In the Northwest Territories, Yukon, and Nunavut, the Non-Insured Health Benefits (NIHB) is a federal program providing coverage for SMA treatments. For the Northwest Territories, benefits are administered through the Alberta Blue Cross. Please see below for coverage details of each of the available SMA treatments.

 

Please discuss your eligibility with your health-care provider.

SMA Treatments:

Evrysdi (generic name: risdiplam)

Coverage criteria can be found here by searching for ‘Risdiplam’ in the ‘Chemical Name’ field. For convenience, full coverage criteria is posted below.

 

Full coverage criteria

 

For the treatment of 5q spinal muscular atrophy (SMA) if the following clinical criteria are met.

 

Initial Coverage (12 months):

– genetic documentation of 5q SMA homozygous gene deletion or compound heterozygote; and

– patient is under the care of a specialist with experience in the diagnosis and management of SMA; and

– patient is symptomatic; and

– is aged between 2 months and 7 months (inclusive); and has genetic documentation of two or three copies of the SMN2 gene

or

– is aged 8 months up to 25 years; and is non-ambulatory; and has genetic documentation of two or three copies of the SMN2 gene

and

– patient does not (or did not) require permanent invasive ventilation* at treatment initiation.

*Permanent invasive ventilation defined as the need for a tracheostomy or requirement of 16 hours or more of non-invasive ventilation (e.g. BiPAP) per day or intubation for more than 21 consecutive days in the absence of, or following the, resolution, of an acute reversible event.

 

A baseline assessment using an age-appropriate scale must be completed prior to EVRYSDI administration using ONE of the following baseline symptom assessment scores:

– Hammersmith Infant Neurological Examination (HINE) Section 2 score

– Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

– Hammersmith Functional Motor Scale-Expanded (HFMSE) score

 

Renewal Coverage (12 months):

Treatment with Evrysdi (risdiplam) should be DISCONTINUED if any of the following occur:

– there is no demonstrated achievement in, or maintenance of, motor milestone function (as assessed using an age-appropriate measurement) after treatment initiation in patients aged between 2 months and 2 years at the time of treatment initiation

– there is no demonstrated maintenance of motor function (as assessed using an age-appropriate measurement) after treatment initiation in patients aged between 2 years and 25 years at the time of treatment initiation

– permanent invasive ventilation is required

 

If a patient has not demonstrated maintenance of motor function based on the current assessment,  then the results should be validated with a confirmatory assessment conducted no longer than 12 weeks later.

 

An assessment must be completed using ONE of the following baseline symptom assessment scores. This should be the same scoring system submitted for the baseline assessment.

– Hammersmith Infant Neurological Examination (HINE) Section 2 score

– Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

– Hammersmith Functional Motor Scale-Expanded (HFMSE) score

 

Combination use of  Evrysdi (risdiplam) and  Spinraza (nusinersen) or Zolgensma (onasemnogene abeparvovec) will not be funded.

Spinraza (generic name: nusinersen)

Coverage criteria can be found here by searching for ‘Nusinersen’ in the ‘Chemical Name’ field. For convenience, full coverage criteria is posted below.

 

Full coverage criteria

 

For the treatment of 5q spinal muscular atrophy (SMA) if the following clinical criteria are met.

 

Initial:

– genetic documentation of 5q SMA homozygous gene deletion, homozygous mutation or compound heterozygote; and

– patient is under the care of a specialist with experience in the diagnosis and management of SMA;

And one of the following:

– patient is pre-symptomatic with genetic documentation of two or three copies of SMN2; or

– patient has had disease duration of less than six months, genetic documentation of two copies of SMN2 and symptom onset was after the first week after birth and on or before seven months of age; or

– patient is under the age of 18 with symptom onset after six months of age and never achieved the ability to walk independently.

And

– patient does not (or did not) require permanent invasive ventilation* at treatment initiation.

*permanent invasive ventilation defined as the use of tracheostomy and a ventilator due to progression of SMA that is not due to an identifiable and reversible cause.

Other requests can be considered on a case-by-case basis.

 

A baseline assessment using an age-appropriate scale must be completed prior to nusinersen administration using one of the following baseline symptom assessment scores:

– Hammersmith Infant Neurological Examination (HINE) section 2 score

– Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

– Hammersmith Functional Motor Scale-Expanded (HFMSE) score

 

Renewal:

Treatment should be discontinued if, prior to the fifth dose or any subsequent dose of nusinersen:

– there is no demonstrated achievement or maintenance in motor milestone function (as assessed using age-appropriate scales) since treatment initiation in patients who were pre-symptomatic at the time of treatment initiation; or

– there is no demonstrated maintenance of motor milestone function (as assessed using age-appropriate scales) since treatment initiation in patients who were symptomatic at the time of treatment initiation; or

– permanent invasive ventilation is required.

 

Reassessments for renewal coverage will be requested before the fifth dose, after 12 months of therapy, then annually.