By Counterforce Health Team in Naglazyme (galsulfase) —

How to Get Naglazyme (Galsulfase) Covered by Cigna in Ohio: Decision Tree, Forms, and Appeals Guide

Answer Box: Getting Naglazyme Covered by Cigna in Ohio

You likely qualify if you have confirmed MPS VI diagnosis through enzyme assay or genetic testing. Cigna requires prior authorization with specific documentation. Your first step today: Contact your prescribing specialist to gather enzyme/genetic test results and complete Cigna's prior authorization form. If denied, Ohio offers two internal appeal levels plus external review through Independent Review Organizations. The external review has a 50-53% success rate for overturning denials.

Table of Contents

  1. How to Use This Guide
  2. Eligibility Quick Check
  3. If You're Likely Eligible: Documentation Checklist
  4. If You're Possibly Eligible: Tests to Request
  5. If Not Yet Eligible: Alternative Options
  6. If Denied: Ohio Appeal Process
  7. Common Denial Reasons & Solutions
  8. Costs and Financial Support
  9. FAQ
  10. Sources & Further Reading

How to Use This Guide

This guide helps patients with MPS VI (mucopolysaccharidosis type VI) and their healthcare teams navigate Cigna's coverage requirements for Naglazyme (galsulfase) in Ohio. Work through the eligibility sections first, then follow the appropriate pathway based on your situation.

Naglazyme is the only FDA-approved enzyme replacement therapy for MPS VI, with proven benefits for walking capacity and stair-climbing ability. At roughly $600,000-$1.8 million annually depending on patient weight, securing insurance coverage is crucial.

Eligibility Quick Check

Answer these questions to determine your coverage pathway:

Diagnosis Confirmed? ✓ or ✗

  • Enzyme assay showing deficient N-acetylgalactosamine 4-sulfatase activity in leukocytes or fibroblasts
  • Genetic testing confirming biallelic pathogenic ARSB gene variants

Prescriber Requirements? ✓ or ✗

  • Specialist prescriber: Geneticist, endocrinologist, metabolic disorder specialist, or lysosomal storage disorder physician

Clinical Documentation? ✓ or ✗

  • Baseline functional assessments (6-minute walk test, pulmonary function tests)
  • Treatment plan specifying 1 mg/kg IV weekly dosing

Results:

  • All ✓: Likely eligible → Go to Documentation Checklist
  • Missing 1-2: Possibly eligible → Go to Tests to Request
  • Missing 3+: Not yet eligible → Go to Alternative Options

If You're Likely Eligible: Documentation Checklist

Required Documents for Cigna Prior Authorization

Diagnostic Evidence (Source: Cigna Policy)

  • Laboratory report showing deficient enzyme activity OR genetic test results
  • Medical records confirming MPS VI diagnosis
  • ICD-10 code E76.29 (Other mucopolysaccharidoses)

Prescriber Documentation

  • Letter from qualified specialist (geneticist, endocrinologist, or metabolic specialist)
  • Treatment plan specifying weekly 1 mg/kg IV infusion
  • Clinical rationale for enzyme replacement therapy

Functional Assessments

  • Baseline 6-minute walk test or 12-minute walk test results
  • Pulmonary function tests (FVC, FEV1)
  • Urinary GAG levels if available

Submission Process

  1. Complete Cigna's prior authorization form (available through provider portal)
  2. Submit via provider portal or fax (verify current submission method)
  3. Include all supporting documentation listed above
  4. Follow up within 5-7 business days for status update
Tip: Request expedited review if treatment delay could worsen your condition. Cigna must respond to expedited requests within 72 hours.

If You're Possibly Eligible: Tests to Request

Missing Diagnostic Confirmation

Request enzyme assay testing:

  • Contact a genetic counselor or metabolic specialist
  • Test measures N-acetylgalactosamine 4-sulfatase activity in blood cells
  • Results typically available within 2-3 weeks

Consider genetic testing:

  • ARSB gene sequencing can confirm diagnosis
  • Useful if enzyme assay results are borderline
  • May be covered by insurance for suspected MPS VI

Missing Functional Assessments

Schedule baseline testing:

  • 6-minute walk test at cardiology or pulmonology clinic
  • Pulmonary function tests (spirometry)
  • Document current functional limitations

Timeline to reapply: 4-6 weeks after completing required testing

If Not Yet Eligible: Alternative Options

Temporary Support Options

Clinical trial participation:

  • Check ClinicalTrials.gov for MPS VI studies
  • Gene therapy trials may be available for some patients

Compassionate use programs:

  • Contact BioMarin at 1-866-906-6100 for expanded access consideration
  • Requires documented medical need and no alternative treatments

Preparing for Exception Requests

Document medical necessity:

  • Gather evidence of disease progression
  • Record functional decline without treatment
  • Obtain specialist recommendations

If Denied: Ohio Appeal Process

Ohio provides multiple appeal levels with strong consumer protections. External review success rates reach 50-53% for overturning denials.

