Product Details

Spinraza

Nusinersen
2.4 mg/mL
Solution for Intrathecal Injection
Single-Use 5-mL Vial (Preservative-Free)

DIN/PIN/NPN

02465663

Manufacturer

Biogen Idec Canada Inc.

Formulary Listing Date

2018-11-20  

Unit Price

9833.3333

Amount MOH Pays

9833.3333

Coverage Status

Exceptional Access Program Product

ODB Formulary Therapeutic Classification

Therapeutic Note

NO

ATC Code

M09AX07

Interchangeable Products

NO  

LU Clinical Criteria

NO  

EAP Criteria

Therapeutic Class Reimbursement Criteria
Metabolic/Genetic Modifiers

Nusinersen

  • Brand(s): Spinraza
  • Dosage Form/Strength: 2.4 mg/mL – 5mL vial Pk
  • Effective date: November 20, 2018 and Updated June 12, 2019

For the treatment of spinal muscular atrophy (SMA) in patients meeting all the following criteria: 

  1. Provides genetic documentation of 5q SMA homozygous gene deletion, homozygous mutation, or compound heterozygote. 

  1. Patient meets one (1) of the following clinical scenarios:
    •     Pre-symptomatic with 2 or 3 copies of the survival motor neuron 2 (SMN2) gene;
    OR
    •     Have had disease duration of less than 6 months, 2 copies of the SMN2 gene, and symptom onset after the first week after birth and on or before 7 months of age;
    OR
    •     Are under the age of 18 with symptom onset after 6 months of age, and never achieved the ability to walk independently. 

  1. Patient does not require permanent invasive ventilation. 

  1. Patient is under the care of a specialist experienced in the diagnosis and management of SMA. 

In addition, symptomatic Type 2 and 3 patients under the age of 18 regardless of ever achieving the ability to walk independently will be considered on a case-by-case basis. 

Other patients who do not meet the expanded funding criteria may be considered in exceptional cases. 

Renewal Criteria: 

Renewal of funding will be considered for patients who have not demonstrated any of the Stopping/discontinuation criteria while on therapy. 

Stopping/Discontinuation Criteria for Spinraza:

These criteria are applicable to patients funded upon meeting either initial or renewal criteria.

An assessment of the response to therapy should be made prior to the fifth dose or every subsequent dose of Spinraza. Treatment should be discontinued upon meeting any of the following circumstances:

  • Patient is not demonstrating/achieving response in motor milestones as assessed using 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), as follows:
    o there is no demonstrated improvement in motor milestone function above pre- treatment scores (as assessed using the HINE Section 2, CHOP INTEND or HFMSE); OR
    o     there is no demonstrated maintenance of motor milestone function (as assessed using HINE Section 2, CHOP INTEND or HFMSE);

    OR

  • Patient requires Permanent Invasive Ventilation (PIV)1

Exclusion criteria: 

  1. Patient has SMA type 4 

  1. Patient has more than four (4) SMN2 gene copies 

  1. Patient with permanent invasive ventilation (PIV)1 

1Permanent Invasive Ventilation (PIV) 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. 

Recommended dose: 

Loading doses:
12 mg administered intrathecally on days 0, 14, 28 and 58 (Note: 4th loading dose is administered approximately 30 days after the 3rd loading dose) 

Maintenance dose:
12 mg administered intrathecally every 4 months starting 4 months after the 4th loading dose. 

Approval duration for initial request: 8 months

Approval duration of Renewals: 1 year

EAP Drug Request Form:

Standard Form for EAP Drug Requests

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