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

How to Get Xenpozyme (Olipudase Alfa-rpcp) Covered by Blue Cross Blue Shield in Texas: Complete Prior Authorization and Appeals Guide

Answer Box: Getting Xenpozyme Covered in Texas

Blue Cross Blue Shield of Texas (BCBSTX) typically requires prior authorization for Xenpozyme (olipudase alfa-rpcp) under the medical benefit using HCPCS code J0218. Most approvals require genetic confirmation of acid sphingomyelinase deficiency (ASMD), baseline organ assessments, and specialist oversight. First step today: verify your specific plan's PA requirements through the BCBSTX provider portal or member services, then gather genetic testing results and specialist consultation notes before submitting your request.

If denied, Texas law provides strong appeal rights including independent external review through the Texas Department of Insurance within 4 months of final denial.

Table of Contents

  1. Coverage Requirements Overview
  2. Prior Authorization Process
  3. Common Denial Reasons & Solutions
  4. Appeals Process in Texas
  5. Required Documentation Checklist
  6. Costs & Patient Assistance
  7. FAQ

Coverage Requirements Overview

Blue Cross Blue Shield of Texas covers Xenpozyme as a medical benefit drug for patients with confirmed acid sphingomyelinase deficiency (ASMD). The medication treats non-CNS manifestations of Niemann-Pick disease types A and B in both adults and children.

Coverage at a Glance

Requirement Details Where to Verify
Prior Authorization Required for most plans BCBSTX Medical Drug List
Formulary Status Medical benefit, HCPCS J0218 Provider portal or member services
ICD-10 Codes E75.240, E75.241, E75.248, E75.249 Texas Medicaid criteria
Specialist Requirement Metabolic/genetic disease specialist Plan-specific PA form
Site of Care Approved infusion center or hospital outpatient Medical Management review

The exact prior authorization process varies by product type—commercial plans may route through eviCore or Carelon, while Medicare Advantage typically uses eviCore for medical drug management.

Prior Authorization Process

Step 1: Verify Coverage and PA Requirements

Before starting treatment, confirm:

  • Your plan covers Xenpozyme under the medical benefit
  • Whether PA is processed by BCBSTX directly, eviCore, or Carelon
  • Current HCPCS code (J0218) and any quantity limits

Action: Log into Availity or call member services to verify benefits and PA vendor.

Step 2: Gather Required Documentation

Based on BCBSTX PA requirements and similar Blue plans, you'll need:

  • Genetic confirmation: SMPD1 mutation testing or enzyme assay showing ASM deficiency
  • Baseline assessments: Imaging showing organ involvement (spleen/liver size), pulmonary function tests if lung disease present
  • Specialist consultation: Note from metabolic disease specialist, medical geneticist, or experienced hematologist
  • Medical necessity letter: Detailed rationale for treatment

Step 3: Submit Complete PA Request

Submit through the appropriate channel:

  • Commercial plans: BCBSTX portal or vendor-specific system
  • Medicare Advantage: Typically eviCore portal
  • Urgent cases: Request expedited review (72-hour decision for emergencies)
Tip: Under Texas House Bill 3459, some providers may qualify for PA exemptions on certain services, though this rarely applies to high-cost specialty drugs.

Common Denial Reasons & Solutions

Missing Diagnostic Confirmation

Denial: "Insufficient evidence of ASMD diagnosis"

Solution: Submit complete genetic testing report showing pathogenic SMPD1 variants OR enzyme assay demonstrating ASM activity <10% of normal. Include specialist note explicitly stating "acid sphingomyelinase deficiency" with appropriate ICD-10 code.

Inadequate Medical Necessity

Denial: "Not medically necessary" or "experimental/investigational"

Solution: Provide detailed letter addressing:

  • Specific organ involvement and severity
  • Symptoms and functional impact
  • Prior supportive care attempts and outcomes
  • Treatment goals and monitoring plan
  • FDA approval status (approved August 31, 2022)

Prescriber/Site Issues

Denial: "Prescriber not qualified" or "inappropriate site of care"

Solution: Ensure prescribing physician is a qualified specialist or co-managing with one. Document infusion site capabilities and safety protocols.

Quantity/Dosing Concerns

Denial: "Exceeds quantity limits"

Solution: Provide current weight, calculated mg/kg dose, and phase of therapy (escalation vs. maintenance). Reference FDA-approved dosing schedule.

