ATTACKS WITH SWELLING AND PAIN
People with HAE experience attacks of swelling that affect various body parts. Swelling attacks occur most commonly in the skin (for example, hands, feet, face), abdomen (gastrointestinal tract), and throat/airway (larynx). Without treatment, attacks can last from two to five days. The frequency and severity of attacks may vary dramatically among people with HAE. It may vary in the same individual during different stages of their life.
HAE-related swelling is NOT the same as swelling caused by allergies and cannot be treated as an allergic reaction. Antihistamines, corticosteroids (prednisone), and epinephrine are ineffective in relieving angioedema symptoms of HAE.
About 25% of people with HAE experience a non- itchy, blotchy, red rash (also can appear as red circles), that often occurs before or during an HAE attack.
Swelling attacks are disfiguring and can be extremely painful and disabling to the point of preventing participation in normal daily activities.
Gastrointestinal attacks usually involve excruciating abdominal pain, nausea, vomiting, and diarrhea caused by swelling in the intestinal wall. These symptoms are distinguishing features of HAE with C1-inhibitor deficiency as recurrent severe abdominal pain is rarely seen in allergic (histaminergic) angioedema.
Episodes of throat/airway swelling are the most dangerous HAE symptoms because the airway can close and cause death by choking.
In fact, studies indicate that the death rate for untreated people with HAE with airway angioedema can be around 30%.
Please note that 50% of people with HAE experience one throat/ airway swelling in their lifetime.
It is important to recognize throat swelling is an emergency that always requires immediate medical attention at the first sign of symptoms, even if effective HAE medication is given at home.