Givosiran (Givlaari)

Policy ID: I-211-005

Section: Injections

Effective Date: March 15, 2020

Revised Date: March 13, 2023

Revision Effective Date: May 01, 2023

Last Reviewed: March 23, 2023

Applies To: Commercial Only

Description

Givosiran (Givlaari ^TM ) 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) 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) 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.

The use of givosiran (Givlaari) for all other indications not listed in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness cannot be established by the available published peer-reviewed literature.

Procedure Code

J0223

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.

Diagnosis Codes

E80.20

E80.21

E80.29

Professional Statements and Societal Positions Guidelines

Not Applicable

ND Committee Review

Internal Medical Policy Committee 3-16-2020 - Effective March 15, 2020

Adopted policy

Internal Medical Policy Committee 7-22-2020 - Effective July 01, 2020

Removed procedure code C9056; and
Added procedure code J0223

Internal Medical Policy Committee 7-22-2021 Annual review, no clinical content change

Internal Medical Policy Committee 1-20-2022

Updated other indications statement to read 'Givosiran (Givlaari) not meeting the criteria as indicated in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness of this service cannot be established by the available published peer-reviewed literature.'

Internal Medical Policy Committee 1-26-2023 Annual review, no clinical content change

Internal Medical Policy Committee 3-23-2023 Effective May 01, 2023

Removed authorization time frames
Updated experimental/investigational statement

Links

References (PDF)

Disclaimer

Current medical policy is to be used in determining a Member's contract benefits on the date that services are rendered. Contract language, including definitions and specific inclusions/exclusions, as well as state and federal law, must be considered in determining eligibility for coverage. Members must consult their applicable benefit plans or contact a Member Services representative for specific coverage information. Likewise, medical policy, which addresses the issue(s) in any specific case, should be considered before utilizing medical opinion in adjudication. Medical technology is constantly evolving, and the Company reserves the right to review and update medical policy periodically.

Policy ID: I-211-004

Section: Injections

Effective Date: March 15, 2020

Revised Date: January 15, 2022

Revision Effective Date: March 01, 2022

Last Reviewed: January 26, 2023

Archived Date: April 30, 2023

Applies To: Commercial Only

Description

Givosiran (Givlaari^TM) 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.

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) not meeting the criteria as indicated in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness of this service cannot be established by the available published peer-reviewed literature.

Procedure Codes

J0223

Diagnosis Codes

E80.20

E80.21

E80.29

Professional Statements and Societal Positions Guidelines

Not Applicable

ND Committee Review

Internal Medical Policy Committee 3-16-2020 - Effective March 15, 2020

Adopted policy

Internal Medical Policy Committee 7-22-2020 - Effective July 01, 2020

Removed procedure code C9056; and
Added procedure code J0223

Internal Medical Policy Committee 7-22-2021 Annual review, no clinical content change

Internal Medical Policy Committee 1-20-2022

Updated other indications statement to read "Givosiran (Givlaari) not meeting the criteria as indicated in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness of this service cannot be established by the available published peer-reviewed literature."

Internal Medical Policy Committee 1-26-2023 Annual review, no clinical content change

Links

Refernces (PDF)

Medical Policies Quick Search