CurrentUnitedHealthcarePolicy IEXD0087.08

Givlaari (givosiran) — Coverage Criteria for Acute Hepatic Porphyria

Policy governing medical benefit coverage criteria for Givlaari (givosiran) for treatment of adults with acute hepatic porphyrias for UnitedHealthcare Individual Exchange plans (with state exceptions).

Policy Summary

PayerUnitedHealthcare

PolicyGivlaari (givosiran) — Coverage Criteria for Acute Hepatic Porphyria

Policy CodePolicy IEXD0087.08

Change TypeUpdated Clinical Evidence and References

Effective DateApr 1, 2025

Next Review Date

Key ActionObtain prior authorization with documentation of diagnosis, attack history or prophylactic hemin use, and provider attestation of baseline hemin/attack frequency.

SourceLink

POLICY UPDATE CHANGES

Updated Clinical Evidence and References sections to reflect the most current information.

2.5 mg/kgFDA recommended dosing

>=2 attacks/6moInitial therapy threshold

70%ENVISION attack reduction

J0223HCPCS

Coverage Criteria for Givlaari (givosiran)

Policy Summary

PayerUnitedHealthcare

PolicyGivlaari (givosiran) — Coverage Criteria for Acute Hepatic Porphyria

Policy CodePolicy IEXD0087.08

Change TypeUpdated Clinical Evidence and References

Effective DateApr 1, 2025

Next Review Date

Key ActionObtain prior authorization with documentation of diagnosis, attack history or prophylactic hemin use, and provider attestation of baseline hemin/attack frequency.

SourceLink

On This Page

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Initial Therapy: Diagnosis of an acute hepatic porphyria (AHP) (i.e., acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, ALA dehydratase deficient porphyria); AND (Patient has active disease as defined by either: >= 2 documented porphyria attacks within the past 6 months OR patient is currently receiving prophylactic hemin to prevent porphyria attacks); AND provider attestation that the patient's baseline (before givosiran is initiated) hemin administration requirements and rate and/or number of porphyria attacks has been documented; AND patient has not had a liver transplant; AND patient will not receive concomitant prophylactic hemin treatment while on Givlaari; AND prescribed by, or in consultation with, a hematologist or a specialist with expertise in the diagnosis and management of AHPs; AND Givlaari dosing is in accordance with the United States Food and Drug Administration approved labeling; AND initial authorization will be for no more than 12 months.

Continuation Therapy

Covered when ALL of the following are met

Continuation Therapy: Patient has previously received Givlaari for the treatment of AHP; AND documentation that the patient has experienced a positive clinical response while on Givlaari; AND patient has not had a liver transplant; AND patient will not receive concomitant prophylactic hemin treatment while on Givlaari; AND prescribed by, or in consultation with, a hematologist or a specialist with expertise in the diagnosis and management of AHPs; AND Givlaari dosing is in accordance with the United States Food and Drug Administration approved labeling; AND reauthorization will be for no more than 12 months.

Patients who have undergone a liver transplant are excluded from coverage under this policy. In addition, concomitant prophylactic hemin treatment while on Givlaari is not permitted and will preclude coverage under the criteria. These restrictions apply to both initiation and continuation of therapy and are intended to reflect safety and efficacy considerations described in the policy.

Providers must attest to the patient’s baseline (pre‑givosiran) hemin administration requirements and the documented rate and/or number of porphyria attacks; absence of that attestation or documentation consistent with the policy criteria is a potential basis for denial.

Givlaari (givosiran) is covered when the initial criteria are met: a confirmed diagnosis of an acute hepatic porphyria (AHP) and either at least 2 documented porphyria attacks in the prior 6 months or current receipt of prophylactic hemin to prevent attacks. Coverage also requires the prescriber to be a hematologist or other specialist with expertise in AHPs or that such a specialist be consulted.

Prior authorization is required. The request must include provider attestation documenting baseline hemin administration requirements and the patient’s attack frequency/number, and dosing must follow the FDA‑approved labeling. Initial authorizations are limited to no more than 12 months. For continuation, documentation of a positive clinical response while on Givlaari is required, and continuation authorizations are also limited to no more than 12 months.

