ModifiedAmerihealth Caritas Vip CarePolicy MA08.112a

Givosiran (Givlaari) and Panhematin (Hemin)

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Medicare Advantage medical-benefit coverage and prior authorization requirements for givosiran (Givlaari) and panhematin (Hemin) in members with acute hepatic porphyria.

Policy Summary

PayerAmerihealth Caritas Vip Care

PolicyGivosiran (Givlaari) and Panhematin (Hemin)

Policy CodePolicy MA08.112a

Change TypeModified

Effective DateSeptember 16, 2025

Last ReviewSep 18, 2026

Next Review DateN/A

Key ActionSubmit all givosiran and panhematin requests for Company review under the policy criteria.

SourceLink

POLICY UPDATE CHANGES

The policy was reviewed and reissued on September 18, 2026 to communicate the Company's continuing position on givosiran (Givlaari).

Panhematin (Hemin) was added to the policy title and eligibility criteria were added for Panhematin therapy.

HCPCS code J1640 for injection, hemin, 1 mg was added to the policy.

ICD-10 diagnosis codes E80.20, E80.21, and E80.29 were removed from the policy.

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ModifiedAmerihealth Caritas Vip CarePolicy MA08.112a

Givosiran (Givlaari) and Panhematin (Hemin)

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Sign up for amerihealth_caritas_vip_care Policy MA08.112a alerts

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Medicare Advantage medical-benefit coverage and prior authorization requirements for givosiran (Givlaari) and panhematin (Hemin) in members with acute hepatic porphyria.

Policy Summary

PayerAmerihealth Caritas Vip Care

PolicyGivosiran (Givlaari) and Panhematin (Hemin)

Policy CodePolicy MA08.112a

Change TypeModified

Effective DateSeptember 16, 2025

Last ReviewSep 18, 2026

Next Review DateN/A

Key ActionSubmit all givosiran and panhematin requests for Company review under the policy criteria.

SourceLink

POLICY UPDATE CHANGES

The policy was reviewed and reissued on September 18, 2026 to communicate the Company's continuing position on givosiran (Givlaari).

Panhematin (Hemin) was added to the policy title and eligibility criteria were added for Panhematin therapy.

HCPCS code J1640 for injection, hemin, 1 mg was added to the policy.

ICD-10 diagnosis codes E80.20, E80.21, and E80.29 were removed from the policy.

2 drugsDrugs addressed by the policy

18+ yearsMinimum age for initial givosiran therapy

16+ yearsMinimum age for panhematin coverage

2 attacks/6 monthsActive disease threshold for initial givosiran therapy

≥4 attacks/yearRecurrent-attack threshold for prophylactic panhematin

Not coveredSpecified experimental or investigational uses

Coverage Criteria

Initial Therapy: Givosiran

Covered when ALL of the following criteria are met:

ALL of the following

The individual is 18 years of age or older.
- ONE of the following
  - Acute intermittent porphyria (AIP).
  - Hereditary coproporphyria (HCP).
  - Variegate porphyria (VP).
  - Aminolevulinic acid dehydratase-deficiency porphyria (ADP).
The individual has documentation of elevated urinary or plasma porphobilinogen (PBG) or delta-aminolevulinic acid (ALA) within the past year.
ONE of the following
- The individual has active symptomatic disease with at least two documented porphyria attacks within the past 6 months.
- The individual is currently receiving prophylactic hemin treatment due to a history of severe or frequent porphyria attacks.

Continuation Therapy: Givosiran

Covered when ALL of the following criteria are met:

ALL of the following

The individual has experienced a clinical response to therapy, such as a reduction in the number of porphyria attacks or a reduction in hemin requirements for acute attacks.
The individual does not have severe or clinically significant transaminase elevations.ALT ≤ 5 times the upper limit of normal

Initial or Prophylactic Therapy: Panhematin (Hemin)

Covered when ALL of the following criteria are met. Prophylactic use is covered for recurrent attacks occurring at least four times per year, with or without symptoms:

ALL of the following

The individual is 16 years of age or older.
- ONE of the following
  - Acute intermittent porphyria (AIP).
  - Hereditary coproporphyria (HCP).
  - Variegate porphyria (VP).
  - ALA dehydratase-deficiency porphyria (ADP).

