By Counterforce Health Team in Naglazyme (galsulfase) —

Getting Naglazyme (Galsulfase) Covered by Aetna in Pennsylvania: Coding, Appeals, and Authorization Guide

Quick Answer: Getting Naglazyme Covered by Aetna in Pennsylvania

Naglazyme (galsulfase) for MPS VI requires prior authorization from Aetna with specific documentation: confirmed diagnosis via enzyme assay or genetic testing, baseline functional assessments (like six-minute walk tests), and specialist involvement. Bill using HCPCS J1458 under the medical benefit at 1 mg per unit. If denied, Pennsylvania's new external review process has overturned 50% of appeals in 2024. Start with Aetna's prior authorization form and gather enzyme/genetic test results plus functional capacity documentation.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Naglazyme (galsulfase) is processed under Aetna's medical benefit, not the pharmacy benefit. This enzyme replacement therapy requires IV infusion in medical settings, making it a "buy-and-bill" medication that providers purchase and bill using medical claims.

Key billing pathway:

  • Medical benefit: Uses HCPCS J1458, billed by infusion centers/hospitals
  • Pharmacy benefit: Not applicable (rare exceptions in specific employer plans)
  • CVS Specialty: May coordinate but doesn't change the medical benefit processing

The medical benefit path means your physician's office or infusion center handles prior authorization and billing, rather than you picking up the medication at a pharmacy.

ICD-10 Documentation Requirements

Primary Diagnosis Code

E76.29 - Other mucopolysaccharidoses (MPS VI/Maroteaux-Lamy syndrome)

This code requires comprehensive documentation to support medical necessity:

Documentation Type Requirement Details
Enzyme Assay Deficient arylsulfatase B activity Must specify tissue tested and reference ranges
Genetic Testing Pathogenic ARSB gene variants Include specific mutations and lab details
Clinical Features Multisystem involvement Joint contractures, skeletal dysplasia, cardiac/respiratory issues
Functional Assessment Baseline capacity measurements Six-minute walk test, stair climbing, endurance metrics

Supporting Documentation

  • Increased urinary glycosaminoglycans (particularly dermatan sulfate)
  • Family history or consanguinity if applicable
  • Age at symptom onset and progression timeline
  • Previous treatment attempts and outcomes
Tip: MPS VI typically presents without intellectual disability—document this distinction to differentiate from other MPS types.

HCPCS Billing and Units Calculation

Core Billing Information

  • HCPCS Code: J1458 (Injection, galsulfase, 1 mg)
  • NDC: 68135-0020-01 (5 mg/5 mL vial)
  • Billing Units: 1 unit = 1 mg administered
  • Dosing: 1 mg/kg body weight weekly

Units Calculation Examples

Patient Weight Weekly Dose Billing Units (J1458) Vials Needed
25 kg 25 mg 25 units 5 vials
45 kg 45 mg 45 units 9 vials
70 kg 70 mg 70 units 14 vials

Modifier Requirements

Current CMS policy does not require JW or JZ modifiers for Naglazyme, as it's not on the single-dose container list requiring wastage documentation. However, verify with your specific Aetna plan, as some may have unique requirements.

Note: Always bill the exact milligrams administered. If a patient receives 45 mg, bill J1458 x 45 units.

Prior Authorization: Building a Clean Request

Aetna requires comprehensive prior authorization using their specific Naglazyme form.

Required Elements for Approval

1. Specialist Involvement

  • Prescription by or consultation with metabolic/lysosomal storage disorder specialist
  • Include specialist's credentials and contact information

2. Diagnostic Confirmation

  • Laboratory report showing arylsulfatase B deficiency
  • Genetic testing results with specific ARSB mutations
  • Clinical summary supporting MPS VI diagnosis

3. Baseline Functional Assessments

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

4. Site of Care Authorization

  • Infusion center certification
  • Staff training documentation
  • Emergency management protocols
  • Monitoring equipment availability

Continuation Criteria

Re-authorization requires evidence of clinical benefit:

  • Stabilization or improvement in functional assessments
  • Maintained or improved walking capacity
  • Reduced hospitalizations or complications
  • Patient/family reported quality of life improvements

Common Coding Pitfalls to Avoid

Unit Conversion Errors

  • Wrong: Billing for vials instead of milligrams
  • Right: Calculate total mg dose, bill that number of J1458 units

Mismatched Benefit Categories

  • Wrong: Submitting to pharmacy benefit or CVS Caremark as retail
  • Right: Process through medical benefit with J-code

Missing Documentation

  • Wrong: Generic MPS diagnosis without subtype specification
  • Right: E76.29 with enzyme/genetic confirmation

Timing Issues

  • Wrong: Requesting authorization after treatment starts
  • Right: Submit PA 2-3 weeks before planned initiation

Site of Care Confusion

  • Wrong: Assuming drug authorization covers administration site
  • Right: Verify both drug and site of care are pre-authorized

Verification with Aetna Resources

Before submitting your prior authorization:

1. Check Current Formulary Status

2. Validate Provider Network Status

  • Ensure infusion site is in-network
  • Confirm specialist credentials are current
  • Verify facility meets Aetna's infusion center standards

3. Review Policy Updates

4. Confirm Submission Process

  • Use provider portal for electronic submission when possible
  • Verify fax numbers are current
  • Confirm required attachments and formats

Appeals Process in Pennsylvania

Pennsylvania introduced a new external review system in January 2024 that has overturned 50% of insurance denials.

