Hereditary Angioedema

What is Hereditary Angioedema?

Hereditary Angioedema (HAE) is a genetic medical condition where a person experiences severe inflammation and swelling. This condition came to medical terms with the help of Canadian physician William Osler’s introduction to HAE in 1888. Moreover, the parts where a person faces angioedema are mostly places like the arms, face, legs, airways, and intestinal tract. Furthermore, swelling in the airways can lead to airway obstruction and trouble maintaining breathing. Also, angioedema causes swelling in the intestinal tract that can lead to situations of vomiting and abdominal pain. 

Additionally, the level of attacks ranges from two weeks to some days. Also, there are three types of Hereditary Angioedema (type I, II, and III). These types can be further understood by their underlying causes along with the levels of C1 protein inhibitor present inside the blood. These types of HAE also have different variety of signs and symptoms. Moreover, this condition affects one in fifty thousand people and the diagnosis of this condition usually appears during childhood itself. Interestingly, this is also interesting to know that type III affects females more, and type I and II affects both sexes equally. 

Hereditary Angioedema can also prove fatal because if a person experiences too much swelling in the airway then it can lead to death as well. But the such a condition is not common, only 25% experience airway swelling. Also, with proper treatment, you can prevent such fatal consequences and live normally and effectively.


What Happens If You have Hereditary Angioedema (HAE)?

Hereditary Angioedema is a genetic condition that is diagnosable in an early stage of childhood itself. So, Hereditary Angioedema frequently extremely swells parts of the body periodically that includes:

  • Genitals
  • Face
  • Lips
  • Larynx
  • Gastrointestinal tract
  • Arms
  • Legs

Other symptoms include:

  • Acute abdominal pain
  • Vomiting
  • Puffiness in the afflicted area without any discomfort or itching
  • Intense pain
  • Itching on intense just beneath the skin
  • Extreme burning sensation
  • Diarrhea
  • Dehydration
  • Hands swell up
  • Fatigue
  • Tingling skin
  • Non-itchy and painless rash
  • Irritation on skin
  • Mood swings
  • Anxiety


What are The Causes of Hereditary Angioedema?

A protein known as the C1 inhibitor is known to have low levels or to act improperly. So, this results in hereditary angioedema (HAE). Also, it has an impact on blood vessels. Moreover, rapid swelling of the face, intestines, larynx, or trachea may occur as a result of a hereditary angioedema episode.


What are The Treatment Methods for Hereditary Angioedema?

Treatment of hereditary angioedema (HAE) includes Replacement therapy or medicines that also help to modulate immunity pertaining to hereditary angioedema. Moreover, replacement therapy is also known as hormone replacement therapy which aids in bringing the deficient amounts of substance present in the body to normal levels properly. 

On the other hand, medicines that are given by doctors to patients with  hereditary angioedema (HAE) are as follows:

  • Berotralstat
  • C1 Esterase Inhibitor (recombinant)
  • C1 Inhibitor (human)
  • Ecallantide
  • Icatibant
  • Kallikrein inhibitor- human
  • Lanadelumab

Medicines to Treat hereditary angioedema



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