CurrentAvMedPolicy N/A

Givlaari (givosiran) prior authorization for acute hepatic porphyria

Authorization form and medical necessity criteria for coverage of Givlaari (givosiran) for members with acute hepatic porphyria; applies to AvMed members requiring medical (J0223) coverage and prescribers initiating therapy.

Policy Summary

PayerAvMed

PolicyGivlaari (givosiran) prior authorization for acute hepatic porphyria

Policy CodePolicy N/A

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionObtain prior authorization and submit supporting documentation including lab results (ALA or PBG), chart notes, prior therapy history, and hemin use as applicable.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsInitial authorization duration

12 monthsContinuation authorization duration

≥18Minimum member age for approval

2 in 6 moMinimum documented attacks

2Quantity limit (vials/mo)

Coverage Criteria for Givlaari (givosiran)

Initial Therapy Criteria

Covered when ALL of the following are met for Initial Authorization (approval = 6 months):

Initial Authorization

Member is ≥ 18 years of age
Prescriber is a hepatologist, hematologist, oncologist or other specialist in treatment of acute hepatic porphyria (ICD-10 codes E80.21 or E80.29)
Clinical diagnosis of acute hepatic porphyria (AIP, VP, HCP, ADP, or other). Diagnosis of non-acute/chronic cutaneous porphyria is excluded from coverage; for off-label diagnoses, supporting literature must be provided.
Diagnosis is based on at least ONE clinical feature: gastrointestinal (abdominal pain, vomiting, constipation, diarrhea); neurologic (extremity or back pain, paresis, mental symptoms, respiratory paralysis); or cardiovascular (tachycardia, systemic arterial hypertension)

Policy Summary

PayerAvMed

PolicyGivlaari (givosiran) prior authorization for acute hepatic porphyria

Policy CodePolicy N/A

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionObtain prior authorization and submit supporting documentation including lab results (ALA or PBG), chart notes, prior therapy history, and hemin use as applicable.

SourceLink

On This Page

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Documentation of elevated urinary or plasma porphobilinogen (PBG) and/or delta-aminolevulinic acid (ALA) within the previous year (submit recent ALA or PBG levels)

History of at least two documented porphyria attacks within the past 6 months AND one of the following: requirement of hospitalization that included IV hemin (J1640), urgent healthcare visit that included IV hemin, treatment with monthly IV hemin at home within the last 6 months, or IV hemin due to severe CNS involvement (e.g., hallucination or seizures)

Member has absence of unacceptable toxicity from givosiran (e.g., anaphylaxis, severe hepatic or renal toxicity, severe injection site reactions)

Member will avoid concomitant use with CYP1A2 or CYP2D6 substrates (pharmacy claims will be verified)

Member will avoid known triggers of porphyria attacks (e.g., alcohol, smoking, exogenous hormones, hypocaloric diet/fasting, certain medications such as barbiturates, hydantoins, sulfa-antibiotics, antiepileptics)

Members currently receiving prophylactic intravenous hemin (J1640) will discontinue hemin within 3 to 6 months of initiation with givosiran

Member has not received and is not awaiting liver transplant

Continuation Therapy Criteria

Continuation of Therapy (approval = 12 months) — Covered when ALL of the following are met:

Continuation Authorization

All criteria for initial approval continue to be met
Absence of unacceptable toxicity from givosiran (examples include anaphylactic reactions, severe hepatic toxicity, severe renal toxicity, severe injection site reactions)
Positive clinical response to givosiran, evidenced by decreased frequency of acute porphyria attacks, decreased hospitalizations/urgent care visits, or decreased requirement for intravenous hemin infusions
Reduction or normalization of biochemical markers (ALA, PBG) compared to baseline (submit current lab results)
No concomitant use of givosiran with prophylactic intravenous hemin therapy
Member has not received a liver transplant

Coverage does not include diagnoses of non-acute or chronic cutaneous porphyria. Only members with a clinical diagnosis of acute hepatic porphyria (AIP, VP, HCP, ADP, or an approved off‑label diagnosis supported by literature) are eligible for Givlaari (givosiran) under this policy; non-acute/chronic cutaneous porphyria is excluded from coverage.

