• Routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE).

• Diagnosis of hereditary angioedema confirmed by a specialist (hematologist, allergist, immunologist)

AND

• Documentation of the following laboratory results:
  • C4 level less than 14 mg/L

AND

• C1 Inhibitor (antigenic) level less than 19.9 mg/dL

OR

• C1 Inhibitor (functional) level less than 72% of the reference range

AND

• Patient requires major dental work or surgical procedure

OR

• Patient experiences more than one severe event per month OR is disabled more than five days per month OR the patient has a history of previous airway compromise

AND

• Failure or clinically significant adverse effects to danazol

• Non-FDA approved indications, which are not listed in the Health Net Approved Indications and Usage Guidelines section, unless there is sufficient documentation of efficacy and safety in the published literature.

• Plasma levels for the diagnosis of hereditary angioedema (HAE) include the following: C4 level less than 14 mg/L (diagnostic); C1 Inhibitor (antigenic) level less than 19.9 mg/dL (diagnostic); C1 Inhibitor (functional) level less than 72% of the reference range (diagnostic).
• C4 is normal between swelling events in only 2% of cases.
• Normal C4 levels: 9-36 mg/dL
• Normal Complement C1 Esterase Inhibitor, Antigen Serum = 21-39 mg/dL
• Normal Complement C1 Esterase Inhibitor, Functional = greater than 67%
• Danazol contraindications include: pediatric patients, undiagnosed abnormal genital bleeding; markedly impaired hepatic, renal, or cardiac function; pregnancy; breast feeding; androgen-dependent tumor; history of or active thrombosis or thromboembolic disease and porphyria. Periodic liver function tests should be performed because modest increases in serum transaminases levels have been reported in patients treated with danazol.
• Danazol failure: Significant frequent angioedema attacks despite androgen prophylaxis
• HAE attack triggers may include minor trauma (such as dental procedures), oral contraceptives, and ACE-Inhibitors.
• Bowen T, Cicardi M, Farkas, H., et al. recommend for short-term prophylaxis: 10 to 20 units per kg one dose 1 hour before surgery or less than 6 hours before procedures (must be given before endotracheal intubation/manipulations) with a second dose of equal amount available during surgery

Vial: 500 units

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