Concurrent use of prophylactic Panhematin (Hemin) with givosiran, or concurrent use of givosiran with prophylactic Panhematin (Hemin), is considered experimental/investigational and is not covered.

Givosiran (Givlaari) is considered experimental/investigational and is not covered when liver transplantation is anticipated or when the individual has a history of recurrent pancreatitis.

Givosiran (Givlaari) and Panhematin (Hemin) are considered experimental/investigational and are not covered when requested for other forms of porphyria, including cutaneous porphyrias such as porphyria cutanea tarda (PCT).

Givosiran (Givlaari) and Panhematin (Hemin) are considered experimental/investigational and are not covered when the applicable policy criteria are not met or for all other indications.

Coding and Clinical Thresholds

CPT Procedure Code Number(s)CPT

N/A

ICD-10 Procedure Code Number(s)ICD-10

N/A

ICD-10 Diagnosis Code Number(s)ICD-10

No codes listed

HCPCS Level II Code Number(s)HCPCS

J0223

Injection, givosiran, 0.5 mg

HCPCS Level II Code Number(s) added in policy revisionHCPCS

J1640

Injection, hemin, 1 mg

Givosiran active symptomatic disease

ConditionGivosiran active symptomatic disease

Minimum activityAt least 2 documented porphyria attacks

Measurement periodWithin the past 6 months

Givosiran transaminase safety threshold

Safety measureAlanine aminotransferase (ALT)

Continuation thresholdALT not greater than 5 times the upper limit of normal

Provider Requirements and Authorization Rules

Prior Authorization

Prior Authorization Required

Prior authorization is required for every request for givosiran (Givlaari) or Panhematin (Hemin). Each request is subject to Company review under the medical-necessity criteria in this policy.

Submit all givosiran and Panhematin requests for Company review before treatment or coverage.

Documentation Required

Medical-Record Documentation

The member’s medical record must support the medical necessity of the requested drug. Records must be made available to the Company upon request for review or audit; failure to provide requested information may result in denial.

Acceptable documentation may include professional provider office records, hospital records, nursing home records, home health agency records, therapy records, and test reports.
Maintain and submit all records requested by the Company, regardless of provider participation status.

Clinical Background

Acute hepatic porphyria (AHP) comprises four inherited disorders involving deficient or abnormal enzymes in heme production. These enzyme abnormalities cause toxic intermediates to accumulate in the liver, resulting in episodic abdominal pain and neurologic or autonomic symptoms. The AHP subtypes addressed by this policy are acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, and aminolevulinic acid dehydratase-deficiency porphyria.

Definitions and Therapies

Acute hepatic porphyria (AHP)

DefinitionA group of four inherited porphyrias caused by gene mutations affecting enzymes involved in heme production

PathophysiologyMissing or deformed genes impair enzymes involved in producing heme

Clinical effectToxic substances accumulate in the liver and can cause unpredictable episodes of pain and other symptoms

Acute intermittent porphyria (AIP)

ClassificationOne of the four acute hepatic porphyria subtypes addressed by the policy

CauseDeficiency of an enzyme involved in heme synthesis

Full name

Initial Therapy Criteria

Givosiran Initial Therapy

Initial givosiran therapy is covered when all of the following criteria are met.

Givosiran initial therapy

Age: The individual is 18 years of age or older.≥18 years
AHP subtype: The individual has acute hepatic porphyria with confirmation of one of the following subtypes: acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, or aminolevulinic acid dehydratase-deficiency porphyria.
Laboratory confirmation: Documentation of elevated urinary or plasma porphobilinogen or delta-ALA within the past year.Within the past year

Continuation Criteria

Givosiran Continuation Criteria

Continuation of givosiran is covered when all of the following criteria are met.

