What should the bleeding plan be for a patient with von Willebrand's (Von Willebrand disease) type bleeding disorder undergoing a colonoscopy due to gastrointestinal (G.I.) bleeding?

Bleeding Management Plan for von Willebrand Disease Patient Undergoing Colonoscopy for GI Bleeding

For patients with von Willebrand disease undergoing colonoscopy for GI bleeding, desmopressin (DDAVP) should be administered 30 minutes prior to the procedure for those with type 1 disease and factor VIII levels >5%, while von Willebrand factor/factor VIII concentrates should be used for patients with type 2, type 3, or those unresponsive to desmopressin. 1, 2

Initial Assessment and Preparation

1. Determine VWD type and severity:

   • Confirm patient's VWD type (1,2, or 3)
   • Check baseline factor VIII levels and VWF activity
   • Review previous response to desmopressin if available 1, 3

2. Hemodynamic assessment:

   • If patient is hemodynamically unstable (shock index >1), prioritize CT angiography before colonoscopy 4, 5
   • Stabilize with fluid resuscitation and blood products as needed
   • Target hemoglobin >7 g/dL (>9 g/dL in patients with cardiovascular disease) 5

Pre-Procedure Management

For Type 1 VWD with Factor VIII >5%:

• Administer desmopressin (DDAVP) 0.3 μg/kg IV 30 minutes before procedure 1, 2
• Monitor factor VIII coagulant activity, ristocetin cofactor activity, and von Willebrand factor antigen during administration 1
• Consider antifibrinolytic therapy (tranexamic acid 500mg-1000mg) as adjunctive treatment 5

For Type 2, Type 3, or Desmopressin Non-responders:

• Administer von Willebrand factor/factor VIII concentrate 2, 3
• Dosing based on severity and type of procedure
• Target VWF:RCo and FVIII levels >50% for minor procedures and >100% for major procedures 3

Colonoscopy Timing and Approach

• For stable patients: Schedule colonoscopy on next available list rather than rushing to perform within 24 hours 4
• For unstable patients: Perform CT angiography first to localize bleeding before planning endoscopic intervention 4, 5
• Ensure adequate bowel preparation with polyethylene glycol solution (preferred over enemas) 4
• Monitor for hypotension and vomiting during bowel preparation 4

During Procedure

• Maintain continuous hemodynamic monitoring
• Have additional doses of hemostatic agents available if needed
• Be prepared for endoscopic interventions based on findings
• Consider tattoo marking of any identified bleeding sources for potential future surgical intervention 4

Post-Procedure Management

• Continue hemostatic coverage for 24-48 hours post-procedure depending on findings and interventions performed
• Monitor for signs of recurrent bleeding
• Maintain hemoglobin targets (>7 g/dL or >9 g/dL for cardiovascular disease patients) 5
• Consider iron supplementation for anemia 5

Special Considerations

• Angiodysplasia: GI bleeding in VWD patients is often related to angiodysplasia and may be refractory to standard treatments 6
• Diagnostic challenges: Multiple radiologic and endoscopic investigations may be needed to identify bleeding sources in VWD patients 6
• Temporary discontinuation of anticoagulants: If patient is on anticoagulants, these should be temporarily suspended during the acute bleeding episode 5

Follow-up

• Schedule gastroenterology follow-up within 2 weeks if discharged 5
• Monitor hemoglobin levels and iron status
• Consider long-term prophylaxis for patients with recurrent GI bleeding 3

This management plan addresses both the hemostatic requirements of the VWD patient and the procedural needs for colonoscopy in the setting of GI bleeding, prioritizing patient safety while enabling effective diagnosis and treatment.