Givosiran (Givlaari) (Medicaid Expansion)

Policy ID: ME-I-211-003-04

Section: Injections

Effective Date: January 01, 2022

Revised Date: February 28, 2025

Revision Effective Date: April 01, 2025

Last Reviewed: March 11, 2025

Applies To: Medicaid Expansion 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.

Policy Application

All claims submitted under this policy's section will be processed according to the policy effective date and associated revision effective dates in effect on the date of service.

Criteria

Coverage is subject to the specific terms of the member's benefit plan.

The use of givosiran (Givlaari) may be considered medically necessary when ALL of the following criteria are met:

The individual must meet FDA-approved label for use (e.g., use outside of studied population will be considered investigational); and
Givosiran (Givlaari) must be prescribed by, or in consult with, a geneticist, hepatologist, hematologist, gastroenterologist, or specialist in acute hepatic porphyria (AHP); and
The individual must have a diagnosis of AHP (e.g., acute intermittent porphyria (AIP), porphyria cutanea tarda (PCT), erythropoietic protoporphyria (EPP)) with one of the following as defined by laboratory reference range:
- Elevated urine porphobilinogen (PBG); or
- Genetic testing confirming a pathogenic mutation e.g. HMBS/PBGD); and
The individual has had two (2) documented porphyria attacks within the past six (6) months requiring hospitalization, urgent healthcare visit, or intravenous hemin administration (number of attacks and days of hemin are documented); and
The individual has not had a liver transplant; and
The individual has addressed identifiable lifestyle triggers (e.g., certain drugs , stress) If the individual is a current tobacco/marijuana user, the individual must have received tobacco/marijuana cessation counseling in the past year; and
If the individual has a history of alcohol use within the past 5 years, one of the following must be met (1, 2 or 3):
1. The individual has a carbohydrate-deficient transferrin (CDT) level < 3% within the past 3 months; or
2. The individual has a phosphatidylethanol (PEth) level < 20 ng/mL; or
3. The individual has submitted two negative alcohol tests with the most recent alcohol test within the past 3 months.

Initial Authorization: Six (6) months

Reauthorization Criteria

Continuation of therapy with givosiran (Givlaari) may be considered medically necessary when ALL of the following criteria is met:

The individual has had a meaningful reduction (e.g., 30%) in each of the following:
- Number of porphyria attacks; and
- Days of Hemin Use; and
- Reduction in ALAS1 levels and urinary ALA and PBG.

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 United States Food and Drug Administration (U.S. 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 11-23-2021- Effective January 1, 2022

Adopted Medicaid Expansion specific policy

Internal Medical Policy Committee 11-29-2022 - Effective January 1, 2023

Updated initial criteria, and
Updated reauthorization criteria, and
Updated experimental/investigational statement

Internal Medical Policy Committee 11-15-2023 Annual Review-no changes in criteria

Internal Medical Policy Committee 11-23-2024 - Effective January 01, 2024

Annual review no clinical content change; and
Added Policy Application

Internal Medical Policy Committee 03-11-2025 - Effective April 01, 2025

Updated initial and reauthorization criteria required based on the DHHS PDL 2025 Version 2

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: ME-I-211-003-03

Section: Injections

Effective Date: January 01, 2022

Revised Date: October 17, 2024

Revision Effective Date: January 01, 2025

Last Reviewed: November 19, 2024

Archived Date: March 31, 2025

Applies To: Medicaid Expansion Only

Description

Policy Application

All claims submitted under this policy's section will be processed according to the policy effective date and associated revision effective dates in effect on the date of service.

Criteria

Coverage is subject to the specific terms of the member's benefit plan.

The use of givosiran (Givlaari) may be considered medically necessary when ALL of the following criteria are met:

The individual must meet FDA-approved label for use (e.g., use outside of studied population will be considered investigational); and
Givosiran (Givlaari) must be prescribed by, or in consult with, a geneticist, hepatologist, hematologist, gastroenterologist, or specialist in acute hepatic porphyria (AHP); and
The individual must have a diagnosis of AHP (i.e., acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), delta-aminolevulinic acid dehydratase deficient porphyria (ADP)) with the following as defined by laboratory reference range (evidenced with submitted documentation):
- Elevated urine porphobilinogen (PBG); and
- Increased aminolevulinic acid (ALA); and
- Genetic testing confirming a mutation; and
The individual has addressed identifiable lifestyle triggers (e.g. certain drugs , smoking, stress); and
The individual has had two (2) documented porphyria attacks within the past six (6) months requiring hospitalization, urgent healthcare visit, or intravenous hemin administration (number of attacks and days of hemin are documented); and
The individual has not had a liver transplant.

Initial Authorization: Six (6) months

Reauthorization Criteria

Continuation of therapy with givosiran (Givlaari) may be considered medically necessary when ALL of the following criteria is met:

The individual has had a meaningful reduction (e.g., 30%) in each of the following:
- Number of porphyria attacks; and
- Days of Hemin Use; and
- Reduction in urinary ALA.

Procedure Code

J0223

Diagnosis Codes

E80.20

E80.21

E80.29

Professional Statements and Societal Positions Guidelines

Not Applicable

ND Committee Review

Internal Medical Policy Committee 11-23-2021- Effective January 1, 2022

Adopted Medicaid Expansion specific policy

Internal Medical Policy Committee 11-29-2022 - Effective January 1, 2023

Updated initial criteria, and
Updated reauthorization criteria, and
Updated experimental/investigational statement

Internal Medical Policy Committee 11-15-2023 Annual Review-no changes in criteria

Internal Medical Policy Committee 11-23-2024 - Effective January 01, 2024

Annual review no clinical content change; and
Added Policy Application

Medical Policies Quick Search

Givosiran (Givlaari) (Medicaid Expansion)

Description

Policy Application

Criteria

Procedure Code

Diagnosis Codes

Professional Statements and Societal Positions Guidelines

ND Committee Review

Links

Disclaimer

Description

Policy Application

Criteria

Procedure Code

Diagnosis Codes

Professional Statements and Societal Positions Guidelines

ND Committee Review

Links

Disclaimer