I have a patient with HAE type 3 diagnosed several years ago. The patient is due to have surgery with endotracheal intubation shortly. Attacks currently occur 2-5 times per month but are generally mild. Prophylactic treatment with Danazol was ineffective in changing the frequency or intensity of attacks. Kalbitor is effective, but only used about once a year for severe attacks. What if any treatment should be initiated prior to surgery? What treatments should be on hand in the PACU and bedside on the floor? Thank you.


Thank you for your inquiry.

The fact that your patient responded to Kalbitor indicates that the pathogenesis is kinin-mediated. I would therefore suggest treating your patient as if they had hereditary angioedema by instituting C1 inhibitor prophylaxis before surgery, the same as one would do for a patient with hereditary angioedema. The principle behind this therapy is illustrated in the reference copied for you below. The drug employed in this reference was Cinryze given at a dose of 1,000 units prior to surgery. This is usually given between one and six hours before the procedure.

In addition, I would recommend that you keep an additional two doses on hand should there be a flare after surgery. Alternatively, since your patient has responded to ecallantide, you could use ecallantide to treat a flare that occurred after surgery.

Thank you again for your inquiry and we hope this response is helpful to you.

W.R. Lumry, P. Busse, J. Baker, M. Davis-Lorton, D. Hurewitz, J.A. Grant, M. White, I. Kalfus et al. Pre-procedure Administration of C1 Esterase Inhibitor (Human) (Cinryze™) for the Prevention of Hereditary Angioedema (HAE) Attacks after Medical, Dental, or Surgical Procedures. Abstract, Journal of Allergy and Clinical Immunology 2011 (February); Vol. 127, Issue 2, Supplement, Page AB234.

Phil Lieberman, M.D.

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