By Counterforce Health Team in Naglazyme (galsulfase) —

The Requirements Checklist to Get Naglazyme (galsulfase) Covered by Aetna (CVS Health) in North Carolina

Answer Box: Getting Naglazyme (galsulfase) Covered by Aetna (CVS Health) in North Carolina

Who's eligible: Patients with confirmed MPS VI (Maroteaux-Lamy syndrome) via enzyme assay or genetic testing. Fastest path: Submit Aetna's precertification form with complete diagnostic documentation 2-3 weeks before treatment. Start today: Contact your metabolic specialist to gather enzyme/genetic test results and baseline functional assessments. If denied, North Carolina's Smart NC external review (1-855-408-1212) offers binding decisions within 45 days (72 hours for urgent cases).

Table of Contents

  1. Who Should Use This Checklist
  2. Member & Plan Basics
  3. Clinical Criteria Requirements
  4. Coding & Billing Information
  5. Documentation Packet
  6. Submission Process
  7. Specialty Pharmacy Routing
  8. After Submission: What to Expect
  9. Denial Prevention Tips
  10. Appeals Process in North Carolina
  11. Quick Reference Checklist

Who Should Use This Checklist

This guide is for patients with MPS VI (Maroteaux-Lamy syndrome) and their healthcare teams seeking Naglazyme (galsulfase) coverage through Aetna (CVS Health) in North Carolina. You'll need this if:

  • You have a confirmed MPS VI diagnosis and need enzyme replacement therapy
  • Your doctor has prescribed Naglazyme for weekly IV infusions
  • You're facing prior authorization requirements or coverage denials
  • You need to transfer to CVS Specialty Pharmacy or another preferred network provider

Expected outcome: With complete documentation, most medically appropriate Naglazyme requests are approved. If denied initially, North Carolina's external review process has helped overturn many specialty drug denials when proper clinical evidence is presented.

Member & Plan Basics

Coverage Verification

Before starting the authorization process:

  • Confirm active coverage: Verify your Aetna plan is active and includes specialty drug benefits
  • Check plan type: Most Aetna plans process Naglazyme under the medical benefit (not pharmacy benefit) since it requires IV infusion
  • Understand your costs: Deductibles and coinsurance apply; high-cost specialty drugs may have separate cost-sharing tiers
Note: Naglazyme costs approximately $600,000-$1.8 million annually based on patient weight, making prior authorization standard across all plans.

Authorization Requirements

All Aetna plans require prior authorization for Naglazyme. The process typically takes 30-45 days for standard reviews, with expedited options available for urgent medical situations.

Clinical Criteria Requirements

FDA-Approved Indication

Naglazyme must be prescribed for its FDA-approved indication: enzyme replacement therapy for MPS VI (mucopolysaccharidosis VI, Maroteaux-Lamy syndrome).

Diagnostic Confirmation

Aetna requires both of the following:

  1. Enzyme assay results showing N-acetylgalactosamine-4-sulfatase (arylsulfatase B) deficiency
  2. Genetic testing confirming pathogenic or likely pathogenic variants in the ARSB gene

Specialist Involvement

The prescription must be from or in consultation with a specialist experienced in metabolic or lysosomal storage disorders. Include the specialist's credentials and contact information.

Baseline Functional Assessments

Document current functional status with:

  • Six-minute walk test results
  • Stair climbing capacity assessment
  • Joint range of motion measurements
  • Cardiac and pulmonary function tests
  • Quality of life assessments (when available)

Dosing Requirements

  • Standard dose: 1 mg/kg IV infusion once weekly
  • Administration: Must occur in a certified infusion center with trained staff and emergency protocols
  • Duration: Ongoing therapy with periodic reassessment

Coding & Billing Information

Required Codes

  • HCPCS J-code: J1458 (Injection, galsulfase, 1 mg per unit)
  • ICD-10 diagnosis: E76.29 (Other mucopolysaccharidoses, including MPS VI)
  • NDC number: 68135-0020-01 (5 mg/5 mL single-dose vial)

Units Calculation

Bill one unit of J1458 per mg administered. For example:

  • 25 kg patient = 25 mg dose = J1458 x 25 units
  • 70 kg patient = 70 mg dose = J1458 x 70 units
Billing Note: Naglazyme is processed under the medical benefit, not pharmacy benefit, due to required IV administration in a medical facility.

