Q:

5/30/2013
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.

A:

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.

Sincerely,
Phil Lieberman, M.D.

AAAAI - American Academy of Allergy Asthma & Immunology