IMPORTANT SAFETY INFORMATION: WARNING: RISK OF ANAPHYLAXIS, HYPERSENSITIVITY AND IMMUNE-MEDIATED REACTIONS, AND RISK OF CARDIORESPIRATORY FAILURE Life-threatening anaphylactic reactions and severe hypersensitivity reactions, presenting as respiratory distress, hypoxia, apnea, dyspnea, bradycardia, tachycardia, bronchospasm, throat tightness, hypotension, angioedema (including tongue or lip swelling, periorbital edema, and face edema), and urticaria, have occurred in some patients during and after alglucosidase alfa infusions. Immune-mediated reactions presenting as proteinuria, nephrotic syndrome, and necrotizing skin lesions have occurred in some patients following alglucosidase alfa treatment. View more

BILLING & REIMBURSEMENT

Treatment with Lumizyme® (alglucosidase alfa) is eligible for reimbursement under most major commercial insurance plans, as well as the Medicare and Medicaid programs. In this section, you will find a list of current Lumizyme billing codes, claim forms and sample letters, plus other information to help you successfully bill and obtain reimbursement from different payers.

Guidelines for Successful Reimbursement

In many cases, physicians must obtain prior authorization for Lumizyme treatment, and insurers often require physicians to document the patient's medical necessity before approving coverage. You may also need to obtain written confirmation of coverage from the insurer before beginning treatment.

Billing for reimbursement for Lumizyme therapy requires using the appropriate codes. The Center for Medicare and Medicaid Services has not yet assigned a permanent HCPCS code (commonly used by many payers) to Lumizyme, although temporary biologic J-codes are available, and their use may streamline the reimbursement process.

However, billing procedures vary from plan to plan, so you are advised to carefully review each patient's coverage, and when necessary, check with the payer about its billing requirements, before initiating treatment. Keep in mind that you may need different claim forms depending on whether the patient receives treatment in a physician's office or outpatient setting.

The following table lists the most commonly used codes when filing claims for reimbursement for Lumizyme® (alglucosidase alfa) treatment:

ICD-10-CM

E74.02 – Pompe Disease

NDC

NDC 58468-0160-1 (Carton of one single-use vial)

NDC

NDC 58468-0160-2 (Carton of ten single-use vials)

HCPCS

J0221 – Injection, alglucosidase alfa (Lumizyme), 10 MG

CPT-4

96365 – Intravenous infusion therapy, prophylaxis,
or diagnosis (specify substance or drug); initial, up to 1 hour
96366 – Each additional hour (list separately in addition to primary code, 96365)

Revenue

260 – General IV therapy service
261 – Infusion pump
258 – IV solutions
636 – Drugs and biologicals requiring an HCPCS code

Coding Glossary

The following are definitions of the different types of coding systems used to classify and identify medical conditions and prescription medications.

  • ICD-10-CM (International Classification of Diseases, 10th Edition) – ICD-10-CM codes represent the patient’s medical condition or reason for treatment (diagnosis code). These codes are used by hospitals and physicians, and are recognized by all insurers.
  • NDC (National Drug Code) – NDCs are codes that identify FDA-approved drugs. The NDC identifies the manufacturer, product, and package size. NDCs are used primarily by retail pharmacies.
  • HCPCS (Healthcare Common Procedure Coding System) – HCPCS codes are assigned by CMS (Center for Medicare and Medicaid Services) and are used by Medicare and most private payers to describe products administered in the physician office or hospital setting.
  • CPT-4 (Current Procedural Terminology) – CPT-4 Codes are used by physicians and hospitals to designate the procedures performed.
  • Revenue Codes – Revenue Codes are used by hospitals to classify services by category, and typically are required by payers when billing infusions in the hospital setting.

This page is for informational purposes only and is subject to change. Healthcare professionals are responsible for complying with reimbursement-related rules and regulations. Providers are also responsible for the accuracy of any claims, invoices, and related documentation submitted to payers.

Resources

Full Prescribing Information (PDF)

Lumizyme Billing Guide (PDF)

 

Indication

LUMIZYME® (alglucosidase alfa) is a hydrolytic lysosomal glycogen-specific enzyme indicated for patients with Pompe disease (GAA deficiency).

