Evinacumab-dgnb (Evkeeza™) Notification

• Evinacumab-dgnb (Evkeeza™) intravenous infusion for administration by a healthcare professional 

• For treatment of adult and pediatric patients, aged 12 years and older, with homozygous familial hypercholesterolemia (HoFH), as an adjunct to other low-density lipoprotein-cholesterol (LDL-C) lowering therapies 
• Limitations of use: Not for patients with other causes of hypercholesterolemia, including those with heterozygous familial hypercholesterolemia (HeFH); cardiovascular morbidity and mortality effects have not been determined

The restricted product(s) may be considered medically necessary when the following criteria are met:

1. The patient is 12 years of age or older; AND
2. The patient has a diagnosis of homozygous familial hypercholesterolemia (HoFH); AND
3. The patient has ONE of the following:
   1. Genetic confirmation of two mutant alleles at the LDLR, Apo-B, PCSK9, or LDLRAP1 gene [medical record documentation required]; OR
   2. History of untreated LDL-C >500 mg / dL (>13 mmol / L) or treated LDL-C ≥300 mg / dL (≥7.76 mmol / L) [medical record documentation required] with ONE of the following:
      1. Clinical manifestations of HoFH (e.g., cutaneous xanthomas, tendon xanthomas, arcus cornea, tuberous xanthomas, or xanthelasma) before 10 years of age; OR
      2. Evidence of elevated LDL-C or total cholesterol (TC) levels consistent with heterozygous FH in both parents (i.e., untreated LDL-C >190 mg / dL [>4.9 mmol / L] and / or untreated TC >250 mg / dL); AND
4. The prescriber has provided a baseline LDL-C level, obtained within 60 days prior to starting treatment with requested agent [medical record documentation required]; AND
5. The patient has failed to achieve a target LDL-C level while treated with and adherent to high-intensity statin therapy or maximally tolerated statin therapy, in combination with ezetimibe and a proprotein convertase subtilisin kexin Type 9 (PCSK9) inhibitor for ≥8 continuous weeks; AND 
   1. ONE of the following:
      1. The patient has been adherent to high-intensity statin therapy (i.e., rosuvastatin ≥20 mg daily, atorvastatin ≥40 mg daily) for ≥8 continuous weeks AND ONE of the following: 
         1. The patient’s LDL-C level after this treatment regimen remains ≥70 mg / dL; OR
         2. The patient has not achieved a 50% reduction in LDL-C from baseline after this treatment regimen; OR
         3. If the patient has atherosclerotic cardiovascular disease (ASCVD), the patient’s non-HDL-C level after this treatment remains ≥100 mg / dL; OR
      2. The patient has been determined to be statin intolerant by meeting one of the following criteria:
         1. The patient experienced statin-related rhabdomyolysis; OR 
         2. The patient experienced skeletal-related muscle symptoms (e.g., myopathy [muscle weakness] or myalgia [muscle aches, soreness, stiffness, or tenderness]) and BOTH of the following:
            1. The skeletal-related muscle symptoms (e.g., myopathy or myalgia) occurred while receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or as combination products); AND
            2. When receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or as combination products) the skeletal-related muscle symptoms (e.g., myopathy, myalgia) resolved upon discontinuation of each respective statin therapy (atorvastatin and rosuvastatin); OR 
         3. The patient experienced elevations in hepatic transaminase while receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or as combination products); OR
      3. The patient has an FDA labeled contraindication or hypersensitivity to atorvastatin and rosuvastatin; AND
   2. For patients receiving concurrent lomitapide (Juxtapid® ) with the requested agent:
      1. The patient has failed to achieve a target LDL-C level while on lomitapide (Juxtapid® ) in combination with the following:
         1. High-intensity or maximally tolerated statin therapy; AND 
         2. Ezetimibe or one additional lipid-lowering medication (e.g., nicotinic acid, bile acid sequestrant, or fibrate); AND
      2. The patient has been taking and adherent to lomitapide (Juxtapid® ) therapy for for ≥8 continuous weeks; AND
   3. ONE of the following:
      1. The patient has tried and had an inadequate response (i.e., LDL-C level ≥70 mg / dL despite treatment) to a PCSK9 inhibitor (i.e., Repatha® [evolocumab] OR Praluent® [alirocumab]) for ≥8 weeks [medical record documentation required]; OR 
      2. The patient has an intolerance, FDA labeled contraindication, or hypersensitivity to BOTH Repatha® (evolocumab) and Praluent® (alirocumab) [medical record documentation required]; OR
      3. The patient has genetic confirmation of two LDLR negative alleles (null homozygous) [medical record documentation required]; AND 
6. The requested agent will be used in conjunction with other LDL-C lowering therapies (e.g., maximally tolerated statin therapy, ezetimibe, PCSK9 inhibitor antibodies, lomitapide, lipoprotein apheresis); AND 
7. The patient does not have any FDA labeled contraindications to the requested agent; AND
8. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., cardiologist, endocrinologist, and/or lipid specialist) or has consulted with a specialist in the area of the patient’s diagnosis; AND 
9. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below); AND
10. For requests for injection or infusion administration of the requested medication in an inpatient or outpatient hospital setting, Site of Care Criteria applies.

