Premera Bluecross ALS Medications Policy Update

Coverage Criteria

Edaravone (generic IV) and Radicava ORS medical necessity

Generic edaravone IV and Radicava ORS (edaravone) may be considered medically necessary when ALL of the following are met:

Edaravone eligible criteria: 1) Diagnosis of amyotrophic lateral sclerosis (ALS) based on: presence of lower motor neuron (LMN) degeneration by clinical, electrophysiological, or neuropathologic examination AND/OR evidence of upper motor neuron (UMN) degeneration by clinical examination AND progressive spread of symptoms or signs within a region or to other regions; AND absence of electrophysiological/pathological or neuroimaging evidence of other disease processes that might explain the findings; 2) Normal respiratory function retained with forced vital capacity (FVC) at least 70% of predicted; 3) Disease duration of 2 years or less; 4) Medication prescribed by or in consultation with a neurologist or ALS specialist.FVC >= 70% predicted

From policy for generic edaravone IV and Radicava ORS.

Qalsody (tofersen) medical necessity

Qalsody (tofersen) intrathecal may be considered medically necessary when ALL of the following are met:

Qalsody initial criteria: 1) Individual is aged 18 years or older; 2) Diagnosis of ALS per policy diagnostic criteria (evidence of LMN and/or UMN degeneration, progressive spread, and exclusion of alternative explanations); 3) Confirmed mutation in the superoxide dismutase 1 (SOD1) gene; 4) Functionality retained for most activities of daily living defined as ALSFRS-R score of 37 or greater; 5) Respiratory function meets slow vital capacity (SVC) of 65% or greater OR forced vital capacity (FVC) of 50% or greater of predicted; 6) Medication prescribed by or in consultation with a neurologist or ALS specialist.SVC >=65% OR FVC >=50%

Disease duration requirement of ≤2 years was removed per 2025 update.

Teglutik/Tiglutik (riluzole) medical necessity

Teglutik (riluzole) oral suspension may be considered medically necessary when ALL of the following are met:

Teglutik criteria: 1) Individual is aged 18 years or older; 2) Has tried generic riluzole tablets first and had inadequate response or intolerance OR documentation that oral suspension formulation is clinically necessary (e.g., difficulty swallowing); 3) Medication prescribed by or in consultation with a neurologist or ALS specialist.see node text

Step requirement for Teglutik: prior trial of generic riluzole tablets required unless clinical necessity for suspension is documented.

Qalsody (tofersen) Initial Therapy

Covered when ALL of the following are met (policy updated):

Qalsody initial therapy: 1) Patient meets the FDA indication for Qalsody (tofersen) for ALS with confirmed SOD1 gene mutation and age requirement per label (≥18 years); 2) Functional status retained for most activities of daily living (ALSFRS-R per label/context); 3) Respiratory function meets SVC ≥65% OR FVC ≥50% of predicted; 4) Other label criteria as applicable; 5) Medication prescribed by or in consultation with a neurologist or ALS specialist.SVC >=65% OR FVC >=50%

2025 updates removed the disease-duration ≤2 years requirement and updated respiratory threshold to include FVC ≥50%.

Edaravone (Radicava / generic) Initial Therapy

Covered when meeting drug-specific FDA prescribing information and policy criteria:

Edaravone initial therapy: 1) Coverage follows Radicava (edaravone) IV and Radicava ORS prescribing information and policy criteria; 2) Generic edaravone IV is subject to the same criteria as Radicava; 3) Functional and respiratory thresholds per product prescribing information and policy (e.g., ALSFRS-R trial-derived thresholds context and percent-predicted FVC requirement reflected in policy); 4) Medication prescribed by or in consultation with a neurologist or ALS specialist.See product PI and policy (e.g., FVC thresholds)

Radicava IV later removed by manufacturer; generic edaravone IV remains available.

Riluzole Products

Covered when following prescribing information and policy criteria:

Riluzole products: Policy includes riluzole formulations such as Teglutik (oral suspension) and previously included branded formulations (e.g., Exservan) which have been removed if discontinued; coverage follows each product's prescribing information; for Teglutik, prior trial of generic riluzole tablets is required unless clinical necessity for suspension is documented.Follow product prescribing information

Exservan was removed after manufacturer withdrawal; Teglutik coverage criteria added in 2024 update.

All uses of the medications described in this policy that are not specifically listed as meeting the coverage criteria are considered investigational. The medications covered by this policy are subject to the product's FDA dosage and administration prescribing information and authorization lengths described in the policy.

