Ep. 178: What Is Hereditary Angioedema (HAE)?
Part of the series: Allergy, Asthma & Immunology Innovations Podcast
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Hereditary angioedema (HAE) is a rare genetic condition that causes attacks of swelling in the skin, the belly, and sometimes the throat. Because it looks like an allergic reaction, it is often missed for years.
Kortney and Dr. Payel Gupta are joined by Dr. Marc Riedl, Professor of Medicine and Clinical Director of the US HAEA Angioedema Center at UC San Diego, to set the stage for our three-part series on HAE. We cover what HAE is, what is happening in the body, and symptoms that tell you it is HAE and not an allergy.
What we cover in our episode about hereditary angioedema
Why HAE is not an allergy. HAE swelling comes from a different system in the body. That's why antihistamines, steroids, and epinephrine don't stop it.
HAE is genetic. HAE comes from a change in one gene, and it can be passed from parent to child. Each child of a parent with HAE has a 50-50 chance of having it. About 1 in 4 people are the first in their family to have it, so family testing matters.
What happens in the body. People with HAE don't have enough working C1 inhibitor, the "brakes" on a chemical called bradykinin. Too much bradykinin makes tiny blood vessels leak fluid, and that fluid is the swelling.
HAE swelling and where it happens. It usually doesn't itch or come with hives, and it can last two to five days. It can show up in the hands, face, feet, belly, or throat. Belly attacks can be very painful, and throat swelling needs emergency care.
Triggers and warning signs. Injury, dental work, estrogen, and stress can set off attacks. Some people notice a red rash that doesn't itch, or tingling, before the swelling starts.
About our guest
Dr. Marc Riedl is Professor of Medicine and Clinical Director of the US HAEA Angioedema Center at the University of California, San Diego, where he is also Clinical Service Chief for Allergy and Immunology. He is the inaugural holder of the Hereditary Angioedema (HAE) Community Endowed Chair in Angioedema Clinical Excellence at UC San Diego. Dr. Riedl is board certified in allergy and immunology and clinical pharmacology, and he has published more than 150 research papers. He spends much of his time caring for and studying people with angioedema.
More resources about HAE
More episodes about swelling and allergic reactions
Timestamps
Our episode explain why and what is HAE
02:17 What is HAE
04:20 Why HAE is not an allergy
06:28 What happens in the body during an HAE attack
11:46 Bradykinin's role in the body
14:12 When swelling happens
15:55 How HAE is passed down in families
19:25 When symptoms usually start
21:57 What HAE swelling looks like
24:28 Where swelling can happen
26:23 Belly attacks
29:46 Throat attacks
31:11 Common HAE triggers
35:25 Early warning signs before an attack
37:48 Does HAE ever go away
39:09 What to do if this sounds like you