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AndembryBlue Cross Blue Shield of Oklahoma

hereditary angioedema (HAE) with normal C1INH (HAE-nI-C1INH)

Preferred products

  • icatibant (generic equivalent for Firazyr)

Initial criteria

  • Diagnosis confirmed as HAE Type 1 or Type 2 by decreased C1-INH protein, C1-INH function, and C4 levels OR mutation in the C1-INH gene
  • Diagnosis confirmed as HAE with normal C1-INH by normal C1-INH protein, C1-INH function, and C4 levels AND mutation in one of: coagulation factor FXII (F12), plasminogen, angiopoietin-1, kininogen-1, heparan sulfate 3-O-sulfotransferase 6, or myoferlin OR confirmed HAE-U by specialist
  • If patient has an FDA labeled indication, the age must be within labeling or supported for that age indication
  • Medications known to cause angioedema (ACE inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate
  • Requested agent will be used to treat acute HAE attacks
  • If brand agent with available generic equivalent is requested (Firazyr vs icatibant), then one of: member plan type eligibility OR stability on brand OR inadequate response to generic OR intolerance/adverse event/contraindication/medical necessity per listed subpoints with chart notes
  • Prescriber is a specialist in allergy/immunology or consulting with one
  • Requested agent will not be used in combination with another acute HAE therapy (Berinert, Ekterly, Firazyr, icatibant, KALBITOR, RUCONEST)
  • Patient has no FDA labeled contraindications
  • Alternate approval for BCBS MT Fully Insured or MT HIM members age <18 years meeting rare-disease literature support criteria
  • Alternate approval for BCBS NM Fully Insured or NM HIM members meeting rare-disease/compendia or literature support criteria

Reauthorization criteria

  • Patient previously approved through plan's prior authorization process
  • Prescriber is a specialist in allergy/immunology or consulting with one
  • Patient has had clinical benefit with requested agent
  • If brand agent with available generic equivalent is requested (Firazyr vs icatibant), same justification criteria as initial approval apply
  • Prescriber has confirmed frequency and severity of attacks and verified patient does not have >1 month supply on hand
  • Requested agent not used in combination with another acute HAE therapy (Berinert, Ekterly, Firazyr, icatibant, KALBITOR, RUCONEST)
  • Patient has no FDA labeled contraindications

Approval duration

6–12 months depending on plan; renewal 12 months