By Counterforce Health Team in Naglazyme (galsulfase) —

How to Get Naglazyme (Galsulfase) Covered by Cigna in Virginia: Complete Coding, Prior Authorization, and Appeals Guide

Quick Answer: Getting Naglazyme Covered by Cigna in Virginia

Naglazyme (galsulfase) requires Cigna prior authorization with confirmed MPS VI diagnosis (ICD-10 E76.29), specialist prescription, and functional assessments. Submit using HCPCS J1458 (1 mg = 1 unit), include enzyme assay results and baseline 6-minute walk test. If denied, file internal appeal within 180 days, then Virginia external review within 120 days through the State Corporation Commission. Start by having your genetics specialist complete Cigna's PA form via CoverMyMeds or provider portal.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit
  2. ICD-10 Mapping for MPS VI
  3. Product Coding: HCPCS J1458 and NDC
  4. Clean Prior Authorization Request
  5. Common Coding Pitfalls
  6. Verification with Cigna Resources
  7. Appeals Process in Virginia
  8. Quick Audit Checklist

Coding Basics: Medical vs. Pharmacy Benefit Paths

Naglazyme (galsulfase) is billed under the medical benefit, not pharmacy benefit, because it's administered as an IV infusion in healthcare settings. This affects how you approach coverage:

Medical Benefit Path (Naglazyme):

Key Difference: Unlike oral specialty drugs that go through pharmacy benefits, Naglazyme requires coordination between your prescribing specialist and the infusion facility for both coding and billing.

ICD-10 Mapping for MPS VI

The primary diagnosis code for mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) is E76.29 (Other mucopolysaccharidoses). This code encompasses both mild and severe forms of MPS VI.

Documentation Requirements for E76.29

Required for diagnosis confirmation:

  • Enzyme assay: Arylsulfatase B (ARSB) activity <10% of normal in fibroblasts or leukocytes
  • Genetic testing: Pathogenic mutations in the ARSB gene
  • Clinical features: Document specific symptoms like dysostosis multiplex, corneal clouding, hepatosplenomegaly

According to clinical guidelines, enzyme assay is typically the first-line diagnostic test, followed by genetic confirmation if enzyme levels are deficient.

Note: Clinical features alone (short stature, joint stiffness, cardiac valve abnormalities) support suspicion but require biochemical or genetic verification for proper coding and treatment approval.

Product Coding: HCPCS J1458 and NDC

HCPCS Code Details

J1458 - Injection, galsulfase, 1 mg

Vial Configuration and Unit Calculation

Naglazyme vial specs:

  • 5 mg/5 mL solution per single-dose vial
  • NDC: 68135-0020-xx
  • Each vial = 5 billable units

Example calculation for a 35 kg patient:

  • Dose needed: 35 mg (35 kg × 1 mg/kg)
  • Vials required: 7 vials (35 mg ÷ 5 mg per vial)
  • Billable units: 35 units

Administration Codes

CPT codes for infusion:

  • 96365: IV infusion, initial, up to 1 hour
  • 96366: Each additional hour

Important: Naglazyme is not on the CMS wastage modifier list, so JW/JZ modifiers are not required for partial vials.

Clean Prior Authorization Request

Required Clinical Documentation

Cigna's PA criteria for Naglazyme include:

Requirement Documentation Needed Source
Confirmed MPS VI diagnosis Enzyme assay results, genetic testing ARSB activity <10% normal
Specialist prescriber Genetics or metabolism specialist Required per Cigna policy
Baseline assessments 6-minute walk test, pulmonary function tests Functional capacity measures
Dosing justification 1 mg/kg weekly, weight-based calculation FDA-approved dosing

Sample PA Request Structure

1. Patient Information

  • Member ID, DOB, diagnosis (E76.29)
  • Prescribing specialist credentials

2. Clinical Justification

  • Enzyme assay results showing ARSB deficiency
  • Baseline functional assessments (walk test, PFTs)
  • Documentation of disease progression without treatment

3. Treatment Plan

  • Weekly infusion schedule at certified facility
  • Monitoring plan for infusion reactions
  • Expected clinical outcomes

Common Coding Pitfalls

Unit Conversion Errors

Mistake: Billing by vial count instead of mg units Fix: Always calculate total mg needed, then convert to J1458 units (1:1 ratio)

Mismatched Diagnosis Codes

Mistake: Using general metabolic disorder codes Fix: Specifically use E76.29 for MPS VI with supporting documentation

Missing Administration Codes

Mistake: Billing only the drug (J1458) without infusion codes Fix: Include CPT 96365/96366 for administration time

Site of Care Issues

Mistake: Home infusion billing without proper justification Fix: Document why hospital/infusion center is medically necessary (anaphylaxis risk)

Clinician Corner: When writing the medical necessity letter, emphasize that Naglazyme carries an FDA warning for life-threatening hypersensitivity reactions, requiring administration in facilities equipped for emergency management.

