What is Hereditary Angioedema (HAE)?
Hereditary angioedema (HAE) is an inherited disorder. Symptoms include:
- Recurrent swelling in the hands, feet, abdomen, genitalia, or face that is not usually painful, but can be severe.
- Painful swelling of the bowel wall (the lining of the intestines) associated with nausea and vomiting.
- Swelling in the throat that can cause respiratory distress.
Causes of 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. A peptide called bradykinin is thought to be responsible for the HAE symptoms of swelling, inflammation and pain.
Patients with HAE do not experience hives.
Hereditary Angioedema Triggers
Pressure, trauma or emotional stress can trigger an attack; however, the trigger of most attacks is unknown.
- Typical attacks last about one to three days.
- If your child develops an acute attack, contact your doctor or go to the emergency room.
Treatment for Hereditary Angioedema Attacks
New medications have been developed to help treat acute HAE attacks.
- Cinryze and Berinert work by blocking bradykinin and are used to treat acute facial and abdominal attacks in patients 12 years and older.
- Kalbitor, which contains a plasma kallikrein inhibitor, is used to treat attacks of hereditary angioedema in patients who are 12 years and older.
- Ruconest (recombinant C1 esterase inhibitor) for patients 13 and older.
- Firazyr (icatibant) - Bradykinin B2-receptor antagonist for patients 18 and older.
- Fresh frozen plasma may also help in treating acute attacks.
Preventing an Attack
- Cinryze, a new medication that contains C1 INH, can be used to prevent an attack in patients 6 years or older.
- Takhzyro I(lanadelumab) Kallikren inhibitor can be used in patients 12 years and older.
- Orladeyo (Berotralstat) Kallikren inhibitor can be used in patients 12 years and older.
- Tranexamic acid (TA) Antifibrolytic agent.
- Epsilon aminocaproic acid (EACA) Antifibrolytic agent.
- Fresh frozen plasma has been used successfully to prevent an attack prior to surgery or a dental procedure.
- Danazol, a type of steroid, is useful for the prevention of an acute attack. ,It is only used in children after puberty and those 16 years and older.
Clinical Studies
Clinical trials investigating new medications for the treatment of HAE are ongoing through the University of Cincinnati.
