CurrentBlue Cross Blue Shield - IowaPolicy 05.04.02

Givlaari (givosiran) for acute hepatic porphyria

Defines prior authorization, initial and continuation medical necessity criteria, required documentation, prescriber specialty, dosing guidance, billing code, and clinical rationale for coverage of Givlaari (givosiran) for treatment of acute hepatic porphyria in adults.

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyGivlaari (givosiran) for acute hepatic porphyria

Policy CodePolicy 05.04.02

Change TypeRevised (billing code added)

Effective DateJun 18, 2020

Next Review Date

Key ActionSubmit prior authorization with documentation of elevated urinary PBG or elevated plasma/fecal porphyrin for initial requests and documentation of positive clinical response for continuation.

POLICY UPDATE CHANGES

Policy lists J0223 (givosiran 0.5 mg) as the billing code (added 7-1-2020).

1Primary HCPCS code listed

>=2Required attacks in 6 months for eligibility

18+Minimum age for coverage

2.5 mg/kgRecommended monthly dose

Coverage Summary

Covered with criteria: prior authorization required for Givlaari (givosiran) for adults with acute hepatic porphyria per the policy effective 2020-06-18; last reviewed 2025-10. Coverage is covered_with_criteria and requires meeting the policy's initial or continuation medical necessity criteria (age ≥18 and other listed thresholds).

Provider Actions

Prior Authorization

Prior authorization required with documentation

Prior authorization is required for Givlaari (givosiran). For initial requests submit confirmation of elevated urinary PBG by quantitative random urine test or an elevated plasma or fecal porphyrin. For continuation requests submit documentation of a positive clinical response compared to baseline (e.g., chart notes, laboratory values).

J0223
Initial: confirmation of elevated urinary PBG by quantitative random urine test or elevated plasma/fecal porphyrin
Continuation: documentation of positive clinical response compared to baseline (chart notes, labs)

Documentation Required

Prescriber specialty requirement

Givlaari must be prescribed by, or in consultation with, a specialist experienced in treating acute hepatic porphyria such as a hepatologist, hematologist, or neurologist.

Billing Rule

Administration setting and safety

Givlaari should be administered subcutaneously by a healthcare professional in a setting with medical support available to manage potential anaphylactic reactions.

J0223

Billing Rule

Dosing limits

Approvals may be subject to dosing limits consistent with FDA-approved labeling. The recommended dose is 2.5 mg/kg administered subcutaneously once monthly.

J0223
Recommended dosing: 2.5 mg/kg subcutaneously once monthly

Clinical Evidence

Nausea 27%common adverse reaction: nausea

Injection site 25%common adverse reaction: injection site reactions

Givlaari has demonstrated clinical benefit in the randomized ENVISION trial, showing approximately 70% fewer porphyria attacks versus placebo during the 6-month double-blind period. Common adverse reactions reported include nausea (27%) and injection site reactions (25%)

Background & Definitions

Givlaari (givosiran) is a double-stranded small interfering RNA that degrades hepatic ALAS1 mRNA via RNA interference, reducing hepatic ALAS1 expression and lowering circulating neurotoxic intermediates ALA and PBG; it is indicated for the treatment of adults with acute hepatic porphyria (AHP).

Active disease: Two documented porphyria attacks within the past 6 months requiring hospitalization, urgent healthcare visit, or intravenous hemin administration at home.

Revision History

2020-07-01material_change

Policy lists J0223 (givosiran 0.5 mg) as the billing code (added 7-1-2020).

2020-06-18effective_date

Policy effective date.

2025-10last_reviewLatest

Policy reviewed (last_review: 2025-10).

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyGivlaari (givosiran) for acute hepatic porphyria

Policy CodePolicy 05.04.02

Change TypeRevised (billing code added)

Effective DateJun 18, 2020

Next Review Date

On This Page

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Givlaari (givosiran) for acute hepatic porphyria

Coverage Summary

Initial Therapy Criteria

Continuation of Therapy Criteria

Provider Actions

Applicable Codes

Clinical Evidence

Background & Definitions

Revision History