Documentation Packet

Medical Necessity Letter Components

Your healthcare provider should include:

  1. Patient demographics and insurance information
  2. Confirmed MPS VI diagnosis with supporting laboratory/genetic evidence
  3. Clinical summary of disease progression and current symptoms
  4. Baseline functional assessments with specific test results
  5. Treatment plan including dosing, schedule, and infusion site details
  6. Monitoring plan for ongoing assessment and reauthorization

Required Attachments

  • Laboratory reports showing enzyme deficiency
  • Genetic testing results confirming ARSB mutations
  • Recent functional assessment reports
  • Specialist consultation notes
  • Previous treatment history (if applicable)

Site of Care Documentation

Include details about the proposed infusion center:

  • Facility certification and staff training
  • Emergency management protocols
  • Monitoring equipment availability
  • Patient safety measures

Submission Process

Forms and Portals

  1. Download Aetna's Naglazyme precertification form
  2. Complete all required sections—incomplete forms cause delays
  3. Submit via Aetna provider portal or fax (verify current number on form)

Common Rejection Causes

  • Missing prescriber signature (stamps not accepted)
  • Incomplete insurance information
  • Missing diagnostic test results
  • Insufficient clinical documentation
  • Wrong form version

Timeline

  • Submit: 2-3 weeks before planned treatment initiation
  • Standard review: 30-45 days for decision
  • Expedited review: 72 hours for urgent medical situations

Specialty Pharmacy Routing

CVS Specialty Enrollment

Since Aetna often requires CVS Specialty for high-cost medications:

  1. Complete the CVS Specialty Lysosomal Storage Disorders enrollment form
  2. Provide complete patient, prescriber, and insurance information
  3. Include prescription details and clinical documentation
  4. Submit with prescriber signature (required)

Transfer Process

If transferring from another specialty pharmacy:

  • Coordinate with both current pharmacy and CVS Specialty
  • Ensure uninterrupted medication supply during transfer
  • Confirm prior authorization transfers with new pharmacy

BioMarin RareConnections Support

BioMarin RareConnections (1-866-906-6100) provides:

  • Enrollment assistance
  • Insurance navigation
  • Prior authorization support
  • Financial assistance programs

After Submission: What to Expect

Confirmation and Tracking

  • Record confirmation numbers from all submissions
  • Track status through Aetna provider portal
  • Follow up if no response within expected timeframes

Approval Process

Once approved:

  • Coordinate with specialty pharmacy for medication delivery
  • Schedule infusion appointments
  • Confirm site of care authorization

Ongoing Requirements

  • Annual reauthorization typically required
  • Updated functional assessments to demonstrate clinical benefit
  • Continued specialist oversight

Denial Prevention Tips

Five Common Pitfalls and Solutions

  1. Incomplete diagnostic documentation
    • Solution: Include both enzyme assay and genetic testing results
  2. Missing baseline functional measures
    • Solution: Complete all required assessments before submission
  3. Inadequate specialist involvement
    • Solution: Ensure prescription is from qualified metabolic specialist
  4. Site of care issues
    • Solution: Verify infusion center meets all safety requirements
  5. Form errors or omissions
    • Solution: Double-check all fields and required signatures

Appeals Process in North Carolina

Internal Appeals with Aetna

If your initial request is denied:

  1. Submit internal appeal within 180 days of denial notice
  2. Include additional clinical documentation addressing denial reasons
  3. Request peer-to-peer review with Aetna medical director
  4. Timeline: 30 days for standard appeals, 72 hours for expedited

Smart NC External Review

North Carolina offers a powerful external review system:

  • Contact: Smart NC at 1-855-408-1212 for free advocacy support
  • Timeline: Submit within 120 days of final internal denial
  • Decision time: 45 days for standard review, 72 hours for expedited
  • Binding: Smart NC decisions are binding on insurers
From our advocates: We've seen several MPS VI cases where initial denials were overturned through Smart NC external review. The key was presenting comprehensive functional assessment data showing the patient's decline without treatment and potential for improvement with Naglazyme. These cases typically benefit from the independent medical expertise that Smart NC provides.

When to Request Expedited Review

Request expedited appeals when:

  • Delay could seriously jeopardize health
  • Patient is experiencing rapid functional decline
  • Treatment interruption poses significant risks

For rare diseases like MPS VI, Counterforce Health helps patients and clinicians turn insurance denials into targeted, evidence-backed appeals by identifying the specific denial basis and crafting point-by-point rebuttals aligned with the plan's own rules.

Quick Reference Checklist

Before You Start

  • Confirm active Aetna coverage
  • Verify specialty drug benefits
  • Identify qualified metabolic specialist
  • Gather insurance cards and member information

Clinical Requirements

  • Enzyme assay showing arylsulfatase B deficiency
  • Genetic testing confirming ARSB mutations
  • Six-minute walk test results
  • Stair climbing assessment
  • Joint range of motion measurements
  • Cardiac and pulmonary function tests
  • Specialist consultation notes

Documentation Packet

  • Complete Aetna precertification form
  • Medical necessity letter
  • Laboratory and genetic test reports
  • Functional assessment results
  • Site of care authorization details
  • Prescriber signature (no stamps)

Submission

  • Submit 2-3 weeks before treatment start
  • Use current form version
  • Include all required attachments
  • Record confirmation numbers
  • Set follow-up reminders

If Denied

  • Review denial letter carefully
  • Submit internal appeal within 180 days
  • Consider peer-to-peer review
  • Contact Smart NC if internal appeals fail
  • Request expedited review if urgent

Disclaimer: This information is for educational purposes only and does not constitute medical advice. Always consult with your healthcare provider and insurance company for specific coverage decisions. Requirements may vary by plan and change over time.

Sources & Further Reading

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