Givosiran continuation

Clinical response: The individual has experienced a clinical response to therapy, such as a reduction in the number of porphyria attacks or a reduction in hemin requirements for acute attacks.
Transaminase safety: The individual does not have severe or clinically significant transaminase elevations; alanine aminotransferase (ALT) is not greater than five times the upper limit of normal.ALT ≤5× ULN

Dosing and Frequency

Givosiran (Givlaari)

Dose2.5 mg/kg

RouteSubcutaneously (SC)

FrequencyOnce every month

Panhematin (Hemin)

Dose1 to 4 mg/kg/day

RouteIntravenously (IV)

DurationFor 3 to 14 days based on clinical signs

Repeat dosing

Administration Site Requirements

Billing Rule

Use an appropriately equipped facility

Givosiran injections must be administered by a healthcare provider in a facility equipped and staffed to handle possible anaphylactic reactions.

Billing Rule

Confirm office anaphylaxis readiness

Givosiran injections should be administered by a healthcare provider in a facility equipped and staffed to manage possible anaphylactic reactions. Confirm that the office has the required staffing and emergency capability before administration.

Policy Summary

PayerAmerihealth Caritas Vip Care

PolicyGivosiran (Givlaari) and Panhematin (Hemin)

Policy CodePolicy MA08.112a

Change TypeModified

Effective DateSeptember 16, 2025

Last ReviewSep 18, 2026

Next Review DateN/A

Key ActionSubmit all givosiran and panhematin requests for Company review under the policy criteria.

SourceLink

On This Page

The individual has documentation of elevated urinary or plasma porphobilinogen (PBG), delta-aminolevulinic acid (ALA), or total porphyrin prior to receiving treatment.

ONE of the following clinical indications

The individual has clinical symptoms suggestive of an acute porphyric attack, such as unremitting abdominal pain, neuropathy, paresis, or tachycardia.
The individual has recurrent porphyria attacks.≥ 4 attacks per year
Prophylactic Panhematin (Hemin) may be used with or without the presence of symptoms.

Applies toContinuation givosiran therapy

Panhematin recurrent attacks

TherapyProphylactic Panhematin (Hemin)

Recurrence thresholdAt least 4 porphyria attacks per year

SymptomsProphylactic use may be medically necessary with or without symptoms

Documentation Required

Drug-Specific Clinical Evidence

Requests must include drug-specific clinical evidence supporting the applicable coverage criteria for the requested therapy.

Givosiran: document that the individual is at least 18 years old, has acute hepatic porphyria with confirmation of AIP, HCP, VP, or ADP, and has elevated urinary or plasma PBG or delta-ALA within the past year. Also document either at least two porphyria attacks within the past 6 months or current prophylactic hemin treatment for severe or frequent attacks.
Panhematin: document that the individual is at least 16 years old, has AHP with confirmation of AIP, HCP, VP, or ADP, has elevated urinary or plasma PBG, delta-ALA, or total porphyrin before treatment, and has clinical symptoms suggestive of an acute porphyric attack. For recurrent attacks, document at least four attacks per year when requesting prophylactic use.

Documentation Required

Exception and Regimen-Change Support

Requests outside the policy’s dosing or frequency requirements require supporting clinical evidence. Regimen changes also require submission for new approval based on the updated member and treatment information.

For an amount or regimen outside the listed dosing and frequency requirements, the prescribing professional provider must submit published peer-reviewed literature supporting the request.
The Company may update dosing and frequency requirements using current FDA information, recognized pharmacology compendia, and published peer-reviewed clinical research.

Note

Evio Clinical Outcomes Monitoring

For approved givosiran treatment, the requesting professional provider or member must attest and agree to provide clinical outcomes data through Evio’s secure web portal as requested. Evio administers outcomes monitoring for this high-cost therapy.

For continuation therapy, document clinical response, such as fewer porphyria attacks or reduced hemin requirements for acute attacks.
Also monitor for severe or clinically significant transaminase elevations, defined as ALT greater than five times the upper limit of normal.

Denial Risk

Givosiran Experimental or Investigational Use

Givosiran is experimental/investigational and not covered when the specified exclusions apply or when the policy criteria are not met.

