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6 Important Facts to Know About Hereditary Angioedema

Hereditary Angioedema (HAE) is a very rare and potentially life-threatening genetic condition. HAE involves recurring attacks of severe swelling (angioedema) in several parts of the body, including the hands, feet, genitals, stomach, face, or throat. Because of the severe swelling, it can restrict breathing and become fatal. These episodes may be triggered by physical trauma or emotional stress; however, swelling can also occur without a known trigger. When untreated, an HAE attack often lasts for three days, sometimes longer, and people with HAE can experience three or more episodes per month. The frequency and severity of attacks vary significantly among individuals. (Source:

To bring awareness to Hereditary Angioedema, we are honored to support HAE Day, happening on May 16th. HAE Day was established to unite the community to raise awareness of HAE. The organization that started HAE Day and continues to raise awareness each year is HAE International. They are a global non-profit network of patient associations dedicated to raising awareness of HAE worldwide while improving people’s lives with HAE.

If you’re interested in learning more about Hereditary Angioedema (HAE), here are six important facts and statistics that may be helpful. 

  1. Hereditary angioedema is a rare disease that results in recurrent attacks of severe swelling that affects males and females in equal numbers. Symptoms typically begin in early childhood. Hereditary Angioedema – NORD (National Organization for Rare Disorders) (

  2. HAE occurs in about 1 in 10,000 to 1 in 50,000 people. Type I is the most common, accounting for approximately 85 percent of cases. Type II occurs in approximately 15 percent of cases. There is no data regarding the incidence of HAE with Normal C1-Inhibitor; however, expert scientists believe that the population represents a small subset of people who suffer from HAE. US Hereditary Angioedema Association (

  3. Symptoms of HAE usually appear early in life, most often by age 13, and may increase in severity after puberty. Because HAE is so rare, it can take as long as ten years to obtain an accurate diagnosis after symptoms are first experienced. US Hereditary Angioedema Association (

  4. When untreated, an HAE attack often lasts for three days, sometimes even longer, and many people with HAE experience three or more swelling attacks every 30 days. The frequency and severity of attacks vary significantly among individuals, even among affected family members. US Hereditary Angioedema Association (

  5. Approximately 20-25% of cases of HAE occur spontaneously, without a previous family history. Being an inherited disorder, there are multiple members in the same family with a diagnosis and multiple generations living with HAE. HAE Infographic-CHAEN (

  6. Currently, eight therapies are available for people with HAE, with more in the pipeline (website These include:
    • BERINERT® C1 Esterase Inhibitor, Human
    • CINRYZE® C1 esterase inhibitor (human)
    • FIAZYR® (incatibant injection)
    • HAEGARDA® C1 Esterase Inhibitor Subcutaneous (Human)
    • KALBITOR® (ecallantide)
    • ORLADEYO™(berotralstat)
    • RUCONEST® C1 esterase inhibitor (recombinant)
    • TAKHZYRO® (lanadelumab-flyo) injection

Orsini Specialty Pharmacy currently prescribes seven of these therapies.

We are honored to play a small part in raising awareness for HAE patients, caregivers, and the providers that support them. Click here, or the button below, to see how we support HAE patients.