HAE Treatment Guidelines

Guidelines from the US Hereditary Angioedema Association (HAEA) Medical Advisory Board were published in 2020 with updated recommendations for the treatment and management of hereditary angioedema (HAE).1

The full publication of the US HAEA guidelines includes recommendations regarding HAE classification, diagnosis, on-demand treatment, prophylactic treatment, special considerations for women and children, development of a comprehensive management and monitoring plan, and assessment of burden of illness.1

Pharmacologic treatment of HAE

The US HAEA guidelines list 4 guiding principles for the overall approach to pharmacologic HAE treatment1:

  1. Availability of effective on-demand acute therapy for all patients
  2. Early treatment to prevent attack progression
  3. Treatment of attacks irrespective of the site of swelling
  4. Incorporation of long-term prophylaxis based on highly individualized decision-making reflecting a physician-patient partnership

On-demand treatment of acute attacks

The 2020 US HAEA guidelines reccomend that all patients with laboratory confirmed HAE should have access to at least 2 standard doses of a FDA approved on-demand medication for treatment of acute HAE attacks.

The guidelines also recommend that on-demand treatment of HAE attacks be self-administered (or administered by a caregiver) whenever feasible.1

The 2020 US HAEA guidelines recommends FIRAZYR as one of the first-line on-demand treatment options.1

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Prophylactic treatment

Short-term prophylaxis is indicated when patients are at increased risk of having an HAE attack associated with triggers such as invasive dental/medical procedures or stressful life events.1

The decision to use long-term prophylactic treatment should reflect the individual needs of the patient. Because HAE severity may change over time, the need to begin or maintain long-term prophylaxis should be periodically revisited and discussed with patients and their caregivers.1

FIRAZYR is not a prophylactic treatment; its use is for on-demand treatment of acute HAE attacks in adults.2 Learn more about one option for prophylactic treatment in patients with HAE.