Hereditary Angioedema (HAE)
What is Hereditary Angioedema (HAE)?
Hereditary angioedema (HAE) is an inherited disease. Symptoms include:
- Recurrent swelling in the arms, legs, abdomen, genitalia, face, or throat which is not usually painful
- Painful swelling of the bowel wall (the lining of the intestines) associated with nausea and vomiting
What causes HAE?
The most common cause of HAE is a decrease in C1 esterase inhibitor (C1 INH), a protein that is part of our body’s normal immune system.
How is HAE different from other causes of angioedema?
Patients with HAE do not experience hives. Allergic reactions to foods or stinging insects, which may cause hives, may also cause angioedema.
What triggers an attack?
Pressure, trauma, or emotional stress can trigger an attack; however, the trigger of most attacks is unknown.
How long does the swelling from an attack last?
Typical attacks last for about 1-3 days.
What should I do if my child develops an acute attack?
If your child develops an acute attack, please contact your physician or go to the emergency room.
How do you treat an acute attack?
- Cinryze, a new medication that contains CI INH, may be used to treat acute attacks
- Fresh frozen plasma may also be used to treat acute attacks
- New medications are currently being developed to help treat acute attacks for HAE
How do you prevent an acute attack?
- Danazol, a type of steroid, is very useful for the prevention of an acute attack
- Cinryze, a new medication that contains CI INH, was recently approved by the FDA to prevent an attack
- Fresh frozen plasma has also been used successfully to prevent an attack prior to surgery or a dental procedure
Are there any clinical studies for this condition?
Clinical trails investigating new medications for the treatment of HAE are ongoing through the University of Cincinnati.
Rev. 6/09
