CurrentCentenePolicy CP PHAR.327

Nusinersen (Spinraza)

Policy governing medical necessity criteria, prior authorization, and coverage rules for nusinersen (Spinraza) for treatment of spinal muscular atrophy (SMA) across Centene lines of business including Commercial, HIM, and Medicaid.

Policy Summary

PayerCentene

PolicyNusinersen (Spinraza)

Policy CodePolicy CP PHAR.327

Change TypeNo material change

Effective Date03.01.17

Next Review Date

Key ActionSubmit prior authorization with documentation (chart notes, labs or other clinical information) demonstrating the member meets all approval criteria.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsapproval duration

12 mgdose per administration

4 dosesinitial dose limit

SMN2 ≤4genetic test requirement

J2326HCPCS

HB 5395

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Coverage Criteria for Nusinersen (Spinraza)

inv-01: Initial Approval Criteria

Covered when ALL of the following are met:

Initial Approval: 1) Diagnosis of spinal muscular atrophy (SMA).

Genetic confirmation: 2) Genetic testing confirming one of the following: a) homozygous deletion of SMN1; b) homozygous mutation in SMN1 (e.g., biallelic mutations of exon 7); or c) compound heterozygous SMN1 mutation (e.g., deletion of SMN1 exon 7 on one allele and mutation on the other). Documentation must also confirm no more than 4 copies of the SMN2 gene.SMN2 ≤ 4 copies

Prescriber: 3) Prescribed by or in consultation with a neurologist.

Baseline motor function testing: 4) Baseline motor function testing appropriate to age documented prior to treatment initiation: for age <2 years, CHOP-INTEND or HINE Section 2; for age ≥2 years, HFMSE, RHS, ULM/RULM, or 6‑Minute Walk Test (6MWT).

See Appendix D for test selection and interpretation.

Respiratory support exclusion: 5) Member does not require tracheostomy or invasive or noninvasive ventilation ≥16 hours/day continuously for >21 days.

Step therapy with Evrysdi: 6) Failure of a trial of Evrysdi (risdiplam) unless contraindicated or clinically significant adverse effects are experienced. For Illinois HIM requests, the step therapy requirement does not apply per IL HB 5395 effective 1/1/2026.

Failure defined as decline in motor function test score(s) from baseline.

Concurrent therapy exclusion: 7) Nusinersen (Spinraza) is not prescribed concurrently with Evrysdi (risdiplam) or onasemnogene abeparvovec-xioi (Zolgensma). If member is currently on Evrysdi, prescriber must attest to Evrysdi discontinuation upon initiation of nusinersen.

Prior Zolgensma treatment: 8) If member has history of Zolgensma treatment, provider must submit both: a) evidence of poor response to Zolgensma (e.g., sustained decrease in CHOP-INTEND score over a 6‑month period); and b) provider attestation of clinical deterioration.

Dose and duration limits: 9) Total dose for initial approval does not exceed 4 intrathecal doses of 12 mg; approval duration 12 months (up to 4 doses).<= 4 doses of 12 mg intrathecal

inv-02: Renewal / Continued Therapy Criteria

Covered when ALL of the following are met:

Eligibility for renewal: 1) Member is currently receiving nusinersen and has 1 to 4 copies of SMN2 or previously met initial approval criteria.SMN2 1–4 copies or prior initial approval

Clinical response: 2) Member is responding positively to therapy as evidenced by motor function testing per age: for age <2 years (CHOP-INTEND) improvement or maintenance of ≥4 points from baseline, or for HINE motor milestone score improvement/maintenance with net more improved categories than worsened; for age ≥2 years, demonstrated improvement or maintenance on HFMSE/RHS (HFMSE/RHS improvement or maintenance, e.g., ≥3 points in some contexts), RULM improvement/maintenance ≥4 points, ULM improvement/maintenance ≥2 points, or 6MWT improvement or maintenance.Specific point changes as above

If first renewal since turning 2 years old, submit baseline HFMSE/RHS/RULM/ULM/6MWT distance recorded at that time.

First renewal at age transition:

Non–FDA indications for nusinersen (Spinraza) that are not specifically addressed in this clinical policy are not authorized unless the provider supplies sufficient documentation demonstrating efficacy and safety per the applicable off‑label use policies: CP.CPA.09 (Commercial), HIM.PA.154 (Health Insurance Marketplace), or CP.PMN.53 (Medicaid), or unless there is an applicable evidence of coverage document that supports the indication.

When state Medicaid coverage provisions conflict with the coverage provisions in this clinical policy, the state Medicaid coverage provisions take precedence. Providers should consult the applicable state Medicaid manual for specific coverage rules and any state‑level requirements that supersede this policy.

The policy acknowledges that the efficacy of nusinersen in patients with fewer than 2 copies of SMN2 has not been demonstrated. It is unknown whether such patients produce sufficient SMN protein for clinical benefit, and use in patients with <2 SMN2 copies should be considered of unknown efficacy unless additional evidence is provided.

Coding and Dosing

HCPCS Codes referencedHCPCS

J2326

HCPCS code referenced in this clinical policy (description not provided in excerpt)

Dosing schedule — loading and maintenance dosing (12 mg intrathecal)

Loading dose (initial series)12 mg intrathecally on days 0, 14, and 28 (three loading doses) with a fourth loading dose of 12 mg intrathecally 30 days after the third loading dose

Maintenance dose12 mg intrathecally every 4 months after completion of loading series

Maximum/standard dose per administration12 mg intrathecally per dose

Total doses referenced for initial approvalInitial approval covers up to 4 loading doses (each 12 mg) over the loading period

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for requests under this policy. Providers must submit a prior authorization request and supporting clinical documentation to demonstrate the member meets all approval criteria. All requests reviewed under this policy require medical director review.

Prior authorization required for Nusinersen (Spinraza) and related requests
All requests require medical director review

Billing Rule

Prior Authorization and Coding

Providers should include relevant billing/coding information with the prior authorization request. HCPCS code J2326 is referenced for informational purposes. Inclusion or exclusion of codes in this policy does not guarantee coverage; providers must reference current professional coding guidance when submitting claims.

Background — Disease and Therapy Context

Spinal muscular atrophy (SMA) is an autosomal recessive genetic disorder caused by mutations in the SMN1 gene. Disease severity generally correlates with age at onset and the number of copies of the SMN2 gene: higher SMN2 copy number typically corresponds to milder phenotypes. For example, approximately 80% of individuals with Type I SMA have 1–2 copies of SMN2, about 82% of those with Type II have 3 copies, and most individuals with Type III have 3–4 copies. SMA is progressive and, without treatment, infants with severe forms show rapid decline in motor function (quantified by instruments such as CHOP‑INTEND), which informs baseline assessment and monitoring requirements in this policy.

Definitions and Key Terms

Nusinersen (Spinraza) — agent description and indication summary

AgentNusinersen (Spinraza), an SMN2-directed antisense oligonucleotide

IndicationIndicated for treatment of spinal muscular atrophy (SMA) in pediatric and adult patients

Route of administrationIntrathecal injection

SMN2 copy number — influence on severity and policy requirement (<=4 copies)

Policy requirementDocumentation of no more than 4 copies of SMN2 is required for approval

Clinical influenceSMN2 copy number correlates with SMA severity; higher SMN2 copy counts generally associate with less severe disease