By Counterforce Health Team in Elaprase (idursulfase) —

Coding That Helps Get Elaprase (Idursulfase) Approved in Virginia with UnitedHealthcare: ICD-10, HCPCS/J-Code, and NDC Guide

Answer Box: Getting Elaprase Covered by UnitedHealthcare in Virginia

Fast track to approval: Use ICD-10 code E76.1 (Hunter syndrome), HCPCS J1743 for billing, and NDC 54092-0700-01. Submit prior authorization through UnitedHealthcare Provider Portal with enzymatic/genetic test results confirming Hunter syndrome diagnosis. If denied, file Virginia external review using Form 216-A within 120 days. First step today: Verify patient weight for precise 0.5 mg/kg dosing calculations and gather diagnostic confirmation documents.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit
  2. ICD-10 Mapping for Hunter Syndrome
  3. Product Coding: HCPCS, NDC, and Units
  4. Clean Request Anatomy
  5. Frequent Coding Pitfalls
  6. Verification with UnitedHealthcare
  7. Quick Audit Checklist
  8. Virginia Appeals Process
  9. FAQ

Coding Basics: Medical vs. Pharmacy Benefit

Elaprase (idursulfase) for Hunter syndrome falls squarely under the medical benefit, not pharmacy benefit, because it requires IV infusion in a clinical setting. This distinction matters for both billing and coverage approval.

Medical Benefit Path:

  • Billed using HCPCS J-code after administration
  • Requires infusion procedure codes (96365, 96366)
  • Subject to medical necessity review
  • Covered when administered in office, hospital outpatient, or infusion center

Why Not Pharmacy Benefit:

  • Not dispensed for home self-administration
  • Requires clinical monitoring for infusion reactions
  • Complex weight-based dosing calculations
Tip: Always verify benefit type with UnitedHealthcare before scheduling the first infusion. Call the provider services line at 888-397-8129 to confirm medical benefit coverage.

ICD-10 Mapping for Hunter Syndrome

The primary diagnosis code for Hunter syndrome is E76.1 (Mucopolysaccharidosis, type II). This code is valid for 2025 and covers all variants of Hunter syndrome.

Documentation Requirements

Your clinical notes should include specific language that supports the E76.1 diagnosis:

Required Elements:

  • Confirmed diagnosis of "Hunter syndrome" or "Mucopolysaccharidosis II (MPS II)"
  • Enzymatic testing showing reduced/absent iduronate-2-sulfatase activity
  • Genetic testing confirming IDS gene pathogenic variant (when available)
  • Clinical features supporting diagnosis (coarse facial features, hepatosplenomegaly, skeletal abnormalities)

Supporting Documentation:

  • Original enzyme assay results
  • Genetic testing report
  • Specialist consultation notes (genetics or metabolic disease)
  • Treatment goals and monitoring plan
Note: UnitedHealthcare requires both clinical suspicion AND laboratory confirmation. Don't rely on clinical features alone—enzymatic or genetic proof is mandatory for coverage approval.

Product Coding: HCPCS, NDC, and Units

Core Codes

  • HCPCS: J1743 (Injection, idursulfase, 1 mg)
  • NDC: 54092-0700-01 (Takeda's packaging)
  • Concentration: 2 mg/mL in 3 mL vials (6 mg per vial)

Weight-Based Dosing Calculations

The FDA-approved dose is 0.5 mg/kg weekly. Here's the step-by-step calculation:

  1. Calculate dose: Patient weight (kg) × 0.5 mg/kg = total dose in mg
  2. Calculate volume: Total dose (mg) ÷ 2 mg/mL = volume needed in mL
  3. Calculate vials: Volume (mL) ÷ 3 mL per vial = number of vials (round up)

Example for 21 kg patient:

  • Dose: 21 kg × 0.5 mg/kg = 10.5 mg
  • Volume: 10.5 mg ÷ 2 mg/mL = 5.25 mL
  • Vials: 5.25 mL ÷ 3 mL = 1.75 vials → Use 2 vials, withdraw 5.25 mL

Billing Units and Modifiers

Units for J1743: Enter the total mg administered as units. For the 21 kg patient above, bill 10.5 units.

Common Modifiers:

  • JW: Drug wastage (for discarded portion from opened vials)
  • 59 or XE: Separate procedure (if same-day services require distinction)
Important: Bill only for the exact mg administered, not the full vial contents. Use modifier JW for documented wastage per UnitedHealthcare guidelines.