Step 1: Internal Appeals with Cigna

First-Level Internal Appeal

  • Deadline: 180 days from denial notice
  • Decision timeline: 30 days (72 hours if expedited)
  • How to file: Call Cigna member services or submit written appeal

Second-Level Internal Appeal

  • Automatic if first appeal denied
  • Timeline: Additional 30 days for decision
  • Include: New clinical evidence, specialist letters, peer-reviewed studies
Peer-to-Peer Review: Request that your prescribing physician speak directly with Cigna's medical director during the internal appeal process.

Step 2: External Review (Ohio Department of Insurance)

Eligibility: Available after exhausting Cigna's internal appeals Timeline: Request within 180 days of final internal denial Success rate: 50-53% overturn rate for denials

How to Request:

  1. Call Ohio Department of Insurance: 1-800-686-1526
  2. Submit external review form and supporting documents
  3. Include: All denial letters, medical records, specialist recommendations

Review Timeline:

  • Standard review: 30 days for decision
  • Expedited review: 72 hours (for urgent cases)
  • Decision is binding on Cigna

More details on Ohio's external review process

Common Denial Reasons & Solutions

Denial Reason How to Overturn Required Documentation
"Diagnosis not confirmed" Submit enzyme assay or genetic test results Laboratory reports showing deficient enzyme activity
"Not medically necessary" Provide functional assessments showing disease impact 6-minute walk test, pulmonary function tests, specialist letter
"Experimental/investigational" Emphasize FDA approval for MPS VI FDA label, clinical trial data showing efficacy
"Step therapy not completed" Document contraindications to alternative treatments Medical records showing why alternatives aren't appropriate
"Prescriber not qualified" Verify specialist credentials Board certification documentation, referral from geneticist

Costs and Financial Support

Annual Treatment Cost: $600,000-$1.8 million (weight-dependent dosing) HCPCS Billing Code: J1458 (per 1 mg administered)

Financial Assistance Options

BioMarin RareConnections Program

  • Copay assistance for eligible patients
  • Prior authorization support
  • Contact: 1-866-906-6100
  • Program details

Foundation Grants

  • National Organization for Rare Disorders (NORD)
  • Patient Advocate Foundation
  • HealthWell Foundation (when funds available)

FAQ

How long does Cigna's prior authorization take in Ohio? Standard decisions within 15 business days; expedited decisions within 72 hours for urgent cases.

What if Naglazyme is non-formulary on my Cigna plan? Request a formulary exception with medical necessity documentation. Non-formulary drugs can still be covered with appropriate clinical justification.

Can I get home infusion for Naglazyme? Cigna generally requires treatment in the least intensive medically appropriate setting. Home infusion may be covered if clinically appropriate and safe.

Does step therapy apply to Naglazyme? No alternative enzyme replacement therapies exist for MPS VI, so traditional step therapy doesn't apply. However, document why supportive care alone is insufficient.

How do I request expedited review? Contact Cigna member services and explain why treatment delay would seriously jeopardize your health. Provide supporting documentation from your specialist.

What happens if external review fails? You retain rights to legal action or regulatory complaints. Some patients pursue federal court appeals under ERISA for employer-sponsored plans.

From our advocates: "We've seen the strongest appeals include both the diagnostic 'proof' that Cigna requires and clear documentation of functional decline without treatment. One family gathered walking test results over six months showing progressive difficulty, which helped demonstrate medical necessity beyond the basic enzyme deficiency."

When navigating complex prior authorizations and appeals for rare disease treatments like Naglazyme, having expert support can make a significant difference. Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into targeted, evidence-backed appeals by analyzing denial letters, identifying specific coverage criteria, and drafting point-by-point rebuttals aligned with each insurer's policies. Their platform streamlines the appeals process by pulling the right clinical evidence and weaving it into compelling cases that meet procedural requirements.

The platform is particularly valuable for specialty drugs like Naglazyme, where appeals require specific documentation of enzyme deficiency, functional assessments, and specialist credentials. Counterforce Health helps ensure nothing falls through the cracks during the complex prior authorization and appeals process.

Sources & Further Reading

Disclaimer: This guide provides general information about insurance coverage and appeals processes. It is not medical advice. Always consult with your healthcare provider about treatment decisions and work with your insurance company's official procedures. Coverage policies may change, so verify current requirements with Cigna and Ohio Department of Insurance directly.

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