Nova Scotia
Treatments

Evrysdi – Risdiplam

Link to more information

Available:

EVRYSDI (risdiplam) has been added to the Nova Scotia Pharmacare The specific criteria for reimbursement are in line with recommendations made by CADTH in 2021.

Criteria
For patients diagnosed with 5q Spinal Muscular Atrophy (SMA) under the care of a specialist with experience in the diagnosis and management of SMA, if the following clinical criteria are met:
Genetic documentation of 5q SMA homozygous gene deletion or compound heterozygote, AND

Patients who:
are symptomatic and have genetic documentation of two or three copies of the SMN2 gene, AND
aged between 2 months and 7 months (inclusive), OR
aged 8 months up to 25 years and are non-ambulatory
Patient is not currently requiring permanent invasive ventilation*, AND A baseline assessment using an age-appropriate scale (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]) must be completed prior to initiation of risdiplam treatment.

For continued coverage, the patient must meet the following criteria:
There is demonstrated achievement or maintenance of motor milestone function (as assessed using age-appropriate scales: the [HINE] Section 2, CHOP INTEND, or HFMSE) after treatment initiation in patients aged between 2 months and 2 years at the time of treatment initiation; OR There is demonstrated maintenance of motor milestone function (as assessed using age-appropriate scales: the HINE Section 2, CHOP INTEND, or HFMSE) after treatment initiation in patients aged between 2 years and 25 years at the time of treatment initiation; AND Patient does not require permanent invasive ventilation*. The decision to discontinue reimbursement should be based on 2 assessments separated by no longer than a 12-week interval. * 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.

Claim Notes:
Coverage for risdiplam will not be provided in combination with other SMA drug therapies or post administration of onasemnogene abeparvovec.
Approval: 12 months
Claims that exceed the maximum claim amount of $9,999.99 must be divided and submitted as separate transactions using the DIN first and then the following PINs:
00904768
00904769
00904770

Spinraza – Nusinersen

Link to more information

Available:

Pediatric:

Initiation Criteria
1. Pre-symptomatic patients with 2 or 3 copies of the SMN2 gene,
OR
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.
OR
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

Adult:

Not available

Zolgensma – Onasemnogene abeparvovec

Available:

No information available at this time.