Provider Alert! Prior Authorization Criteria for Enzyme Replacement Therapy

Date: January 28, 2022

Attention: Providers

Effective Date: March 1, 2022

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: 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.

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.org

For access to all provider alerts, log into:
www.thecheckup.org or www.texaschildrenshealthplan.org/for-providers.