Vimizim™ (elosulfase alfa) injection, for intravenous use, is a hydrolytic lysosomal glycosaminoglycan (GAG) specific enzyme indicated for patients with Mucopolysaccharidosis type IVA (MPS IVA: Morquio A syndrome). Vimizim™ received approval from the Food and Drug Administration (FDA) on February 14, 2014.
Vimizim™ is supplied as a concentrated solution for infusion in a 5mg/5ml (1mg / mL) single-use vial, requiring dilution. As approved by the FDA, the recommended dose is 2mg per kg, administered intravenously over a minimum range of 3.5 to 4.5 hours, based on infusion volume, once every week. Pre-treatment with antihistamines, with or without antipyretics, is recommended 30-60 minutes prior to the start of the infusion.
Understanding Vimizim Reimbursement: J3590 and C9399 Billing
In the absence of a national coverage determination (NCD) or local coverage determination (LCD) specifically for elosulfase alfa, Medicare may consider coverage when the drug is administered incident to a physician service or in a hospital setting. It is imperative that the patient’s medical record clearly supports the diagnosis of Mucopolysaccharidosis, type IVA (MPS IVA; Morquio A syndrome) using the appropriate ICD-10-CM code(s) such as E76.211 (Morquio A syndrome), aligning with current FDA guidance for its use and administration.
Providers must ensure thorough documentation, not only of the diagnosis but also of the drug’s administration and the patient’s response, to support claims for J3590 (injection, elosulfase alfa, 0.5 mg) or C9399 (unclassified drug or biological). Adherence to current Medicare billing guidelines and rare disease drug coverage policies is crucial for appropriate reimbursement.