Treating Acute Attacks

As a person with hereditary angioedema (HAE) , you should become your own advocate. Recognize the symptoms of HAE attacks. Learn as much as you can about treatment. Monitor your symptoms. And work with your doctor to choose the best treatment plan for you. The following information about disease treatment is important for you to know.

Acute attacks

Prior to 2010, there was no replacement therapy readily accessible in Canada for acute attacks of HAE. Berinert® is the first and only C1 esterase inhibitor available in Canada since October 2010.

  • Berinert® (C1 Esterase Inhibitor, Human) is indicated for the treatment of acute abdominal or facial attacks of hereditary angioedema (HAE) of moderate and severe intensity.

C1-INH product with indication for acute attacks

C1-INH (complement 1 esterase inhibitor) therapy works by replacing the missing or malfunctioning C1-INH protein in patients with a C1-INH deficiency. C1-INH for acute attacks is given when an attack occurs, optimally at the first sign of the attack (prodrome). The most frequent side effects from the clinical trial included nausea, diarrhea, abdominal pain, and muscle spasms.

Other treatment options

Before C1-INH was available, solvent detergent plasma and fresh-frozen plasma ( FFP) were administered for acute HAE attacks. Additional treatment options include epinephrine, which only provides temporary relief, and androgens and antifibrinolytics, which don’t take effect until several days have passed and have undesirable side effects. Neither corticosteroids nor antihistamines have been shown to have any real effect on HAE attacks.

Pain medications may be helpful during an acute attack. Ask your doctor about their use.

Acute laryngeal edema

Depending on the symptoms and the sites of the angioedema, intensive support may be necessary, including intravenous fluids. This is often the case during a laryngeal edema (swelling) attack, which must be controlled to protect the airways. C1-INH may be administered for this type of attack. But if the laryngeal edema has progressed rapidly, a tracheotomy (TRAKE-ee-OTT-o-mee) may have to be performed to allow oxygen to flow into the lungs. A tracheotomy is an incision through the neck into the windpipe that is made when the upper airway is blocked.

Emergencies that require immediate medical attention

These can be the first indications of a potentially life-threatening attack:

  • Hoarse voice or laryngitis
  • Difficulty in swallowing
  • Feeling of tightness
  • Voice changes

Recording information

Record information about your HAE attacks and treatment in a diary/journal. This will help you and your doctor monitor and manage your condition more effectively.

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Tools and resources

Find additional information on HAE, explore your family tree, or find the meanings of terms used to explain the causes, symptoms, treatment, or management of HAE. Use our HAE tools and resources.