Sickle Cell Crises
Steven Elsbecker DO
Peds ICU Presentation
November 15th 2013
What We Will Discuss
• Prevalence and epidemiology of Sickle Cell Disease
• Various Sickle Cell Disease complications
• Th...
What We Won’t Discuss
• Chronic outpatient management
• Biochemical and molecular details
• Genetic inheritance details
Epidemiology
• Hgb SS affects 1:5000 Sub-Saharan African Americans
• 1:36000 Hispanic-Americans
• 44 States currently perf...
Sickle Cell
Complications
Skin
• Stasis Ulcers
Neuro
• CVA 2/2 vaso-occlusion
Eye
• Retinopathy 2/2 vaso-occlusion
Pulmonary
• Embolism, Infarct, Infection
Vascular
• Vaso-occlusion causing downstream
ischemia anywhere in the body
Liver
• Hepatitis 2/2 blood transfusion
cholestasis, hepatic sequestration
Gall Bladder
• Cholelithiasis/Cholecystitis
Spleen
• Splenic Sequestration
Genitourinary
• Hematuria, decreased fertility,
impotence, priapism
Bone
• Infarct / Aseptic Necrosis
osteomyelitis, Aplastic Crisis
Reproductive
• Decreased fertility,
placental insufficiency
Immunologic
• Relative immunodeficiency
Erythrocytes
• Hemolysis
The Sickle Cell
Crises
The Crises
• These 4 general categories cause the most morbidity and mortality
• Splenic Sequestration Crisis
• Hemolytic ...
Splenic Sequestration Crisis
• Splenic engorgement
• Rapid drop in Hgb
• Circulatory collapse
• 3-24 hours
• Transfusion/E...
Hemolytic Crisis
• Acute, accelerated breakdown
• Oxidative stress
• Infection
• Hypoxia…
• More common with
concomitant G...
Aplastic Crisis
• Supply:Demand mismatch
• Most commonly follows Parvo B19
• Reticulocyte count
• Critical drops in Hgb
Vaso-Occlusive Crisis
• The cause of nearly all SCD complications
• Abnormal cells stack in small vessels
• Shunt = Ischem...
Acute Chest Syndrome
• Concomitant infarction and infection
• High morbidity and mortality
• Treat pain, hypoxia and infec...
Questions?
• Platt OS, Brambilla DJ, Rosse WF, et al. (June 1994). "Mortality in sickle cell disease. Life expectancy and ...
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Hgb ss crisis elsbecker

  1. 1. Sickle Cell Crises Steven Elsbecker DO Peds ICU Presentation November 15th 2013
  2. 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. 3. What We Won’t Discuss • Chronic outpatient management • Biochemical and molecular details • Genetic inheritance details
  4. 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. 5. Sickle Cell Complications
  6. 6. Skin • Stasis Ulcers
  7. 7. Neuro • CVA 2/2 vaso-occlusion
  8. 8. Eye • Retinopathy 2/2 vaso-occlusion
  9. 9. Pulmonary • Embolism, Infarct, Infection
  10. 10. Vascular • Vaso-occlusion causing downstream ischemia anywhere in the body
  11. 11. Liver • Hepatitis 2/2 blood transfusion cholestasis, hepatic sequestration
  12. 12. Gall Bladder • Cholelithiasis/Cholecystitis
  13. 13. Spleen • Splenic Sequestration
  14. 14. Genitourinary • Hematuria, decreased fertility, impotence, priapism
  15. 15. Bone • Infarct / Aseptic Necrosis osteomyelitis, Aplastic Crisis
  16. 16. Reproductive • Decreased fertility, placental insufficiency
  17. 17. Immunologic • Relative immunodeficiency
  18. 18. Erythrocytes • Hemolysis
  19. 19. The Sickle Cell Crises
  20. 20. The Crises • These 4 general categories cause the most morbidity and mortality • Splenic Sequestration Crisis • Hemolytic Crisis • Aplastic Crisis • Vaso-occlusive Crisis
  21. 21. Splenic Sequestration Crisis • Splenic engorgement • Rapid drop in Hgb • Circulatory collapse • 3-24 hours • Transfusion/Exchange
  22. 22. Hemolytic Crisis • Acute, accelerated breakdown • Oxidative stress • Infection • Hypoxia… • More common with concomitant G6PD deficiency
  23. 23. Aplastic Crisis • Supply:Demand mismatch • Most commonly follows Parvo B19 • Reticulocyte count • Critical drops in Hgb
  24. 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. 25. Acute Chest Syndrome • Concomitant infarction and infection • High morbidity and mortality • Treat pain, hypoxia and infection • Macrolides or Quinilones
  26. 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.
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