Evrysdi — Blue Cross Blue Shield of Montana

spinal muscular atrophy (SMA)

Initial criteria

Patient has a diagnosis of spinal muscular atrophy (SMA)
Patient has a deletion or mutation at the survival motor neuron 1 (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: (A) If symptomatic, symptom onset was evident prior to age 18 years OR (B) If asymptomatic, patient has no more than 4 copies of SMN2
Patient has had at least ONE of the following baseline functional assessments based on patient age and motor ability: CHOP-INTEND; HINE-2; HFMSE; 6MWT; BSID; MFM32; or RULM
Patient does NOT require invasive ventilation or tracheostomy
Patient has NOT received gene therapy for the requested indication (e.g., Zolgensma [onasemnogene abeparvovec-xioi])
If patient has used Spinraza (nusinersen) in the last four months, they will complete a four-month washout period between the last Spinraza dose and initiation of therapy with the requested agent
Patient will NOT be using the requested agent in combination with Spinraza (nusinersen)
Prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist, geneticist) or has consulted with a specialist
Patient does NOT have any FDA labeled contraindications to the requested agent

Patient has been previously approved for the requested agent through the plan’s Prior Authorization process
Patient has had improvements or stabilization from baseline with the requested agent as indicated by one of the following functional assessments: CHOP-INTEND; HINE-2; HFMSE; 6MWT; BSID; MFM32; or RULM
Patient does NOT require invasive ventilation or tracheostomy
Patient has NOT received gene therapy for the requested indication (e.g., Zolgensma [onasemnogene abeparvovec-xioi])
Patient will NOT be using the requested agent in combination with Spinraza (nusinersen)
Prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist, geneticist) or has consulted with a specialist
Patient does NOT have any FDA labeled contraindications to the requested agent

12 months