By Counterforce Health Team in Xenpozyme (olipudase alfa-rpcp) —

How to Get Xenpozyme (olipudase alfa-rpcp) Covered by Blue Cross Blue Shield in Pennsylvania: Complete Coding, Appeals, and Authorization Guide

Answer Box: Getting Xenpozyme Covered in Pennsylvania

Xenpozyme (olipudase alfa-rpcp) requires prior authorization from Blue Cross Blue Shield plans in Pennsylvania. Use ICD-10 codes E75.240 (type A), E75.241 (type B), or E75.244 (type A/B) for ASMD diagnosis. Submit PA with genetic confirmation, baseline organ assessments, and specialist consultation. If denied, Pennsylvania's Independent External Review has a 53% overturn rate for specialty drug appeals. First step: Contact your BCBS plan's provider services to obtain current PA forms and criteria.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Xenpozyme is covered under the medical benefit for provider-administered infusions, not the pharmacy benefit. This distinction is crucial across Blue Cross Blue Shield plans including Highmark (western PA) and Independence Blue Cross (Philadelphia region).

Medical Benefit Coverage

  • Administration Setting: Outpatient infusion centers only
  • Place of Service Codes: POS 19 (off-campus outpatient hospital) or POS 22 (on-campus outpatient hospital)
  • Billing Method: Provider bills insurer directly using HCPCS J-codes
  • Prior Authorization: Required through medical benefit PA process

Why Not Pharmacy Benefit?

Xenpozyme requires professional IV administration every 2 weeks with careful dose escalation over 8-9 weeks. The complex infusion protocol and monitoring requirements make it unsuitable for pharmacy benefit coverage or self-administration.

Tip: Always verify which BCBS plan you have in Pennsylvania. Highmark covers western PA while Independence Blue Cross serves the Philadelphia area. Each has slightly different PA requirements.

ICD-10 Mapping for ASMD

Accurate diagnosis coding is essential for Xenpozyme approval. Use these specific ICD-10-CM codes for acid sphingomyelinase deficiency (ASMD):

ICD-10 Code Description When to Use
E75.240 Niemann-Pick disease type A Infantile neurovisceral form with CNS involvement
E75.241 Niemann-Pick disease type B Visceral form without CNS involvement
E75.244 Niemann-Pick disease type A/B Intermediate form with mixed features

Documentation Requirements

Your medical records must include:

  • Genetic confirmation: SMPD1 mutation testing results
  • Enzyme assay: ASM activity <10% of normal
  • Clinical symptoms: Progressive hepatosplenomegaly, pulmonary involvement
  • Specialist consultation: From metabolic disease specialist or medical geneticist
Note: Avoid using the parent code E75.24 alone—it's non-billable. Always specify the exact type (A, B, or A/B) based on clinical presentation and testing.

Product Coding: HCPCS, NDC, and Units

HCPCS J-Code

J0218 (Injection, olipudase alfa-rpcp, 1 mg) - Permanent code effective April 1, 2023

NDC Numbers

  • 58468-0050-01: 20 mg single-dose vial
  • 58468-0051-xx: 4 mg lyophilized powder single-dose vial

Dosing and Units Calculation

Adult Dosing (8-week escalation):

Week Dose (mg/kg) 70kg Adult Example J0218 Units
0-2 0.1-0.3 7-21 mg 7-21
14+ 3.0 (maintenance) 210 mg 210

Billing Formula: J0218 units = Total dose in mg = Patient weight (kg) × dose (mg/kg)

Maximum Coverage: Some plans limit to 344 units per 14-day period

Administration Codes

  • 96365: IV infusion, initial (≤90 minutes)
  • 96366: Each additional 30 minutes

Clean Request Anatomy

A successful PA request includes these essential components:

Required Documentation Checklist

✅ Patient Information

  • Insurance ID and group number
  • Complete contact information
  • Date of birth

✅ Clinical Documentation

  • ICD-10 diagnosis code (E75.240, E75.241, or E75.244)
  • Genetic testing results showing SMPD1 mutations
  • Enzyme assay confirming ASM deficiency
  • Baseline imaging (spleen/liver size)
  • Pulmonary function tests if applicable

✅ Prescriber Information

  • DEA and NPI numbers
  • Specialty credentials (metabolic disease, genetics, hematology)
  • Contact information for peer-to-peer review

✅ Treatment Plan

  • Dose escalation schedule
  • Infusion site details
  • Monitoring plan with response metrics

Frequent Coding Pitfalls

Common Mistakes That Cause Denials

1. Unit Conversion Errors

  • Wrong: Billing vial quantity instead of mg units
  • Right: Calculate total mg dose for J0218 units

2. Mismatched Place of Service

  • Wrong: Home infusion (POS 12)
  • Right: Outpatient infusion center (POS 19/22)

3. Missing Baseline Documentation

  • Wrong: Diagnosis code only
  • Right: Genetic confirmation + enzyme assay + imaging

