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 is 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 below. Use of the following codes 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.
Risk of Acute Cardiorespiratory Failure: Patients with acute underlying respiratory illness and compromised cardiac and/or respiratory function may be at risk of acute cardiorespiratory failure. Caution should be exercised when administering alglucosidase alfa to patients susceptible to fluid volume overload. Appropriate medical support and monitoring measures should be available during infusion and some patients may require longer observation times.
Risk of Cardiac Arrhythmia and Sudden Cardiac Death during General Anesthesia for Central Venous Catheter Placement: Caution should be used when administering general anesthesia for the placement of a central venous catheter intended for alglucosidase alfa infusion.
Risk of Antibody Development: As with all therapeutic proteins, there is potential for immunogenicity. There is some evidence to suggest that some patients who develop high and sustained IgG antibody titers may experience reduced clinical efficacy. 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.
Pregnancy: Based on animal data, alglucosidase alfa may cause fetal harm.
Please see the Full Prescribing Information for complete details, including boxed WARNING.