CurrentCignaPolicy IP0790

Spinal Muscular Atrophy - Gene Therapy Itvisma

Defines Cigna's coverage and prior authorization requirements for one-time intrathecal Itvisma for treatment of spinal muscular atrophy in pediatric and adolescent patients, including clinical, laboratory, and prescriber requirements.

Policy Summary

PayerCigna

PolicySpinal Muscular Atrophy - Gene Therapy Itvisma

Policy CodePolicy IP0790

Change TypeNew policy

Effective Date02/12/2026

Next Review DateN/A

Key ActionObtain prior authorization and submit required documentation confirming genetic testing, SMN2 copy number, antibody titer, liver/renal labs, and prior therapy status.

SourceLink

POLICY UPDATE CHANGES

New policy established for Itvisma gene therapy for spinal muscular atrophy.

one-timeapproved dose frequency

1.2 x 10^14recommended dose

2 to <18 yrsFDA age range

90 daysapproval duration

J3590billing code

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Coverage Criteria — Itvisma (onasemnogene abeparvovec-brve intrathecal)

FDA-Approved Indication (Initial/Single Dose)

Approve for a one-time (per lifetime) single dose if the patient meets ALL of the following (A through O):

A: Patient is ≥ 2 years to < 18 years of age

B: Patient has not received Itvisma or Zolgensma (onasemnogene abeparvovec-xioi) in the past (verification in claims history required)

If no claims history available, the prescribing physician must confirm prior gene therapy was not received

C: Genetic test confirming diagnosis of spinal muscular atrophy with pathogenic variants in SMN1 (documentation required)

Pathogenic variants may include homozygous deletion, compound heterozygous mutation, or other rare mutations

D.i: Patient has two or three SMN2 gene copies
D.ii: Patient has four SMN2 gene copies AND copy number determined by a quantitative assay capable of distinguishing 4 vs ≥ 5 copies

E: Baseline anti-AAV9 antibody titers ≤ 1:50 (documentation required)≤ 1:50

F.i: Alanine aminotransferase (ALT) ≤ 2 times the upper limit of normalALT ≤ 2x ULN
F.ii: Aspartate aminotransferase (AST) ≤ 2 times the upper limit of normalAST ≤ 2x ULN
F.iii: Total bilirubin ≤ 2 times the upper limit of normalTotal bilirubin ≤ 2x ULN
F.iv: Prothrombin time ≤ 2 times the upper limit of normalPT ≤ 2x ULN

G: Renal function assessment within past 30 days with creatinine < 1.0 mg/dL (documentation required)Creatinine < 1.0 mg/dL

H: Complete blood count within past 30 days meeting BOTH: WBC ≤ 20,000 cells/mm3 AND hemoglobin within normal reference range (documentation required)WBC ≤ 20,000; Hgb normal for age/gender

Reference ranges vary by laboratory, age, and gender

I: Patient does not have hepatitis B

J: Patient does not have hepatitis C

K: Patient is not human immunodeficiency virus (HIV) positive

L: If currently receiving or previously received Spinraza (nusinersen), prescribing physician confirms Spinraza will be discontinued

M: If currently receiving or previously received Evrysdi (risdiplam), prescribing physician confirms Evrysdi will be discontinued

N: Medication prescribed by a physician who has consulted with or who specializes in management of patients with spinal muscular atrophy and/or neuromuscular disorders

O: If criteria A through N are met, approve one dose of Itvisma (1.2 x 10^14 vector genomes intrathecal) as a one-time (per lifetime) single dose1.2 x 10^14 vector genomes intrathecal

Itvisma is considered not medically necessary for any use outside the FDA‑approved indication described in this policy. The policy explicitly excludes treatment of patients who have complete paralysis of all limbs, those with permanent ventilator dependence, and patients with prior receipt of gene therapy. This list is not exhaustive and may be updated as new evidence becomes available.

