ModifiedUnitedHealthcarePolicy CSPA2026D0059O

Spinraza® (Nusinersen) (for Pennsylvania Only) – Community Plan Medical Benefit Drug Policy

UnitedHealthcare Community Plan Medical Benefit Drug Policy for Spinraza (nusinersen) applicable only to Pennsylvania; defines initial and continuation authorization criteria, excluded indications, applicable HCPCS and ICD-10 codes, and operational/billing constraints (loading and maintenance dose limits and administration settings).

Policy Summary

PayerUnitedHealthcare

PolicySpinraza® (Nusinersen) (for Pennsylvania Only) – Community Plan Medical Benefit Drug Policy

Policy CodePolicy CSPA2026D0059O

Change TypeRevised coverage criteria; supporting information updated

Effective DateMar 1, 2026

Next Review Date

Key ActionSubmit medical records including baseline motor exam and genetic testing, ensure prescriber is or consults a neurologist experienced in SMA, document prior gene therapy and clinical decline if applicable; do not request inpatient admission solely to administer Spinraza.

SourceLink

POLICY UPDATE CHANGES

Revised coverage criteria; added criterion requiring the provider does not request a planned inpatient admission for the sole purpose of administering Spinraza

Updated Background, Clinical Evidence, and References sections to reflect most current information

2Dose authorization limits (loading / 12-month maintenance)

3Required baseline/response assessment options (HINE-2, HFMSE, RULM, CHOP INTEND, prescriber assessment)

1State applicability (Pennsylvania only)

Coverage summary

Coverage stance: covered_with_criteria. Scope: UnitedHealthcare Community Plan Medical Benefit Drug Policy for Spinraza (nusinersen) applicable only to Pennsylvania. Subject: Spinraza (nusinersen) coverage for spinal muscular atrophy. Policy number: CSPA2026D0059O. Effective date / last review: March 1, 2026.

Authorizations are limited per thresholds: No more than 4 loading doses for initial authorization. No more than 3 maintenance doses (12 months) for reauthorization.

Initial therapy — medically necessary criteria

Spinraza is proven and medically necessary for treatment of SMA when ALL of the following are met:

ALL of the following

Diagnosis of spinal muscular atrophy by, or in consultation with, a neurologist with expertise in the diagnosis of SMA
Submission of genetic testing confirming 5q SMN1 mutation/deletion
- Homozygous gene deletion or mutation (e.g., homozygous deletion of exon 7 at locus 5q13)
- Compound heterozygous mutation (e.g., deletion of SMN1 exon 7 (allele 1) and mutation of SMN1 (allele 2))
Gene replacement therapy history
- No prior gene replacement therapy: Patient has not previously received gene replacement therapy for the treatment of SMA (e.g., Zolgensma (onasemnogene abeparvovec-xioi))
- If previously received gene replacement therapy
  - Patient recently received gene replacement therapy within the previous 6 months
  - Patient has experienced a declination in clinical status since receipt of gene replacement therapy
Concomitant therapy restriction: Patient is not receiving concomitant chronic SMN-modifying therapy (e.g., Evrysdi (risdiplam))
Prescriber expertise: Spinraza is prescribed by, or in consultation with, a neurologist with expertise in the treatment of SMA
Administration: Spinraza is to be administered intrathecally by, or under the direction of, healthcare professionals experienced in performing lumbar punctures
Dosing compliance: Spinraza dosing for SMA is in accordance with the United States Food and Drug Administration approved labeling
Inpatient admission limitation: Provider does not request a planned inpatient admission for the sole purpose of administering Spinraza
Newly added criterion per 03/01/2026 revision
Loading dose limit: Initial authorization will be for no more than 4 loading dosesNo more than 4 loading doses
Baseline assessments
- Hammersmith Infant Neurological Exam Part 2 (HINE-2) (infant to early childhood)
- Hammersmith Functional Motor Scale Expanded (HFMSE)
- Revised Upper Limb Module (RULM) Test
- Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

Continuation therapy — medically necessary criteria

Reauthorization is approved when ALL of the following are met:

