Hereditary Angioedema (HAE) is an inherited deficiency of functional C-1-esterase inhibitor (C-1-INH) and is characterized by unpredictable recurrent episodes of painful often disabling swelling of the abdomen or face or extremities.
In the recent past several new drugs have become available to treat this condition. One of the most exciting ones is Ruconest,
which is a C-1-esterase inhibitor made by recombinant technology. Thus, it works by replacing what the patient fails
to make (or fails to make in a functional protein). By way of explanation C-1-esterase inhibitor is a “gate-keeper” or “watch-dog” for inappropriate activation of our complement system proteins. If the complement system is activated when it is not needed, it makes the individual sick by causing the tissue swelling which is the hallmark of HAE.
In women with HAE, variations in hormones such as the menstrual cycle or pregnancy can bring on attacks. A case in point was recently published in the medical literature of a 23-year-old woman who experienced multiple attacks of angioedema during her pregnancy. Because Ruconest is technically not a drug but a pure replacement protein it is felt to be safe to use during pregnancy. At 38 weeks the woman had a severe attack as she was going into labor. Because of concern for the fetus an ultrasound was done and the baby was also having an attack of facial angioedema. The woman received Ruconest and as her swelling subsided so too did her infant sons swelling. By the time he was delivered he was back to normal. This is the first documented account of fetal angioedema successfully treated while treating the mother.