4. Incorrect Benefit Category

  • Wrong: Submitting to pharmacy benefit
  • Right: Medical benefit with proper HCPCS codes

BCBS Pennsylvania Plan Verification

Highmark (Western PA)

  • Coverage Area: Pittsburgh, Erie, western counties
  • PA Requirements: Prior authorization required for HCPCS J0218
  • Contact: Provider services portal or customer service
  • Key Policy: Site-of-service restrictions apply

Independence Blue Cross (Eastern PA)

  • Coverage Area: Philadelphia and southeastern counties
  • Updates: Precertification changes effective January 2026
  • Contact: Provider communication portal
  • Verification: Check current medical benefit drug list

How to Verify Your Specific Plan

  1. Check member ID card: Look for plan name and member services number
  2. Call member services: Verify medical vs. pharmacy benefit coverage
  3. Provider portal: Access current PA forms and criteria
  4. Confirm PA vendor: Some plans use third-party vendors like eviCore
Important: Pennsylvania has multiple BCBS affiliates. Always confirm which specific plan covers your patient before submitting PA requests.

Appeals Playbook for Pennsylvania

Internal Appeals (First Step)

Timeline: 30 days for standard review, 72 hours for expedited How to File: Provider portal, fax, or written request Required: Copy of denial letter, additional clinical documentation

Pennsylvania External Review (Final Step)

Pennsylvania's Independent External Review Program offers a powerful second chance for denied specialty drugs.

Key Statistics: 53% overturn rate in first six months of operation

Review Type Timeline When to Use
Standard 45 days from assignment Most denials
Expedited 72 hours Life/health at serious risk

Filing External Review

  1. Eligibility: Must have Final Adverse Benefit Determination letter
  2. Deadline: Within 4 months of final denial
  3. How to File: Online at pa.gov external review portal
  4. Required Documents: Insurance card, denial letter, medical records
  5. Cost: Free to consumers

Strengthening Your Appeal

Include evidence that Xenpozyme is:

  • FDA-approved for your specific ASMD type
  • Medically necessary based on genetic confirmation
  • The only available treatment for ASMD
  • Prescribed by qualified specialist

Counterforce Health helps patients and clinicians navigate complex prior authorization and appeals processes by turning insurance denials into targeted, evidence-backed appeals. Our platform analyzes denial letters and creates point-by-point rebuttals aligned with each plan's specific criteria, significantly improving approval rates for specialty medications like Xenpozyme.

Quick Audit Checklist

Before submitting your PA or appeal, verify:

Coding Accuracy

  • Correct ICD-10 code (E75.240, E75.241, or E75.244)
  • HCPCS J0218 with accurate unit calculation
  • Proper NDC number for vial size
  • Appropriate place of service code

Documentation Completeness

  • Genetic testing results (SMPD1 mutations)
  • Enzyme assay showing ASM deficiency
  • Baseline organ assessments
  • Specialist consultation notes
  • Treatment plan with monitoring schedule

Administrative Details

  • Correct BCBS plan identification
  • Current PA form (check for 2024-2025 updates)
  • Prescriber credentials and contact info
  • Infusion site approval and coordination

FAQ

Q: How long does BCBS prior authorization take in Pennsylvania? A: Standard PA decisions typically take 30 days, with expedited reviews available within 72 hours when delay could jeopardize health.

Q: What if Xenpozyme is non-formulary on my BCBS plan? A: Request a formulary exception with medical necessity documentation. Emphasize that no FDA-approved alternatives exist for ASMD.

Q: Can I request an expedited appeal if my condition is worsening? A: Yes, both internal appeals and Pennsylvania's external review offer expedited timelines (72 hours) when health is at serious risk.

Q: Does step therapy apply to Xenpozyme? A: Generally no, since Xenpozyme is the only FDA-approved treatment for ASMD. However, some plans may require documentation of supportive care attempts.

Q: What happens if my external review is denied? A: Pennsylvania's external review decision is final and binding. For employer plans, you may have additional options under federal ERISA law—consult legal aid.

Q: How much does Xenpozyme cost without insurance? A: WAC pricing ranges from $1,515 per 4mg vial to $7,576 per 20mg vial. Sanofi offers financial assistance through their CareConnect program.

Q: Can I get help with the appeals process? A: Yes, contact the Pennsylvania Health Law Project for free consumer assistance, or consider services like Counterforce Health that specialize in insurance appeals.

Q: What if I need treatment while appealing? A: Sanofi's patient assistance program may provide temporary access. Also ask your provider about compassionate use options during appeals.

Sources & Further Reading

Disclaimer: This guide provides general information about insurance coverage and coding for educational purposes. It is not medical advice or a guarantee of coverage. Insurance policies vary by plan and individual circumstances. Always consult with your healthcare provider and insurance plan for specific coverage decisions. For additional help with appeals in Pennsylvania, contact the Pennsylvania Insurance Department's consumer services or visit pa.gov for official resources.

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