I checked with a high volume HAE center and received the following response. In general, women with HAE do not swell as a consequence of labor and delivery. Therefore, we do not typically give a prophylactic treatment before delivery unless she has a known history of swelling with delivery. With that said, it is always good practice to have an additional dose of C1INH available in the delivery room to give if the patient begins to swell. If the patient is to have a C-section, then I would be inclined to give an extra dose of C1INH shortly before the procedure unless she receives her scheduled dose within 12 hours of the procedure.
You state that her HAE improves between pregnancies. If she does not take C1INH prophylaxis between pregnancies, I would not taper the prophylaxis off too quickly as there is a significant risk of swelling in the postpartum period as the hormonal levels re-equilibrate. She should be closely observed for swelling for 3 days after birth.
Caballero et al, International Journal of Women’s Health, Sept 2014
Dr. Jacqueline A. Pongracic, FAAAAI
Dr. Bruce Zuraw