CurrentSecurity Health Plan of Wisconsin, Inc.Policy SHP-0514

Givlaari (givosiran) Coverage for Acute Hepatic Porphyria

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This policy governs prior authorization, dosing, renewal, and billing requirements for subcutaneous Givlaari (givosiran) used to treat acute hepatic porphyria. It applies to providers submitting requests for covered members, including initial and renewal authorizations.

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyGivlaari (givosiran) Coverage for Acute Hepatic Porphyria

Policy CodePolicy SHP-0514

Change TypeNo material clinical or coverage changes

Effective DateN/A

Last ReviewJan 6, 2026

Next Review DateN/A

Key ActionSubmit supporting medical records, laboratory results, diagnostic evidence, and other required documentation through the prior-authorization portal or by fax.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsPrior authorization validity

≥2 attacks/6 monthsQualifying attack pathway

2.5 mg/kg monthlyCovered dose

756/monthMaximum billable units

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CurrentSecurity Health Plan of Wisconsin, Inc.Policy SHP-0514

Givlaari (givosiran) Coverage for Acute Hepatic Porphyria

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Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyGivlaari (givosiran) Coverage for Acute Hepatic Porphyria

Policy CodePolicy SHP-0514

Change TypeNo material clinical or coverage changes

Effective DateN/A

Last ReviewJan 6, 2026

Next Review DateN/A

Key ActionSubmit supporting medical records, laboratory results, diagnostic evidence, and other required documentation through the prior-authorization portal or by fax.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsPrior authorization validity

≥2 attacks/6 monthsQualifying attack pathway

2.5 mg/kg monthlyCovered dose

756/monthMaximum billable units

18 yearsMinimum approval age

3–6 monthsHemin discontinuation window

Coverage Criteria

Initial Therapy

Initial coverage is provided when ALL of the following criteria are met.

Initial therapy

Universal criteria: Patient is at least 18 years old; will avoid known triggers of porphyria attacks, including alcohol, smoking, exogenous hormones, hypocaloric diet or fasting, and specified porphyrogenic medications; and has not had or is not anticipating a liver transplant.
Definitive acute hepatic porphyria diagnosis: Patient has acute hepatic porphyria, including acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, or ALA dehydratase-deficient porphyria, evidenced by elevated urinary or plasma PBG and ALA levels within the previous year OR a mutation in an affected gene identified by molecular genetic testing.Laboratory evidence within the previous year OR affected-gene mutation
ONE of: Patient has at least two documented porphyria attacks during the previous six months OR one severe attack with central, peripheral, or autonomic nervous system involvement during the previous six months OR is currently receiving off-label hemin for attack prophylaxis.≥2 attacks in 6 months OR 1 severe attack in 6 months OR current prophylactic hemin
Patients currently receiving prophylactic intravenous hemin must discontinue hemin within 3 to 6 months after initiating givosiran.

Renewal Therapy

Renewal coverage is provided when ALL of the following criteria are met.

Continuation therapy

Ongoing eligibility: Patient continues to meet the applicable universal and other indication-specific criteria identified for initial approval.
Safety: There is no unacceptable toxicity from givosiran.No unacceptable toxicity
Examples include anaphylactic reactions, severe hepatic or renal toxicity, severe injection-site reactions, increased blood homocysteine levels, and acute pancreatitis.
Clinical response: Disease response is evidenced by a decrease in the frequency of acute porphyria attacks, hospitalizations or urgent-care visits, and/or the need for intravenous hemin infusions for acute attacks.Documented improvement from baseline

Coverage is excluded for a patient who has had or is anticipating a liver transplant.

For renewal, givosiran must not be used concurrently with prophylactic intravenous hemin therapy.

