Prior Authorization Criteria for Enzyme Replacement Therapy

Date: January 28, 2022 Attention: Providers Effective Date: March 1, 2022Providers 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: Effective March 1, 2022, HHSC will require prior authorization for all enzyme replacement therapies for Medicaid and CHIP. Enzyme replacement therapy (ERT) is a medical treatment that replaces a specific enzyme deficient or absent in the body. The following enzyme replacement therapies are listed with the FDA-approved indications, age restrictions and diagnosis codes (as applicable).

Agalsidase beta (Fabrazyme) is indicated in clients age 2 years and older with Fabry disease. Diagnosis code: E7521.
Alglucosidase alfa (Lumizyme) is indicated for clients with Pompe disease (GAA deficiency). Diagnosis code: E7402.
Alglucosidase alfa-ngpt (Nexviazyme) is indicated for clients who are one year of age and older with late onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency). Diagnosis code: E7402.
Cerliponase alfa (Brineura) is indicated to slow the loss of ambulation in symptomatic pediatric clients 3 years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2). Diagnosis code: E754.
Elosulfase alfa (Vimizim) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for clients age 5 years and older with Mucopolysaccharidosis type IVA. Diagnosis code: E76210.
Galsulfase (Naglazyme) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for clients with Mucopolysaccharidosis VI (MPS VI; Maroteaux-Lamy syndrome). Diagnosis codes: E7629.
Idursulfase (Elaprase) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for clients with Hunter syndrome (Mucopolysaccharidosis II, MPS II). Diagnosis code: E761.
Imiglucerase (Cerezyme) is indicated for long-term enzyme replacement therapy for clients age 2 and above with a confirmed diagnosis of Type 1 Gaucher disease (diagnosis code: E7522) that results in one or more of the following conditions:
- Anemia
- Thrombocytopenia
- Bone disease
- Hepatomegaly or splenomegaly
Laronidase (Aldurazyme) is indicated in clients with Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I (MPS I) and clients with the Scheie form who have moderate to severe symptoms. Diagnosis codes: E7601, E7602, and E7603.
Protein C Concentrate, human (Ceprotin) is indicated in pediatric and adult clients with severe congenital Protein C deficiency for the prevention and treatment of venous thrombosis and purpura fulminans. Diagnosis code: D6859.
Sebelipase alfa (Kanuma) is indicated for the treatment of pediatric and adult clients with a diagnosis of Lysosomal Acid Lipase (LAL) deficiency. Diagnosis code: E755.
Taliglucerase alfa (Elelyso) is indicated for long-term enzyme replacement therapy for adult clients with a diagnosis of Type 1 Gaucher disease. Diagnosis code: E7522.
Velaglucerase alfa (Vpriv) is indicated for long-term replacement therapy for pediatric and adult clients with Type 1 Gaucher disease. Diagnosis code: E7522.

How this impacts providers: Prior authorization approval for any of the enzyme replacement therapy listed above will be considered when the following criteria are met:

A request for the specific enzyme replacement therapy
The laboratory evidence of the enzyme deficiency

Here are the Healthcare Common Procedure Coding System (HCPCS) codes that may be reimbursed for Enzyme Replacement Therapy services.

C9085	J0180	J0567	J0221
J1322	J1458	J1743	J1786
J1931	J2724	J2840	J3060
J3385

Next steps for providers: Prescribers should adjust their prescribing patterns accordingly and share this update with their staff as well If you have any questions, please email TCHP Pharmacy Department at: TCHPPharmacy@texaschildrens.orgFor access to all provider alerts,log into: www.thecheckup.org or www.texaschildrenshealthplan.org/for-providers.