By Counterforce Health Team in Lumizyme (alglucosidase alfa) —

Complete Guide: Getting Lumizyme (Alglucosidase Alfa) Covered by UnitedHealthcare in Texas - ICD-10 Codes, HCPCS Billing, and Appeals Process

Quick Answer: Getting Lumizyme Covered by UnitedHealthcare in Texas

UnitedHealthcare requires prior authorization for Lumizyme (alglucosidase alfa) in Texas. The fastest path to approval requires: (1) confirmed Pompe disease diagnosis with ICD-10 code E74.02, (2) genetic testing or enzyme assay documentation, and (3) medical necessity letter from a specialist. Submit through UnitedHealthcare Provider Portal with HCPCS code J0221 for billing (10 mg units). If denied, Texas law guarantees external review by an Independent Review Organization (IRO). Start today: Gather diagnostic test results and contact your prescribing physician to initiate the prior authorization request.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Lumizyme (alglucosidase alfa) is typically covered under UnitedHealthcare's medical benefit rather than pharmacy benefit, since it's administered by IV infusion in clinical settings.

Medical Benefit Coverage

  • HCPCS J-code: J0221 (injection, alglucosidase alfa, 10 mg per unit)
  • Billing location: Hospital outpatient, infusion center, or physician office
  • Prior authorization: Required for all UnitedHealthcare plans
  • Coinsurance: Typically 20% after deductible for commercial plans

Pharmacy Benefit (Rare)

  • NDC required: NDC 58468-0077-01 for 50 mg vials
  • Use cases: "White bagging" arrangements where specialty pharmacy ships to provider
  • Coverage: Less common; requires special arrangement with OptumRx
Note: Always verify benefit type with UnitedHealthcare before submitting claims, as coverage pathways can vary by plan design.

ICD-10 Mapping for Pompe Disease

Primary Diagnosis Code

E74.02 - Pompe disease (Glycogen storage disease type II)

This code encompasses:

  • Cardiac glycogenosis
  • Type II glycogen storage disease
  • Acid α-glucosidase deficiency

Supporting Documentation Requirements

Your medical records must include:

  1. Diagnostic confirmation via genetic testing (GAA gene mutations) OR enzyme assay showing deficient acid alpha-glucosidase activity
  2. Clinical phenotype description (infantile-onset vs. late-onset)
  3. Symptom documentation such as:
    • Progressive muscle weakness
    • Respiratory insufficiency
    • Hypertrophic cardiomyopathy (infantile-onset)
    • Hepatomegaly
Tip: Include specific test values and reference ranges in your documentation. UnitedHealthcare reviewers look for quantitative evidence supporting the E74.02 diagnosis.

Product Coding: HCPCS, J-Codes, and NDC

HCPCS Billing Calculations

J0221 represents 10 mg of alglucosidase alfa. Here's how to calculate billing units:

Standard dosing: 20 mg/kg every 2 weeks

Example calculation for 68 kg patient:

  • Total dose: 68 kg × 20 mg/kg = 1,360 mg
  • Billing units: 1,360 mg ÷ 10 mg = 136 units
  • Vials needed: 1,360 mg ÷ 50 mg per vial = 27.2 → 28 vials (round up)

Coverage Limits

UnitedHealthcare typically allows up to 230 billing units (2,300 mg) every 14 days, which accommodates patients up to approximately 115 kg.

When Modifiers Apply

  • -JW modifier: Required when discarding unused drug from single-use vials
  • -GA modifier: Use when patient has signed an Advance Beneficiary Notice (Medicare patients)

Clean Request Anatomy

A complete prior authorization request includes:

Required Clinical Elements

Component Details Documentation
Diagnosis ICD-10: E74.02 Genetic test results or enzyme assay
Prescriber Specialist preferred Neurologist, geneticist, or metabolic specialist
Dosing 20 mg/kg every 2 weeks Weight-based calculation with units
Medical necessity Treatment rationale Letter addressing FDA-approved indication
Prior therapies Step therapy compliance Documentation of alternatives tried/failed

Sample Medical Necessity Letter Points

Your physician's letter should address:

  1. Confirmed Pompe disease diagnosis with test results
  2. Clinical symptoms impacting quality of life
  3. Treatment goals (stabilization, functional improvement)
  4. Monitoring plan for safety and efficacy
  5. Contraindications to alternative therapies (if step therapy applies)

Frequent Billing Pitfalls

Unit Conversion Errors

Common mistake: Billing total milligrams instead of 10 mg units

  • ❌ Wrong: 1,360 mg patient dose = 1,360 units
  • ✅ Correct: 1,360 mg patient dose = 136 units (1,360 ÷ 10)

Weight Documentation Mismatches

Claims are often denied when billed units don't align with documented patient weight. Ensure:

  • Current weight is documented in medical record
  • Calculation shows appropriate dosing (20 mg/kg)
  • Units billed match the calculated dose

