Currentblue cross blue shield - tennesseePolicy N/A

Itvisma (intrathecal onasemnogene abeparvovec) for spinal muscular atrophy

Covers use of Itvisma for treatment of spinal muscular atrophy (SMA) in specified pediatric and adult populations when approval criteria are met; applies to BlueCross BlueShield of Tennessee members and their providers.

Policy Summary

Payerblue cross blue shield - tennessee

PolicyItvisma (intrathecal onasemnogene abeparvovec) for spinal muscular atrophy

Policy CodePolicy N/A

Change TypeAdded FDA-approved indication

Effective DateJun 2, 2026

Next Review DateN/A

Key ActionObtain prior authorization with genetic confirmation of bi-allelic SMN1 variants and documentation of SMN2 copy number and baseline labs.

SourceLink

POLICY UPDATE CHANGES

Itvisma is indicated for treatment of SMA in adults and pediatric patients 2 years of age and older with confirmed SMN1 mutation.

1 dosemaximum authorized total doses for SMA treatment

>=2 yrsminimum age for FDA indication

≤3SMN2 gene copies required

<=1:50anti-AAV9 antibody titer requirement

6/2/2026

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Itvisma (intrathecal onasemnogene abeparvovec)

inv-01: Initial therapy - SMA

Covered when ALL of the following are met for treatment of SMA:

Overall age and indication: Member has a genetically confirmed diagnosis of SMA with bi-allelic pathogenic SMN1 variants; Itvisma indicated for patients >= 2 years of ageage >= 2 years

FDA-approved indication applies to adults and pediatric patients 2 years and older

Dose limitation: Authorization of one dose total may be granted1 dose total

Single total dose policy

Clinical onset: Member's onset of clinical signs and symptoms occurred at 6 months of age or olderonset >= 6 months

Specifies later-onset disease inclusion

SMN2 copy number: Member has 3 or less copies of SMN2 geneSMN2 <= 3 copies

Copy number requirement

Age at administration: Member is 2 years of age or older at the time of treatment administrationage >= 2 years

Policy applies to patients aged 2 years and older

Ventilation status: Member does not require invasive ventilation, prolonged noninvasive ventilation (>6 hours awake or >12 hours total per 24-hour period), or tracheostomyno chronic invasive or prolonged NIV/tracheostomy

Respiratory support exclusion

Anti-AAV9 antibody: Member has anti-AAV9 antibody titer <= 1:50 as determined by ELISA binding immunoassaytiter <= 1:50

Pre-treatment serology requirement

Procedural and infection contraindications: No contraindication to lumbar puncture and no active infectious process prior to treatmentno LP contraindication; no active infection

Procedural safety criteria

Allergy and serious illness: Member has no history of allergy or hypersensitivity to the treatment regimen or excipients and no serious concomitant illness (e.g., severe liver or kidney disease, symptomatic cardiomyopathy)no allergy/serious comorbidity

Safety exclusions

Baseline assessments and monitoring: Baseline liver function, platelet count, troponin I, creatinine, neurologic evaluation, and HFMSE assessment have been performed and will be monitored after administration as clinically appropriatebaseline labs and HFMSE documented

Required monitoring

Prior therapies: If the member is on nusinersen (Spinraza) or risdiplam (Evrysdi), those therapies must be discontinued prior to administrationdiscontinue other SMA disease-modifying therapies

Avoid concurrent disease-modifying therapy

Vaccination status: Member's vaccination status will be up to date prior to Itvisma administrationup-to-date vaccinations

Immunization requirement

No prior gene therapy: Member has not received Itvisma, Zolgensma, or other gene therapy previouslyno prior gene therapy

Prior gene therapy exclusion

All indications for Itvisma outside of the FDA‑approved indication for treatment of spinal muscular atrophy (SMA) in patients 2 years of age and older with a confirmed SMN1 mutation are considered experimental/investigational and not medically necessary. Requests for coverage for non‑SMA indications will be denied.

Use of Itvisma for any indication other than the FDA‑approved treatment of SMA in adults and pediatric patients 2 years of age and older with confirmed SMN1 mutation is considered not medically necessary. Providers seeking authorization for uses outside this labeled population should not expect coverage and should document that the indication falls within the FDA‑approved SMA population to avoid denial.

Laboratory and Test Requirements

Anti‑AAV9 antibody titer — requirement

Anti-AAV9 antibody titer requirement≤ 1:50 as determined by ELISA (Enzyme‑linked Immunosorbent Assay) binding immunoassay

Assay method specifiedELISA binding immunoassay (enzyme‑linked immunosorbent assay)

TimingMust be assessed prior to Itvisma administration (pre‑treatment serology)

Consequence of not meeting thresholdRequests do not meet criteria if titer > 1:50 (may be denied)

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization required. Genetic confirmation of SMA (bi-allelic SMN1 pathogenic variants), documentation of SMN2 copy number, baseline assessments, and other PA requirements must be submitted to initiate review.

Genetic confirmation of SMA (bi-allelic SMN1 pathogenic variants) required
SMN2 copy number documentation required (laboratory assay e.g., quantitative PCR or MLPA)
Baseline assessments: liver function, platelet count, troponin I, creatinine, neurologic evaluation, HFMSE

Prior Authorization

Discontinue Other SMA Therapies Before Itvisma

If the member is currently receiving nusinersen (Spinraza) or risdiplam (Evrysdi), these therapies must be discontinued prior to administration of Itvisma.

Discontinue nusinersen (Spinraza) prior to Itvisma administration
Discontinue risdiplam (Evrysdi) prior to Itvisma administration

Definitions and Assessment Tools

Genetically confirmed SMA — definition

DefinitionPresence of bi‑allelic pathogenic variants in the SMN1 gene (deletions or point mutations) as demonstrated by genetic testing

Documentation requiredGenetic testing results demonstrating bi‑allelic pathogenic SMN1 variants must be submitted with prior authorization request

Role in coverage decisionGenetic confirmation is a mandatory criterion for authorization of Itvisma for SMA

HFMSE — assessment for SMA

What it isHammersmith Functional Motor Scale–Expanded (HFMSE): a standardized motor function assessment used for baseline and follow‑up evaluations in SMA

Documentation requiredHFMSE assessment results must be included in prior authorization submission and baseline assessment

Background

Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder caused by pathogenic variants in the SMN1 gene. Disease severity is modified by the number of copies of the paralogous SMN2 gene. Intrathecal onasemnogene abeparvovec (Itvisma) is a gene therapy administered intrathecally and is indicated by the FDA for treatment of SMA in adults and pediatric patients 2 years of age and older with confirmed SMN1 mutation. The policy covers Itvisma only when the FDA‑labeled SMA indication and associated authorization criteria are met; other uses are considered investigational and not medically necessary.