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(Carton of one single-use vial)|
|NDC||NDC(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
The codes here are provided for informational purposes only and are not intended to substitute for the physician’s independent diagnosis or treatment of each patient. Providers are responsible for the accuracy and validity of any claims, invoices, and related documentation submitted to payers. Physicians should contact the payer if they have any specific questions about coverage or payment. Any specific guidance or direction on the submission of claims offered by the payer supersede the codes listed above. Use of the following codes listed above does not guarantee reimbursement.
If you would like to confirm the most recent billing codes, have questions about which codes to use, or need assistance submitting claims or other supporting documentation, contact a CareConnectPSS Case Manager:, option 3
Following are definitions of the different types of coding systems used to classify and identify medical conditions and prescription medications.
LUMIZYME® (alglucosidase alfa) is a hydrolytic lysosomal glycogen-specific enzyme indicated for patients with Pompe disease (GAA deficiency).
WARNING: RISK OF ANAPHYLAXIS, HYPERSENSITIVITY AND IMMUNE-MEDIATED REACTIONS, AND RISK OF CARDIORESPIRATORY FAILURE
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.
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.