By Counterforce Health Team in Lumizyme (alglucosidase alfa) —

The Requirements Checklist to Get Lumizyme (Alglucosidase Alfa) Covered by UnitedHealthcare in North Carolina: Forms, Appeals, and Timeline

Answer Box: Getting Lumizyme Covered by UnitedHealthcare in North Carolina

UnitedHealthcare requires prior authorization for Lumizyme (alglucosidase alfa) with confirmed Pompe disease diagnosis (ICD-10: E74.02), GAA enzyme deficiency testing, and specialist prescriber. Submit via the UnitedHealthcare Provider Portal with complete clinical documentation. If denied, North Carolina residents can appeal through UHC's internal process, then request external review through Smart NC at 1-855-408-1212 within 120 days.

First step today: Verify active UHC coverage and gather GAA enzyme test results, genetic testing, and pulmonary function tests from your specialist.

Table of Contents

  1. Who Should Use This Checklist
  2. Member & Plan Basics
  3. Clinical Criteria Requirements
  4. Coding and Billing Information
  5. Documentation Packet Essentials
  6. Submission Process
  7. Appeals Playbook for North Carolina
  8. Common Denial Reasons & How to Fix Them
  9. Costs & Financial Assistance
  10. Quick Reference Checklist

Who Should Use This Checklist

This guide is for patients with confirmed or suspected Pompe disease seeking UnitedHealthcare coverage for Lumizyme (alglucosidase alfa) in North Carolina, along with their caregivers and healthcare providers.

Use this checklist if you:

  • Have a confirmed Pompe disease diagnosis requiring enzyme replacement therapy
  • Received a UnitedHealthcare denial for Lumizyme
  • Are preparing an initial prior authorization request
  • Need to appeal a coverage decision in North Carolina

Expected outcomes: With complete documentation following this checklist, most medically appropriate Lumizyme requests receive approval within 15 business days. Appeals through North Carolina's Smart NC program overturn approximately 50% of denials.

Member & Plan Basics

Verify Your Coverage

Before starting the prior authorization process:

  • Check active enrollment: Log into MyUHC.com or call member services (number on your insurance card)
  • Confirm plan type: State-regulated plans qualify for North Carolina external review; self-funded employer plans may not
  • Review deductible status: Lumizyme falls under medical benefits with typical 20% coinsurance after deductible

Prior Authorization Requirements

UnitedHealthcare requires prior authorization for all Lumizyme requests, with annual renewal for continuation therapy.

Clinical Criteria Requirements

Diagnosis Confirmation

ICD-10 Code: E74.02 (Pompe disease/glycogen storage disease type II)

Required testing documentation:

  • GAA enzyme activity assay: <1% of normal mean (infantile-onset) or <40% of normal (late-onset)
  • Genetic testing: GAA gene mutation analysis (if enzyme testing inconclusive)
  • Clinical symptoms: Muscle weakness, respiratory insufficiency, or cardiomyopathy

Age-Specific Criteria

Patient Type Key Requirements
Infantile-onset GAA <1% normal; hypertrophic cardiomyopathy; respiratory/skeletal weakness
Late-onset GAA <40% (two assays or genetic confirmation); FVC 30-79% predicted; 6-minute walk test ≥40m; lower extremity weakness

Prescriber Requirements

Lumizyme must be prescribed by a specialist experienced in treating Pompe disease, typically:

  • Neurologist
  • Geneticist
  • Metabolic specialist
  • Pediatric specialist (for infantile cases)

Coding and Billing Information

HCPCS Coding

Primary Code: J0221 - Alglucosidase alfa (Lumizyme), 10 mg

Billing calculation: Standard dosing is 20 mg/kg every 2 weeks

  • Example: 68 kg patient = 1,360 mg total dose = 136 billing units
  • UnitedHealthcare typically allows up to 230 units (2,300 mg) every 14 days

Site of Care

Lumizyme is administered via IV infusion in clinical settings:

  • Hospital outpatient departments
  • Infusion centers
  • Physician offices with infusion capabilities

Documentation Packet Essentials

Core Documents Required

  1. Completed prior authorization form (obtain current version from UHC Provider Portal)
  2. Medical necessity letter from prescribing specialist
  3. Diagnostic test results:
    • GAA enzyme assay report
    • Genetic testing results (if applicable)
    • Pulmonary function tests
    • 6-minute walk test results
  4. Clinical notes documenting symptoms and functional status
  5. Treatment history (if applicable)

Medical Necessity Letter Components

Your specialist should include these elements:

Clinical Documentation Checklist:Patient demographics and insurance informationConfirmed Pompe disease diagnosis with ICD-10 code E74.02GAA enzyme deficiency test results with laboratory reference rangesClinical symptoms and functional assessmentsRationale for Lumizyme therapy per FDA labelingProposed dosing schedule (20 mg/kg every 2 weeks)Monitoring plan and expected outcomesPrescriber credentials and contact information