ALL of the following

Diagnosis of spinal muscular atrophy by, or in consultation with, a neurologist with expertise in the diagnosis of SMA
Prior Spinraza: Patient has previously received Spinraza therapy
Gene replacement therapy history
- No prior gene replacement therapy: Patient has not previously received gene replacement therapy for the treatment of SMA (e.g., Zolgensma (onasemnogene abeparvovec-xioi))

Not proven / not medically necessary

ANY of the following

Exclusion: non-5q SMA: Spinal muscular atrophy without chromosome 5q mutations or deletions
Exclusion: routine post-gene therapy treatment: Routine concomitant treatment of SMA in patients who have previously received gene replacement therapy (e.g., Zolgensma)

Applicable codes

Drug HCPCSHCPCS

J2326

Injection, nusinersen, 0.1 mg

Relevant ICD-10 diagnosis codes (for reference)ICD-10

G12.0	Infantile spinal muscular atrophy, type I [Werdnig-Hoffmann]
G12.1	Other inherited spinal muscular atrophy
G12.9	Spinal muscular atrophy, unspecified

Provider actions & operational requirements

Prior Authorization

Prior authorization required with documentation

Prior authorization is required. Submit medical records including baseline exam and genetic testing confirming 5q SMN1 mutation/deletion. Prescriber must be or consult a neurologist experienced in SMA. Required codes: J2326 (Injection, nusinersen, 0.1 mg) and diagnosis codes G12.0, G12.1, G12.9. Required documentation: baseline exam (HINE-2, HFMSE, RULM, or CHOP INTEND as appropriate), genetic testing confirming SMN1 mutation/deletion, and evidence of neurologist involvement; document prior gene therapy and clinical decline if applicable.

HCPCS: J2326
ICD-10: G12.0, G12.1, G12.9
Baseline exam: HINE-2, HFMSE, RULM, or CHOP INTEND (as appropriate)
Genetic testing confirming 5q SMN1 mutation/deletion
Prescriber neurologist involvement; document prior gene therapy and clinical decline if applicable

Clinical evidence highlights

51% vs 0%ENDEAR primary motor milestone responder rate: 51% nusinersen vs 0% sham (interim analysis)

47% / 63%ENDEAR survival benefit: 47% reduction in risk of death or permanent ventilation; 63% reduction in risk of death

~3.9 ptsCHERISH HFMSE change: Mean improvement ~3.9 points at 15 months vs decrease in control

Clinical trials listed include ENDEAR, CHERISH, NURTURE and early/other studies (CS1, CS2, CS3, CS10, CS12).

SMN2 copy number correlates with clinical phenotype and is used to inform urgency of treatment in presymptomatic infants; patients with fewer SMN2 copies generally have more severe disease and require more urgent treatment.

Background & definitions

Spinraza (nusinersen) is a modified antisense oligonucleotide directed at SMN2 that increases exon 7 inclusion in SMN2 mRNA and production of full-length SMN protein; it is FDA-approved for treatment of SMA in pediatric and adult patients caused by 5q SMN1 mutations/deletions.

Clinical trials demonstrated benefit across SMA populations: improvements in motor milestones and a higher motor milestone responder rate in infantile-onset disease (ENDEAR), improved event-free and overall survival in infants, and clinically meaningful HFMSE score improvements in later-onset SMA (CHERISH and supporting CS-series studies).

Term	Definition
Gene replacement therapy	Therapy such as onasemnogene abeparvovec-xioi (Zolgensma) used to deliver a functional copy of SMN1.
Loading doses / maintenance doses	Spinraza FDA-labeled dosing regimen includes multiple loading doses followed by maintenance dosing; policy limits initial authorization to ≤ 4 loading doses and reauthorization to ≤ 3 maintenance doses (12 months).

Revision history

03/01/2026revisedLatest

Revised coverage criteria; added criterion requiring the provider does not request a planned inpatient admission for the sole purpose of administering Spinraza. Initial authorization limited to no more than 4 loading doses; reauthorization limited to no more than 3 maintenance doses (12 months).

