Evrysdi — Blue Cross Blue Shield of Alabama

Initial criteria

• Patient has a diagnosis of spinal muscular atrophy (SMA)
• Patient has a deletion or mutation at the SMN1 gene on chromosome 5q confirmed by genetic testing (medical records required)
• Patient has a diagnosis of probable SMA Type 1, 2, or 3, AND ONE of the following: • If symptomatic, symptom onset was evident prior to age 18 years • If asymptomatic, patient has no more than 4 copies of SMN2 (medical records required)
• Patient retains meaningful voluntary motor function (e.g., manipulate objects using upper extremities, ambulate, etc.) (medical records required)
• Patient has had at least ONE of the following baseline (prior to starting therapy) functional assessments (medical records required): • Motor function/milestones assessment using at least one validated scale (HINE, HFMSE, CHOP‑INTEND, BSID‑III, 6MWT, ULM, MFM32, or RULM) OR • Respiratory function tests (e.g., FVC) OR • Exacerbations necessitating hospitalization and/or antibiotic therapy for respiratory infection in the preceding year/timeframe OR • Patient weight (for patients without gastrostomy tube)
• Patient does NOT have advanced disease (e.g., complete limb paralysis, permanent ventilation support, etc.)
• Patient has NOT received gene therapy for the requested indication (e.g., Zolgensma [onasemnogene abeparvovec‑xioi]) (medical records required)
• If patient has used Spinraza (nusinersen) in the last four months, they will complete a four‑month washout period between last Spinraza dose and initiation of therapy with requested agent
• Patient will NOT use requested agent in combination with Spinraza (nusinersen) for the requested indication (medical records required)
• Prescriber is a specialist in the area of patient’s diagnosis (e.g., neurologist, geneticist) or has consulted with such a specialist
• Patient does NOT have any FDA‑labeled contraindications to requested agent

Reauthorization criteria

• Patient has been previously approved for requested agent through plan’s Prior Authorization process
• Patient has responded to therapy compared to baseline with at least ONE of the following: • Stability or improvement in motor function/milestones by validated scale (HINE, HFMSE, CHOP‑INTEND, BSID‑III, 6MWT, ULM, MFM32, or RULM) OR • Stability or improvement in respiratory function tests (e.g., FVC) OR • Reduction in exacerbations necessitating hospitalization and/or antibiotic therapy for respiratory infection in preceding year/timeframe OR • Stable or increased patient weight (for patients without gastrostomy tube) OR • Slowed rate of decline in the aforementioned measures
• Patient has NOT received gene therapy for the requested indication (e.g., Zolgensma [onasemnogene abeparvovec‑xioi])
• Patient will NOT use requested agent in combination with Spinraza (nusinersen) for the requested indication
• Prescriber is a specialist in the area of patient’s diagnosis (e.g., neurologist, geneticist) or has consulted with such a specialist
• Patient does NOT have any FDA‑labeled contraindications to requested agent

Approval duration

12 months