Ekterly — Blue Cross Blue Shield of Kansas

Initial criteria

• The patient has a diagnosis of hereditary angioedema (HAE) due to C1INH deficiency (HAE-C1INH Type 1 or Type 2) confirmed by appropriate laboratory or genetic testing OR the patient has HAE with normal C1INH evidenced by normal C1-INH and C4 levels and either a pathogenic mutation in F12, plasminogen, angiopoietin-1, kininogen-1, heparan sulfate 3-O-sulfotransferase 6, or myoferlin genes, OR diagnosis confirmed by an HAE specialist
• If the patient has an FDA labeled indication, then ONE of the following: (A) patient's age is within FDA labeling OR (B) there is support for use for the patient's age
• Medications known to cause angioedema (i.e., ACE inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate
• The requested agent will be used to treat acute HAE attacks
• If the request is for a brand agent with an available generic equivalent, ONE of the following applies: (A) patient has intolerance or hypersensitivity to the generic equivalent, (B) patient has an FDA labeled contraindication to the generic that is not expected with the brand, or (C) there is support for the use of the brand agent over the generic equivalent
• The prescriber is a specialist in the area of the patient's diagnosis (e.g., allergist, immunologist) or has consulted with a specialist
• The patient will NOT use the requested agent in combination with another agent indicated for the treatment of acute HAE attacks (i.e., Berinert, Ekterly, Firazyr, icatibant, KALBITOR, RUCONEST)
• The patient does NOT have any FDA labeled contraindications to the requested agent

Reauthorization criteria

• The patient has been previously approved for the requested agent through the plan's Prior Authorization process
• The prescriber is a specialist in the area of the patient's diagnosis (e.g., allergist, immunologist) or has consulted with a specialist
• The patient has had clinical benefit with the requested agent
• If the request is for a brand agent with an available generic equivalent, ONE of the following applies: (A) patient has intolerance or hypersensitivity to the generic equivalent, (B) patient has an FDA labeled contraindication to the generic that is not expected with the brand, or (C) there is support for the use of the brand agent
• The prescriber has verified that the patient does not have greater than a 1-month supply (sufficient for 2 acute HAE attacks) on hand
• The patient will NOT use the requested agent in combination with another agent indicated for treatment of acute HAE attacks (i.e., Berinert, Ekterly, Firazyr, icatibant, KALBITOR, RUCONEST)
• The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

initial 6 months; renewal 12 months