Evrysdi — Blue Cross Blue Shield of Kansas

spinal muscular atrophy (SMA)

Initial criteria

Diagnosis of spinal muscular atrophy (SMA)
Deletion or mutation at the 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: (A) If symptomatic, symptom onset evident prior to age ≤ 18 years OR (B) If asymptomatic, patient has no more than 4 copies of SMN2
At least ONE baseline (prior to starting therapy) functional assessment based on patient age and motor ability: CHOP-INTEND OR HINE-2 OR HFMSE OR 6MWT OR BSID OR MFM32 OR RULM test
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) for the requested indication
Prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist, geneticist) or has consulted with a specialist in the area of the patient’s diagnosis
Patient does NOT have any FDA labeled contraindications to the requested agent

Patient previously approved for the requested agent through the plan’s Prior Authorization process
Patient has had improvements or stabilization from baseline (prior to starting therapy) with the requested agent as indicated by one of the following functional assessments: CHOP-INTEND OR HINE-2 OR HFMSE OR 6MWT OR BSID OR MFM32 OR RULM test
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) for the requested indication
Prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist, geneticist) or has consulted with a specialist in that area
Patient does NOT have any FDA labeled contraindications to the requested agent

12 months