Internal Appeals (Required First Step)

Timeline: Submit within 180 days of denial Process:

  1. Call Aetna member services to initiate
  2. Submit written appeal with supporting documentation
  3. Include updated clinical evidence
  4. Request peer-to-peer review if available

Standard Decision: 30-45 days Expedited Decision: 72 hours for urgent cases

Pennsylvania External Review

If Aetna upholds the denial, Pennsylvania residents can request independent external review:

Eligibility: Commercial insurance plans (not self-funded employer plans) Timeline: 4 months from final internal denial Process:

  1. Submit request at pa.gov/reviewmyclaim or call 1-877-881-6388
  2. PID determines eligibility within 5 business days
  3. Independent Review Organization (IRO) assigned
  4. Submit additional documentation within 15 business days
  5. Decision within 45 days (72 hours if urgent)

Success Rate: 50-53% of appeals overturned in favor of consumers Binding: IRO decisions are final and enforceable

From our advocates: We've seen families succeed by organizing all enzyme testing, genetic reports, and functional assessments into a clear timeline showing disease progression. The key is demonstrating that Naglazyme isn't experimental—it's FDA-approved ERT with established clinical benefits for confirmed MPS VI patients.

When to Escalate Further

If external review fails, contact:

  • Pennsylvania Insurance Department Consumer Services
  • Pennsylvania Attorney General's Office (healthcare division)
  • Consider consulting healthcare advocacy organizations

Pre-Submission Audit Checklist

Before submitting your Naglazyme prior authorization:

Diagnosis Documentation

  • E76.29 ICD-10 code properly documented
  • Enzyme assay results showing arylsulfatase B deficiency
  • Genetic testing confirming ARSB mutations
  • Clinical summary supporting MPS VI diagnosis

Coding Accuracy

  • J1458 HCPCS code for billing
  • NDC 68135-0020-01 for product identification
  • Correct unit calculation (1 mg = 1 billing unit)
  • Medical benefit pathway confirmed

Clinical Requirements

  • Specialist involvement documented
  • Baseline functional assessments completed
  • Six-minute walk test or equivalent capacity measure
  • Treatment goals clearly stated

Administrative Elements

  • Prior authorization form completed in full
  • Site of care authorization requested
  • Provider network status verified
  • Current formulary status confirmed

Supporting Documentation

  • Laboratory reports attached
  • Genetic testing results included
  • Functional assessment data provided
  • Specialist consultation notes attached

FAQ

How long does Aetna prior authorization take for Naglazyme in Pennsylvania? Standard prior authorization decisions are typically made within 30-45 days. Expedited reviews for urgent cases are completed within 72 hours. Submit requests 2-3 weeks before planned treatment initiation.

What if Naglazyme is non-formulary on my Aetna plan? Naglazyme may require formulary exception documentation. Provide evidence that it's FDA-approved for MPS VI, demonstrate medical necessity, and show that no formulary alternatives exist for this rare condition.

Can I request an expedited appeal if Aetna denies coverage? Yes, if there's urgent medical need. Document why delays would cause significant health deterioration. Expedited appeals must be decided within 72 hours for urgent cases.

Does step therapy apply to Naglazyme for MPS VI? Step therapy typically doesn't apply since Naglazyme is the only FDA-approved enzyme replacement therapy for MPS VI. If step therapy is required, document the lack of therapeutic alternatives.

What's the difference between medical and pharmacy benefit for Naglazyme? Naglazyme is covered under the medical benefit because it requires IV infusion in medical settings. It's billed using HCPCS J1458 rather than processed through pharmacy benefit channels.

How much does Naglazyme cost without insurance coverage? Annual costs range from approximately $600,000 to $1.8 million+ depending on patient weight, as dosing is 1 mg/kg weekly. Actual net costs vary based on negotiations and patient assistance programs.

What patient assistance is available for Naglazyme? BioMarin offers patient assistance programs through their RareConnections program. Additional support may be available through rare disease foundations and state pharmaceutical assistance programs.

Can I appeal to Pennsylvania if I have a self-funded employer plan? No, Pennsylvania's external review process only applies to state-regulated commercial insurance plans. Self-funded employer plans are regulated federally under ERISA and have different appeal processes.

Healthcare coverage is complex, especially for rare conditions like MPS VI. Counterforce Health helps patients, clinicians, and specialty pharmacies navigate insurance denials by creating targeted, evidence-backed appeals. Their platform ingests denial letters, plan policies, and clinical notes to draft point-by-point rebuttals aligned with each payer's specific requirements, significantly improving approval rates for specialty medications like Naglazyme.

When facing coverage challenges, having the right documentation and following proper procedures can make the difference between approval and denial. Pennsylvania's new external review system provides an additional safety net, with independent medical experts overturning half of insurance denials in 2024.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance coverage decisions depend on individual plan terms and medical circumstances. Always consult with your healthcare provider and insurance company for specific coverage determinations. For additional help with appeals in Pennsylvania, contact the Pennsylvania Insurance Department Consumer Services or consider working with healthcare advocacy organizations like Counterforce Health that specialize in insurance appeals for specialty medications.

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