CurrentCignaPolicy N/A

Spinal Muscular Atrophy - Evrysdi Prior Authorization Policy

Defines Cigna prior authorization criteria, documentation, and coverage limitations for Evrysdi (risdiplam) for treatment of spinal muscular atrophy for pediatric and adult patients; applies to Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicySpinal Muscular Atrophy - Evrysdi Prior Authorization Policy

Policy CodePolicy N/A

Change TypeAdded tablet formulationminor clarification

Effective Date

Next Review DateOct 2, 2024

Key ActionObtain prior authorization and provide required documentation including genetic testing, SMN2 copy number, baseline and follow-up motor assessments, dosing information, and verification of prior therapies to approve Evrysdi for 4 months.

SourceLink

POLICY UPDATE CHANGES

Evrysdi tablets (5 mg) were added to the policy.

Note regarding verification in claims history was revised to account for when claims history is not available.

4 monthsapproval duration

0.15–5 mg/daydosing range

2–4 copiesSMN2 focus

NCapplicable state

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Coverage Criteria for Evrysdi (risdiplam)

Initial Therapy (FDA-Approved Indication)

Approve for 4 months if the patient meets ALL of the following:

Initial Therapy criteria: i. Baseline motor ability assessment has been performed from ONE of the following: Bayley Scales of Infant and Toddler Development; CHOP-INTEND; HFMSE; HINE-2; MFM-32; RULM; OR WHO motor milestone scale [documentation required]; AND ii. Genetic test confirming diagnosis of SMA with bi-allelic pathogenic variants in SMN1 [documentation required]; AND iii. Patient meets ONE of the following: (a) SMN2 = 2 or 3 copies [documentation required]; OR (b) Patient has 4 SMN2 copies [documentation required] AND objective signs consistent with SMA Types 1, 2, or 3 [documentation required]; AND iv. For patients currently receiving or who have received nusinersen (Spinraza), the prescribing physician confirms Spinraza will be discontinued; AND v. Patient has not received Zolgensma (verification in claims history required; if no claims history, physician confirmation that patient has not previously received Zolgensma is acceptable); AND vi. Female patients of reproductive potential: (a) not currently pregnant; AND (b) will use effective contraception during treatment and for 1 month after the last Evrysdi dose; AND vii. Dosing within the past 1 month meets ONE of the following based on current weight: 0.15 mg/kg once daily if <2 months; 0.2 mg/kg once daily if 2 months to <2 years; 0.25 mg/kg once daily if ≥2 years and <20 kg; OR 5 mg once daily if ≥2 years and ≥20 kg; AND viii. Medication is prescribed by a physician who has consulted with or specializes in the management of patients with SMA and/or neuromuscular disorders.

Continuation Therapy (Patients currently receiving Evrysdi)

Approve for 4 months if the patient meets ALL of the following:

Continuation criteria: i. Genetic test confirming diagnosis of SMA with bi-allelic pathogenic variants in SMN1 [documentation required]; AND ii. Patient meets ONE of the following: (a) SMN2 = 2 or 3 copies [documentation required]; OR (b) Patient has 4 SMN2 copies [documentation required] AND objective signs consistent with SMA Types 1, 2, or 3 [documentation required]; AND iii. For patients currently receiving or who have received nusinersen (Spinraza), the prescribing physician confirms Spinraza will be discontinued; AND iv. Patient has not received Zolgensma (verification in claims history required; if no claims history, physician confirmation that patient has not previously received Zolgensma is acceptable); AND v. Female patients of reproductive potential: (a) not currently pregnant; AND (b) will use effective contraception during treatment and for 1 month after the last Evrysdi dose; AND vi. Dosing within the past 1 month meets ONE of the following based on current weight: 0.15 mg/kg once daily if <2 months; 0.2 mg/kg once daily if 2 months to <2 years; 0.25 mg/kg once daily if ≥2 years and <20 kg; OR 5 mg once daily if ≥2 years and ≥20 kg; AND vii. Medication is prescribed by a physician who has consulted with or specializes in the management of patients with SMA and/or neuromuscular disorders; AND viii. Patient must have ONE of the following: (a) a positive clinical response (improvement or stabilization) from pretreatment baseline within the past 4 months on ONE of the following assessments: Bayley, CHOP-INTEND, HFMSE, HINE-2, MFM-32, RULM, OR WHO motor milestone scale [documentation required]; OR (b) the prescribing physician documents the patient continues to benefit from Evrysdi on the most recent assessment within the past 4 months (examples: improved pulmonary function, improved bulbar function, reduced need for respiratory support, fewer respiratory infections or complications, or prevention of permanent assisted ventilation) [documentation required].