Concomitant prophylactic intravenous hemin therapy is not permitted with givosiran treatment. Members must not use givosiran in combination with prophylactic IV hemin as a condition of coverage.

Coding and Quantity Limits

Covered HCPCS CodesHCPCSCovered

J0223

Givosiran (Givlaari) injection (Medical)

Related HCPCS Codes (hemin)HCPCS

J1640

Hemin for injection, porphyria

Relevant ICD-10 CodesICD-10

E80.21	Acute intermittent porphyria
E80.29	Other acute hepatic porphyrias

Quantity limit

Quantity limit (pharmacy)Givlaari 189 mg/mL single‑dose vial: 2 vials every month

Maximum dose units per monthMax 288 mg every month

Product form/strength referencedGivlaari 189 mg/mL single‑dose vial for injection

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior authorization required for Givlaari (J0223)

Prior authorization is required for Givlaari (givosiran) when billed as J0223 (medical). Documentation of prior IV hemin use (J1640) may be requested to support criteria related to prior hospitalizations or hemin infusions.

Drug requested listed as Givlaari (givosiran) Subcutaneous (J0223) (Medical).
Requirement to document IV hemin (J1640) for certain clinical history criteria.

Step Therapy

Prior therapy verification — samples do not meet preauthorization

Use of samples to start therapy does not meet the plan's step-edit/preauthorization criteria. Previous therapies will be verified through pharmacy paid claims or submitted chart notes.

Samples provided to initiate therapy will not satisfy preauthorization requirements.
Prior therapies may be confirmed via pharmacy paid claims or chart documentation.

Documentation Required

Required supporting documentation (labs, diagnostics, chart notes)

Submit supporting documentation with the authorization request, including recent laboratory results (ALA or PBG), diagnostics, chart notes, prior therapy history, and documentation of attack frequency and any hemin use as applicable.

Recent urinary or plasma PBG and/or ALA levels (within the previous year).
Chart notes documenting at least two porphyria attacks in the past 6 months and details of hospitalizations/urgent visits or IV hemin use.
Documentation of baseline and follow-up biochemical markers and clinical response.

Denial Risk

Incomplete or illegible requests may delay or deny authorization

Authorization can be delayed or denied if the request information is incomplete, incorrect, or illegible; ensure all required fields are complete and legible on the prior authorization form.

The prescribing physician must sign and clearly print their name; preprinted stamps are not valid.
Incomplete, incorrect, or illegible information may delay authorization.

Background

Givosiran (Givlaari) is an RNA interference therapeutic indicated for patients with acute hepatic porphyria (AHP) subtypes (including acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and ALA dehydratase deficient porphyria) to reduce the frequency of porphyria attacks. For initial authorization (approval = 6 months), the policy requires the member be ≥ 18 years old, treated by an appropriate specialist, have biochemical confirmation of elevated urinary or plasma ALA or PBG within the prior year, and a clinical history of at least two porphyria attacks in the past 6 months with specified hemin-related events. Continuation criteria include absence of unacceptable toxicity, clinical improvement (fewer attacks, hospitalizations, or hemin infusions), and reduction or normalization of biochemical markers; continuation approvals are for 12 months.

Definitions

Acute hepatic porphyria (AHP) — definition and subtypes

DefinitionAcute hepatic porphyria (AHP) — a group of hepatic porphyrias characterized by acute neurovisceral attacks; includes several subtypes

Included subtypesAcute intermittent porphyria (AIP); Variegate porphyria (VP); Hereditary coproporphyria (HCP); ALA dehydratase deficient porphyria (ADP)

ExclusionsNon‑acute/chronic cutaneous porphyria is excluded from coverage