Important Safety Information

WARNING: RISK OF ANAPHYLAXIS, HYPERSENSITIVITY AND IMMUNE-MEDIATED REACTIONS, AND RISK OF CARDIORESPIRATORY FAILURE

  • Life-threatening anaphylactic reactions and severe hypersensitivity reactions, presenting as respiratory distress, hypoxia, apnea, dyspnea, bradycardia, tachycardia, bronchospasm, throat tightness, hypotension, angioedema (including tongue or lip swelling, periorbital edema, and face edema), and urticaria, have occurred in some patients during and after alglucosidase alfa infusions. Immune-mediated reactions presenting as proteinuria, nephrotic syndrome, and necrotizing skin lesions have occurred in some patients following alglucosidase alfa treatment. Closely observe patients during and after alglucosidase alfa administration and be prepared to manage anaphylaxis and hypersensitivity reactions. Inform patients of the signs and symptoms of anaphylaxis, hypersensitivity reactions, and immune-mediated reactions and have them seek immediate medical care should signs and symptoms occur.
  • Infantile-onset Pompe disease patients with compromised cardiac or respiratory function may be at risk of serious acute exacerbation of their cardiac or respiratory compromise due to fluid overload, and require additional monitoring.

Warnings and Precautions

Anaphylaxis and Hypersensitivity Reactions: Life-threatening anaphylaxis and hypersensitivity reactions have been observed in some patients during and after treatment with alglucosidase alfa. If anaphylaxis or severe hypersensitivity reactions occur, immediately discontinue infusion and institute appropriate medical treatment. Appropriate medical support and monitoring measures should be available during infusion.

Immune-Mediated Reactions: Monitor patients for the development of systemic immune-mediated reactions involving skin and other organs. If immune-mediated reactions occur, consider discontinuation of the administration of alglucosidase alfa, and initiate appropriate medical treatment.

Risk of Acute Cardiorespiratory Failure: Patients with acute underlying respiratory illness or compromised cardiac and/or respiratory function may be at risk of serious exacerbation of their cardiac or respiratory compromise during infusions. Appropriate medical support and monitoring measures should be readily available during alglucosidase alfa infusion, and some patients may require prolonged observation times that should be individualized based on the needs of the patient.

Risk of Cardiac Arrhythmia and Sudden Cardiac Death during General Anesthesia for Central Venous Catheter Placement: Administration of general anesthesia can be complicated by the presence of severe cardiac and skeletal (including respiratory) muscle weakness. Therefore, caution should be used when administering general anesthesia. Ventricular arrhythmias and bradycardia, resulting in cardiac arrest or death, or requiring cardiac resuscitation or defibrillation have been observed in infantile-onset Pompe disease patients with cardiac hypertrophy during general anesthesia for central venous catheter placement.

Risk of Antibody Development: Patients with infantile-onset Pompe disease should have a cross-reactive immunologic material (CRIM) assessment early in their disease course and be managed by a clinical specialist knowledgeable in immune tolerance induction in Pompe disease to optimize treatment. CRIM status has been shown to be associated with immunogenicity and patients’ responses to enzyme replacement therapies. There is evidence to suggest that some patients who develop high and sustained IgG antibody titers, including CRIM-negative patients, may experience reduced clinical alglucosidase alfa treatment efficacy.

Monitoring: Laboratory Tests: Patients should be monitored for IgG antibody formation every 3 months for 2 years and then annually thereafter.

Adverse Reactions

The most frequently reported adverse reactions (≥ 5%) in clinical trials were hypersensitivity reactions and included: anaphylaxis, rash, pyrexia, flushing/feeling hot, urticaria, headache, hyperhidrosis, nausea, cough, decreased oxygen saturation, tachycardia, tachypnea, chest discomfort, dizziness, muscle twitching, agitation, cyanosis, erythema, hypertension/increased blood pressure, pallor, rigors, tremor, vomiting, fatigue, and myalgia.

Please see the Full Prescribing Information for complete details, including boxed WARNING.