New Brunswick

Evrysdi – Risdiplam

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Effective May 24- , 2022 – According to Bulletin 1008, EVRYSDIⓇ (risdiplam) will be covered through the New Brunswick Drug Formulary. For the treatment of 5q spinal muscular atrophy (SMA), if the following criteria are met:
• Genetic documentation of 5q SMA homozygous gene deletion, or compound heterozygous mutation; and
• Patient is not requiring permanent invasive ventilation; and
• Patient who is symptomatic with two or three copies of the SMN2 gene and is:
– 2 months to 7 months of age, or
– 8 months to 25 years of age and non-ambulatory.

Discontinuation Criteria:
• There is failure to demonstrate maintenance in motor milestone function as assessed using
age-appropriate scales since treatment initiation; or
• permanent invasive ventilation is required Clinical Notes: 1. An age-appropriate scale is defined as the Hammersmith Infant Neurological Examination (HINE) Section 2, Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND), or Hammersmith Functional Motor Scale-Expanded (HFMSE). New Brunswick Drug Plans 5 May 2022 2. A baseline assessment using an age-appropriate scale must be completed prior to initiation of treatment. 3. Yearly assessments must be completed using an age-appropriate scale no more than 12 weeks prior to the renewal date. 4. Permanent invasive ventilation is defined as the use of tracheostomy and a ventilator due to progression of SMA that is not due to an identifiable and reversible cause.

Spinraza – Nusinersen

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Initiation Criteria
1. Pre-symptomatic patients with 2 or 3 copies of the SMN2 gene,
2. Had the disease for <6 months, 2 copies of SMN2, and symptom onset after the first week after birth and on, or before seven months of age.
3. Patients under the age of 18 with symptom onset > 6 months of age, and never achieved the ability to walk independently. †

Stopping Criteria
1. For those pre-symptomatic at initiation: no improvement/maintenance on HINE-2, CHOP INTEND, or HFMSE, OR
2. For those symptomatic at initiation: no maintenance in HINE-2, CHOP INTENT, or HFMSE, OR
3. Permanent invasive ventilation required


“Requests for patients who do not meet the above criteria may be considered on a case-by-case basis.”

Zolgensma – Onasemnogene abeparvovec

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  • Genetic documentation of 5q SMA with biallelic mutations in the survival motor neuron 1 (SMN1) gene; and
  • Patient is 180 days of age or younger at the time onasemnogene abeparvovec is administered; and
  • Patient is pre-symptomatic or symptomatic with one to three copies of the survival motor neuron 2 (SMN2) gene; and
  • Patient does not require permanent ventilatory support (invasive or non-invasive) or a permanent feeding tube.

Clinical Note:

  • Permanent ventilatory support is defined as the need for a tracheostomy or requirement of 16 hours or more of respiratory assistance per day (via non-invasive ventilatory support) for 14 or more consecutive days in the absence of an acute reversible illness excluding perioperative ventilation.

Claim Notes:

  • The patient must be under the care of a specialist experienced in the diagnosis and treatment of SMA.
  • No treatment with nusinersen, risdiplam or other medications indicated for the treatment of SMA will be considered after the patient has received a dose of onasemnogene abeparvovec.
  • Approvals will be limited to one lifetime administraton of 1.1 x 1014 vector genomes/kg.
  • Patients who have received a prior dose of onasemnogene abeparvovec accessed by any mechanism (e.g. private insurance plan, clinical trial, compassionate access) will not be funded.
  • Patients with 4 or more copies of the SMN2 gene will not be funded.