First described in 1882 by Heinrich Quincke, angioedema was regarded for more than one century as a rare disease.
Subsequently, it was established that the hereditary form of the disease (Hereditary Angioedema, HAE) is caused by a deficiency of C1-Inhibitor due to alterations in the encoding SERPING1 gene and Bradykinin was recognized as the principal mediator of HAE symptoms. Over the past decade, however, impressive research advances uncovered an astonishing complexity of the disease.
As a result, nowadays, kinin-mediated angioedema is considered as adverse family of
disorders, whose the only common characteristics is an unpredictable clinical expression by
spontaneous, recurrent, self-limiting but potentially life-threatening attacks of localized edema of subcutaneous and submucosal tissues. –“Hereditary Kinin-Mediated Angioedema: Current Challenges”, Arije Ghannam, June 30, 2016.
I am neither a doctor or researcher, I am a patient who inherited nonallergic kinin mediated hereditary angioedema from my father who suffered with this disease his entire life. The article I have written presents what I have learned from my own doctors, from reading research about HAE as soon as it is published, from other patients who have this rare disease, and from my own experience with HAE.
I think of Hereditary Angioedema attacks in terms of stages or phases. There are four stages to an Hereditary Angioedema attack:
- The prodromal stage
- The initial symptom stage
- The maximum symptom stage
- The swelling and inflammation reduction phase
These phases or stages of an attack tend to be similar for any attack location.
The first stage, the prodrome – symptom(s) experienced just prior to the onset of an attack, last from an hour to several hours, and can include unexplainable sudden-onset anxiety and
sometimes “feelings of impending doom,” depressive feelings that can be severe, even with
thoughts of suicide with a plan, mental confusion – like difficulty remembering how to unlock
your door. This has happened to me a few times in the past when my attacks were quite severe.
I had to purposely think: get correct key out, put it in the lock, turn it, put hand on latch and
open. When that happened the first time, I was worried that I might be developing a brain
illness, but my doctor told me I could experience those kinds of neurological symptoms at the beginning of an attack, and that when it happens I must use my rescue med ASAP.
Otherprodromal symptoms include extreme fatigue, hunger, muscle aches, tingling before lip or tongue swelling, frustration with everything and everyone, negativity, anger, and the skin rash called “erythema marginatum”. It’s a lacy-looking rash that doesn’t hurt and isn’t itchy. It just appears anywhere on the body.
People with HAE are cautioned to ALWAYS treat their prodromes with HAE-specific rescue
meds as soon as they become evident.
About the Author: Teresa has a Masters Degree in Psychiatry worked as Federal Research Compliance Officer, before having to retire due to her rare disease. She has lived on the East coast as well as in the mid-west, and has become an advocate for HAE patients across the nation and in other countries. A grandchild also has a separate ultra-rare genetic disorder, and she supports research, advocacy and social awareness for the rare community.