Concurrent use of prophylactic Panhematin with givosiran.
Anticipated liver transplantation.
History of recurrent pancreatitis.
Requests for other forms of porphyria, including cutaneous porphyrias such as porphyria cutanea tarda (PCT).
All other indications when the policy criteria are not met.

Denial Risk

Panhematin Experimental or Investigational Use

Panhematin is experimental/investigational and not covered when the specified exclusions apply or when the policy criteria are not met.

Concurrent use of givosiran with prophylactic Panhematin.
Requests for other forms of porphyria, including cutaneous porphyrias such as porphyria cutanea tarda (PCT).
All other indications when the policy criteria are not met.

Denial Risk

Insufficient Documentation or Dosing Support

Incomplete records or inadequate support for the requested dose, frequency, or regimen may result in denial. The Company may conduct postpayment reviews and audits of claims for givosiran or Panhematin.

Submit medical records demonstrating medical necessity and the applicable drug-specific criteria.
For requests outside dosing and frequency requirements, submit published peer-reviewed literature supporting the requested amount or regimen.
Failure to produce requested documentation may result in denial for the drug.

Prior Authorization

Unapproved Dose or Regimen Change

A new approval is required when member information or the approved treatment regimen changes. Accurate member information is necessary for the Company to approve the requested dose and frequency.

Submit changes in member information, dose, frequency, or regimen to the Company for new approval as part of utilization management.
Examples include changes in member weight or an incomplete therapeutic response.

Acute intermittent porphyria

Hereditary coproporphyria (HCP)

ClassificationOne of the four acute hepatic porphyria subtypes addressed by the policy

AbbreviationHCP

Disease groupHereditary coproporphyria is an acute hepatic porphyria

Variegate porphyria (VP)

ClassificationOne of the four acute hepatic porphyria subtypes addressed by the policy

AbbreviationVP

Disease groupVariegate porphyria is an acute hepatic porphyria

ADP

AbbreviationADP

Full nameAminolevulinic acid dehydratase-deficiency porphyria

ClassificationOne of the four acute hepatic porphyria subtypes addressed by the policy

Acute hepatic porphyria (AHP)

DefinitionA group of genetic disorders caused by missing or abnormal enzymes involved in heme production

ResultToxic substances accumulate in the liver

SymptomsAccumulation can cause episodic pain and other symptoms

Panhematin (Hemin)

ProductHemin injection

MechanismRapidly downregulates aminolevulinate synthase 1 (ALAS1) expression in the liver

Therapeutic effectReduces overproduction of aminolevulinic acid (ALA) and porphobilinogen (PBG) and may ameliorate acute or recurrent attacks

Givosiran (Givlaari)

Drug classALAS1-directed small interfering RNA

MechanismCauses degradation of ALAS1 mRNA in hepatocytes through RNA interference

Therapeutic effectReduces circulating levels of the neurotoxic intermediates ALA and PBG

Disease activity or prior treatment

Active symptomatic disease: At least two documented porphyria attacks within the past six months.≥2 attacks within 6 months
Prophylactic hemin: Currently receiving prophylactic hemin treatment because of a history of severe or frequent porphyria attacks.

Panhematin Initial or Prophylactic Therapy

Panhematin is covered for acute AHP attacks when all acute-attack criteria are met, or prophylactically for recurrent attacks.

Panhematin therapy

Acute attack treatment
- Age: The individual is 16 years of age or older.≥16 years
- AHP subtype: The individual has acute hepatic porphyria with confirmation of one of the following subtypes: acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, or ALA dehydratase-deficiency porphyria.
- Laboratory confirmation: Documentation before treatment of elevated urinary or plasma PBG, delta-ALA, or total porphyrin.Prior to treatment
- Clinical attack symptoms: Clinical symptoms suggestive of an acute porphyric attack, such as unremitting abdominal pain, neuropathy, paresis, or tachycardia.
Prophylactic treatment: The individual has recurrent porphyria attacks at least four times per year; prophylactic panhematin is medically necessary with or without the presence of symptoms.≥4 attacks per year

In more severe cases, the dose may be repeated no earlier than every 12 hours