Duration of Approval: 180 days (6 months)

1. The patient was approved through Blue Cross NC initial criteria for approval; OR 
2. The patient would have met initial criteria for approval at the time they started therapy; AND
3. The patient has had clinical benefit with the requested agent demonstrated by a reduction in LDL-C level from baseline prior to starting therapy with the requested agent [medical record documentation required]; AND 
4. ONE of the following: 
   1. The patient is currently adherent to high-intensity statin therapy (i.e. rosuvastatin ≥20 mg, atorvastatin ≥40 mg); OR 
   2. The patient has been determined to be statin intolerant by meeting one of the following criteria:
      1. The patient experienced statin-related rhabdomyolysis; OR
      2. The patient experienced skeletal-related muscle symptoms (e.g., myopathy [muscle weakness] or myalgia [muscle aches, soreness, stiffness, or tenderness]) and BOTH of the following: 
         1. The skeletal-related muscle symptoms (e.g., myopathy or myalgia) occurred while receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or as combination products); AND
         2. When receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or as combination products) the skeletal-related muscle symptoms (e.g., myopathy, myalgia) resolved upon discontinuation of each respective statin therapy (atorvastatin and rosuvastatin); OR
      3. The patient experienced elevations in hepatic transaminase while receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or as combination products); OR 
   3. The patient has an FDA labeled contraindication or hypersensitivity to atorvastatin and rosuvastatin; AND
5. The requested agent will be used in conjunction with other LDL-C lowering therapies (e.g., maximally tolerated statin therapy, ezetimibe, PCSK9 inhibitor antibodies, lomitapide, lipoprotein apheresis); AND 
6. The patient does not have any FDA labeled contraindications to the requested agent; AND
7. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., cardiologist, endocrinologist, and/or lipid specialist) or has consulted with a specialist in the area of the patient’s diagnosis; AND 
8. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below); AND 
9. For requests for injection or infusion administration of the requested medication in an inpatient or outpatient hospital setting, Site of Care Criteria applies.

Duration of Approval: 365 days (1 year)

1. For requests for injection or infusion administration in an inpatient setting, the injection or infusion may be given if the above medical necessity criteria are met AND the inpatient admission is NOT for the sole purpose of administering the injection or infusion; OR 
2. For requests for injection or infusion administration in an outpatient hospital setting, the injection or infusion may be given if the above medical necessity criteria are met AND ONE of the following must be met: 
   1. History of mild adverse events that have not been successfully managed through mild pre-medication (e.g., diphenhydramine, acetaminophen, steroids, fluids, etc.); OR
   2. Inability to physically and cognitively adhere to the treatment schedule and regimen complexity; OR
   3. New to therapy, defined as initial injection or infusion OR less than 3 months since initial injection or infusion; OR
   4. Re-initiation of therapy, defined as ONE of the following:
      1. First injection or infusion after 6 months of no injections or infusions for drugs with an approved dosing interval less than 6 months duration; OR
      2. First injection or infusion after at least a 1-month gap in therapy outside of the approved dosing interval for drugs requiring every 6 months dosing duration; OR
   5. Requirement of a change in the requested restricted product formulation; AND 
3. If the Site of Care Medical Necessity Criteria in #1 or #2 above are not met, the injection or infusion will be administered in a home-based infusion or physician office setting with or without supervision by a certified healthcare professional.

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

October 2021: Criteria change: Added Site of Care medical necessity criteria. Policy notification given 8/2/2021 for effective date 10/1/2021.

July 2021: Criteria update: Added HCPCS code C9079 to dosing reference table effective 7/1/2021, deleted C9399 termed 6/30/2021.

June 2021: Criteria change: Added maximum units; medical policy formatting change. Policy notification given 4/16/2021 for effective date 6/16/2021.

Further historical criteria changes and updates available upon request from Medical Policy and/or Corporate Pharmacy