Provider Alert! Zolgensma Update – new TMPPM criteria pending publication

Attention: Neurologists and SMA specialists

Effective Date: December 1, 2020 

Call to action:  Prescribers should be aware of an update to prior authorization criteria for Zolgensma (onasemnogene abeparvovec) drug HCPCS code J3399. Zolgensma is indicated for treatment of pediatric patients (<2 years of age) with Spinal Muscular Atrophy (SMA).

Effective December 1, diagnosis of Type I SMA (diagnosis code G120) will require three or less copies of SMN-2. Previous criteria required two or fewer copies. All other criteria will remain the same.

How this impacts providers: Prescribers should adjust their prescribing patterns accordingly and communicate these changes to their staff.

Next steps for providers: Prior authorization requests for procedure code J3399 must be submitted with a Special Medical Prior Authorization (SMPA) Request Form.  The completed Special Medical Prior Authorization (SMPA) Request Form must be maintained by the provider in the member’s medical record and is subject to retrospective review.  Providers should communicate these changes to their staff.

Zolgensma prior authorization criteria:

Zolgensma must be prescribed by, or in consultation with, a neurologist or a specialist in SMA.

Zolgensma is not a benefit for members with a tracheostomy or invasive ventilator support.

Prior authorization of therapy will be approved (one treatment per lifetime) for members who meet the following criteria:

Member has documented diagnosis of SMA based on the following:

• Client is 24 months of age or younger.
• Onset of clinical signs and symptoms is consistent with SMA from birth up to six months of age
• Medical record supports any of the following mutation or deletion of genes in chromosome 5q
  • Homozygous gene deletion of the survival motor neuron 1 (SMN1) gene (e.g., absence of SMN1 gene)
  • Homozygous mutation of the SMN1 gene (e.g., biallelic mutation of exon 7)
  • Compound heterozygous mutation in the SMN1 gene (e.g., deletion of SMN1 exon 7 [allele 1] and mutation of SMN1 [allele 2])
• Confirmed diagnosis of Type I SMA (diagnosis code G120) based on gene mutation analysis with biallelic SMN1 mutation (deletion or point mutation) and three or less copies of SMN2
• Documentation contains the Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorder (CHOP-INTEND) score to evaluate the client’s motor skills.
• Baseline documentation supports an AAV9 antibody titer of 1:50 or lower, as determined by enzyme-linked immunosorbent assay (ELISA) binding immunoassay.
• Physician attestation supports that the client has not received prior Zolgensma therapy.

If nusinersen (Spinraza) (procedure code J2326) has been previously prescribed, the prescriber must provide documentation of one of the following before switching to Zolgensma therapy:

• Evidence of clinical deterioration (e.g., decreased physical function and CHOP-INTEND score) while on Spinraza therapy
• Prescriber’s attestation that Spinraza therapy has been discontinued

If you have any questions, please email Provider Network Management at: providerrelations@texaschildrens.org.

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www.thecheckup.org or www.texaschildrenshealthplan.org/for-providers.