Products that have been withdrawn or discontinued by the manufacturer are removed from the policy and are not covered as branded products when they are no longer marketed. Examples noted in the policy history include Relyvrio (sodium phenylbutyrate and taurursodiol) and other manufacturer-discontinued formulations; the policy also documents removals and clarifications in subsequent updates.

When a branded product is discontinued, generic alternatives may remain available and covered if included in the policy (for example, generic edaravone IV was added while Radicava IV was later removed after discontinuation).

Requests for use of any medication listed in this policy that do not meet the specified diagnostic, functional, respiratory, or other drug-specific criteria are considered investigational / not medically necessary. This includes any unlisted indications or uses outside the documented coverage criteria.

When a manufacturer discontinues a branded product, that branded formulation is not available for coverage under this policy. The policy history documents these changes (for example, removal of Exservan after the product was discontinued and removal of Radicava IV when the manufacturer discontinued it), and coverage decisions reflect availability at the time of review.

Coding

HCPCS CodesHCPCS

J1301	Injection, edaravone, 1 mg
J1304	Injection, tofersen (Qalsody), 1 mg

HCPCS / historical codingHCPCS

J1301	HCPCS code previously added (referenced in history)
J1304	HCPCS code added 01/01/24
J3490	Previously used code for reporting Qalsody; J3490 added back per history
C9157	HCPCS code added 10/01/23 (later termed)

Respiratory function thresholds — edaravone and Qalsody thresholds summarized

Edaravone (Radicava/generic) respiratory thresholdForced vital capacity (FVC) is at least 70% of predicted

Qalsody (tofersen) respiratory thresholdSlow vital capacity (SVC) of 65% or greater OR FVC of 50% or greater of predicted

Source policy effective changesGeneric edaravone IV added with same criteria as Radicava; Radicava IV later discontinued (generic remains)

Respiratory function — updated Qalsody respiratory criterion (SVC ≥65% or FVC ≥50%)

Qalsody updated respiratory criterionSlow vital capacity (SVC) of 65% or greater OR forced vital capacity (FVC) of 50% or greater of predicted

Related functional requirement

Provider Actions and Requirements

Prior Authorization

Prior Authorization and Benefit Routing

Prior authorization is required for all medications listed in this policy. Route of benefit: Qalsody (tofersen) and generic edaravone IV are managed under the medical benefit; Radicava ORS (edaravone), Teglutik (riluzole), and Tiglutik (riluzole) are managed under the pharmacy benefit. Confirm appropriate benefit routing and submit prior authorization requests per payer procedures.

Prior authorization required for all drugs in policy
Medical benefit: Qalsody (tofersen), generic edaravone IV
Pharmacy benefit: Radicava ORS (edaravone), Teglutik, Tiglutik

Billing Rule

Prior Authorization and Coding

Prior authorization approvals are contingent on meeting the policy’s clinical criteria. Coding changes and product availability may affect billing — verify current HCPCS/NDC/CPT coding at time of submission and note that Radicava (edaravone) IV and Exservan (riluzole) have been removed/withdrawn from the market; generic edaravone IV was added.

Background

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting upper and lower motor neurons and leading to progressive muscle weakness and paralysis. This policy addresses coverage for disease-modifying and supportive pharmacologic therapies for ALS and ties coverage to specific diagnostic confirmation, functional status, and respiratory thresholds referenced in clinical trial inclusion criteria and product labeling.

Coverage determinations in this policy require documentation of ALS diagnosis per the policy diagnostic criteria (evidence of lower motor neuron degeneration by clinical/electrophysiologic/neuropathologic examination and/or upper motor neuron degeneration by clinical exam, progressive spread of symptoms, and exclusion of alternative explanations) and adherence to drug-specific eligibility requirements such as age, respiratory function, and functional status.

Policy history notes reflect changes in available products and criteria over time (for example, addition of Teglutik coverage criteria in 2024 and updates in 2025 to respiratory thresholds and product availability), and clinicians should follow the product FDA prescribing information and the documentation requirements when requesting authorization.

