Exchange
  • 22. Hemolytic Crisis • Acute, accelerated breakdown • Oxidative stress • Infection • Hypoxia… • More common with concomitant G6PD deficiency
  • 23. Aplastic Crisis • Supply:Demand mismatch • Most commonly follows Parvo B19 • Reticulocyte count • Critical drops in Hgb
  • 24. Vaso-Occlusive Crisis • The cause of nearly all SCD complications • Abnormal cells stack in small vessels • Shunt = Ischemia = Pain • Can cause any symptom 2/2 end organ ischemia
  • 25. Acute Chest Syndrome • Concomitant infarction and infection • High morbidity and mortality • Treat pain, hypoxia and infection • Macrolides or Quinilones
  • 26. Questions? • Platt OS, Brambilla DJ, Rosse WF, et al. (June 1994). "Mortality in sickle cell disease. Life expectancy and risk factors for early death". N. Engl. J. Med. 330 (23): 1639– 44.doi:10.1056/NEJM199406093302303. ISSN 0028-4793. PMID 7993409. • http://ericksontribune.com/2007/10/mds-launch-network-to-treat-sickle-cell/ • Wellems TE, Hayton K, Fairhurst RM (September 2009). "The impact of malaria parasitism: from corpuscles to communities". J. Clin. Invest. 119 (9): 2496– 505.doi:10.1172/JCI38307. PMC 2735907. PMID 19729847. • Malowany JI, Butany J (February 2012). "Pathology of sickle cell disease". Seminars in Diagnostic Pathology 29 (1): 49–55. doi:10.1053/j.semdp.2011.07.005. PMID 22372205. • "BestBets: How long should an average sickle cell crisis last?". Retrieved 2010-11-27. • Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009-05-28). Robbins and Cotran Pathologic Basis of Disease, Professional Edition: Expert Consult - Online (Robbins Pathology) (Kindle Locations 33498-33499). Elsevier Health. Kindle Edition. • Glassberg J (August 2011). "Evidence-based management of sickle cell disease in the emergency department". Emergency Medicine Practice 13 (8): 1–20; quiz 20.PMID 22164362. • Anie KA, Green J (2012). "Psychological therapies for sickle cell disease and pain". In Anie, Kofi A. Cochrane Database of Systematic Reviews (Online) 2: CD001916.doi:10.1002/14651858.CD001916.pub2. PMID 22336781.