TIP: The World Allergy Organization guidelines state that every patient with HAE should be considered for home therapy and self-administration training.

Long-term prophylaxis

Ongoing treatment used to prevent symptoms in patients not adequately managed with acute therapy13

The treatment options for long-term HAE prophylaxis are C1-INH concentrate or androgens. Both have been shown to reduce HAE attack frequency.13 Choice of treatment depends on contraindications, adverse events, risk factors for adverse effects, tolerance, response, and dose required to control attacks.13

Short-term prophylaxis

Used to prevent edema when a predictable stressor (eg, dental work) is planned13,14

Short-term prophylaxis is generally limited to patients in unusual circumstances, particularly those about to undergo a surgical or dental procedure. Currently, however, there are no therapies approved for short-term prophylaxis.13

2012 WAO guidelines list C1-INH concentrate and androgens as short-term prophylactic options. If used, C1-INH should be administered 1 to 6 hours before the procedure. Short-term prophylaxis with an androgen should begin 5 days pre-procedure and continue 2 to 5 days post-procedure.13

Acute treatment

Treats attacks as they occur to reduce morbidity and prevent mortality13

The 2012 WAO guidelines recommend C1-INH, ecallantide, or icatibant for on-demand treatment of HAE attacks.13

C1-INH concentrate works by replacing the deficient C1-INH protein, thereby helping to regulate activation of all cascade systems involved in bradykinin production and release during attacks.13

Ecallantide inhibits kallikrein activity and the progression of edema in HAE attacks.13

lcatibant blocks bradykinin activation, thereby preventing angioedema attacks.13

If none of these drugs are available, solvent detergent-treated plasma or frozen plasma (if a safe supply is available) should be used.13

On-demand therapies are typically approved for use in adults and adolescents; one is also approved for use in pediatric patients.

Emergency treatment

Steps to take for laryngeal or abdominal attacks

Emergency treatment during an acute attack can be extremely challenging because, unlike allergic reactions, swelling related to HAE does not respond to epinephrine, antihistamines, or glucocorticoids.2 C1-INH, administered as early as possible, has been proven effective for the emergent treatment of HAE attacks.15

Laryngeal attacks

Acute HAE attacks involving laryngeal swelling are potentially fatal. Attacks of this type must be treated immediately in the hospital—not in a local clinic—in case emergency intubation or tracheotomy is necessary.16

Involvement of the upper airway usually begins slowly. Voice alteration and dysphagia indicate high risk of total airway obstruction. If there is suspicion of airway involvement, begin treatment immediately.9

Abdominal attacks

Acute abdominal HAE attacks can include severe pain that may mimic appendicitis, bowel rupture, or bowel obstruction. It is very important that HAE be correctly diagnosed in such cases.10

Pain management using NSAIDs is often effective.

Quality-of-life issues with HAE

For patients with HAE, suffering goes beyond the physical. Most feel a loss of control due to frequent, unpredictable attacks and the fear that they may face a life-threatening HAE attack. Many patients suffer from depression, fear, and anxiety, especially if they lack understanding of their condition.17

Healthcare professionals should clearly understand and communicate the issues around HAE and the urgency of treatment.

Additional considerations

Monitoring of "trigger" medications13

Because various medications, such as estrogen-containing oral contraceptives, hormone replacement therapy, and ACE inhibitors, can contribute to the onset of attacks, medication history and selection should be carefully reviewed when treating patients with HAE attacks.

Dental or surgical procedures13

Short-term prophylaxis (using C1-INH, for example) should be considered for patients scheduled to undergo a dental or surgical procedure.

Help maintain quality of life across the care continuum

  • Recognize the key role of the specialist nurse in education and support
  • Foster effective communication among team members
  • Ensure dissemination of information to patients and team
    • Discuss the need for trigger identification and avoidance
  • Network and share information among all specialties treating angioedema
  • Encourage patients to connect with the US Hereditary Angioedema Association (HAEA)

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