Definitions

ALS diagnostic criteria — LMN/UMN evidence and diagnostic standards referenced

Required diagnostic evidenceEvidence of lower motor neuron (LMN) degeneration by clinical, electrophysiological, or neuropathologic examination and/or evidence of upper motor neuron (UMN) degeneration by clinical examination

Progression requirementProgressive spread of symptoms or signs within a region or to other regions as determined by history or examination

Exclusion of alternativesAbsence of electrophysiological/pathological or neuroimaging evidence of other disease processes that might explain LMN/UMN signs

ALSFRS-R — instrument description and scoring

Instrument descriptionALS Functional Rating Scale–Revised (ALSFRS-R) is a 12-item instrument evaluating fine motor, gross motor, bulbar, and respiratory function with each item scored 0–4 (higher scores = greater function)

Scoring use in policy

Initial Therapy Criteria

Initial therapy context

Clinical trial-derived inclusion characteristics align with initial therapy criteria used for edaravone and tofersen:

Initial therapy context (edaravone): Edaravone trial inclusion: functionality retaining most activities of daily living (scores ≥2 on each ALSFRS-R item), percent-predicted FVC ≥80% in the pivotal trial, definite or probable ALS per El Escorial criteria, and disease duration ≤2 years; these characteristics provide context for initial therapy eligibility in policy though policy FVC threshold for edaravone is set at ≥70% predicted.Trial: FVC >=80% (context); Policy: FVC >=70%

Use clinical-trial inclusion criteria to align expectations for initial therapy documentation.

Initial therapy context (tofersen): Tofersen pivotal trial inclusion included SVC >65% predicted and disease duration ≤2 years; policy-aligned initial therapy for Qalsody now requires SVC ≥65% or FVC ≥50% and removed the ≤2 year disease-duration restriction.SVC >=65% or FVC >=50%

Trial context informs policy thresholds and ALSFRS-R functional requirements.

Continuation / Reauthorization Criteria

Re-authorization

Re-authorization may be approved when chart notes demonstrate continued positive clinical response:

Re-authorization condition: Chart notes must demonstrate the individual continues to show a positive clinical response, documented by the ability to perform most activities of daily living.see node text

Length of re-authorization approvals vary by drug; non-formulary exception reviews may be approved up to 12 months; other reviews commonly up to 6–12 months based on drug.

Continuation / Reauthorization

Continuation and reauthorization follow the original product criteria:

Continuation / Reauthorization: Continuation authorizations follow initial coverage criteria and product prescribing information; non-formulary exception requests may be approved for up to 12 months.Up to 12 months for non-formulary exceptions

Step Therapy

Step	Requirement	Notes
1
Prior trial of generic riluzole tablets is required before Teglutik (riluzole) oral suspension.
Unless documentation is provided that the suspension formulation is clinically necessary (e.g., difficulty swallowing) per policy.

Step	Requirement	Notes
1
No specific step therapy sequence is documented for other products in the policy.
Policy history and updates add or remove product coverage (e.g., Exservan removed after manufacturer discontinuation; generic alternatives may remain).

Quantity Limits

All listed ALS drugs — quantity limits summary (inventory blank summary)

Inventory — all listed ALS drugsPolicy lists multiple ALS drugs including generic edaravone IV; Radicava ORS (edaravone); Qalsody (tofersen); Teglutik/Tiglutik (riluzole); Exservan and Relyvrio noted as removed/withdrawn

Quantity limits summaryNo specific per-drug quantity limits documented in policy; quantity limits section lists 'All listed ALS drugs' without numeric limits

Authorization duration noteNon-formulary exception authorizations for listed drugs may be approved up to 12 months

Site of Care

Billing Rule

Benefit routing and typical administration settings by product

Site of care and benefit routing differ by product: Qalsody (tofersen) and generic edaravone IV are managed through the medical benefit and are typically administered in a medical setting (infusion center or home infusion as appropriate). Radicava ORS (oral suspension) and riluzole formulations (Teglutik/Tiglutik) are managed through the pharmacy benefit for outpatient oral administration.

Qalsody and generic edaravone: medical benefit — typically infusion center or medically supervised setting.
Radicava ORS and riluzole suspensions/tablets: pharmacy benefit — oral formulations dispensed via pharmacy.

Billing Rule

Administer IV or intrathecal products per FDA prescribing information at appropriate site of care

Medications subject to FDA dosing and administration (e.g., IV edaravone) should be administered at the site of care indicated in the product prescribing information; infusion-center administration is appropriate for IV products.

Follow prescribing information for site-of-care directions for IV edaravone and intrathecal Qalsody.
Document site-of-care decisions in the medical record consistent with the product PI.

OpenPayer

Amyotrophic Lateral Sclerosis (ALS) Medications

Coverage Criteria

Coding

Provider Actions and Requirements

Background

Definitions

Initial Therapy Criteria

Continuation / Reauthorization Criteria

Step Therapy

Quantity Limits

Site of Care