Evrysdi (risdiplam) is considered experimental, investigational, or unproven for any uses not specified in this policy. Specific exclusions include patients with complete paralysis of all limbs and patients with permanent ventilator dependence, for whom available data are insufficient to demonstrate clinical benefit.

Coding and Dosing Details

Covered/Drug Codesmixed

NDC/HCPCS not specified

No explicit billing codes provided in document.

Treatment approval duration

Approval durationInitial and continuation approvals are granted for 4 months when criteria are met.

Documentation window for response (continuation)Evidence of clinical response must be documented within the most recent 4 months for reauthorization.

Policy statement locationSpecified under FDA‑Approved Indication — Initial Therapy and Continuation Therapy sections.

Weight-based dosing threshold

Weight threshold for fixed dosing>= 20 kg = fixed 5 mg once daily dosing for patients >= 2 years of age.

Dosing for <20 kg and >=2 years0.25 mg/kg once daily if >= 2 years and weighs < 20 kg.

Provider Requirements and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required. Approvals are for 4 months when criteria are met. Because of the specialized skills required for evaluation and diagnosis of patients treated with Evrysdi, the medication must be prescribed by a physician who has consulted with or who specializes in spinal muscular atrophy and/or neuromuscular disorders.

Approval duration: 4 months (1 month = 30 days).
Prescriber requirement: Physician who has consulted with or specializes in SMA/neuromuscular disorders.

Documentation Required

Documentation is required as noted in the clinical criteria. Baseline and follow-up motor ability assessments must be documented (examples: Bayley Scales of Infant and Toddler Development, CHOP-INTEND, HFMSE, HINE-2, MFM-32, RULM, or WHO motor milestone scale). Genetic testing confirming bi-allelic pathogenic variants in SMN1 and documentation of SMN2 copy number (or equivalent supporting evidence) are also required. For continuation requests, documentation of response or benefit to Evrysdi therapy within the past 4 months is required.

Definitions

SMN2 copy number

DefinitionSMN2 copy number: the number of SMN2 gene copies influences SMA phenotype severity and is used to guide treatment decisions and eligibility in this policy.

2–3 copiesPatients with two or three SMN2 copies meet the policy criterion for treatment when other requirements are met (documentation required).

4 copiesPatients with four SMN2 copies require documentation of objective signs consistent with SMA Types 1–3 in addition to SMN2 copy documentation.

Clinical relevanceHigher SMN2 copy number generally correlates with milder phenotypic expression and is considered when determining eligibility and expected phenotype.

Background

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by loss-of-function mutations or deletion in the SMN1 gene leading to degeneration of lower motor neurons and progressive muscle weakness. Disease severity is modified by the number of copies of the SMN2 gene, with higher SMN2 copy number generally associated with milder phenotypes.

Evrysdi (risdiplam) is an orally administered SMN2 splicing modifier developed to increase production of functional SMN protein from SMN2 and is indicated for treatment of SMA in infants, children, and adults. Clinical use and coverage in this policy are guided by genetic confirmation of bi-allelic SMN1 pathogenic variants, documentation of SMN2 copy number, and objective motor assessments to establish baseline status and response to therapy.