Coding and Billing Requirements

HCPCS CodeHCPCSCovered

J0223

Injection, givosiran, 0.5 mg: 1 billable unit = 0.5 mg

NDCNDCCovered

71336-1001-xx

Givlaari 189 mg/mL in a single-dose vial for injection

Covered Diagnosis CodesICD-10Covered

E80.20	Unspecified porphyria
E80.21	Acute intermittent (hepatic) porphyria
E80.29	Other porphyria

Maximum Billable Units

Maximum monthly limit756 billable HCPCS units every month

Billing measureHCPCS billable units

Limit periodMonthly

Acute Attack Frequency

Minimum qualifying historyAt least 2 documented porphyria attacks

Lookback periodPrevious 6 months

Documentation requirement

Authorization and Documentation

Prior Authorization

12-Month Prior Authorization

Prior authorization is required. When approval criteria are met, initial authorization is valid for 12 months (365 days) and may be renewed every 12 months (365 days).

Documentation Required

Required Clinical Documentation

Submit supporting clinical documentation with every authorization request through the PA web portal or by fax. Documentation must support medical necessity and, when applicable, include the diagnosis, step-therapy history, clinical markers, and genetic or mutational testing.

Medical records and chart notes
Laboratory results and confirmatory diagnostics
Diagnosis and relevant clinical markers
Step-therapy information
Genetic or mutational testing when applicable

Therapy Overview

Givlaari (givosiran) is administered by subcutaneous injection to reduce hepatic heme-pathway activity in patients with acute hepatic porphyria. The policy applies to acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and ALA dehydratase-deficient porphyria when the required diagnostic evidence and clinically significant attack history are documented. Ongoing coverage requires clinical and biochemical response and avoidance of concurrent prophylactic intravenous hemin therapy.

Clinical Definitions

Acute Hepatic Porphyria (AHP)

DefinitionA group of acute hepatic porphyrias

Included typeAcute intermittent porphyria

Included typeVariegate porphyria

Included typeHereditary coproporphyria

Included typeALA dehydratase-deficient porphyria

Severe Attack

DefinitionA porphyria attack involving nervous system manifestations

Initial Approval Criteria

Initial therapy requires ALL universal and indication-specific criteria to be met.

Initial approval

Universal criteria: Patient is at least 18 years old; will avoid known triggers of porphyria attacks, including alcohol, smoking, exogenous hormones, hypocaloric diet or fasting, and specified medications such as barbiturates, hydantoins, sulfa antibiotics, and antiepileptics; and has not had or is not anticipating a liver transplant.Age ≥18 years
Diagnosis: Patient has a definitive diagnosis of acute hepatic porphyria, including acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, or ALA dehydratase-deficient porphyria, evidenced by elevated urinary or plasma PBG and ALA levels within the previous year OR a mutation in an affected gene identified by molecular genetic testing.Laboratory evidence within the previous year OR affected-gene mutation

Renewal Criteria

Renewal requires ALL continuation criteria to be met.

Givlaari renewal

Ongoing eligibility: Patient continues to meet the universal and other indication-specific criteria identified for initial approval.
No unacceptable toxicity: There is no unacceptable toxicity from givosiran.
Examples include anaphylactic reactions, severe hepatic or renal toxicity, severe injection-site reactions, increased blood homocysteine levels, and acute pancreatitis.
Clinical disease response: There is a decrease in the frequency of acute porphyria attacks, hospitalizations or urgent-care visits, and/or the need for intravenous hemin infusions for acute attacks.Documented improvement from baseline

Quantity Limits

DrugGivlaari (givosiran)

Maximum quantity756 billable HCPCS units

Quantity periodEvery month

Administration Site and Method

Note

Administer Givlaari in the office

Givlaari must be administered by a healthcare professional as a subcutaneous injection only. The recommended administration is 2.5 mg/kg once monthly based on actual body weight.

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyGivlaari (givosiran) Coverage for Acute Hepatic Porphyria

Policy CodePolicy SHP-0514

Change TypeNo material clinical or coverage changes

Effective DateN/A

Last ReviewJan 6, 2026

Next Review DateN/A

Key ActionSubmit supporting medical records, laboratory results, diagnostic evidence, and other required documentation through the prior-authorization portal or by fax.

