Orladeyo – NC Standard

Restricted Product(s):

• Orladeyo™ (berotralstat)

FDA Approved Use:

• For prophylaxis to prevent attacks of hereditary angioedema (HAE) in adults and pediatric patients 12 years of age and older.

Criteria for Approval of Restricted Product(s):

Initial Coverage

1. The patient has a diagnosis of Hereditary Angioedema (HAE) as confirmed through complement testing (Please submit medical record documentation to demonstrate C4 level, C1-inhibtor level, and C1-INH function where applicable); AND
   1. If HAE-I, please demonstrate low C4 and C1-inhibitor level; AND
   2. If HAE-II, please demonstrate low C4 with a normal C1-inhibitor level and a decreased C1-INH function; AND
   3. If HAE-III, please demonstrate normal C4 and C1-inhibitor and family history of angioedema; AND
2. The patient is 12 years of age or older; AND
3. Treatment dosing details have been provided for review (including patient’s current weight and dosing schedule); AND
4. Prescriber attests that patient has experienced attacks of a severity and/or frequency such that they would clinically benefit from prophylactic therapy (e.g., 2 or more attacks within the last 2 months) (medical record documentation required); AND
5. The patient will be treated with Orladeyo for routine prophylaxis and NOT for treatment of acute HAE attacks; AND
6. The patient will not be treated with another anti-HAE medication in the prevention of HAE attacks (Haegarda, Takhzyro, Cinryze); AND
7. The prescribed dose and quantity are appropriate based on intended use and FDA labeling; AND
8. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., allergist, immunologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis; AND
9. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.

Duration of Approval: 365 days (1 year)

Continuation Coverage

1. The patient has been previously approved with Blue Cross and Blue Shield of North Carolina (Blue Cross NC) or would have met initial criteria for approval upon the start of therapy; AND
2. The patient has shown a reduction in HAE attacks since initiation of the requested agent (medical record documentation required); AND
3. The patient will not be treated with another anti-HAE medication in the prevention of HAE attacks (Haegarda, Takhzyro, Cinryze); AND
4. The prescribed dose and quantity are appropriate based on intended use and FDA labeling; AND
5. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., allergist, immunologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis.

Duration of Approval: 365 days (1 year)

Quantity Limitations:

Quantity limitations apply to brand and associated generic products.

Quantity Limit Exception Criteria:

1. The quantity (dose) requested is for documented titration purposes at the initiation of therapy (authorization for a 90 day titration period); AND
2. The prescribed dose cannot be achieved using a lesser quantity of a higher strength; AND
3. The quantity (dose) requested does not exceed the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert; OR
4. If the quantity (dose) requested exceeds the maximum FDA labeled dose, when specified, or to the safest studied dose per the manufacturer’s product insert, then the prescriber must submit documentation in support of therapy with a higher dose for the intended diagnosis (submitted documentation may include medical records OR fax form which reflects medical record documentation that shows the length of time the requested dose has been used, and what other medications and doses have been tried and failed).

Duration of Approval: 365 days (1 year)

References:

All information referenced is from FDA package insert unless otherwise noted below.

Policy Implementation/Update Information:

Criteria and treatment protocols are reviewed annually by the Blue Cross NC P&T Committee, regardless of change. This policy is reviewed in Q2 annually.

April 2025: Criteria change: Removed trial and failure requirement Haegarda or Takhzyro.

July 2022: Criteria change: Added step through Haegarda or Takhzyro for HAE prophylactic treatment.

April 2022: Criteria change: Added evaluation of frequency/severity of previous HAE attacks to warrant prophylactic treatment. Added patient will NOT be using the requested agent in combination with another anti-HAE agent. Added prescriber is a specialist or consulted a specialist. Added dose and quantity are appropriate based on FDA labeling.

December 2020: Original utilization management criteria issued.