Missing Prior Authorization

UnitedHealthcare requires PA for all Lumizyme prescriptions. Common issues:

  • Submitting claims before PA approval
  • PA expires before treatment completion
  • Dose changes without updated authorization

Verification Steps

Before submitting your request:

Check Formulary Status

  1. Log into UnitedHealthcare Provider Portal
  2. Verify Lumizyme is covered under medical benefit
  3. Confirm current PA requirements and forms

Validate Coding

  • ICD-10: E74.02 for Pompe disease
  • HCPCS: J0221 for billing (10 mg units)
  • NDC: 58468-0077-01 if required by payer
  • Units calculation: Total mg dose ÷ 10

Documentation Checklist

  • Genetic testing or enzyme assay results
  • Medical necessity letter from specialist
  • Current patient weight and dosing calculation
  • Prior therapy documentation (if step therapy applies)
  • Treatment monitoring plan

Pre-Submission Audit Checklist

Clinical Documentation Review

  • E74.02 diagnosis clearly documented
  • Diagnostic test results included (genetic or enzyme)
  • Specialist consultation notes present
  • Medical necessity letter addresses FDA indication
  • Patient weight current and accurate

Coding Verification

  • J0221 units calculated correctly (total mg ÷ 10)
  • Dosing aligns with 20 mg/kg standard
  • Modifiers applied appropriately (-JW, -GA if needed)
  • NDC included if required by plan

Administrative Elements

  • Prior authorization submitted and approved
  • Provider NPI and credentials verified
  • Site of care appropriate for IV infusion
  • Billing address and contact information current

Appeals Process in Texas

If UnitedHealthcare denies your Lumizyme request, Texas law provides strong appeal rights.

Internal Appeals (First Step)

Timeline: File within 180 days of denial Process:

  1. Submit written appeal addressing denial reasons
  2. Include additional clinical documentation
  3. Request expedited review for urgent medical needs
  4. UnitedHealthcare has 30 days to respond (pre-service) or 60 days (post-service)

External Review (Independent Review Organization)

When available: Medical necessity denials, experimental/investigational determinations Timeline: Request within 4 months of final internal denial Process:

  1. UnitedHealthcare provides IRO request form with denial letter
  2. Submit completed form with supporting documentation
  3. IRO reviews within 20 days (5 days for urgent cases)
  4. Decision is binding on UnitedHealthcare
Important: Self-funded (ERISA) employer plans may be exempt from Texas IRO process and follow federal appeal procedures instead.

Getting Help in Texas

  • Texas Department of Insurance: 1-800-252-3439
  • Office of Public Insurance Counsel: 1-877-611-6742
  • TDI IRO Information Line: 1-866-554-4926

When patients and clinicians need help navigating complex insurance denials for specialty medications like Lumizyme, Counterforce Health provides targeted support by analyzing denial letters and crafting evidence-based appeals that align with payer-specific requirements and state regulations.

FAQ

How long does UnitedHealthcare prior authorization take for Lumizyme in Texas? Standard PA reviews typically complete within 72 hours. Expedited reviews for urgent medical needs can be processed within 24 hours when properly documented.

What if Lumizyme is not on my UnitedHealthcare formulary? Lumizyme requires prior authorization regardless of formulary status. Non-formulary medications may require additional documentation but can still be covered with medical necessity justification.

Does step therapy apply to Lumizyme in Texas? Step therapy requirements vary by plan type. Newly enrolled Medicare Advantage members may need to try alternative enzyme replacement therapies first, unless contraindicated or previously failed.

Can I request an expedited appeal if my Lumizyme is denied? Yes, if delays would jeopardize your health. Submit expedited appeal requests with physician certification of urgent medical need. Texas IRO also offers expedited external review (5-day turnaround).

What happens if I exceed the 230-unit coverage limit? Doses exceeding standard limits require additional prior authorization with clinical justification. Document medical necessity for higher doses based on patient-specific factors.

How do I calculate HCPCS units for different patient weights? Use this formula: (Patient weight in kg × 20 mg/kg) ÷ 10 = billing units. Always round up to the nearest whole unit.

When to Escalate

Contact Texas regulators if:

  • UnitedHealthcare fails to respond within required timeframes
  • Appeal rights are not properly communicated
  • You believe the denial violates state or federal patient protection laws

File complaints with:

For complex cases involving rare disease medications, specialized platforms like Counterforce Health can help generate comprehensive appeals that address both clinical requirements and regulatory standards specific to Texas insurance law.

Sources & Further Reading

Disclaimer: This information is for educational purposes and does not constitute medical or legal advice. Insurance coverage decisions depend on individual plan benefits and clinical circumstances. Always consult with your healthcare provider and insurance plan for personalized guidance. Coverage policies and appeal procedures may change; verify current requirements with official sources.

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