SourceLink

On This Page

Biochemical response: ALA and PBG biochemical markers show reduction or normalization compared with baseline.Reduction or normalization versus baseline

No concurrent prophylactic hemin: Patient will not use givosiran in combination with prophylactic intravenous hemin therapy.No concurrent prophylactic IV hemin

Attacks must be documented

Severe Attack Alternative

Qualifying alternative1 severe porphyria attack

Required involvementCentral, peripheral, or autonomic nervous system (CNS, PNS, or ANS) involvement

Lookback periodPrevious 6 months

Diagnostic Laboratory Timing

Required markersElevated porphobilinogen (PBG) and delta-aminolevulinic acid (ALA)

Specimen typesUrinary or plasma levels

Testing timeframeWithin the previous year

Dose

Dose2.5 mg/kg

RouteSubcutaneous injection

FrequencyOnce monthly

Weight basisActual body weight

Documentation Required

Diagnosis and Disease-Severity Evidence

Document a definitive acute hepatic porphyria (AHP) diagnosis and the disease-severity evidence supporting treatment. The record must establish qualifying biochemical or genetic evidence and meet at least one specified attack-history or hemin-treatment criterion.

AHP diagnosis, including acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, or ALA dehydratase deficient porphyria
Elevated urinary or plasma PBG and ALA levels within the previous year, or an affected-gene mutation identified by molecular genetic testing
At least two documented porphyria attacks during the previous six months; or one severe attack with CNS, PNS, or ANS involvement during the previous six months; or current off-label hemin for attack prophylaxis
For patients receiving prophylactic intravenous hemin, document the plan to discontinue hemin within 3 to 6 months after givosiran initiation

Documentation Required

Renewal Response Documentation

For renewal, submit evidence of clinical and biochemical response while confirming that the patient continues to meet applicable criteria and has no unacceptable toxicity.

Reduced frequency of acute porphyria attacks
Fewer hospitalizations or urgent-care visits
Reduced need for intravenous hemin infusions for acute attacks
Reduction or normalization of ALA and PBG compared with baseline

Unsupported block type: denial_risk

Denial Risk

Failure to Meet Initial Approval Criteria

Initial authorization may be denied when the patient does not meet the policy’s required approval criteria. The request must establish the patient’s age, trigger-avoidance plan, transplant status, definitive diagnosis, qualifying disease severity, and any required transition from prophylactic hemin.

Patient is at least 18 years of age
Patient will avoid known porphyria triggers, including alcohol, smoking, exogenous hormones, hypocaloric diet or fasting, and specified medications
Patient has not had and is not anticipating a liver transplant
Definitive AHP diagnosis with qualifying laboratory or molecular genetic evidence
Required attack history or severity evidence is present
Patients receiving prophylactic intravenous hemin will discontinue it within 3 to 6 months after starting givosiran

Denial Risk

Failure to Meet Renewal Criteria

Renewal may be denied when response or safety requirements are not met, or when therapy is used with prophylactic intravenous hemin. Continued authorization also requires ongoing satisfaction of applicable initial and indication-specific criteria.

Unacceptable toxicity, such as anaphylaxis, severe hepatic or renal toxicity, severe injection-site reactions, increased homocysteine, or acute pancreatitis
Inadequate reduction in attacks, hospitalizations or urgent-care visits, or need for intravenous hemin
No reduction or normalization of ALA or PBG compared with baseline
Concurrent prophylactic intravenous hemin therapy

Manifestation categoriesCentral, peripheral, or autonomic nervous system involvement

ExamplesHallucinations or seizures

Diagnostic Markers

Primary biochemical markersDelta-aminolevulinic acid (ALA) and porphobilinogen (PBG)

Specimen typesUrinary or plasma

Laboratory criterionElevated ALA and PBG levels within the previous year

Additional diagnostic evidenceA mutation in an affected gene identified by molecular genetic testing

ONE of: Patient has a history of at least two documented porphyria attacks during the previous six months OR a history of one severe attack with CNS, PNS, or ANS involvement during the previous six months OR is currently receiving off-label hemin for attack prophylaxis.≥2 attacks in 6 months OR 1 severe attack in 6 months OR current prophylactic hemin

Biochemical response: ALA and PBG biochemical markers are reduced or normalized compared with baseline.Reduction or normalization versus baseline

No concurrent prophylactic intravenous hemin: Patient will not use givosiran in combination with prophylactic intravenous hemin therapy.No concurrent prophylactic IV hemin