Criteria
Coverage is subject to the specific terms of the member’s benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.
Evinacumab-dgnb (Evkeeza) may be considered medically necessary when the following criteria are met:
- Individual is 12 years of age or older; and
- Evinacumab-dgnb (Evkeeza) will be prescribed by or in consultation with a cardiologist, endocrinologist, or lipid specialist; and
- The individual will continue on current lipid lowering treatment regimen in combination with evinacumab-dgnb (Evkeeza); and
- There is genetic confirmation or clinical documentation of homozygous familial hypercholesterolemia (see table 1 below); and
- Individual has failure of or intolerance to statin therapy (see table 2 below); and
- Individual has failure of proprotein convertase substilisin kexin 9 (PCSK9) inhibitor (e.g., alirocumab or evolocumab based upon FDA approval for age) for at least three (3) months (see table 3 below).
Table 1
Documentation of Homozygous Familial Hypercholesterolemia (must meet either genetic confirmation or clinical confirmation column) |
Genetic Confirmation | Clinical Documentation (ONE from each of the following) |
Two (2) mutant alleles at the LDLR, ApoB, PCSK9, or LDLRAP1 gene locus | ONE of the following untreated lab values |
Untreated total cholesterol of greater than 500 mg/dL |
Untreated LDL-C of greater than 400 mg/dL |
Attestation of ONE of the following |
Cutaneous or tendon xanthoma before age 10 years |
Evidence of heterozygous familial hypercholesterolemia in both parents |
Table 2
Statin Therapy Failure (must meet either statin failure or statin intolerance column) |
Statin Failure (ONE of the following) | Statin Intolerant (One of the following) |
17 years of age or younger LDL-C greater than 135 mg/dL, despite use of a maximally tolerated statin | Statin related rhabdomyolysis or skeletal-related muscle symptoms while receiving at least two (2) separate trials of different statins which resolved upon discontinuation of the statins |
18 years of age or older LDL-C greater than 100 mg/dL, despite use of a maximally tolerated statin | Creatinine kinase (CK) increase to 10 times upper limit of normal (ULN) during any one (1) course of statin therapy |
Liver function tests (LFTs) increase to 3 times upper limit of normal (ULN) during any one (1) course of statin therapy |
Hospitalization due to severe statin-related adverse event, such as rhabdomyolysis during any one (1) course of statin therapy |
Table 3
PCSK9 Failure or Contraindication/Adverse Event (must meet either column) |
PCSK9 Treatment Failure | PCSK9 Contraindication/Adverse Event |
13 to 17 years of age LDL-C greater than 135 mg/dL, despite use of a PCSK9 inhibitor (evolocumab) for at least three (3) months | Contraindication to or adverse event from PCSK9 inhibitor therapy |
18 years of age or older LDL-C greater than 100 mg/dL, despite use of a PCSK9 inhibitor (alirocumab or evolocumab) for at least three (3) months | |
Evinacumab-dgnb (Evkeeza) for any other indication is considered experimental/investigational and therefore, not covered. The safety and/or efficacy cannot be established by review of the available published peer-reviewed literature.
Procedure Codes