By Counterforce Health Team in Naglazyme (galsulfase) —

How to Get Naglazyme (Galsulfase) Covered by Cigna in North Carolina: Prior Authorization Guide and Appeals Process

Answer Box: Getting Naglazyme (Galsulfase) Covered by Cigna in North Carolina

Cigna requires prior authorization for Naglazyme (galsulfase) with enzyme/genetic confirmation of MPS VI diagnosis. Submit through Express Scripts/Accredo with specialist documentation including baseline functional assessments (6-minute walk test, pulmonary function). If denied, North Carolina's Smart NC external review overturns 81.7% of specialty drug denials. First step today: Contact your metabolic specialist to gather enzyme assay results and call Cigna at the number on your member ID card to request prior authorization forms.

Table of Contents

  1. Coverage Requirements at a Glance
  2. Step-by-Step: Fastest Path to Approval
  3. When Naglazyme Isn't Initially Approved
  4. Formulary Exception Process
  5. Appeals Playbook for North Carolina
  6. Common Denial Reasons & Solutions
  7. Cost Assistance Programs
  8. FAQ

Coverage Requirements at a Glance

Requirement What It Means Where to Find It Source
Prior Authorization Required Cigna requires approval before dispensing Express Scripts/Accredo specialty pharmacy Cigna Formulary Exception Process
Confirmed MPS VI Diagnosis Enzyme assay showing <10% normal arylsulfatase B activity OR genetic confirmation Lab results from metabolic specialist Cigna Coverage Policy
Specialist Prescription Medical geneticist or metabolic disease specialist required Provider credentials verification EmblemHealth Policy
Baseline Functional Assessment 6-minute walk test, pulmonary function tests, cardiac evaluation Clinical notes from specialist UnitedHealthcare Policy
Dosing Limit Maximum 1 mg/kg per week Patient weight calculation HealthNet Policy

Step-by-Step: Fastest Path to Approval

1. Gather Essential Documentation (Patient + Specialist)

Timeline: 1-2 weeks before requesting PA

  • Enzyme assay results showing deficient arylsulfatase B activity
  • Genetic testing confirming ARSB gene mutations (if available)
  • Baseline functional assessments: 6-minute walk test, pulmonary function tests
  • Current weight for dosing calculation
  • Specialist consultation notes confirming MPS VI diagnosis

2. Submit Prior Authorization Request (Specialist/Clinic Staff)

Timeline: 48-72 hours before planned infusion

  • Call Cigna at member services number on ID card
  • Request PA forms or submit via Express Scripts provider portal
  • Fax completed forms to Express Scripts: 1-877-328-9660
  • Include all documentation from Step 1

3. Cigna Review Process

Timeline: 72 hours standard; 24 hours expedited

  • Cigna reviews against coverage criteria
  • Approval loads into Accredo pharmacy system
  • Denial triggers appeal rights notification

4. If Approved: Schedule Infusion

Timeline: Immediate

  • Contact Accredo Specialty Pharmacy: 1-800-803-2523
  • Schedule weekly 1 mg/kg IV infusions at approved facility
  • Ensure premedication protocol for infusion reactions

When Naglazyme Isn't Initially Approved

Naglazyme (galsulfase) is the only FDA-approved enzyme replacement therapy for MPS VI—there are no direct alternatives or substitutes. However, Cigna may initially deny coverage for several reasons that can be addressed through their formulary exception process.

Understanding "No Alternatives" for MPS VI

Unlike other conditions where multiple treatment options exist, mucopolysaccharidosis type VI has a unique treatment landscape:

  • Naglazyme is the sole approved ERT for this rare genetic condition
  • No other enzyme replacement therapies target arylsulfatase B deficiency
  • Supportive care only (physical therapy, orthopedic interventions, cardiac monitoring) cannot replace enzyme replacement
  • Investigational treatments (gene therapy, substrate reduction therapy) remain in clinical trials

This "no alternatives" status actually strengthens your case for formulary exceptions and appeals.

Formulary Exception Process

When Cigna places Naglazyme on a non-preferred tier or excludes it entirely, you can request a formulary exception based on medical necessity.

Exception Criteria That Apply to Naglazyme

Physician must certify in writing that formulary alternatives:

  • Are ineffective for the patient's condition
  • Have caused or are likely to cause adverse reactions
  • Are contraindicated based on patient characteristics
  • Are not in the patient's best interest

Since no formulary alternatives exist for MPS VI, your specialist should emphasize that Naglazyme is the only available treatment option.

Submission Process

Method Contact Information Timeline
Phone Call Cigna member services (ID card number) 24-72 hours
Fax Express Scripts: 1-877-328-9660 72 hours standard
Online Portal Evernorth Prior Authorization 24-72 hours

Expedited reviews available within 24 hours for life-threatening conditions or ongoing treatment.

