Treating Acute Attacks

The second method of treating HAE is to treat acute attacks as they occur. For this method to work effectively, you need to become your own best advocate by learning to recognize the symptoms of your 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 to know.

Acute attacks

Prior to 2009, no specific treatments were available in the United States to treat acute attacks of HAE. However, there are now several treatment options specifically indicated to treat HAE attacks:4

  • C1-INH (Complement 1 esterase inhibitor protein) concentrate for acute abdominal, facial or laryngeal HAE attacks in adults and adolescents
  • Plasma kallikrein inhibitor for acute HAE attacks in individuals 12 years of age and older
  • Bradykinin B2 receptor antagonist for acute HAE attacks in adults 18 years of age and older

C1-INH product

C1-INH 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. Adverse events associated with products that replace C1-INH are headache, nausea, rash, vomiting, impairment of the sense of taste, and increase in the severity of pain associated with HAE.

Kallikrein inhibitor

A kallikrein inhibitor works by blocking the generation of the enzyme kallikrein and its byproduct, bradykinin, which is thought to cause HAE attacks. Kallikrein inhibitors have been associated with such side effects as headache, nausea, diarrhea, pyrexia, injection-site reactions, and nasopharyngitis.

Bradykinin B2 Reception Antagonist

Bradykinin is a protein that is thought to be responsible for the characteristic HAE symptoms of localized swelling, inflammation, and pain. Bradykinin B2 receptor antagonists are drugs that bind to the bradykinin B2 receptor and prevent bradykin from activating this receptor. This blocks bradykin from inducing the clinical symptoms of an acute, episodic attack of HAE. The most commonly reported adverse reactions associated with a bradykinin B2 receptor antagonist were injection site reactions, pyrexia, transaminase increase,

Other treatment options

Before multiple products were available, fresh-frozen plasma ( FFP) was administered for acute HAE attacks. Additional treatment options include epinephrine, which provides only temporary relief, and androgens and antifibrinolytics, which don’t take effect until several days have passed and could 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 attacks

The most dangerous angioedema attacks from HAE are laryngeal attacks, those affecting the throat and airways. Because the swelling that results from an acute laryngeal attack can close the airways, it is vital that the attack be controlled as quickly as possible. Patients need to get immediate medical attention if they notice any of the following signs, which could mean that a potentially life-threatening laryngeal attack is beginning:3

  • A hoarse voice or laryngitis
  • Difficulty swallowing
  • Feeling of tightness in the upper airways
  • Other 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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