CurrentcignaPolicy IP0063

Spinal Muscular Atrophy - Evrysdi

Defines Cigna prior authorization, coverage criteria, duration of approval, documentation requirements, and exclusions for Evrysdi (risdiplam) for treatment of spinal muscular atrophy (SMA) in pediatric patients and adults.

Policy Summary

Payercigna

PolicySpinal Muscular Atrophy - Evrysdi

Policy CodePolicy IP0063

Change TypeRevisions (documentation, durations, exclusions)

Effective DateMar 15, 2026

Next Review Date

Key ActionPrior Authorization is required; provide required documentation (motor assessment, genetic testing showing bi-allelic pathogenic SMN1 variants, SMN2 copy number, and verification of no prior onasemnogene therapies).

SourceLink

POLICY UPDATE CHANGES

Policy title updated from 'Risdiplam' to 'Spinal Muscular Atrophy - Evrysdi'; Evrysdi oral tablets added.

For patients currently receiving Evrysdi, approval duration updated to 1 year (from 4 months); criteria for continuation updated.

Annual revision 12/15/2024: No criteria changes (administrative).

2Approval durations (months/years)

3SMN2 copy scenarios explicitly allowed

3Conditions listed as not covered

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Coverage Summary

Scope: Defines Cigna prior authorization, coverage criteria, duration of approval, documentation requirements, and exclusions for Evrysdi (risdiplam) for treatment of spinal muscular atrophy (SMA) in pediatric patients and adults. Payer: cigna; Policy: Spinal Muscular Atrophy - Evrysdi; Policy number: IP0063.

Coverage stance: covered_with_criteria per policy requirements and prior authorization procedures.

FDA indication summary: Evrysdi (risdiplam) is an SMN2 splicing modifier indicated for the treatment of spinal muscular atrophy in pediatric patients and adults.

Effective date: 2026-03-15. Last review: 2025-08-15.

Initial Therapy Criteria

A) Initial Therapy

Approval duration: Approve for 4 months
Approval duration = 4 months (1 month = 30 days)
Baseline motor ability assessment
- Bayley Scales of Infant and Toddler Development
- CHOP-INTEND: Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND)
- HFMSE: Hammersmith Functional Motor Scale Expanded (HFMSE)
- HINE-2: Hammersmith Infant Neurological Exam Part 2 (HINE-2)
- MFM-32: Motor Function Measure-32 Items (MFM-32)
- RULM:
Genetic confirmation: Genetic test confirming the diagnosis of spinal muscular atrophy with bi-allelic pathogenic variants in the survival motor neuron 1 (SMN1) gene
[documentation required]; Note: Pathogenic variants may include homozygous deletion, compound heterozygous mutation, or a variety of other rare mutations.
SMN2 copy criteria
- Two or three SMN2 copies: Patient has two or three survival motor neuron 2 (SMN2) gene copies
  [documentation required]
- Four SMN2 copies with objective signs: Patient has four survival motor neuron 2 (SMN2) gene copies AND patient has objective signs consistent with spinal muscular atrophy Types 1, 2, or 3
  [documentation required]
Prior gene therapy: Patient has not received Zolgensma (onasemnogene abeparvovec-xioi) or Itvisma (onasemnogene abeparvovec-brve) in the past
[verification in claims history required] Note: If no claim for Zolgensma or Itvisma is present (or if claims history is not available), the prescribing physician confirms that the patient has not previously received Zolgensma or Itvisma.
Specialist prescribing requirement: Medication is prescribed by a physician who has consulted with a specialist or who specializes in the management of patients with spinal muscular atrophy and/or neuromuscular disorders

Continuation Therapy Criteria

B) Patient Currently Receiving Evrysdi

Approval duration: Approve for 1 year
Approval duration = 1 year
Genetic confirmation: Genetic test confirming the diagnosis of spinal muscular atrophy with bi-allelic pathogenic variants in the survival motor neuron 1 (SMN1) gene
[documentation required]; Note: Pathogenic variants may include homozygous deletion, compound heterozygous mutation, or a variety of other rare mutations.
SMN2 copy criteria (current therapy)
- Two or three SMN2 copies: Patient has two or three survival motor neuron 2 (SMN2) gene copies

Unproven Uses and Conditions Not Covered

Applicable Codes

ProductNDCCovered

No codes listed

Applicable diagnosis codesICD-10Covered

No codes listed

Administration/infusion codesCPT/HCPCS

No codes listed

Provider Actions & Authorization Requirements

Prior Authorization

Prior authorization required

Prior Authorization is required for benefit coverage of Evrysdi. Approvals are provided for specified durations.

Initial approval duration: 4 months
Continuation (patient currently receiving Evrysdi) approval duration: 1 year

Documentation Required

Documentation of diagnostic and assessment testing

Provide required documentation to support coverage requests for Evrysdi, including baseline motor ability assessment, genetic testing, SMN2 copy documentation, and verification regarding prior gene therapies.

Baseline motor ability assessment from one of: Bayley Scales of Infant and Toddler Development; CHOP-INTEND; HFMSE; HINE-2; MFM-32; RULM; World Health Organization motor milestone scale

Clinical Evidence Summary

FIREFISHType 1 improvements (sitting, survival w/o permanent ventilation)

SUNFISH (Part 2)Month 12 motor & upper limb benefits vs placebo

RAINBOWFISHPresymptomatic: high proportion sitting unsupported at 12 months

FIREFISH: Phase studies in infants with Type 1 SMA (symptom onset 28 days to 3 months) required genetic confirmation of SMN1 loss-of-function and enrolled patients with two SMN2 copies; showed improvements in ability to sit and increased survival without permanent ventilation (infantile population).