Coding and Diagnosis References

Applicable HCPCSHCPCS

J0223

Injection, givosiran, 0.5 mg.

Diagnosis Codes referencedICD-10

E80.0	Hereditary erythropoietic porphyria.
E80.1	Porphyria cutanea tarda.
E80.20	Unspecified porphyria.
E80.21	Acute intermittent (hepatic) porphyria.
E80.29	Other porphyria

Minimum documented porphyria attacks

Threshold>= 2 porphyria attacks in the prior 6 months

Attack definition used in ENVISION trialAttacks requiring hospitalization, urgent healthcare visit, or IV hemin administration at home

Alternate qualifying pathwayCurrent receipt of prophylactic hemin to prevent porphyria attacks (meets active disease requirement)

Provider Requirements and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization required. Documentation must demonstrate diagnosis of an acute hepatic porphyria (AHP) and either a history of attacks or current prophylactic hemin use before givosiran initiation. Initial authorizations are limited to a maximum of 12 months.

Required for initial and continuation therapy
Initial authorization: up to 12 months
Reauthorization: up to 12 months

Documentation Required

Required Documentation

Providers must attest to baseline hemin administration requirements and document the rate and/or number of porphyria attacks prior to initiating givosiran. For continuation, documentation must show a positive clinical response while on therapy.

Provider attestation of baseline hemin administration requirements (prophylactic or treatment) and documented attack rate/number
For initial therapy: documentation of ≥2 porphyria attacks in the past 6 months OR current prophylactic hemin use
For continuation therapy: evidence of prior givosiran use and positive clinical response

Documentation Required

Concomitant Therapy Restriction

Concomitant prophylactic hemin is not permitted while a patient is receiving givosiran. Patients receiving prophylactic hemin at baseline may be eligible if documented, but prophylactic hemin must be discontinued during givosiran therapy.

Patient will not receive concomitant prophylactic hemin treatment while on Givlaari (givosiran)
Baseline prophylactic hemin use may support initial eligibility but must be stopped once givosiran is started

Denial Risk

Triggers for Possible Denial

Applications lacking required diagnosis, insufficient attack documentation, missing provider attestation of baseline hemin use/attack rate, or evidence of liver transplant are at higher risk for denial.

No documented diagnosis of an acute hepatic porphyria (AHP)
Insufficient documentation of attack frequency (fewer than 2 attacks in past 6 months) and no baseline prophylactic hemin use
Missing provider attestation of baseline hemin administration requirements and attack rate/number
Patient has had a liver transplant
Prescriber is not a hematologist or specialist with AHP expertise
Prescribed dosing not consistent with FDA labeling

Definitions

AHP — definition and contextual attributes

DefinitionAcute hepatic porphyria (AHP): a family of ultra-rare genetic hepatic enzyme deficiencies in the heme biosynthesis pathway leading to acute neurovisceral attacks and long-term complications such as hypertension, chronic kidney disease, and hepatocellular carcinoma.

Common presenting featuresSevere unexplained abdominal pain often with nausea, vomiting, neurologic symptoms (confusion, seizures, weakness), and dark or reddish urine.

Pathophysiology relevant to therapyGivosiran is an siRNA that degrades hepatic ALAS1 mRNA, reducing ALA and PBG levels associated with attacks.

Background

Acute hepatic porphyrias (AHP) are a family of ultra‑rare, inherited hepatic enzyme deficiencies in the heme biosynthesis pathway that produce episodic neurovisceral attacks—typically severe abdominal pain with neurologic features—and can lead to long‑term complications such as hypertension, chronic kidney disease, and hepatocellular carcinoma. Givlaari (givosiran) is an RNA interference therapy that targets hepatic ALAS1 mRNA to reduce the neurotoxic intermediates ALA and PBG and thereby decrease the frequency of porphyria attacks.

In clinical practice and in the pivotal trials, active disease for purposes of initiating therapy has been defined by attack burden (for example, >= 2 attacks in the prior 6 months) or by the need for prophylactic hemin to prevent recurrent attacks. This clinical context underpins the policy’s coverage criteria and documentation requirements.