Orladeyo — Blue Cross Blue Shield of Montana

Initial criteria

• ONE of the following: (A) The requested agent is eligible for continuation of therapy AND the prescriber states the patient has been treated with the requested agent (starting on samples is not approvable) within the past 90 days AND is at risk if therapy is changed OR (B) ALL of the following:
• 1. The patient has a diagnosis of hereditary angioedema (HAE) due to C1INH deficiency [HAE-C1INH (Type 1 or Type 2)] evidenced by ONE of the following: (A) Diagnosis confirmed with measurements of C1-INH protein level, C1-INH function level, and C4 level meeting Type 1 or Type 2 HAE definitions OR (B) Diagnosis confirmed by mutation in the C1-INH gene altering protein synthesis and/or function.
• 2. The requested agent is being prescribed for HAE prophylaxis.
• 3. The patient has a history of at least three moderate to severe acute HAE attacks per month (e.g., airway swelling, severe abdominal pain, painful facial swelling).
• 4. If the patient has an FDA labeled indication, then ONE of the following: (A) The patient’s age is within FDA labeling for the requested indication OR (B) There is support for using the requested agent for the patient’s age for the requested indication.
• 5. If TAKHZYRO is requested, age and dosing requirements apply: (A) Adults/≥12 years—criteria for initiation, duration, attack freedom status, and dose adjustment (300 mg q4w or q2w). (B) Age 6–<12—criteria for initiation, duration, attack freedom status, and dose adjustment (150 mg q4w or q2w). (C) Age 2–<6—eligible without exclusions stated prior.
• 6. Medications known to cause angioedema (ACE inhibitors, estrogens, angiotensin receptor blockers) have been evaluated and discontinued when appropriate.
• 7. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., allergist, immunologist) or has consulted with one.
• 8. The patient will NOT be using the requested agent in combination with another agent indicated for prophylaxis of HAE attacks (Andembry, CINRYZE, Dawnzera, HAEGARDA, Orladeyo, TAKHZYRO).
• 9. The patient does NOT have any FDA labeled contraindications to the requested agent.
• Alternative approval path: (A) For BCBS MT Fully Insured or MT HIM members under age 18, no FDA contraindications, with indication supported by two peer-reviewed journal articles and age bracket support in two peer-reviewed journals, OR (B) For BCBS NM Fully Insured or NM HIM members meeting rare disease and literature/compendia support criteria, OR (C) For Ohio Fully Insured or HIM Shop members meeting literature/compendia support for safe and effective proposed use.

Reauthorization criteria

• 1. The patient has been previously approved for the requested agent through the plan’s Prior Authorization process.
• 2. The prescriber is a specialist in the relevant area or has consulted with one.
• 3. The patient has had clinical benefit as evidenced by: decreased frequency of acute HAE attacks from baseline OR decreased use of on-demand therapy.
• 4. The patient will NOT be using the requested agent in combination with another agent indicated for prophylaxis of HAE attacks (Andembry, Dawnzera, CINRYZE, HAEGARDA, Orladeyo, TAKHZYRO).
• 5. If TAKHZYRO is requested, same age and maintenance/dose adjustment criteria as initial approval.
• 6. The patient does NOT have any FDA labeled contraindications to the requested agent.

Approval duration

12 months (BCBSIL and BCBSMT); other plans: 3 months for CINRYZE, 4 months for HAEGARDA, 6 months for Andembry or Orladeyo, 9 months for TAKHZYRO