03/01/2026clarified

Updated Background, Clinical Evidence, and References sections to reflect most current information.

policy_history_note

Archived prior policy version CSPA2025D0059N.

Policy Summary

PayerUnitedHealthcare

PolicySpinraza® (Nusinersen) (for Pennsylvania Only) – Community Plan Medical Benefit Drug Policy

Policy CodePolicy CSPA2026D0059O

Change TypeRevised coverage criteria; supporting information updated

Effective DateMar 1, 2026

Next Review Date

SourceLink

On This Page

If previously received gene replacement therapy

Patient has previously received gene replacement therapy
Patient has experienced a declination in clinical status that represented a potential failure or abatement of gene therapy efficacy

Concomitant therapy restriction: Patient is not receiving concomitant chronic SMN-modifying therapy (e.g., Evrysdi (risdiplam))

Demonstration of positive clinical response

HINE-2 milestones: One of the following: improvement or maintenance of previous improvement in ability to kick; improvement or maintenance of previous improvement in any other HINE-2 milestone (excluding voluntary grasp); exhibited improvement or maintenance of previous improvement in more HINE motor milestones than worsening (net positive improvement); achieved and maintained any new motor milestones when they would otherwise be unexpected to do so; continued clinical benefit based on the prescriber's assessment
HFMSE: One of the following: improvement or maintenance of previous improvement in score from pretreatment baseline; achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so; continued clinical benefit based on the prescriber's assessment
RULM: One of the following: improvement or maintenance of previous improvement in score from pretreatment baseline; achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so; continued clinical benefit based on the prescriber's assessment
CHOP INTEND: One of the following: improvement or maintenance of previous improvement in score from pretreatment baseline; achieved and maintained any new motor milestone from pretreatment baseline when they would otherwise be unexpected to do so; continued clinical benefit based on the prescriber's assessment
Alternative: gene therapy declination: Both of the following: Patient was prescribed Spinraza due to clinical declination after receipt of gene therapy; Continued clinical benefit based on the prescriber's assessment

Prescriber expertise: Spinraza is prescribed by, or in consultation with, a neurologist with expertise in the treatment of SMA

Administration: Spinraza is to be administered intrathecally by, or under the direction of, healthcare professionals experienced in performing lumbar punctures

Dosing compliance: Spinraza dosing for SMA is in accordance with the United States Food and Drug Administration approved labeling

Inpatient admission limitation: Provider does not request a planned inpatient admission for the sole purpose of administering Spinraza

Newly added criterion per 03/01/2026 revision

Reauthorization maintenance dose limit: Reauthorization will be for no more than 3 maintenance doses (12 months)No more than 3 maintenance doses (12 months)

Documentation Required

Baseline and follow-up motor assessments

Provide baseline motor assessments (unless patient is <2 months of age) and for reauthorization provide recent results demonstrating a positive clinical response from pretreatment baseline on one of the accepted assessments or the prescriber's assessment.

Acceptable assessments: HINE-2, HFMSE, RULM, CHOP INTEND
Baseline assessments are not necessary for patients <2 months; initial assessments shortly post-therapy may serve as baseline
Reauthorization requires documentation of positive response from pretreatment baseline on one of the listed assessments or prescriber's assessment

Billing Rule

Administration setting limitation

Spinraza must be administered intrathecally by, or under the direction of, healthcare professionals experienced in performing lumbar punctures. Do not request a planned inpatient admission for the sole purpose of administering Spinraza.

Intrathecal administration by clinicians experienced with lumbar puncture
Planned inpatient admission solely for administration is prohibited

Documentation Required

Concomitant therapy restriction

Document that the patient is not receiving concomitant chronic SMN‑modifying therapy when requesting coverage for Spinraza.

Confirm and document patient is not on chronic SMN‑modifying therapy (e.g., risdiplam/Evrysdi) at time of request

Operational limits / thresholds

Initial authorization loading dose limitNo more than 4 loading doses

Reauthorization maintenance dose limitNo more than 3 maintenance doses (12 months)

State applicabilityPennsylvania only