Emergency Treatment of HAE

Emergency treatment of hereditary angioedema (HAE) during an acute attack can be extremely challenging because, unlike an allergic reaction, swelling related to HAE does not respond to epinephrine, antihistamines, or glucocorticoids.2

C1-INH replacement, kallikrein inhibitor therapy and BK2 receptor antagonist are all available in the US for the treatment of acute HAE attacks.12 Prior to these treatments, fresh-frozen plasma (FFP) was administered for acute HAE attacks.2, 15

Laryngeal attacks

The most urgent acute HAE attacks are those involving laryngeal swelling, which are potentially lethal. Attacks of this type must be treated immediately in the hospital—not in a local clinic—in case emergency intubation or tracheotomy is necessary.2, 13

Involvement of the upper airway usually begins slowly, but cases of progression within 20 minutes have been reported. Voice alteration and dysphagia indicate high risk of total airway obstruction. If there is suspicion of airway involvement, begin treatment immediately.

Abdominal attacks

Severe pain can often accompany abdominal attacks. In fact, an acute abdominal HAE attack can mimic appendicitis, a bowel rupture, or a bowel obstruction. Many patients have undergone unnecessary surgeries before HAE was correctly diagnosed.1

In abdominal HAE attacks during which severe pain is noticeable, pain management using analgesics is often effective. C1-INH, BK2 receptor antagonist and kallikrein inhibitor can also be used to treat these types of attacks. However, it is very important that HAE be correctly diagnosed in such cases.1

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Emergency treatment of HAE

Swelling related to HAE does not respond to commonly used emergency medications, such as epinephrine, antihistamines, or glucocorticoids. See how to treat an acute attack

US Hereditary Angioedema Association (HAEA)

Learn about the HAEA, a non-profit organization that provides a wide range of services, including clinical trial placement, physician referrals, education, and individualized patient case management. Visit HAEA.org