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.6

In 2010, Berinert® a C1-INH replacement therapy became available in Canada for the treatment of acute HAE attacks.15 Prior to this treatment, solvent detergent treated plasma (SDP) or less safe frozen plasma was administered for acute HAE attacks.5

Laryngeal attacks

The most important urgent acute HAE attacks are those involving laryngeal swelling, which is 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 necessary17. Intubation is to be considered early in progressive laryngeal edema.5

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.17,18 For more information, click (here).

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. In addition, the white blood cell count may increase during an attack, further complicating the clinical scenario. Many patients have undergone unnecessary surgeries before HAE was correctly diagnosed. As with any group of people, those with HAE can indeed develop appendicitis or other acute bowel symptoms, so caution is advised.6

C1-INH can be used to treat these types of attacks. Pain management using non-steroidal anti-inflammatory drugs (NSAIDs) is often effective.

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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.

Canadian HAE Network

Canadian Hereditary Angioedema Network (CHAEN)/ Rèseau Canadien d'Angioédème Héréditaire (RCAH) is a coalition of Canadian physicians interested in advancing the care of Hereditary Angioedema. Visit haecanada.com.