Prior Authorization Criteria Update for Zolgensma Scheduled for February 1

Date: November 28, 2022 Attention: Providers Effective date: February 1, 2023 Providers should monitor the Texas Children’s Health Plan (TCHP) Provider Portal regularly for alerts and updates associated to the COVID-19 event.  TCHP reserves the right to update and/or change this information without prior notice due to the evolving nature of the COVID-19 event.Call to action: Texas Children’s Health Plan (TCHP) will update the prior authorization criteria for Zolgensma (J3399) effective February 1, 2023. TCHP will remove the Spinal Muscular Atrophy (SMA) type and the number of SMN 2 copies language from the Zolgensma clinical policy. HHSC updated the clinical policy and prior authorization criteria to read:

• Confirmed diagnosis of SMA (diagnosis code G120) based on gene mutation analysis with bi-allelic SMN1 mutation (deletion or point mutation).

How this impacts providers:

1. Prior authorization approval for Zolgensma (onasemnogene abeparvovec – xioi) treatment of spinal muscular atrophy will be considered once all the following criteria are met:

• Client is 24 months of age or younger
• Medical record supporting the mutation or deletion of genes in chromosome5q
  • Homozygousgene deletion of the SMN1 gene (e.g., absence of SMN1 gene)
  • Homozygousmutation of the SMN1 gene (e.g., biallelic mutation of exon 7)
  • Compoundheterozygous mutation in the SMN1 gene (e.g., deletion of SMN1 exon 7 [allele 1] and mutation of SMN1 [allele 2]
• Confirmed diagnosis of SMA (diagnosis code G120) based on gene mutation analysis with bi-allelic SMN1 mutation (deletion or point mutation)
• The administration of Zolgensma (onasemnogeneabeparvovec-xioi) may cause serious liver injury or failure. Providers must meet the following to administer the drug:
  • The client’s liver function must be examined by clinical examination and laboratory testing (e.g., hepatic aminotransferases (aspartate aminotransferase (AST) and alanine aminotransferase (ALT), total bilirubin, and prothrombin time) prior to infusion of Zolgensma.
  • Systemic corticosteroid must be administered prior to and after 5th administration of the drug.
  • Provider must continue to monitor client’s liver function at least 3 months after the infusion of the drug.
• Evaluation of motor skill and function must be documented using a standardized test; however, it is not a prerequisite for therapy and should not delay treatment. Standardized testing tools that may be used to evaluate motor skill/function include, but are not limited to:
  • Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND)score
  • Bayley Scale of Infant and Toddler Development screening test
  • WHO Multicenter Growth Reference Study (WHO MGRS)
• Baseline documentation of AAV9 antibody titer of 1:50 or lower, as determined by ELISA binding immunoassay

2. If Spinraza (nusinersen) (procedure code J2326) or Evrysdi (risdiplam) was previously prescribed, the prescriber must provide documentation of one of the following prior to switching to Zolgensma (onasemnogene abeparvovec-xioi) therapy:

• Evidence of clinical deterioration (e.g., decreased physical function and motor skill/function test scores) while on Spinraza (nusinersen) therapy or Evrysdi (risdiplam) therapy OR
• Prescriber attestation to the discontinuation of Spinraza (nusinersen) therapy or Evrysdi (risdiplam).

3. Physician attestation that client has not received prior Zolgensma (onasemnogene abeparvovec-xioi) therapy.

Next steps for providers: Refer to the Outpatient Drug Services Handbook Chapter of the Texas Medicaid Provider Procedure Manual for more details on the clinical policy and prior authorization requirements. If you have any questions, please email Provider Relations at: providerrelations@texaschildrens.org.For access to all provider alerts,log into: www.thecheckup.org or www.texaschildrenshealthplan.org/for-providers