By Counterforce Health Team in Lumizyme (alglucosidase alfa) —

Coding That Helps Get Lumizyme (alglucosidase alfa) Approved in Texas with Blue Cross Blue Shield: ICD-10, HCPCS/J-Code, NDC Guide

Answer Box: Essential Coding for Lumizyme Approval

Getting Lumizyme (alglucosidase alfa) approved by Blue Cross Blue Shield of Texas requires precise coding: ICD-10 E74.02 (Pompe disease), HCPCS J0221 (10 mg units), and proper NDC 58468-0160-xx. Prior authorization is required for most BCBS Texas plans. Submit via Availity Authorization & Referrals or Blue Approvr with confirmed diagnosis (GAA enzyme assay/genetic testing), weight-based dosing (20 mg/kg every 2 weeks), and clinical documentation. First step today: Verify your patient's exact BCBS plan type and check the current PA grid for J0221 requirements.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit Paths
  2. ICD-10 Mapping for Pompe Disease
  3. Product Coding: HCPCS, J-Code, and NDC Overview
  4. Clean Request Anatomy: Example PA with Callouts
  5. Frequent Pitfalls: Unit Conversions and Code Mismatches
  6. Verification Steps: Cross-Check with BCBS Resources
  7. Quick Audit Checklist: Pre-Submission Review
  8. Appeals Process for Coding-Related Denials

Coding Basics: Medical vs. Pharmacy Benefit Paths

Lumizyme is an IV-infused enzyme replacement therapy that's processed under the medical benefit for virtually all Blue Cross Blue Shield of Texas plans. This means it's billed on professional (CMS-1500) or facility (UB-04) claims using HCPCS codes, not through retail pharmacy systems.

Medical Benefit Characteristics

  • Billing method: Buy-and-bill using HCPCS J-codes
  • Site of care: Hospital outpatient, infusion center, or physician office
  • Prior authorization: Required through medical management, not pharmacy benefit managers
  • Claims processing: Professional/facility claims with revenue codes
Note: Some BCBS plans use "medical pharmacy" vendors for specialty biologics, but Lumizyme still bills with J-codes under the medical benefit structure.

ICD-10 Mapping for Pompe Disease

Primary Diagnosis Code

E74.02 – Pompe disease is the billable, specific ICD-10-CM code for acid α-glucosidase deficiency. This code has remained unchanged since 2016 and is valid through 2025.

Supporting Documentation for E74.02

To support accurate coding and prevent denials:

  • GAA enzyme assay showing <40% of lab-specific normal mean
  • Genetic testing confirming biallelic pathogenic GAA variants
  • Clinical phenotype documentation (cardiomyopathy, skeletal muscle weakness, respiratory involvement)
  • Age at onset (infantile vs. late-onset Pompe disease)

While E74.02 is primary, you may need additional codes for complications:

  • I42.0 – Dilated cardiomyopathy (if cardiac involvement)
  • G71.2 – Congenital myopathies (if muscle weakness is prominent)
  • J44.1 – Chronic obstructive pulmonary disease with acute exacerbation (if respiratory complications)

Product Coding: HCPCS, J-Code, and NDC Overview

HCPCS J-Code

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

  • Each billable unit = 10 mg
  • Total units = total mg administered ÷ 10
  • Used on Box 24D (CMS-1500) or appropriate revenue code (UB-04)

NDC Numbers

Lumizyme 50 mg single-use vials:

  • 58468-0160-1 – carton of 1 vial
  • 58468-0160-2 – carton of 10 vials

Report in 11-digit format (5-4-2) with unit of measure UN (unit) and quantity matching actual vials used.

Units Calculation Formula

Clinical dose: Weight (kg) × 20 mg/kg = total mg HCPCS units: Total mg ÷ 10 mg = billing units Vials needed: Total mg ÷ 50 mg (round up to whole vial)

Example: 68 kg patient

  • Dose: 68 × 20 = 1,360 mg
  • Billing units: 1,360 ÷ 10 = 136 units J0221
  • Vials needed: 1,360 ÷ 50 = 27.2 → 28 vials

Clean Request Anatomy: Example PA with Callouts

Sample Prior Authorization Request

Patient Information:

  • Name: [Patient Name]
  • DOB: [Date]
  • BCBS ID: [Member ID]
  • ICD-10: E74.02 (Pompe disease)

Drug Information:

  • Drug: Lumizyme (alglucosidase alfa)
  • HCPCS: J0221
  • NDC: 58468-0160-1
  • Dose: 20 mg/kg IV every 2 weeks
  • Units per dose: [Calculate based on weight]

Clinical Documentation:

  1. Confirmed diagnosis via GAA enzyme assay and/or genetic testing
  2. Current weight and calculated dose
  3. Baseline assessments (pulmonary function, motor function, cardiac status)
  4. Treatment goals and monitoring plan
Counterforce Health Tip: Our platform automatically calculates correct HCPCS units and identifies the specific clinical criteria each BCBS plan requires, helping prevent common coding errors that lead to denials.

