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SajazirCareFirst (Caremark)

Treatment of acute attacks of hereditary angioedema (HAE) in adults age ≥ 18 years

Initial criteria

  • Medication will not be used in combination with any other medication used for the treatment of acute HAE attacks
  • Member has C1 inhibitor deficiency or dysfunction as confirmed by laboratory testing AND meets one of the following criteria: C1 inhibitor (C1-INH) antigenic level below the lower limit of normal OR normal C1-INH antigenic level and low C1-INH functional level (functional C1-INH less than 50% or below the lower limit of normal as defined by the laboratory)
  • OR Member has normal C1 inhibitor as confirmed by laboratory testing AND meets one of the following criteria: Member has an F12, angiopoietin-1, plasminogen, kininogen-1 (KNG1), heparan sulfate-glucosamine 3-O-sulfotransferase 6 (HS3ST6), or myoferlin (MYOF) pathogenic variant confirmed by genetic testing OR Member has a documented family history of angioedema and angioedema was refractory to a trial of high-dose antihistamine therapy (e.g., cetirizine 40 mg per day or equivalent) for at least one month
  • Other causes of angioedema have been ruled out (e.g., ACE inhibitor–induced angioedema, estrogen-related angioedema, allergic angioedema)
  • Medication prescribed by or in consultation with a prescriber specializing in management of HAE

Reauthorization criteria

  • Member continues to meet the initial approval criteria
  • Member has experienced a reduction in severity and/or duration of acute attacks
  • Prophylaxis considered based on attack frequency, severity, comorbid conditions, and quality of life

Approval duration

12 months