Appeals Process in Texas

Texas provides strong patient protection through a structured appeals process with independent external review.

Internal Appeal (First Level)

  • Deadline: 180 days from denial for commercial plans
  • Decision timeline: 30 days for pre-service requests
  • How to file: Follow instructions on denial letter or submit via BCBSTX appeals process

Expedited Appeals

Available when delay could seriously jeopardize health:

  • Decision timeline: 72 hours
  • Eligibility: Progressive disease, organ dysfunction risk, or treatment interruption concerns

External Review (Independent Review Organization)

If internal appeal fails, Texas law provides access to binding external review:

  • Eligibility: Denials based on medical necessity, appropriateness, or experimental status
  • Deadline: 4 months from final internal denial
  • Timeline: 20 days standard, 5 days urgent
  • Cost: Insurer pays IRO fee
  • Outcome: Binding on the plan if overturned

To request: Contact Texas Department of Insurance at 1-800-252-3439 or Office of Public Insurance Counsel at 1-877-611-6742.

Note: Self-funded employer plans follow federal ERISA rules and may have different appeal processes. Check your Summary Plan Description.

Required Documentation Checklist

Essential Documents

  • Completed PA form with prescriber signature and date
  • Genetic testing report (SMPD1) or enzyme assay results
  • Specialist consultation note confirming ASMD diagnosis
  • Baseline imaging (MRI/ultrasound showing spleen/liver size)
  • Pulmonary function tests (if lung involvement)
  • Medical necessity letter with treatment rationale
  • Current weight and calculated dosing
  • Site of care information and capabilities

Medical Necessity Letter Components

Your specialist should address:

  1. Confirmed ASMD diagnosis with test results
  2. Organ involvement severity with objective measures
  3. Symptom burden and functional impact
  4. Prior treatments and their outcomes
  5. Treatment goals and expected benefits
  6. Monitoring plan for safety and efficacy
  7. Why Xenpozyme is appropriate for this patient

Costs & Patient Assistance

Manufacturer Support

Sanofi offers patient assistance through the Xenpozyme support program. Contact Xenpozyme.com for:

  • Financial assistance applications
  • Prior authorization support
  • Nursing and infusion coordination

Foundation Grants

Several rare disease foundations provide grants for ASMD patients:

  • National Niemann-Pick Disease Foundation
  • EveryLife Foundation for Rare Diseases
  • Patient Advocate Foundation

State Resources

Texas residents may qualify for additional support through state programs, though Texas has not expanded Medicaid, limiting some options compared to other states.

Frequently Asked Questions

How long does BCBSTX prior authorization take?

Standard PA decisions typically take 30 days for pre-service requests. Expedited reviews are decided within 72 hours when delay could jeopardize health.

What if Xenpozyme is not on my formulary?

As a medical benefit drug, Xenpozyme may not appear on pharmacy formularies. Check the medical drug benefit list and PA requirements instead.

Can I appeal if my doctor isn't a specialist?

Yes, but you'll need to demonstrate the prescriber's knowledge of ASMD or obtain co-management with a qualified specialist. Many appeals succeed when this documentation is added.

What's the difference between commercial and Medicare Advantage appeals?

Medicare Advantage follows federal Medicare appeal rules with different timelines and external review processes through Medicare's Independent Review Entity (IRE), not the Texas IRO system.

How much does Xenpozyme cost without insurance?

Wholesale acquisition cost ranges from approximately $1,515 for a 4mg vial to $7,576 for a 20mg vial. Monthly costs can exceed $30,000 depending on patient weight and dosing phase.

Can I request expedited external review?

Yes, if you meet criteria for urgent review (delay would seriously jeopardize health), you can request expedited external review concurrent with expedited internal appeal.

About Counterforce Health: Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into successful appeals by crafting evidence-based, targeted rebuttals that align with each plan's specific criteria. Our platform identifies denial reasons and creates point-by-point responses using FDA labeling, peer-reviewed studies, and specialty guidelines to support medical necessity arguments.

When facing complex prior authorization requirements for medications like Xenpozyme, having expert support can make the difference between approval and denial. Counterforce Health specializes in navigating these challenges for rare disease treatments.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Insurance coverage varies by plan and individual circumstances. Always consult your healthcare provider and insurance plan documents for specific guidance. For assistance with appeals and coverage issues, contact the Texas Department of Insurance or qualified healthcare advocates.

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