Additional examples of situations considered not medically necessary include use of Itvisma in patients with complete paralysis of all limbs, patients with permanent ventilator dependence, and patients who have previously received any gene therapy (Itvisma has not been studied in this population).

Coding and Laboratory Thresholds

Considered Medically Necessary when criteria metHCPCSCovered

J3590

Unclassified biologics

inv-05: AAV9 antibody titer

AAV9 antibody titerBaseline anti‑AAV9 antibody titers ≤ 1:50 (documentation required)

Test timingMeasured prior to dosing as part of baseline evaluation

DocumentationResult must be provided in prior authorization documentation

inv-06: Creatinine

CreatinineCreatinine < 1.0 mg/dL (renal function assessment within the past 30 days)

TimingMeasured within 30 days prior to dosing

Documentation

Provider Actions, Prior Authorization & Documentation

Prior Authorization

Prior Authorization Required

Prior Authorization is required for benefit coverage of Itvisma. Approval is recommended only when all clinical, laboratory, and prescriber criteria are met. Approvals are provided for a one-time (per lifetime) single dose; authorization duration is 90 days to allow preparation and administration. Claims submitted without applicable covered codes or without meeting documented criteria will be denied.

Prior authorization required before claim submission
Approval duration: 90 days (one-time, per lifetime single dose)
Claims lacking covered codes or required criteria will be denied

Note

Concurrent Therapy Confirmation

For patients currently receiving or with prior treatment with Spinraza (nusinersen) or Evrysdi (risdiplam), the prescribing physician must confirm that further therapy with those agents will be discontinued prior to approval of Itvisma.

Confirm discontinuation of Spinraza when applicable

Background

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by biallelic pathogenic variants in the SMN1 gene that result in reduced survival motor neuron protein and motor neuron loss. Disease severity varies and is influenced by SMN2 copy number, with presentations ranging from severe infantile forms to later‑onset phenotypes. Itvisma is a gene therapy intended for treatment of SMA; this policy defines the coverage scope and exclusions, including populations for whom evidence is insufficient to support benefit such as those with complete paralysis, permanent ventilator dependence, or prior gene therapy exposure.

Definitions

inv-18: SMN1 pathogenic variant

DefinitionBiallelic deletion or pathogenic variant in the SMN1 gene identified by genetic testing (documentation required)

ExamplesMay include homozygous deletion, compound heterozygous mutation, or other rare mutations

Role in diagnosisPresence of SMN1 pathogenic variants confirms genetically diagnosed spinal muscular atrophy for therapy eligibility

inv-19: SMN2 copy number

SMN2 copy number relevanceSMN2 gene copy number influences disease severity; higher copy number generally associated with milder disease

Eligibility criteriaPatient must have either two or three SMN2 copies, or four copies if determined by a quantitative assay capable of distinguishing 4 vs ≥5 copies (documentation required)

Documentation Required

Required Documentation

Documentation to support the request is required and must be submitted with the prior authorization request or made available in the medical record. Acceptable documentation includes, but is not limited to: chart notes, genetic and laboratory test results, imaging or medical test reports, claims history, prescription records, and other patient-specific information. Specific documentation required per criteria includes confirmation of SMN1 pathogenic variant, SMN2 copy number (and quantitative assay details when 4 copies are reported), baseline anti-AAV9 antibody titer, recent liver function tests (ALT, AST, total bilirubin, prothrombin time), renal function (creatinine), complete blood count (WBC and hemoglobin), and verification of no prior Itvisma or Zolgensma use when claims history is available. All documentation must include patient-identifying information.

Genetic test confirming SMN1 pathogenic variant
SMN2 copy number documentation; quantitative assay verification when 4 copies
Baseline anti-AAV9 antibody titer results
Liver function tests within 30 days: ALT, AST, total bilirubin, prothrombin time
Renal function assessment within 30 days: creatinine
Complete blood count within 30 days: WBC and hemoglobin
Verification of no prior Itvisma or Zolgensma (claims history or prescriber attestation)