Prior Authorization Criteria Update for Zolgensma

Date: September 20, 2022 Attention: All ProvidersEffective Date: November 11, 2022Providers should monitor the Texas Children’s Health Plan (TCHP) Provider Portal regularly for alerts and updates associated with 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) would like to inform providers that effective November 1, 2022, the Health and Human Services Commission (HHSC) will update the prior authorization criteria for Zolgensma (J3399). The following are the newly added updates to the Zolgensma (onasemnogene abeparvovec – xioi) clinical policy:

• The administration of onasemnogene abeparvovec-xioi (Zolgensma) 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) before infusion of Zolgensma.
  • Systemic corticosteroid must be administered prior to and after the administration of the drug.
  • The provider must continue monitoring the client’s liver function at least 3 months after the drug infusion.

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

• The client is 24 months or younger.
• Medical record supporting the mutation or deletion of genes in chromosome 5q
  • Homozygous gene deletion of the 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 bi-allelic SMN1 mutation (deletion or point mutation) and 3 or less copies of SMN2.
• The administration of onasemnogene abeparvovec-xioi (Zolgensma) 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) before infusion of Zolgensma.
  • Systemic corticosteroid must be administered before and after the administration of the drug.
  • The provider must continue monitoring the client’s liver function at least 3 months after the drug infusion.
• Evaluation of motor skills and function must be documented using a standardized test. However, it is not a therapy prerequisite and should not delay treatment. Standardized testing tools that may be used to evaluate motor skill or 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.
• Physician attestation that client has not received prior onasemnogene abeparvovec-xioi (Zolgensma) therapy.
• If nusinersen (Spinraza) (procedure code J2326) or risdiplam (Evrysdi) has been previously prescribed, the prescriber must provide documentation of one of the following before switching to onasemnogene abeparvovec-xioi (Zolgensma) therapy:
  • Evidence of clinical deterioration (e.g., decreased physical function and motor skill/function test scores) while on nusinersen (Spinraza) therapy or risdiplam (Evrysdi) therapy OR
  • The prescriber attests to discontinuing nusinersen (Spinraza) or risdiplam (Evrysdi) 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 Network Management at: providerrelations@texaschildrens.org. For access to all provider alerts,log into: http://www.thecheckup.org or www.texaschildrenshealthplan.org/for-providers.