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BerinertHighmark

Hereditary angioedema (HAE) with normal C1INH (formerly Type III)

Preferred products

  • generic icatibant

Initial criteria

  • age ≥ 5 years for Berinert; age ≥ 12 years for Ekterly; age ≥ 18 years for Firazyr/Sajazir (icatibant); age ≥ 13 years for Ruconest
  • Medication is used for the treatment of acute HAE attacks and not for prophylactic treatment
  • Prescribed by or in consultation with an allergist, immunologist, or a provider who specializes in hereditary angioedema
  • C4, C1INH antigen, and C1INH functional levels within normal limits per lab reference range
  • Meets at least one of: family history of HAE; FXII mutation; angiopoietin-1 mutation; plasminogen mutation; kininogen-1 mutation; myoferlin mutation; heparan sulfate-glucosamine 3-O-sulfotransferase 6 mutation
  • History of at least one symptom of moderate or severe angioedema attack (e.g., airway swelling, severe abdominal pain, facial swelling, nausea/vomiting, painful facial distortion) in absence of concomitant hives or angioedema-causing medication
  • Medications known to cause angioedema (e.g., ACE inhibitors, estrogens, ARBs) evaluated and discontinued when appropriate
  • Not on two acute therapies simultaneously (e.g., Berinert, Ekterly, Firazyr, Kalbitor, or Ruconest)
  • For Berinert or Ruconest: if age ≥ 18 years, therapeutic failure, contraindication, or intolerance to plan-preferred generic icatibant AND prescriber submits member's weight
  • For Ekterly if age ≥ 18 years: either therapeutic failure, contraindication, or intolerance to plan-preferred generic icatibant OR documentation member is unable to use self-administered injection due to (diagnosed needle phobia via psychiatric evaluation) OR (physical/cognitive impairment causing high risk of inappropriate administration)
  • For brand Firazyr: therapeutic failure or intolerance to generic icatibant

Reauthorization criteria

  • Member has experienced a decrease in frequency of HAE attacks from baseline OR significant improvement in severity or duration of attacks from baseline
  • If brand Firazyr requested: therapeutic failure or intolerance to generic icatibant

Approval duration

up to 12 months