Prem Menon, M.D.     Vimla Menon, M.D.
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Asthma, Allergy and Immunology Center

Asthma, Allergy and Immunology Center
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Baton Rouge, LA 70808
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Prem Menon, M.D.
Telephone: (225) 766-6931
1-800-Dr-Menon (1-800-376-3666)
Fax: (225) 766-9413

Vimla Menon, M.D.
Telephone: (225) 766-6931
Fax: (225) 766-9413

Home | Allergy Information | Hereditary Angioedema )HAE)

Hereditary Angioedema (HAE)

Hereditary Angioedema (HAE) is a rare and potentially life-threatening genetic disease that causes episodes of severe swelling (edema) in hands, feet, arms, legs, face and the gastrointestinal tract. An estimated 6-10,000 people in the United States have HAE, and many patients remain undiagnosed for years due to the rarity of the disease.

The swelling episodes of HAE are often confused with allergic reaction, but HAE cannot effectively be treated using standard allergy medications including corticosteroids, antihistamines, and epinephrine.

HAE episodes may occur without any apparent reason and may vary in its presentation in different individuals.

However, possible triggers may include:

  • Stress
  • Trauma
  • Anxiety
  • Illnesses such as the cold and flu
  • Surgery (particularly dental surgery)

Women may also experience easing or worsening of HAE symptoms during menstruation and pregnancy due to hormonal changes.

Swelling episodes may last 2-5 days if left untreated. Swelling in the gastrointestinal tract can causes severe abdominal pain, nausea, and vomiting. Swelling in the throat (laryngeal edema) can close the airway and cause death by asphyxiation.

Patients should seek medical treatment immediately if the throat starts to swell or breathing is compromised.

Diagnosis

HAE is diagnosed with a blood test that measures C1 Esterase inhibitor [C1INH] and C4 protein levels in the blood. Once diagnosed with HAE, it is important to see an HAE medical specialist in order to have an individualized treatment plan.

HAE can be passed down genetically and affects multiple members of the same family. There are three subtypes of familial HAE:

  1. Type I familial HAE is caused by a known genetic mutation that causes a quantitative deficiency of a C1INH protein.
  2. Type II familial HAE is caused by a known genetic mutation that affects the functionality of C1INH protein
  3. Type III HAE is either caused by an unknown genetic mutation or Factor XII mutation.

Not everyone with AE (Angioedema) is born with gene mutations. Some patients acquire AE due to an underlying malignancy or disease that causes the production of anti- C1INH autoantibodies that results in depletion of C1INH. Angioedema can be caused by ACE- Inhibitor blood pressure medications. Angioedema may also be due to allergic or non-allergic causes.

Treatment

There are currently five medications approved by the FDA to treat HAE patients:

  • Berinert® and Ruconest®: delivered intravenously and treats HAE symptoms once they have begun
  • Kalbitor® and Firazyr®: delivered by subcutaneous injection and treats HAE symptoms once they have begun
  • CinryzeTM: delivered intravenously and helps prevent HAE symptoms.

Older therapies such as attenuated androgens and fresh frozen plasma are no longer front-line treatments. There are other HAE treatments currently undergoing clinical trials that may be available in the near future.

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