EVKEEZA (evinacumab-dgnb)

EVKEEZA (evinacumab-dgnb)

Medical Administration - intravenous

Diagnosis considered for coverage:

• Indicated as an adjunct to other low-density lipoprotein-cholesterol (LDL-C) lowering therapies for the treatment of adult and pediatric patients, aged 12 years and older, with homozygous familial hypercholesterolemia (HoFH).

Limitations of Use:

• The safety and effectiveness of Evkeeza™ have not been established in patients with other causes of hypercholesterolemia, including those with heterozygous familial hypercholesterolemia (HeFH). The effects of Evkeeza™ on cardiovascular morbidity and mortality have not been determined.

Coverage Criteria:

For diagnosis of Homozygous Familial Hypercholesterolemia (HoFH):

• Dose does not exceed 15 milligrams per kilogram of bodyweight infused once every 4 weeks, AND
• Patient is 12 years of age or older, AND
• Prescribed by a cardiologist, endocrinologist, or lipid specialist, AND
• Medical records (e.g., chart notes, laboratory values) document diagnosis of homozygous familial hypercholesterolemia (HoFH) as confirmed by one of the following:
  • Genetic confirmation of two mutant alleles at the LDLR, APOB, PCSK9, or LDLRAP1 gene locus, OR
  • Both of the following:
    • One of the following:
      • Untreated/pre-treatment LDL-C greater than 500 mg/dL
      • Treated LDL-C greater than 300 mg/dL
    • One of the following:
      • Xanthoma before 10 years of age
      • Evidence of heterozygous familial hypercholesterolemia in both parents
• Patient has failed to achieve a low-density lipoprotein-cholesterol (LDL-C) goal of less than 100 mg/dL (or 70 mg/dL in HoFH patients with clinical cardiovascular disease (CVD)) (recent lab documentation required) despite the use of both of the following:
  • One of the following:
    • Patient is currently treated with maximally tolerated statin therapy plus ezetimibe, OR
    • Patient is unable to tolerate statin therapy as evidenced by one of the following intolerable and persistent (i.e., more than 2 weeks) symptoms
      • Myalgia (muscle symptoms without CK elevations)
      • Myositis (muscle symptoms with CK elevations less than 10 times upper limit of normal [ULN]), OR
    • Patient has a labeled contraindication to all statins as documented in medical records, OR
    • Patient has experienced rhabdomyolysis or muscle symptoms with statin treatment with CK elevations greater than 10 times ULN, AND
  • One of the following:
    • Patient has been treated with PCSK9 therapy or did not respond to PCSK9 therapy
    • Physician attests that the patient is known to have two LDL-receptor negative alleles (little to no residual function) and therefore would not respond to PCSK9 therapy
    • Patient has a history of intolerance or contraindication to PCSK9 therapy
    • Patient has previously been treated with Juxtapid® (lomitapide)
    • Patient has previously been treated with lipoprotein apheresis, AND
• Patient will continue other traditional lipid-lowering therapies (e.g., maximally tolerated statins, ezetimibe) in combination with Evkeeza™

Reauthorization Criteria:

For diagnosis of Homozygous Familial Hypercholesterolemia (HoFH):

• Dose does not exceed 15 milligrams per kilogram of bodyweight infused once every 4 weeks, AND
• Prescribed by a cardiologist, endocrinologist or lipid specialist, AND
• Medical records (e.g., chart notes, laboratory values) document LDL-C reduction while on Evkeeza™ therapy, AND
• Patient will continue other traditional lipid-lowering therapies (e.g., maximally tolerated statins, ezetimibe) in combination with Evkeeza™

Coverage Duration:

• Initial: 6 months
• Reauthorization: 1 year

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information:

• Warnings and precautions include embryo-fetal toxicity.  Based on the findings in animal reproduction studies, Evkeeza™ may cause fetal harm when administered to pregnant patients.  Advise patients who may become pregnant of the risk to a fetus.  Consider obtaining a pregnancy test prior to initiating treatment with Evkeeza™.  Advise patients who may become pregnant to use effective contraception during treatment with Evkeeza™ and for at least 5 months following the last dose of Evkeeza™.  

Policy Updates:

• 6/15/2021 – New policy approved by P&T.

References:

• Evkeeza Prescribing Information. Regeneron Pharmaceuticals, Inc. Tarrytown, NY. February 2021.
• Raal FJ, Rosenson RS, Reeskamp LF, et al. Evinacumab for homozygous familial hypercholesterolemia. N Engl J Med. 2020;383(8):711-720. doi:10.1056/NEJMoa2004215
• Raal FJ, Santos RD. Homozygous familial hypercholesterolemia: current perspectives on diagnosis and treatment. Atherosclerosis. 2012;223:262-8.
• Cuchel M, Bruckert E, Ginsberg HN, et al. Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society. Eur Heart J. 2014;35:2146-57.
• Harada-Shiba M, Arai H, Ishigaki Y, et al. Guidelines for Diagnosis and Treatment of Familial Hypercholesterolemia 2017. J Atheroscler Thromb. 2018;25(8):751-770. doi:10.5551/jat.CR003
• Grundy SM, Stone NJ, Bailey AL, et al. 2018 AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/APhA/ASPC/NLA/PCNA Guideline on the Management of Blood Cholesterol: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019; 73:e285-e350.
• Rosenson RS, Durrington PD. Familial hypercholesterolemia in adults: Treatment. UpToDate. http://www.utdol.com. Updated September 14, 2020. Accessed March 26, 2021.
• Adam RC, Mintah IJ, Alexa-Braun CA, et al. Angiopoietin-like protein 3 governs LDL-cholesterol levels through endothelial lipase-dependent VLDL clearance. J Lipid Res. 2020;61(9):1271-1286. doi:10.1194/jlr.RA120000888
• France M, Rees A, Datta D, et al. HEART UK statement on the management of homozygous familial hypercholesterolaemia in the United Kingdom. Atherosclerosis. 2016;255:128-139. doi:10.1016/j.atherosclerosis.2016.10.017