SUNFISH (Part 2): Phase 3, double-blind, randomized, placebo-controlled trial in later-onset SMA (Type 2 and Type 3) where most patients had three SMN2 copies; demonstrated benefits at Month 12 in motor function and upper limb motor performance (later-onset population).

RAINBOWFISH: Study in presymptomatic infants (up to 6 weeks at first dose) with varying SMN2 copy numbers; high proportions achieved ability to sit without support at 12 months (presymptomatic population).

Background & Definitions

Evrysdi (risdiplam) is an SMN2 splicing modifier indicated for treatment of spinal muscular atrophy in pediatric patients and adults. The disease is an autosomal recessive disorder caused by deletion or loss-of-function mutations in the SMN1 gene, and SMN2 copy number modifies phenotype.

Mechanism: Risdiplam modifies SMN2 splicing to increase production of functional SMN protein (SMN2 splicing modifier).

Indication details: Clinical trials (FIREFISH, SUNFISH, RAINBOWFISH) support efficacy across infantile-onset (Type 1), later-onset (Types 2 and 3), and presymptomatic populations; genetic confirmation of bi-allelic pathogenic variants in SMN1 is required for diagnosis documentation.

Disease description: Loss of SMN protein from SMN1 leads to degeneration of lower motor neurons; higher SMN2 copy number generally correlates with milder phenotypic expression.

Term	Definition
Bi-allelic pathogenic variants	Pathogenic variants including homozygous deletion, compound heterozygous mutation, or other rare mutations in SMN1 predictive of loss of function.

Revision History

2026-03-15revisedLatest

Selected revision: Itvisma added to prior gene therapy exclusions with note that if no claim is present (or claims history not available) the prescribing physician must confirm the patient has not previously received Itvisma; examples of pathogenic variants note updated. Approval duration for patients currently receiving Evrysdi updated to 1 year (from 4 months). (Selected Revision date 03/15/2026).

2026-03-15revisedLatest

Policy title updated from 'Risdiplam' to 'Spinal Muscular Atrophy - Evrysdi'; Evrysdi oral tablets added. Updated documentation requirements; initial authorization duration changed from 6 months to 4 months; removed 6-Minute Walk Test; genetic language changed to 'bi-allelic pathogenic variants'; added 'patient has not received Zolgensma' criterion; removed several prior restrictions (e.g., contraception, hepatic impairment); removed follow-on Evrysdi criteria for those previously treated with Zolgensma. (Material change).

Policy Summary

Payercigna

PolicySpinal Muscular Atrophy - Evrysdi

Policy CodePolicy IP0063

Change TypeRevisions (documentation, durations, exclusions)

Effective DateMar 15, 2026

Next Review Date

SourceLink

On This Page

Revised Upper Limb Module (RULM) test

WHO motor milestone scale: World Health Organization motor milestone scale

Four SMN2 copies with objective signs: Patient has four survival motor neuron 2 (SMN2) gene copies AND patient has objective signs consistent with spinal muscular atrophy Types 1, 2, or 3

[documentation required]

Specialist prescribing requirement: Medication is prescribed by a physician who has consulted with a specialist or who specializes in the management of patients with spinal muscular atrophy and/or neuromuscular disorders

[documentation required]

Clinical response requirement

Positive clinical response on listed exams: Patient has had a positive clinical response (improvement or stabilization) from pretreatment baseline status within the past 4 months on ONE of the following exams: Bayley Scales of Infant and Toddler Development; Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND); Hammersmith Functional Motor Scale Expanded (HFMSE); Hammersmith Infant Neurological Exam Part 2 (HINE-2); Motor Function Measure-32 Items (MFM-32); Revised Upper Limb Module (RULM) test; OR World Health Organization motor milestone scale
[documentation required]
Physician-assessed response: According to the prescribing physician, the patient has responded to recent (i.e., within the past 4 months) physician monitoring/assessment tools
[documentation required]

Genetic test confirming diagnosis of SMA with bi-allelic pathogenic variants in SMN1 (pathogenic variants may include homozygous deletion, compound heterozygous mutation, or other rare mutations)

Documentation of SMN2 copy number (two or three copies; or four copies with objective signs consistent with SMA Types 1–3)

Verification that the patient has not received onasemnogene gene therapies (Zolgensma or Itvisma) when required

Documentation Required

Verification of prior gene therapy

Verify claims history for prior receipt of onasemnogene gene therapies (Zolgensma or Itvisma). If no claim is present or claims history is unavailable, the prescribing physician must confirm the patient has not previously received Zolgensma or Itvisma.

Check claims for Zolgensma (onasemnogene abeparvovec-xioi) or Itvisma (onasemnogene abeparvovec-brve)
If claims are unavailable or no claim found, obtain prescribing physician confirmation that patient has not received these gene therapies

Documentation Required

Specialist prescribing requirement

Evrysdi must be prescribed by a physician who has consulted with or who specializes in the management of patients with spinal muscular atrophy and/or neuromuscular disorders. Approval requires this prescribing condition.

Denial Risk

Denial if not accompanied by covered codes/documentation

Claims submitted without covered diagnosis or procedure codes under this policy will be denied as not covered. Documentation submitted must include patient-specific identifying information.

Claims lacking covered codes will be denied
Documentation must include patient-specific identifying information (e.g., chart notes, lab results, claims records, prescription receipts)

2024-12-15revised

Annual administrative revision: No criteria changes.