Frequent Pitfalls: Unit Conversions and Code Mismatches

Common Billing Errors

Error Consequence Correction
Using vials as billing units (28 vials = 28 units) Under-billing, revenue loss Use mg-based calculation (1,360 mg = 136 units)
Rounding up HCPCS units Over-billing, audit risk Bill actual mg administered ÷ 10
Outdated weight in calculations Incorrect dosing, denials Update weight before each infusion
Wrong frequency documentation Medical necessity denials Confirm every 2 weeks per FDA label
Missing NDC-HCPCS alignment Claims edits, processing delays Verify NDC matches J0221 requirements

Wastage Documentation

For single-use vials, document:

  • Total vials opened and lot numbers
  • Actual mg administered
  • Wasted amount (if applicable, with JW modifier)

Some BCBS plans require separate billing lines for documented wastage using the same J0221 code with modifier JW.

Verification Steps: Cross-Check with BCBS Resources

Plan-Specific Verification

  1. Identify plan type: Commercial, ASO, Medicaid, Medicare Advantage
  2. Check PA requirements: Use current BCBS Texas PA code lists
  3. Verify submission method: Availity, Blue Approvr, or delegated vendor (like eviCore)

Key Resources

Submission Channels

  1. Availity Authorization & Referrals (preferred electronic method)
  2. Blue Approvr (automated medical PA tool)
  3. Phone: PA number on member ID card

Quick Audit Checklist: Pre-Submission Review

Documentation Review

  • ICD-10 E74.02 confirmed with diagnostic test results
  • Current patient weight documented in kg
  • Dose calculation shows 20 mg/kg every 2 weeks
  • HCPCS units match actual mg administered ÷ 10
  • NDC format is 11-digit (58468-0160-xx)
  • Vial count rounds up from mg calculation
  • Baseline assessments support medical necessity

Coding Accuracy

  • J0221 units = administered dose ÷ 10 mg
  • NDC matches vial strength and package size
  • Diagnosis code aligns with confirmed Pompe disease
  • Frequency matches FDA-approved dosing
  • Site of care meets plan requirements

Authorization Alignment

  • PA request matches calculated dose and units
  • Plan type verified (commercial, Medicaid, etc.)
  • Submission method confirmed for plan
  • Required clinical criteria addressed

Internal Appeal Process

If Lumizyme is denied due to coding issues:

  1. Review denial reason - often "not medically necessary" or "dosing exceeds guidelines"
  2. Gather documentation - enzyme assays, genetic testing, weight records
  3. Submit internal appeal within 60 days to:
    • Mail: Blue Cross Blue Shield of Texas, ATTN: Complaints and Appeals Department, P.O. Box 660717, Dallas, TX 75266-0717
    • Fax: 1-855-235-1055
    • Phone: Customer Advocate 1-888-657-6061

External Review Rights

Texas law provides Independent Review Organization (IRO) review for medical necessity denials:

  • Available after internal appeal completion
  • Deadline: Within 4 months of final internal denial
  • Cost: No charge to patient; BCBS pays IRO fees
  • Binding: IRO decision is final and binding on BCBS

Contact Texas Department of Insurance Consumer Help Line at 1-800-252-3439 for guidance on external review rights.

"From Our Advocates" Sidebar

In our experience helping families navigate Pompe disease coverage, the most successful appeals combine precise coding with compelling clinical narratives. We've seen cases where initial denials for "excessive dosing" were overturned simply by including updated weight documentation and recalculated HCPCS units that matched FDA labeling. The key is ensuring every number on your claim tells the same clinical story.

Counterforce Health helps patients, clinicians, and specialty pharmacies get prescription drugs approved by turning insurance denials into targeted, evidence-backed appeals. Our platform automatically identifies denial reasons and drafts point-by-point rebuttals aligned to each plan's specific rules, pulling the right clinical evidence and operational details payers expect. Learn more about our coverage support services.

Sources & Further Reading

Disclaimer: This guide is for informational purposes only and does not constitute medical, legal, or financial advice. Insurance coverage decisions are made by individual payers based on specific plan terms and medical circumstances. Always consult with healthcare providers and insurance representatives for personalized guidance. For assistance with Texas insurance complaints or appeals, contact the Texas Department of Insurance at 1-800-252-3439.

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