Description

Nusinersen (Spinraza™) is a survival motor neuron-2 (SMN2)-directed antisense oligonucleotide given intrathecally, indicated for the treatment of spinal muscular atrophy (SMA) in pediatric and adult patients.

Criteria

Coverage is subject to the specific terms of the member’s benefit plan.

Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.

Nusinersen (Spinraza) may be considered medically necessary for individuals with SMA type I, II, or III caused by mutations in chromosome 5q when the following criteria are met:

• Individual has had SMA diagnostic test results confirming 0 copies of SMN1; or
• Molecular genetic testing of 5q SMA for any of the following:
  • homozygous gene deletion; or
  • homozygous conversion mutation; or
  • compound heterozygote; and
• The prescriber has provided documentation confirming the individual has two or more copies of SMN2 gene as determined by genetic testing; and
• If the patient has ONE of the following:
  • Type I or II and the prescriber has provided documentation indicating the patient had onset of SMA symptoms at or before 21 months of age; or
  • Type III and the prescriber has provided documentation indicating the patient’s onset of SMA symptoms occurred after 18 months of age; and
• The patient has not received Zolgensma (onasemnogene abeparvovec-xioi); and
• Individual has baseline documentation of Hammersmith Infant Neurologic Exam (HINE) or Upper Limb Module (ULM) or Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) or Hammersmith Function Motor Scale Expanded (HFMSE); and
• Nusinersen (Spinraza) is prescribed by or in consultation with a neurologist with experience and expertise in the treatment and ongoing management with pediatric and adult patients with spinal muscular atrophy.

Continuation of treatment with nusinersen (Spinraza) beyond 6 months after initiation of therapy, and every 6 months thereafter, may be considered medically necessary for the treatment of spinal muscular atrophy when individuals meet BOTH of the following:

• When initial therapy was determined to meet the above criteria; and
• When there is documentation of clinically significant improvement in spinal muscular atrophy-associated symptoms (for example, progression, stabilization, or decreased decline in motor function) compared to the predicted natural history trajectory of disease; and
• The patient has not received Zolgensma (onasemnogene abeparvovex-xioi).

The use of nusinersen (Spinraza) for any other indication other than spinal muscular atrophy caused by mutations in chromosome 5q is considered experimental/investigational and therefore non-covered due to the lack of scientific evidence to support efficacy and safety.

Procedure Codes

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes

Covered Diagnosis Codes for J2326

Professional Statements and Societal Positions Guidelines

NA