CurrentBlue Cross Blue Shield - MassachusettsPolicy 044

Spinal muscular atrophy medications (Evrysdi/Spinraza)

Defines prior authorization clinical criteria, dosing limits, reauthorization requirements, and billing/coding guidance for Evrysdi (risdiplam) and Spinraza (nusinersen) for treatment of spinal muscular atrophy (SMA) for BCBSMA commercial members. Applies to outpatient pharmacy (Rx) and medical (intrathecal nusinersen) benefits as specified.

Policy Summary

PayerBlue Cross Blue Shield - Massachusetts

PolicySpinal muscular atrophy medications (Evrysdi/Spinraza)

Policy CodePolicy 044

Change TypeReformatted; prior addition of Evrysdi

Effective DateJul 1, 2023

Next Review Date

Key ActionSubmit prior authorization requests with genetic test results and specialist prescriber information via BCBSMA Pharmacy Operations by phone (1-800-366-7778) or fax (1-800-583-6289).

POLICY UPDATE CHANGES

Policy last updated 7/1/2023 (reformatted policy).

Evrysdi (risdiplam) was added to policy in a prior update.

2SMA medications addressed

4ICD-10 diagnosis codes listed

1Authorization duration (years)

Coverage Summary

Scope: This policy (Policy Number 044, effective 2023-07-01) defines prior authorization clinical criteria, dosing limits, reauthorization requirements, and billing/coding guidance for Evrysdi (risdiplam) and Spinraza (nusinersen) for treatment of spinal muscular atrophy (SMA) for BCBSMA commercial members; it applies to outpatient pharmacy (Rx) and medical benefits (intrathecal nusinersen) as specified.

Quick facts & thresholds

Authorization durationUp to 1 year per approval; reauthorization requires documentation of clinically meaningful improvement in motor milestones

Evrysdi age-based dosing (policy text)<2 months: 0.15 mg/kg daily oral; 2 months to <2 years: 0.2 mg/kg daily oral; 2 years and older: policy text incomplete for exact dose

Spinraza dosing schedule12 mg intrathecal: 4 loading doses (first 3 at 14-day intervals; 4th loading dose 30 days after 3rd); maintenance dose every 4 months thereafter

Number of SMA medications addressed2 (Evrysdi and Spinraza)

ICD-10 diagnosis codes listed4 codes (G12.0, G12.1, G12.8, G12.9)

Evrysdi (risdiplam) - Initial Therapy Criteria

Evrysdi (risdiplam) - Initial Authorization

Covered when ALL of the following criteria are met:

ALL of the following

Diagnosis of spinal muscular atrophy confirmed by genetic testing demonstrating bi-allelic mutations in the survival motor neuron 1 (SMN1) gene
Deletion of both copies of SMN1 or pathogenic variants in both copies of SMN1 confirm SMA diagnosis (Bi-allelic SMN1 mutations).
SMN2 copy documentation
- If patient is symptomatic, documentation of a genetic test confirms 2, 3 or 4 copies of the SMN2 gene
- If patient is asymptomatic, documentation of a genetic test confirms minimum of 2 but less than 4 copies of the SMN2 gene
Either deletion of both copies of the SMN1 gene OR identification of pathogenic variant(s) in both copies of the SMN1 gene
The prescription is written by a board certified or board eligible Neurologist
Dose is limited to FDA approved dosing
Policy lists age-based dosing: <2 months: 0.15 mg/kg daily oral dosing; 2 months to <2 years: 0.2 mg/kg daily oral dosing; 2 years and older: per FDA approved dosing.
Patient is not on permanent ventilator dependence
Patient is not receiving concurrent treatment with Spinraza (nusinersen) or Zolgensma (onasemnogene abeparvovec)

Evrysdi (risdiplam) - Reauthorization Criteria

Evrysdi (risdiplam) - Reauthorization

Reauthorization requires ALL of the following:

ALL of the following

Same genetic and eligibility criteria as initial approval are met
Documentation to support clinically meaningful improvement in motor milestones during previous treatment period
Reauthorization will require documentation of clinically meaningful improvement in motor milestones.

Spinraza (nusinersen) - Initial Therapy Criteria

Spinraza (nusinersen) - Initial Authorization

Covered when ALL of the following criteria are met:

ALL of the following

Diagnosis of spinal muscular atrophy confirmed by genetic testing demonstrating bi-allelic mutations in the survival motor neuron 1 (SMN1) gene
Policy describes compound heterozygous mutations, deletion, or pathogenic variants in SMN1 as confirming diagnosis.
SMN2 copy documentation
- If patient is symptomatic, documentation of a genetic test confirms 2, 3 or 4 copies of the SMN2 gene
- If patient is asymptomatic, documentation of a genetic test confirms minimum of 2 but less than 4 copies of the SMN2 gene
Pathogenic variant in one copy and deletion of the second copy of the SMN1 gene
The prescription is written by a board certified or board eligible Neurologist
Patient is not receiving concurrent treatment with Evrysdi (risdiplam) or Zolgensma (onasemnogene abeparvovec)
Dose is limited to FDA approved dosing of 12 mg administered intrathecally per treatment with 4 loading doses; first three loading doses at 14-day intervals, 4th loading dose 30 days after 3rd, maintenance dose every 4 months thereafter

Spinraza (nusinersen) - Reauthorization Criteria

Spinraza (nusinersen) - Reauthorization

Reauthorization requires ALL of the following:

ALL of the following

Same genetic and eligibility criteria as initial approval are met
Documentation to support clinically meaningful improvement in motor milestones during previous treatment period
Reauthorization will require documentation of clinically meaningful improvement in motor milestones.

