By Counterforce Health Team in Naglazyme (galsulfase) —

Coding That Helps Get Naglazyme (Galsulfase) Approved by Blue Cross Blue Shield in New Jersey: ICD-10, HCPCS J-Code, and NDC Guide

Answer Box: Getting Naglazyme Covered in New Jersey

Naglazyme (galsulfase) requires prior authorization from Blue Cross Blue Shield in New Jersey and is covered under medical benefits using HCPCS J1458. Key coding requirements: ICD-10 E76.29 (MPS VI), J1458 (1 unit = 1 mg), weight-based dosing (1 mg/kg weekly), and enzyme assay documentation. Submit PA requests through Horizon BCBS provider portal with diagnosis confirmation, baseline functional measures, and billing details. If denied, use New Jersey's IHCAP external review process through Maximus Federal Services within 120 days.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Naglazyme (galsulfase) is exclusively billed under medical benefits through Blue Cross Blue Shield in New Jersey, not pharmacy benefits. This IV-only enzyme replacement therapy requires specialized infusion administration over several hours, making it unsuitable for traditional pharmacy dispensing.

Coverage Path Comparison

Benefit Type Naglazyme Coverage Billing Method Prior Auth
Medical Benefit ✅ Covered via J1458 Physician/infusion center billing Required
Pharmacy Benefit ❌ Not applicable N/A - IV only N/A

Horizon Blue Cross Blue Shield lists Naglazyme on their office-administered specialty medications requiring medical necessity and authorization review (MNAR).

ICD-10 Mapping for MPS VI

The primary diagnosis code for Mucopolysaccharidosis Type VI (Maroteaux-Lamy syndrome) is E76.29 (Other mucopolysaccharidoses). This billable code explicitly includes:

  • Mucopolysaccharidosis type VI
  • Maroteaux-Lamy syndrome (mild and severe forms)
  • N-acetylgalactosamine-4-sulfatase deficiency

Documentation Requirements for E76.29

To support this diagnosis code, medical records must include:

Biochemical Confirmation:

  • Arylsulfatase B (ASB) enzyme activity <10% of normal in cultured fibroblasts or leukocytes
  • Normal activity of a different sulfatase (excludes multiple sulfatase deficiency)
  • Elevated urinary dermatan sulfate (>100 µg/mg creatinine in rapidly progressive forms)

Genetic Testing:

  • Pathogenic biallelic mutations in ARSB gene
  • Over 130 known mutations confirming autosomal recessive inheritance

Clinical Features:

  • Short stature and dysostosis multiplex
  • Joint stiffness and corneal clouding
  • Hepatosplenomegaly and cardiac valve abnormalities
Tip: Include both enzyme assay results and genetic confirmation in your PA submission. Orphanet reports that complete diagnostic workup strengthens medical necessity documentation.

Product Coding: HCPCS, NDC, and Units

HCPCS J-Code: J1458

J1458 = Injection, galsulfase, 1 mg

Each billable unit equals exactly 1 mg of galsulfase. Standard MPS VI dosing is 1 mg/kg body weight IV once weekly, so:

Units Calculation: Patient weight in kg = Number of J1458 units per dose

Examples:

  • 25 kg pediatric patient = 25 units per weekly dose
  • 50 kg adult patient = 50 units per weekly dose
  • 70 kg adult patient = 70 units per weekly dose
Note: Many payers cap coverage at 115 units every 7 days. Doses exceeding this limit may require additional justification.

NDC and Vial Information

  • NDC: 68135-0020-01
  • Strength: 5 mg/5 mL single-dose vial
  • Units per vial: 5 HCPCS units (J1458)
  • Manufacturer: BioMarin Pharmaceutical

Associated CPT Codes for Infusion

When billing for the administration service, use:

  • 96365: IV infusion, initial service (up to 1 hour)
  • 96366: IV infusion, each additional hour

Clean Request Anatomy

Example Prior Authorization Request

Patient Information:

  • Diagnosis: E76.29 (Mucopolysaccharidosis type VI)
  • Weight: 45 kg
  • Requested dose: 45 mg (45 units J1458) IV weekly

Clinical Documentation:

  • Enzyme assay: ASB activity 2% of normal (reference lab report)
  • Genetic testing: Compound heterozygous ARSB mutations
  • Baseline 6-minute walk test: 180 meters
  • Urinary dermatan sulfate: 250 µg/mg creatinine

Billing Details:

  • HCPCS: J1458 x 45 units per dose
  • Frequency: Once weekly (52 doses annually = 2,340 units/year)
  • Site of service: Hospital outpatient infusion center
  • NDC: 68135-0020-01

When working with complex coverage challenges like Naglazyme approval, Counterforce Health helps patients and providers navigate insurance denials by creating targeted, evidence-backed appeals that align with each payer's specific requirements.

