What is Hereditary Angioedema (HAE)?

Hereditary angioedema (HAE) is a rare genetic condition that causes swelling attacks that can affect areas of the body, including the hands and feet, stomach, face, and throat.

Although HAE is rare, affecting only 1 in 10,000 to 1 in 50,000 people in the world, it can be potentially life-threatening, especially when the swelling occurs in the throat area, which can interfere with breathing and requires immediate emergency care. HAE attacks can be unpredictable, however the goal of preventive therapy is to reduce how often HAE attacks occur and how severe they are.

When do symptoms start?

It varies, but in one study, nearly half of the people reported their symptoms began by age 10 and over three-fourths reported symptoms before the age of 21. Reports also show increased frequency of attacks during adolescence.

What causes HAE?

Most people with HAE either don't have enough of a protein called C1 esterase inhibitor (C1-INH), or the protein doesn't work properly. This deficiency or dysfunction can lead to episodes of swelling. These two causes can be classified as Type 1 or Type 2.

Type 1 HAE

Type 1 is the most common and caused by having too little C1-INH circulating in the blood.

Type 2 HAE

Type 2 means a person's body makes enough C1-INH, but the protein doesn't work as it should.

CINRYZE (C1 esterase inhibitor [human]) was the first preventive therapy indicated to help prevent HAE attacks in children (6 to 12 years old).

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