Provider Alert! Clinical Criteria for Xenpozyme Procedure Code J0218 effective May 1, 2023

Provider Alert!

Provider Alert! Clinical Criteria for Xenpozyme Procedure Code J0218 effective May 1, 2023

Date: April 6, 2023

Attention: All Providers

Effective Date: May 1, 2023

Call to action: Effective May 1, 2023, the Health and Human Services Commission (HHSC) will begin the incorporation of prior authorization criteria for Xenpozyme (procedure code J0218) to the Enzyme Replacement Therapy policy. Xenpozyme is the first therapy indicated specifically for the treatment of non-central nervous system manifestations of acid sphingomyelinase deficiency (ASMD) in adult and pediatric patients, and is currently the only approved treatment for this disease.

Authorization Requirements

1. Member has acid sphingomyelinase deficiency (ASMD), diagnosis code E75.241 or E75.244

2. Documentation of baseline transaminase assessment prior to treatment

3. Documentation of alanine transaminase (ALT) and aspartate aminotransferase (AST) levels before initiation of therapy, during any dose escalations of therapy and before any missed doses

4. Verification of negative pregnancy status for female clients of reproductive potential prior to therapy initiation

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.

Why is this important?

Texas Children’s Health Plan (TCHP) recognizes we may serve potentially impacted patients in our membership. We want to ensure that the member meets clinical evidence for treatment.

Next steps for providers: Prescribers should share this communication with their staff. Provider must submit documentation (such as office chart notes, lab results, or other clinical information) supporting that the member has met all approval criteria in support for Xenpozyme approval.

Note: If request is for a non-FDA approved dose or indication, medical rational must be submitted in support of therapy (such as high-quality peer reviewed literature, acceptable compendia or evidence based practice guidelines) and exceptions will be considered on a case-by-case basis.

If you have any questions, please email TCHP Pharmacy at:

For access to all provider alerts,log into: or

Share this post