Sickle Cell CrisesSteven Elsbecker DO

Peds ICU Presentation

November 15th 2013

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

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 perform neonatal screening for sickle cell trait and disease

• 1:12 African Americans are carries of the sickle cell trait

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 CellCrises

The Crises

• These 4 general categories cause the most morbidity and mortality

• Splenic Sequestration Crisis

• Hemolytic Crisis

• Aplastic Crisis

• Vaso-occlusive Crisis

Splenic Sequestration Crisis

• Splenic engorgement

• Rapid drop in Hgb

• Circulatory collapse

• 3-24 hours

• Transfusion/Exchange

Hemolytic Crisis

• Acute, accelerated breakdown

• Oxidative stress• Infection

• Hypoxia…

• More common with

concomitant G6PD deficiency

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 = Ischemia = Pain

• Can cause any symptom 2/2

end organ ischemia

Acute Chest Syndrome

• Concomitant infarction and infection

• High morbidity and mortality

• Treat pain, hypoxia and infection• Macrolides or Quinilones

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.