Verification with Cigna Resources

Pre-Submission Checks

1. Formulary Status

2. Provider Network

  • Confirm infusion facility is in-network
  • Verify specialist has Cigna credentials

3. PA Requirements

Cross-Reference Tools

  • Cigna Provider Portal: Real-time PA status
  • CoverMyMeds: Electronic PA submission
  • Member Services: 800-882-4462 for coverage verification

Appeals Process in Virginia

Internal Appeals with Cigna

Timeline: File within 180 days of initial denial

Process:

  1. Submit appeal with additional documentation
  2. Standard review: Up to 60 days
  3. Expedited review: 72 hours (if delay risks health)

Required documents:

  • Original denial letter
  • Updated clinical records
  • Peer-reviewed literature supporting medical necessity

Virginia External Review

If Cigna's internal appeals are exhausted, Virginia residents can file for external review through the State Corporation Commission.

Key Details:

  • Deadline: 120 days from final internal denial
  • Cost: Free for Virginia residents
  • Timeline: 45 days standard, 72 hours expedited
  • Decision: Binding on Cigna

Required Forms:

Contact: Virginia SCC Bureau of Insurance at 877-310-6560

From our advocates: We've seen several MPS VI patients succeed in Virginia external review by including detailed functional assessments showing disease progression. The independent medical reviewers particularly value before-and-after walk test data and specialist letters explaining why enzyme replacement therapy is the only FDA-approved treatment option.

Quick Audit Checklist

Before submitting your PA request:

✓ Diagnosis: E76.29 with enzyme assay documentation
✓ Prescriber: Genetics/metabolism specialist credentials verified
✓ Coding: J1458 units calculated correctly (mg = units)
✓ Dosing: 1 mg/kg weekly with patient weight documented
✓ Site: Infusion facility network status confirmed
✓ Assessments: Baseline 6-minute walk test and PFTs included
✓ Safety: Anaphylaxis management plan documented
✓ Submission: PA form complete via CoverMyMeds or provider portal

When Prior Authorization Gets Denied

Common Denial Reasons & Solutions

Denial Reason Fix Strategy Required Documentation
Unconfirmed diagnosis Submit enzyme assay results ARSB activity <10% normal
Not medically necessary Provide functional decline data Walk test, PFT trends
Experimental treatment Cite FDA approval Package insert, clinical trials
Site of care issues Justify infusion center need Anaphylaxis risk documentation

Escalation Resources

Virginia-Specific Support:

  • Managed Care Ombudsman: Informal resolution assistance
  • Virginia Poverty Law Center: Legal help for complex denials
  • SCC Consumer Services: Guidance on external review process

Cost Considerations and Support

Estimated annual cost: $600,000-$1.8M+ depending on patient weight

Financial assistance options:

  • BioMarin patient assistance programs
  • Rare disease foundation grants
  • Virginia Medicaid (if eligible after expansion)

Insurance optimization:

  • Verify in-network infusion centers
  • Understand copay/coinsurance structure
  • Consider Medicare Part B if dual-eligible

About Counterforce Health: Counterforce Health helps patients and clinicians navigate complex prior authorization and appeals processes for specialty medications. Our platform analyzes denial letters and creates evidence-backed appeals tailored to specific payer policies, helping turn insurance denials into approvals through targeted, clinical documentation.

For patients facing Naglazyme denials, Counterforce Health can help identify the specific denial basis—whether it's PA criteria, step therapy, or medical necessity—and draft point-by-point rebuttals aligned to Cigna's own coverage rules, incorporating the right clinical evidence and procedural requirements for Virginia appeals.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions depend on individual circumstances and plan specifics. Always consult with your healthcare provider and insurance plan for guidance specific to your situation. For official appeals guidance in Virginia, contact the State Corporation Commission Bureau of Insurance at 877-310-6560.

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