Givosiran (Givlaari)

Section: Injections
Effective Date: March 15, 2020
Last Reviewed: March 16, 2020

Description

Givosiran (GivlaariTM) is an aminolevulinate synthase 1-directed small interfering RNA that causes degradation of aminolevulinate synthase 1 (ALAS1) mRNA in hepatocytes through RNA interference, reducing the elevated levels of liver ALAS1 mRNA. This leads to reduced circulating levels of neurotoxic intermediates aminolevulinic acid (ALA) and porphobilinogen (PBG), factors associated with attacks and other disease manifestation of acute hepatic porphyria (AHP). Givosiran (Givlaari) is, therefore, indicated for the treatment of adults with AHP.

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.

Givosiran (Givlaari) for an initial six (6) month authorization may be considered medically necessary when ALL of the following criteria are met:

  • Individual is 18 years of age or older; and
  • Individual is diagnosed with AHP [acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), delta-aminolevulinic acid dehydratase deficient porphyria (ADP), or no identified mutation] by at least ONE of the following:
    • Elevated urine porphobilinogen (PBG); or
    • Increased aminolevulinic acid (ALA); or
    • Genetic testing confirming a mutation; and
  • Individual has a history of symptoms of AHP chronically or during previous acute attack(s) (e.g., abdominal, back, and/or chest pain, weakness, paralysis, respiratory failure, confusion, anxiety, depression, memory loss, fatigue, hallucinations, seizures, skin lesions, hypertension, tachycardia, nausea, vomiting, constipation, hyponatremia, neuropathic pain, sensory loss); and
  • Individual has not had a prior liver transplantation; and
  • Prescribed by or in consultation with a geneticist, hepatologist, hematologist, gastroenterologist, neurologist, or any healthcare provider with experience managing AHP.

Reauthorization Criteria

Givosiran (Givlaari) for an additional reauthorization period of 12 months may be considered medically necessary when ALL of the following criteria are met:

  • Individual has experienced a positive clinical response indicated by an improvement of the previously reported laboratory value(s) and symptoms; and
  • Prescribed by or in consultation with a geneticist, hepatologist, hematologist, gastroenterologist, neurologist, or any healthcare provider with experience managing AHP; and
  • Individual has not had a prior liver transplantation.

Givosiran (Givlaari) for any other indication is considered not medically necessary and therefore, not covered.

Procedure Codes

J3490

Outpatient HCPCS (C Codes)

C9056*

*effective 4/1/2020

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes

E80.20 E80.21 E80.29

Professional Statements and Societal Positions Guidelines

NA

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