Submission Process

Primary Submission Method

UnitedHealthcare Provider Portal: uhcprovider.com

  • Upload all documents digitally
  • Track authorization status in real-time
  • Receive determination notifications

Alternative Methods

  • Phone: OptumRx at 888-397-8129 for pharmacy benefit questions
  • Fax: Use fax number provided on current PA form (verify with portal)

Timeline Expectations

Review Type Standard Timeline Expedited Timeline
Initial Review 15 business days 72 hours (urgent cases)
Additional Information 15 days from receipt 72 hours
Appeal Response 30 days (pre-service) 72 hours

Appeals Playbook for North Carolina

Internal Appeals Process

Level 1 Standard Appeal

  • Deadline: 180 days from denial date
  • Submit via: UHC Provider Portal or MyUHC.com member portal
  • Response time: 30 days (pre-service) or 60 days (post-service)
  • Include: Enhanced medical necessity letter with additional clinical evidence

Level 2 Peer-to-Peer Review

  • Request within: 21 days (outpatient) or 3 business days (inpatient)
  • Process: Prescribing specialist discusses case with UHC medical director
  • Duration: 5-10 minute scheduled call
  • Often resolves: Cases without formal written appeal

North Carolina External Review

After exhausting UHC internal appeals, North Carolina residents can request external review through Smart NC.

Smart NC Contact Information:

  • Phone: 1-855-408-1212 (free advocacy assistance)
  • Website: NC Department of Insurance
  • Eligibility: State-regulated plans; medical necessity denials
  • Deadline: 120 days from final internal denial
  • Cost: Free (insurer pays fees)

Timeline:

  • Standard review: 45 days maximum
  • Expedited review: 72 hours (urgent cases like delayed Lumizyme)
  • Success rate: Approximately 50% of denials overturned statewide
From our advocates: We've seen Pompe disease cases succeed in external review when families included objective functional decline data (like declining 6-minute walk distances over months) alongside the genetic testing. The independent reviewers particularly value seeing how delays in enzyme replacement therapy could worsen irreversible muscle damage.

Common Denial Reasons & How to Fix Them

Denial Reason Fix Strategy Required Documentation
Missing genetic/enzyme confirmation Submit complete diagnostic workup GAA assay results with lab reference ranges
Non-specialist prescriber Transfer care to qualified specialist Referral to neurologist/geneticist
Incomplete dosing justification Provide weight-based calculation Patient weight, dosing formula (20 mg/kg q2w)
"Experimental" determination Cite FDA approval and guidelines FDA label, peer-reviewed studies
Step therapy requirement Document contraindications to alternatives Medical reasons other ERTs inappropriate

Costs & Financial Assistance

Manufacturer Support

Sanofi Patient Assistance:

  • Lumizyme Patient Support Program
  • Website: lumizyme.com (verify current programs)
  • Eligibility: Income-based assistance for qualifying patients

Additional Resources

  • National Organization for Rare Disorders (NORD): Financial assistance programs
  • HealthWell Foundation: Copay assistance for eligible rare disease patients
  • North Carolina prescription assistance programs: Contact Smart NC for local resources

Quick Reference Checklist

Before You Start

  • Verify active UnitedHealthcare coverage
  • Confirm plan type (state-regulated vs. self-funded)
  • Gather insurance card and member ID

Clinical Requirements

  • Confirmed Pompe disease diagnosis (ICD-10: E74.02)
  • GAA enzyme deficiency test results
  • Specialist prescriber (neurologist/geneticist)
  • Pulmonary function tests (for late-onset)
  • 6-minute walk test results (for late-onset)

Documentation Package

  • Current UHC prior authorization form
  • Medical necessity letter from specialist
  • Complete diagnostic test results
  • Clinical notes documenting symptoms
  • Treatment history (if applicable)

Submission

  • Submit via UHC Provider Portal
  • Track authorization status
  • Note confirmation numbers
  • Set calendar reminder for follow-up

If Denied

  • Request peer-to-peer review within 21 days
  • File Level 1 internal appeal within 180 days
  • Gather additional clinical evidence
  • Contact Smart NC if internal appeals exhausted

Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into targeted, evidence-backed appeals. Our platform analyzes denial letters and plan policies to draft point-by-point rebuttals aligned with payer requirements, pulling the right clinical evidence and procedural details to strengthen your case. Visit counterforcehealth.org to learn how we can help streamline your prior authorization and appeals process.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions vary by individual circumstances and plan details. Always consult with your healthcare provider and contact UnitedHealthcare directly for current policy information and personalized guidance. For additional support with North Carolina insurance appeals, contact Smart NC at 1-855-408-1212.

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