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Hereditary Angioedema (HAE)

Hereditary Angioedema (HAE)

Hereditary angioedema (HAE) is an inherited disorder. Symptoms include:

  • Recurrent swelling in the arms, legs, belly, genitalia, throat or face. Swelling in the throat can cause severe problems with breathing.
  • Nausea and vomiting due to painful swelling of the bowel wall (the lining of the intestines)

Causes of HAE

The most common cause of HAE is a defect in the gene that controls a blood protein, called CI inhibitor. This protein is part of our body’s normal immune system.

Hereditary Angioedema Triggers

The first symptoms of HAE usually show up in childhood and may get worse during the teen years. Certain triggers may bring on an attack, including trauma, emotional stress, surgery or minor illnesses, like the cold or flu; however, the trigger of most attacks is unknown.

  • Typical attacks last about 1-3 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 attacks for HAE.

  • Cinryze and Berinert are used to treat acute facial and belly attacks in patients 12 years and older.
  • Kalbitor is used to treat attacks in patients who are 12 years and older.
  • Fresh frozen plasma may also help in treating acute attacks.

Preventing an Attack

  • Danazol, a type of steroid, is useful for the prevention of an acute attack.
  • Cinryze is used to prevent an attack in patients 18 years or older.
  • Fresh frozen plasma has been used successfully to prevent an attack prior to surgery or a dental procedure

Last Updated 05/2018

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