General Exclusions & Limitations

General Exclusions / Limitations

ALL of the following

We do not cover the medications listed above for other conditions not listed above
Requests based exclusively on the use of samples will not meet coverage criteria; additional clinical information demonstrating medical necessity must be submitted

Applicable Codes

HCPCS codes (nusinersen)HCPCSCovered

J2326

Injection, nusinersen 0 mg

ICD-10 diagnosis codes considered medically necessary when submitted with the HCPCS code aboveICD-10Covered

G12.0	Infantile spinal muscular atrophy_type [Werdnig-Hoffman]
G12.1	Other inherited spinal muscular atrophy
G12.8	Other spinal muscular atrophies and related syndromes
G12.9	Spinal muscular atrophy_unspecified

Provider Actions & Administrative Requirements

Prior Authorization

Prior authorization required

Prior authorization is required for Evrysdi (risdiplam) and Spinraza (nusinersen). Requests may be submitted to BCBSMA Pharmacy Operations by phone (1-800-366-7778) or fax (1-800-583-6289) using the Formulary Exception/Prior Authorization form.

Documentation Required

Genetic testing documentation

Submit genetic testing results demonstrating bi-allelic SMN1 mutations and documentation of SMN2 copy number per symptomatic/asymptomatic criteria: if symptomatic, report 2, 3, or 4 SMN2 copies; if asymptomatic, report a minimum of 2 but less than 4 SMN2 copies. These results must be included as part of the prior authorization.

Documentation Required

Specialist prescriber requirement

The prescription must be written by a board certified or board eligible Neurologist; include the prescriber's board certification/eligibility in the documentation submitted for prior authorization.

Denial Risk

Concurrent therapy exclusion

Claims may be denied if the member is receiving concurrent treatment with another SMA disease-modifying therapy. Do not request coverage if the member is receiving Evrysdi, Spinraza (nusinersen), or Zolgensma (onasemnogene abeparvovec-xioi).

Prior Authorization

Authorization duration

If approved, prior authorization will be granted for up to one year. Reauthorization requires the same eligibility criteria and submission of documentation demonstrating clinically meaningful improvement in motor milestones during the prior treatment period.

Billing Rule

Provider coding guidance

Report services using current industry-standard procedure, revenue, and diagnosis codes, including modifiers where applicable. Note there is no specific CPT code for nusinersen administration; use the applicable HCPCS/diagnosis codes per policy.

Billing Rule

Specialty pharmacy requirement

BCBSMA members (other than certain Medex/Medicare prescription plans) are required to fill prescriptions for these medications at an in-network retail specialty pharmacy; verify member plan limitations and use the BCBSMA retail specialty pharmacy network for fills.

Documentation Required

Samples not acceptable

Requests based exclusively on the use of samples will not meet coverage criteria. Additional clinical information demonstrating medical necessity must be submitted with the prior authorization request.

Background & Definitions

Background: This policy covers FDA‑approved disease‑modifying therapies for SMA (Evrysdi and Spinraza). Coverage is based on genetic confirmation of SMA (bi‑allelic SMN1 mutations with SMN2 copy number documentation per symptomatic/asymptomatic criteria), requires prescriptions to be written by a board‑certified or board‑eligible Neurologist, and mandates evidence of clinical benefit (documentation of clinically meaningful improvement in motor milestones) for reauthorization.

Term	Definition
Bi-allelic SMN1 mutations	Deletion of both copies of SMN1 or pathogenic variants in both copies of SMN1 confirming SMA diagnosis.

Revision History

2019-05-01added

Evrysdi (risdiplam) added to policy (historical action noted in policy history).

2022-08-01revised

Updated to include new age dosing of Evrysdi (policy history notes August 2022 update with new age dosing).

2023-07-01reformattedLatest

Policy reformatted; policy last updated and effective 7/1/2023 (policy displays Policy last updated 7/1/2023 and 'Action = Reformatted Policy').

Policy Summary

PayerBlue Cross Blue Shield - Massachusetts

PolicySpinal muscular atrophy medications (Evrysdi/Spinraza)

Policy CodePolicy 044

Change TypeReformatted; prior addition of Evrysdi

Effective DateJul 1, 2023

Next Review Date

Key ActionSubmit prior authorization requests with genetic test results and specialist prescriber information via BCBSMA Pharmacy Operations by phone (1-800-366-7778) or fax (1-800-583-6289).

On This Page

OpenPayer

Spinal muscular atrophy medications (Evrysdi/Spinraza)

Coverage Summary

Evrysdi (risdiplam) - Initial Therapy Criteria

Evrysdi (risdiplam) - Reauthorization Criteria

Spinraza (nusinersen) - Initial Therapy Criteria

Spinraza (nusinersen) - Reauthorization Criteria

General Exclusions & Limitations

Applicable Codes

Provider Actions & Administrative Requirements

Background & Definitions

Clinical Evidence Notes

Revision History