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A Happy Ending

A Happy Ending. CP Case Conference Stephanie Chen 2/24/2012. 6/30/11 – UMHS ED. HPI 16 yo F 6 days non-specific symptoms No fevers OSH ED last weekend PMH Depression, ulcers PE Afebrile Trace cervical LAD, no organomegaly A&P Resolving viral illness D/C home. 7/3/11 – UMHS ED.

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A Happy Ending

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  1. A Happy Ending CP Case Conference Stephanie Chen 2/24/2012

  2. 6/30/11 – UMHS ED • HPI • 16 yo F • 6 days non-specific symptoms • No fevers • OSH ED last weekend • PMH • Depression, ulcers • PE • Afebrile • Trace cervical LAD, no organomegaly • A&P • Resolving viral illness • D/C home

  3. 7/3/11 – UMHS ED • HPI • “Legs are giving out” • Paresthesias • Neck stiffness & HA • PE • Afebrile • Negative Kernig & Brudzinski • No organomegaly • MSK/Neuro • Proximal > Distal B weakness UE and LE • Intact sensation • DTR absent B LE, 1+ B UE

  4. 7/3/11 – UMHS ED Course • Labs • LP: 1 WBC, 1 RBC, protein 317 • AST 71, ALT 128 • EBV titers pending • CBC and CPK normal • Peds Neuro • Admit to PICU

  5. Guillain-Barre Syndrome • Disease course • Progressive, ascending, symmetrical weakness • May  neuromuscular respiratory failure • 25% require artificial ventilation • Autonomic instability • Recovery often takes weeks to months • 3.5-12% die during acute stage • Bimodal age peaks • 20’s and 70’s • Incidence: 1-2 per 100,000/year

  6. Causes • Autoimmune pathogenesis suggested • Infection • 2/3 with infection in 6 weeks prior to symptoms • C. jejuni in 1/3 of patients • Influenza Vaccination • Associated with 1/1,000,000 risk of GBS

  7. Treatment • Supportive Care • Ambulatory pts with AIDP, spontaneous recovery anticipated • Corticosteroids • No benefit when used alone • IVIg • Affect time course, not extent, of recovery • Plasmapheresis • Affect time course, not extent, of recovery • First therapeutic modality to impact disease favorably • Relapses in 10% • 2-3 weeks after IVIg or TPE • After relapse, additional therapy with TPE can be helpful

  8. IVIg for GBS • IVIg within 2 weeks of onset hastens recovery as much as plasmapheresis • No difference in frequency of adverse events • Significantly more likely to be completed

  9. UMHS PICU • Intubated 7/5/11 • Completed 5th IVIg treatment 7/8/11 • Extubated 7/14/11 • Improving strength • Gen Peds

  10. UMHS PICU • Reintubated 7/20/11 • Persistent LE weakness • EMG consistent with GBS • Repeat IVIg x 2 doses • Worsening respiratory function • Little to no strength improvement • Apheresis request 8/1/11

  11. 78 yo woman • Failed 5 day IVIg course • 7 days after IVIg completion  TPE • Gradual improvement after 1st procedure • Weaned off mechanical ventilation on 8th day • May be benefit from TPE after IVIg in refractory cases

  12. Therapeutic Plasmapheresis (TPE) for GBS • Most effective when initiated within 7 days of disease onset • TPE vs. supportive care • Accelerates motor recovery • Decreases time on ventilator • International Randomized Trial • TPE vs. IVIg vs. TPE followed by IVIg • All three equivalent in mean disability improvement at 4 weeks and time to walk without assistance

  13. TPE Technical Notes • 5-6 procedures QOD • 5% albumin replacement • More susceptible to volume shifts, BP and HR changes

  14. 1st Procedure • 30 minutes post • Hypotension to 64/39 • 75/50 earlier in day • Responded to 1500 cc fluid bolus

  15. Clinical Course • Plasmapheresis course • Hypotension controlled • Strength clearly improved by 4th treatment • Laptop ventilator by 8/7/11 • Post-plasmapheresis course • Pressure dressing applied to stoma 8/30/11 • D/C home 9/1/11

  16. February 2012 • Complete recovery of motor function and DTR • Middle of cheerleading season

  17. References • Szczepiorkowski ZM, Winters JL, et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach from the Apheresis Applications Committee of the American Society for Apheresis. Journal of Clincial Apheresis 2010; 25:83-177. • Hughes RAC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database of Systematic Reviews 2010, Issue 6. Art. No.: CD002063. • DynaMed[Internet]. Ipswich (MA): EBSCO Publishing. 1995 – 2011. Guillain-Barre Syndrome; [updated 2011 Sep 01; cited 2012 Feb 19]. Available from: http://web.ebscohost.com.proxy.lib.umich.edu/dynamed/detail?vid=3&hid=110&sid=c30084cb-8818-4622-a0d6-038b0b8c207b%40sessionmgr15&bdata=JnNpdGU9ZHluYW1lZC1saXZlJnNjb3BlPXNpdGU%3d#db=dme&AN=116758. • Buzzigoli SB, Genovesi M, et al. Plasmapheresis treatment in Guillain-Barre Syndrome: potential benefit over intravenous immunoglobulin. Anaesth Intensive Care 2010; 38:387-89. • Winters JL, Brown D, Hazard E, Chainani A and Andrzejewski C. Cost-minimization analysis of the direct costs of TPE and IVIg in the treatment of Guillain-Barre syndrome. BMC Health Services Research 2011; 11:101.

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