Clean Request Anatomy

Prior Authorization Request Components

A complete PA request includes these coded elements:

Patient Information:

  • ICD-10: E76.1 (Hunter syndrome)
  • Patient weight and calculated dose
  • Age (UnitedHealthcare requires ≥16 months)

Drug Information:

  • HCPCS: J1743
  • NDC: 54092-0700-01
  • Units: Exact mg per patient weight
  • Frequency: Weekly infusions

Clinical Documentation:

  • Enzymatic test results showing IDS deficiency
  • Genetic testing (when available)
  • Specialist prescriber information
  • Infusion monitoring plan

Infusion Procedure Codes

Always include these with J1743:

  • 96365: IV infusion, up to 1 hour (primary)
  • 96366: Each additional hour (if infusion >1 hour)

Frequent Coding Pitfalls

Unit Conversion Errors

  • Wrong: Billing full vial contents (6 units) when patient needs 4.5 mg
  • Right: Bill 4.5 units for J1743, use JW modifier for 1.5 mg wastage

Mismatched Codes

  • Wrong: Using generic "mucopolysaccharidosis" codes instead of E76.1
  • Right: Always specify E76.1 for Hunter syndrome

Missing Documentation

  • Wrong: Submitting PA without enzymatic confirmation
  • Right: Include both enzyme assay and genetic testing results

Dosing Mistakes

  • Wrong: Rounding up to convenient doses (e.g., 5 mg instead of 4.2 mg)
  • Right: Calculate exact weight-based dose using 0.5 mg/kg

Verification with UnitedHealthcare

Pre-Submission Checklist

Before submitting your PA request, verify these elements through UnitedHealthcare resources:

Provider Portal Checks:

  1. Confirm J1743 requires PA for your patient's plan
  2. Verify NDC 54092-0700-01 is recognized
  3. Check for any quantity limits or frequency restrictions
  4. Review current medical policy for Hunter syndrome

Contact Information:

Cross-Reference Resources

UnitedHealthcare Policy Documents:

Quick Audit Checklist

Before submitting any Elaprase request, review these items:

✓ Patient Information

  • Correct patient weight documented
  • Age ≥16 months confirmed
  • Insurance eligibility verified

✓ Diagnosis Coding

  • ICD-10 E76.1 used consistently
  • Enzymatic test results attached
  • Genetic testing included (if available)

✓ Drug Coding

  • HCPCS J1743 with correct units (mg)
  • NDC 54092-0700-01 specified
  • Wastage modifier JW used if applicable

✓ Clinical Documentation

  • Specialist prescriber identified
  • Medical necessity narrative included
  • Infusion monitoring plan detailed
  • Prior therapy attempts documented (if any)

✓ Procedural Codes

  • Infusion codes 96365/96366 included
  • Site of care specified
  • Administration plan outlined

Virginia Appeals Process

When UnitedHealthcare denies your properly coded Elaprase request, Virginia offers a robust external review process.

Timeline and Forms

Internal Appeals First:

  • UnitedHealthcare allows up to 180 days for internal appeals
  • Submit through provider portal or member services
  • Expedited review available for urgent cases (≤72 hours)

External Review Process:

  • File Form 216-A within 120 days of final denial
  • Virginia State Corporation Commission Bureau of Insurance
  • Standard review: 45 days; Expedited: 72 hours

Contact Information:

  • Virginia SCC Bureau of Insurance
  • Phone: 1-877-310-6560
  • Email: [email protected]
  • Fax: (804) 371-9915
Virginia Advantage: The state's external review process has successfully overturned many specialty drug denials, particularly when clinical documentation is thorough and coding is accurate.

Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals. Our platform analyzes denial letters, identifies the specific basis for denial, and drafts point-by-point rebuttals aligned to UnitedHealthcare's own coverage policies, helping patients get the medications they need faster.

FAQ

Q: How long does UnitedHealthcare PA take for Elaprase in Virginia? A: Standard PA decisions are typically made within 15 business days. Expedited reviews for urgent cases can be completed in 72 hours when clinical urgency is documented.

Q: What if my weight-based calculation results in partial vials? A: Always round up vials purchased but bill only for mg administered. Use modifier JW for documented wastage. For a patient needing 4.2 mg, buy one 6 mg vial, administer 4.2 mg, and waste 1.8 mg with proper documentation.

Q: Can I request an expedited appeal if Elaprase is denied? A: Yes, if your physician certifies that delaying treatment could seriously jeopardize health. Use Form 216-C for expedited external review in Virginia.

Q: Does step therapy apply to Elaprase? A: No. UnitedHealthcare does not require step therapy for Elaprase because there are no FDA-approved alternatives for Hunter syndrome enzyme replacement therapy.

Q: What counts as medical necessity for Hunter syndrome treatment? A: Confirmed diagnosis via enzymatic or genetic testing, documented clinical features, specialist prescriber involvement, and appropriate weight-based dosing calculations.

Q: Is genetic testing required if enzyme testing confirms Hunter syndrome? A: While enzymatic testing may be sufficient for diagnosis, genetic testing strengthens the medical necessity case and is recommended for comprehensive documentation, especially for family planning purposes.

From our advocates: We've seen Virginia patients successfully overturn UnitedHealthcare denials by ensuring their enzymatic test results clearly showed IDS deficiency levels and their weight-based dosing calculations were precisely documented in the medical records. Attention to these coding details often makes the difference between approval and denial.

For patients and families navigating insurance coverage for rare disease treatments, Counterforce Health provides expert support in transforming denials into successful appeals. Our platform ingests denial letters and clinical documentation to create targeted rebuttals that address payer-specific requirements, helping streamline the approval process for essential medications like Elaprase.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Always consult with healthcare providers and insurance representatives for patient-specific guidance. Coverage policies and requirements may change; verify current information with UnitedHealthcare and Virginia regulatory authorities.

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