Frequent Coding Pitfalls

Unit Conversion Errors

❌ Common Mistake: Billing for vials instead of mg

  • Incorrect: 9 units for 45 kg patient (thinking 9 vials)
  • ✅ Correct: 45 units for 45 kg patient (45 mg total dose)

Mismatched Diagnosis Codes

❌ Avoid These Codes:

  • E76.1 (Mucopolysaccharidosis type II - Hunter syndrome)
  • E76.0 (Mucopolysaccharidosis type I - Hurler/Scheie)
  • E76.3 (Mucopolysaccharidosis type IV - Morquio)

✅ Use: E76.29 specifically for MPS VI

Missing Documentation Elements

Common PA rejections occur when submissions lack:

  • Confirmed enzyme deficiency results
  • Baseline functional assessments (6-minute walk test, pulmonary function)
  • Prior therapy documentation (if applicable)
  • Weight-based dosing justification

Site of Service Issues

Ensure your billing location matches Blue Cross Blue Shield's site-of-care requirements:

  • Hospital outpatient departments typically preferred
  • Physician offices may need additional authorization
  • Home infusion rarely covered due to complexity

Verification with Blue Cross Blue Shield

Pre-Submission Checklist

Before submitting your PA request, verify these elements through Horizon BCBS provider resources:

  1. Formulary Status: Confirm Naglazyme remains on the specialty medication list
  2. PA Requirements: Check current medical policy #027 for enzyme replacement therapies
  3. Quantity Limits: Verify maximum units per timeframe
  4. Site Restrictions: Confirm approved administration locations

Provider Portal Verification

Access the Horizon provider portal to:

  • Check real-time PA status
  • Download current forms
  • View specific plan requirements
  • Submit documentation electronically
Clinician Corner: When submitting medical necessity letters, emphasize functional improvements documented in clinical trials. Include baseline 6-minute walk test results and planned monitoring schedule to demonstrate ongoing medical necessity.

Quick Audit Checklist

Pre-Submission Review

✅ Diagnosis Coding

  • E76.29 (Other mucopolysaccharidoses) documented
  • Enzyme assay results <10% normal included
  • Genetic testing confirmation attached

✅ Product Coding

  • J1458 units = patient weight in kg
  • Weekly dosing frequency specified
  • NDC 68135-0020-01 referenced
  • Total annual units calculated correctly

✅ Clinical Documentation

  • Baseline functional assessments completed
  • Prior therapy failures documented (if applicable)
  • Treatment goals clearly stated
  • Monitoring plan outlined

✅ Administrative Requirements

  • PA form completely filled out
  • Prescriber information accurate
  • Site of service appropriate
  • Insurance verification current

Appeals Process in New Jersey

If Blue Cross Blue Shield denies your Naglazyme request, New Jersey offers robust appeal options through the Independent Health Care Appeals Program (IHCAP).

Internal Appeals (First Step)

Submit appeals directly to Blue Cross Blue Shield within their specified timeframe (typically 60 days). Include:

  • Complete medical records supporting MPS VI diagnosis
  • Peer-reviewed literature on Naglazyme efficacy
  • Detailed medical necessity letter addressing denial reasons

External Review Through IHCAP

After completing internal appeals, you can request external review through Maximus Federal Services, New Jersey's independent review organization.

Key Deadlines:

  • Standard appeals: 120 days from final internal denial
  • Expedited appeals: File immediately for urgent cases

Filing Options:

Review Timeline:

  • Preliminary review: Within 5 business days
  • Full review: Within 45 days (48 hours for expedited)
  • Decision binding on Blue Cross Blue Shield

Organizations like Counterforce Health specialize in creating comprehensive appeal packages that address specific payer denial reasons while incorporating the clinical evidence and regulatory requirements that external reviewers expect to see.

FAQ

Q: How long does Blue Cross Blue Shield prior authorization take in New Jersey? A: Standard PA reviews typically take 3-5 business days. Expedited reviews for urgent cases are completed within 24-48 hours.

Q: What if Naglazyme is non-formulary on my specific plan? A: Request a formulary exception through your plan's standard process. Emphasize the lack of therapeutic alternatives for MPS VI treatment.

Q: Can I request an expedited appeal if my patient's condition is deteriorating? A: Yes, New Jersey allows expedited appeals when delays would jeopardize health. File immediately with supporting clinical documentation.

Q: Does step therapy apply to Naglazyme? A: Step therapy typically doesn't apply since there are no alternative enzyme replacement therapies for MPS VI. However, verify with your specific Blue Cross Blue Shield plan.

Q: What if my patient was previously treated outside New Jersey? A: Include all prior treatment records and outcomes in your PA submission. Out-of-state treatment history supports continued therapy medical necessity.

Q: How do I calculate annual costs for budget planning? A: Multiply weekly units by 52 weeks. For a 50 kg patient: 50 units × 52 weeks = 2,600 units annually. At approximately $500/unit, this equals ~$1.3M annually.

Q: Are there patient assistance programs available? A: BioMarin offers patient support programs. Contact their specialty pharmacy hub for eligibility information and application assistance.

Q: What documentation is required for continuation of therapy? A: Submit annual reviews including updated functional assessments, treatment response data, and ongoing medical necessity justification.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical advice. Coverage policies vary by specific insurance plan and may change over time. Always verify current requirements directly with your Blue Cross Blue Shield plan and consult with healthcare providers for medical decisions. For assistance with insurance appeals and coverage challenges, contact the New Jersey Department of Banking and Insurance at 1-800-446-7467.

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