CurrentCignaPolicy IP0118

Givosiran (Givlaari) for Acute Hepatic Porphyria — Medical Necessity and Coverage Criteria

Medical necessity and coverage criteria for use of givosiran (Givlaari) in treating adults with acute hepatic porphyria under Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyGivosiran (Givlaari) for Acute Hepatic Porphyria

Policy CodePolicy IP0118

Change TypeNo material change

Effective Date

Next Review Date

Key ActionObtain prior authorization with documentation of age ≥18, diagnostic confirmation (clinical features plus elevated ALA or PBG), a qualifying porphyria attack in the last 6 months, and specialist involvement.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

>=18minimum patient age for coverage

2.5 mg/kgrecommended dose (monthly)

Up to 6 moauth duration (initial)

Up to 12 moauth duration (reauth)

J0223HCPCS code

Coverage Criteria for Givosiran (Givlaari)

Policy Summary

PayerCigna

PolicyGivosiran (Givlaari) for Acute Hepatic Porphyria

Policy CodePolicy IP0118

Change TypeNo material change

Effective Date

Next Review Date

SourceLink

On This Page

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Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

AHP treatment criteria: 1) Age 18 years or older; 2) Diagnosis of acute hepatic porphyria confirmed by BOTH: (a) demonstrated clinical features associated with AHP (e.g., neurovisceral symptoms, blistering lesions, hepatic involvement, peripheral neuropathy, abdominal pain, constipation, muscle weakness, pain in the arms and legs) AND (b) ONE of: elevated urinary aminolevulinic acid (ALA) > upper limit of normal OR elevated urinary or plasma porphobilinogen (PBG) > upper limit of normal; 3) History of one porphyria attack in the last 6 months that required hospitalization, urgent healthcare visit, or intravenous hemin administration; 4) Medication is prescribed by, or in consultation with, a gastroenterologist, hepatologist, medical geneticist, or a physician who specializes in acute hepatic porphyria.

Dosing: Up to 2.5 mg/kg subcutaneously no more frequently than once every 30 days.

Continuation Therapy — Reauthorization covered when ALL of the following are met

Reauthorization covered when ALL of the following are met

Reauthorization criteria: Original medical necessity criteria continue to be met AND there is documentation of beneficial response (for example, reduction in porphyria attacks, improvement of signs and symptoms, decrease in hemin administration).

Reauthorization duration: up to 12 months.

Conditions Not Covered / Experimental

Not covered uses: Any other use is considered experimental, investigational, or unproven.

Criteria will be updated as new published data are available.

Any use of givosiran (Givlaari) that does not meet the specific medical necessity criteria outlined in this policy is considered experimental, investigational, or unproven. This includes indications, patient populations, dosing regimens, or treatment durations outside the covered criteria. The policy will be updated as new published data become available.

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for givosiran (Givlaari). Initial approvals may be granted for up to 6 months; reauthorization approvals may be granted for up to 12 months. Submit prior authorization requests with complete documentation that meets the medical necessity criteria (see Medical Necessity and Reauthorization Criteria) and includes diagnostic and clinical evidence as described below.

Initial approval duration: up to 6 months
Reauthorization approval duration: up to 12 months

Note

Context Regarding Existing Therapies

Treatment decisions should take into account existing, guideline-recommended therapies for acute hepatic porphyria (AHP). Hemin therapy (e.g., Panhematin) is recommended for prevention and treatment of acute attacks and may be used prophylactically for patients with frequent attacks (≥4 attacks/year) or on an as‑needed basis. Prophylactic hemin needs periodic reassessment (every 6–12 months) due to risks such as iron overload and potential hepatic damage. Carbohydrate loading may be used in early attacks though evidence is limited. Women with cyclic attacks may benefit from hormonal management or GnRH analogs in addition to consideration of prophylactic hemin.

Denial Risk

Triggers for Denial

Requests lacking the following documentation are at high risk for denial. Ensure the prior authorization includes: confirmation the patient is ≥18 years old; documentation of clinical features consistent with AHP; biochemical confirmation (elevated urinary ALA or elevated urinary/plasma PBG); documentation of history of at least one porphyria attack in the past 6 months that required hospitalization, urgent healthcare visit, or IV hemin; and that the medication is prescribed by or in consultation with an appropriate specialist (gastroenterologist, hepatologist, medical geneticist, or a physician specializing in AHP).

Denial triggers: age < 18 years
Denial triggers: absence of documentation confirming diagnosis of AHP (clinical features plus elevated urinary ALA or elevated urinary/plasma PBG)
Denial triggers: no history of a qualifying porphyria attack in the past 6 months (hospitalization, urgent care visit, or IV hemin)
Denial triggers: prescriber is not an appropriate specialist and no documented consultation

Background

Acute hepatic porphyrias (AHPs) are a group of liver-based enzyme deficiencies that produce episodic neurovisceral symptoms such as severe abdominal pain, neuropathy, and, in some types, cutaneous manifestations. AHPs include disorders like acute intermittent porphyria and other hepatic porphyrias characterized by accumulation of neurotoxic intermediates aminolevulinic acid (ALA) and porphobilinogen (PBG).

Givosiran is an aminolevulinate synthase 1-directed small interfering RNA (siRNA) that reduces hepatic ALAS1 mRNA, lowering levels of ALA and PBG and thereby decreasing the frequency of acute porphyria attacks. Clinical trials of givosiran generally enrolled adults with recurrent attacks (usually requiring at least two attacks in the prior 6 months), and treatment decisions should reflect the demonstrated benefits in similar populations.

For coverage under this policy, confirm that the patient meets criteria such as age ≥18 years, biochemical confirmation of AHP (elevated urinary ALA or urinary/plasma PBG), and an appropriate clinical history of recent porphyria attacks; specific medical necessity requirements are detailed in the coverage criteria section.

Definitions

AHP — Acute hepatic porphyria (definitions and key terms)

DefinitionAcute hepatic porphyria (AHP) — a subgroup of porphyrias caused by a hepatic enzyme deficiency (includes acute intermittent porphyria [AIP], variegate porphyria [VP], 5-aminolevulinic acid dehydratase deficiency porphyria [ALAD], and hereditary coproporphyria [HCP]) characterized by acute neurovisceral symptoms with or without cutaneous manifestations.

PathophysiologyLiver-based enzyme deficiency leading to accumulation of neurotoxic intermediates (aminolevulinic acid [ALA] and porphobilinogen [PBG]) that cause episodic neurovisceral symptoms.

Common clinical manifestationsIntermittent acute neurovisceral symptoms such as severe abdominal pain, constipation, muscle weakness, limb pain, insomnia, emotional/psychiatric symptoms, tachycardia, and hypertension; VP and HCP may also have photosensitivity.

Typical diagnostic confirmationDiagnosis requires clinical features consistent with AHP plus biochemical confirmation (elevated urinary ALA > ULN or elevated urinary or plasma PBG > ULN).

Relevant patient populationAdults aged ≥ 18 years (policy applies to patients 18 years or older).

Treatment targetTherapies aim to reduce hepatic ALAS1 activity and lower circulating ALA and PBG to prevent or decrease frequency/severity of acute attacks.

OpenPayer

Givosiran (Givlaari) for Acute Hepatic Porphyria — Medical Necessity and Coverage Criteria

Coverage Criteria for Givosiran (Givlaari)

Coding and Billing

Provider Actions, Prior Authorization, and Documentation

Background

Definitions