Appeals Playbook for North Carolina

North Carolina offers exceptionally strong consumer protections through the Smart NC program, which has an 81.7% success rate for specialty drug appeals.

Internal Appeals (First Level)

Timeline: File within 180 days of denial Process:

  1. Submit written appeal to Cigna member services
  2. Include additional clinical documentation
  3. Request peer-to-peer review with specialist
  4. Cigna has 30 days to respond (72 hours for expedited)

Smart NC External Review (Second Level)

Timeline: File within 120 days of final internal denial Success Rate: 81.7% for specialty drugs Cost: Free to patients

How to File:

  1. Contact Smart NC: 1-855-408-1212
  2. Submit external review request form (available here)
  3. Smart NC assigns Independent Review Organization (IRO)
  4. Decision within 45 days standard; 72 hours expedited
From Our Advocates: We've seen multiple cases where North Carolina patients initially denied Naglazyme coverage by major insurers successfully obtained approval through Smart NC's external review process. The key was submitting comprehensive clinical documentation showing the lack of treatment alternatives and functional decline without enzyme replacement therapy. These cases typically resolved within 30-45 days of filing the external review request.

Required Documentation for Appeals

  • Original denial letter with specific denial reasons
  • Complete medical records showing MPS VI diagnosis
  • Specialist letter explaining medical necessity
  • FDA labeling for Naglazyme showing approved indication
  • Clinical studies supporting enzyme replacement therapy efficacy
  • Documentation of functional decline without treatment

Common Denial Reasons & Solutions

Denial Reason How to Overturn Required Documentation
"Experimental/Investigational" Submit FDA approval documentation FDA labeling, approval date (2005)
"Lack of Medical Necessity" Provide specialist letter with clinical rationale Metabolic specialist consultation, functional assessments
"Insufficient Diagnostic Evidence" Submit complete enzyme/genetic testing Enzyme assay results, genetic testing report
"No Prior Authorization" Resubmit with complete PA packet All required forms via proper channels
"Site of Care Issues" Clarify infusion center requirements Facility credentials, administration protocols

Most Effective Appeal Strategy: Emphasize that Naglazyme is the only approved treatment for MPS VI, making denial equivalent to denying all treatment options for a serious genetic condition.

Cost Assistance Programs

Annual Cost: Approximately $600,000-$1.8 million based on patient weight Manufacturer Support: BioMarin Co-Pay Assistance Program

  • Eligible commercially insured patients may pay as little as $0
  • Income and insurance requirements apply
  • Application available through specialist office

Additional Resources:

  • Patient Advocate Foundation: Financial assistance for rare disease treatments
  • National Organization for Rare Disorders (NORD): Emergency financial assistance
  • HealthWell Foundation: Copayment assistance programs

At Counterforce Health, we help patients and clinicians turn insurance denials into successful appeals by analyzing denial letters against specific plan policies and drafting evidence-backed rebuttals. Our platform identifies the exact denial basis—whether PA criteria, medical necessity, or formulary status—and provides targeted documentation strategies that address each payer's specific requirements.

FAQ

How long does Cigna prior authorization take for Naglazyme in North Carolina? Standard review: 72 hours. Expedited review: 24 hours for urgent cases. Submit 48-72 hours before planned infusion to avoid delays.

What if Naglazyme is not on Cigna's formulary? Request a formulary exception emphasizing that no alternatives exist for MPS VI. Success rates are high when properly documented with specialist support.

Can I request an expedited appeal in North Carolina? Yes. Both Cigna internal appeals and Smart NC external reviews offer expedited pathways. For Smart NC, expedited decisions come within 72 hours for urgent cases.

What counts as medical necessity for Naglazyme? Confirmed MPS VI diagnosis via enzyme assay or genetic testing, plus documentation of functional impairment that enzyme replacement therapy can address. Cigna policy details here.

Does step therapy apply to Naglazyme? Generally no, since no alternative treatments exist for MPS VI. However, some plans may require documentation of supportive care attempts before approving ERT.

How do I find a qualified specialist in North Carolina? Contact medical genetics programs at major academic medical centers like Duke University or UNC Chapel Hill. The National Society of Genetic Counselors maintains a provider directory.

What if my employer plan is self-funded? Self-funded plans may not be subject to North Carolina's external review process. Check with your HR department about plan governance and available appeal rights.

Can Smart NC help with the appeal process? Yes. Smart NC staff provide free guidance on filing external reviews, gathering documentation, and communicating with insurers. Call 1-855-408-1212 for assistance.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance coverage policies vary by plan and individual circumstances. Always consult with your healthcare provider and insurance company for specific coverage determinations. For free assistance with North Carolina insurance appeals, contact Smart NC at 1-855-408-1212.

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