Von Willebrand's Disease (vWD)

 

 

Considerations 

 

  • High risk for perioperative bleeding

  • Sequelae of bleeding in enclosed spaces (joints, intracranial, pericardium, thorax)

  • Potential contraindication to neuraxial anesthesia & analgesia

  • Consultation with hematology for factor optimization (replacement/supplementation)

 

 

Goals

 

  • Optimize factor activity & coagulation profile in perioperative period

  • Techniques for minimizing perioperative blood loss

 

 

Optimization 

 

  • Consultation with hematologist for appropriate factor management

  • Schedule OR early in the week & early in the day (ensure all lab/blood bank/consultant resources available)

  • Assay factor levels 48h prior to OR & restore levels to 40% of normal prior to surgery, as dictated by the surgical procedure (see tables below). Key trough factor VIII levels:

    • Obstetric > 50%

    • Minor surgery > 30%

    • Major surgery > 50% 

  • Repeat factor assay after initial administration to confirm factor activity (within 2 hours of expected OR start) 

  • Continue to monitor factor activity level intra-operatively as dictated by clinical situation

  • Avoid all anti-platelet medications

  • Consider antifibrinolytics during perioperative period (up to 3-5 days)

 

Management

 

  • DDAVP 0.3 mcg/kg (provides 3-5 fold increase in activity); only if known responder:

    • Maximum effect 30 min post dose but levels remain elevated for 6-8 hours

    • DDAVP works by stimulating the release of vWF from endothelial cells

  • Factor VIII-vWF concentrates (Humate P)

  • Platelet concentrates (contains vWF)

  • Recombinant factor VIII

  • Recombinant factor VIIa

  • Emergency: cryoprecipitate (contains vWF, FVIII, FXIII, fibronectin, fibrinogen)

 

 

Pregnancy 

 

  • Establish baseline factor VIII, vWF, ristocetin cofactor early in pregnancy & at 34 weeks

  • During pregnancy, prophylactic treatment if factor VIII level < 25% (typically levels increase with pregnancy)

  • Know the patient's type & factor VIII level:

    • Responder: at labour onset, DDAVP 0.3mcg/kg IV q12h

    • Non-responder: Humate P or cryoprecipitate

  • Labour & delivery/cesarean section: maintain levels > 50% of normal

  • Post-partum: follow levels & treat if < 25% or significant hemorrhage

  • Regional: epidural safe if factor VIII & vWF > 50%

 

 

Treatment Choices Based on Sub-types 

 

 

 

 

 

 

 

 

 

 

Recommended Dosages of Factor VIII for Patients with Severely Reduced (<10%) Factor Levels 

 

 

 

 

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