To read part 1 of Teresa’s post, click here.
The sooner you treat [during an HAE attack], the better HAE meds work!
The second stage of attacks is the swelling that is generally painless, without the severe,
urgent symptoms that will come later, and can last an hour or a day, but usually not that long.
Early on in the attack the swelling can be evident, as in facial swelling, or less evident in some other locations where the symptoms can be mistaken as symptoms of some other condition. For example, swelling in the abdomen can be mistaken for GERD with the taste of acid in the back of the mouth, heartburn, bad breath.
Early throat swelling symptoms can be mistaken for the cough from post-nasal drip, dry throat, or onset of a cold or flu.
The early swelling symptoms are the most difficult and the most dangerous HAE attack symptoms to correctly identify, especially if it is the patient’s first swelling in that particular location.
Patients can mistake these early symptoms as unimportant, and emergency room medical staff will very often look askance at any patient presenting with these seemingly minor symptoms, thus delaying treatment. This happens even when the patient informs the staff that they have a rare disease that they have already treated with their rescue med, but it is progressing nonetheless to the point where medical intervention is necessary.
The third stage is the maximum symptom stage, lasting from hours to a day, or longer. Bradykinin (a peptide of the kinin group of proteins in the blood), is now flooding the body
causing capillary permeability, swelling, inflammation, and intense pain if the swelling is located in internal organs such as the gastrointestinal tract where swelling in the bowel wall lining can cause complete obstruction.
Throat swelling will proceed to marked lowering of the voice, constriction of the throat causes
breathing to become a mere whistling as air tries to move through the ever narrowing airway.
Medical emergency staff will often misdiagnose abdominal swelling attacks as “acute abdomen” and perform unnecessary surgical procedures. This was quite common until recently when more doctors are reading about this rare disease, but it still happens to this day.
Throat swelling attacks are routinely misdiagnosed as allergic conditions and treating emergency physicians are baffled when the patient continues to worsen despite antihistamines.
Medical staff will even delay intubation until it is too late. Too many HAE patients report telling medical staff that their relative that they’ve just rushed to the ER has hereditary angioedema and that treating it as an allergic condition will not work, only to watch their loved one die in the ER.
“… approximately 50% of patients… have previously had their conditions misdiagnosed, most commonly as allergic angioedema or appendicitis. Misdiagnosis results in marked delays in receiving the correct diagnosis, during which time patients cannot access effective, lifesaving treatment.”- Misdiagnosis trends in patients with hereditary angioedema from the real-world clinical setting. Andrea Zanichelli, et.al., August 10, 2016.
The last stage is the swelling and inflammation reduction stage, and lasts from several hours to 1-3+ days. The more swelling there is, the longer it takes to go away. Inflammation can last even longer in patients with frequent abdominal attacks, resulting in HAE-induced chronic irritable bowel syndrome. It is my experience with swelling attacks located in the abdomen and other organs, and in the throat, that it is best for me to use caution for several days following the attacks. This is not the case with extremity swelling, or facial swelling.
Hereditary angioedema not only causes life-threatening swelling attacks, but also PTSD from near-death attacks and from the blithe disregard and disrespect from medical professionals who know nothing about HAE and refuse to even google the term while their HAE patient awaits.
Hereditary angioedema can cause HAE-induced anemia, and other illnesses. Most patients with severe, frequent HAE attacks report being diagnosed with anxiety and depressive disorders. Nonallergic hereditary angioedema is a potentially lethal disease that demands attention and respect from patients and the doctors who treat them.
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.
