What to prescribe for a patient with type 1 von Willebrand disease who is about to undergo wisdom teeth extraction?

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Professor of Medicine/Hematology, University of Washington

This month, Barbara A. Konkle, MD, answers a question about what to prescribe for a patient with type 1 von Willebrand disease who is about to undergo wisdom teeth extraction.

CLINICAL DILEMMA

I have a patient with type 1 von Willebrand disease (vWD) who is about to undergo extraction of three impacted wisdom teeth. Her activity level is undetectable, and her factor 8 is 21%. Is 40 µ/kg 1 hour prior to the procedure, and 20 µ/kg every 8 hours for three days after the procedure, with aminocaproic acid as needed, acceptable prophylaxis?

EXPERTS MAKE THE CALL

In the scenario you describe, removal of three impacted wisdom teeth is considered major surgery, and thus the recommended dose would be higher than you describe, although dosed less frequently given the von Willebrand factor (vWF) half-life. Ideally, there would be a better measurement of vWF activity and vWF antigen, but for the recommendation, given the FVIII level, I will presume the patient has severe type 1 vWD.

There is no extensive outcome data or standard treatment regimens for severe vWD, and products differ some in their dosing recommendations. For the described patient, I would recommend either Humate-P 80 µ/kg or Wilate 60 µ/kg just prior to the extraction, one-half that dose every 12 hours for five doses (so for 3 days) (data from Wilate trials suggest you might be able to go to once daily after 2 days), then an additional dose once daily for two more days.

Antifibrinolytic therapy is important in oral surgery, and I would begin it as soon as the patient can take medication by mouth, using aminocaproic acid 4-5 gm every 6 hours (I tell my patients that if there is no oozing after a few days, they can decrease to every 8 hours, but I give them a large enough prescription to cover the more frequent dosing.) or tranexamic acid 1,300 mg every 8 hours. The latter tends to be better tolerated. I would continue the antifibrinolytic therapy for one week, but give the patient a prescription for 10 days in case she stops and needs to restart due to oozing.

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Disclaimer: ASH does not recommend or endorse any specific tests, physicians, products, procedures, or opinions, and disclaims any representation, warranty, or guaranty as to the same. Reliance on any information provided in this article is solely at your own risk.

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