Evrysdi — Blue Cross Blue Shield of Illinois

spinal muscular atrophy (SMA) Type 1, 2, or 3

Initial criteria

Diagnosis of spinal muscular atrophy (SMA)
Deletion or mutation at SMN1 gene on chromosome 5q confirmed by genetic testing (medical records required)
Diagnosis of probable SMA Type 1, 2, or 3 AND ONE of the following: symptomatic with onset before age 18 years OR asymptomatic with no more than 4 copies of SMN2
At least one baseline functional assessment (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 SMA (e.g., Zolgensma [onasemnogene abeparvovec-xioi])
If patient has used Spinraza (nusinersen) in last four months, a four-month washout period must be completed prior to initiating requested agent
Requested agent will NOT be used in combination with Spinraza (nusinersen)
Prescriber is a neurologist, geneticist, or has consulted with such a specialist
No FDA labeled contraindications to requested agent

Previously approved for requested agent through the plan’s prior authorization process
Improvement or stabilization from baseline with requested agent as demonstrated by one of the functional assessments (CHOP-INTEND, HINE-2, HFMSE, 6MWT, BSID, MFM32, RULM)
Patient does NOT require invasive ventilation or tracheostomy
Patient has NOT received gene therapy for SMA (e.g., Zolgensma [onasemnogene abeparvovec-xioi])
Patient will NOT use requested agent in combination with Spinraza (nusinersen)
Prescriber is a specialist (neurologist, geneticist) or has consulted with one
No FDA labeled contraindications

12 months