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

ICD-10 Codes and Billing Guide: Getting Xenpozyme Covered by UnitedHealthcare in Florida

Answer Box: Getting Xenpozyme Covered by UnitedHealthcare in Florida

Quick Path to Approval: Xenpozyme requires prior authorization through UnitedHealthcare with specific ICD-10 codes (E75.240-E75.244), genetic testing confirmation, and medical benefit billing. Submit via OptumRx at 1-833-547-2030 with complete clinical documentation including ASMD diagnosis confirmation, baseline spleen imaging, and specialist consultation notes. Appeals must be filed within 180 days if denied, with external review available through Florida Department of Financial Services.

First Step Today: Contact your metabolic disease specialist to confirm proper ICD-10 coding and gather required genetic testing results before submitting your prior authorization request.

Table of Contents

  1. Medical vs. Pharmacy Benefit Pathways
  2. ICD-10 Coding for ASMD Diagnosis
  3. HCPCS J-Code and NDC Billing
  4. Clean Prior Authorization Request
  5. Common Coding Pitfalls
  6. UnitedHealthcare Verification Steps
  7. Pre-Submission Audit Checklist
  8. Appeals Process in Florida
  9. FAQ

Medical vs. Pharmacy Benefit Pathways

Xenpozyme (olipudase alfa-rpcp) coverage through UnitedHealthcare depends on whether it's processed under your medical or pharmacy benefit. Understanding this distinction is crucial for proper billing and approval.

Medical Benefit Coverage applies when Xenpozyme is provider-administered in outpatient settings, physician offices, or specialty infusion centers. This pathway typically offers better coverage for infused specialty medications and follows UnitedHealthcare's medical prior authorization process through OptumRx.

Pharmacy Benefit Coverage may apply for Medicare Advantage members through either Part B medical benefit or Part D pharmacy benefit, depending on your specific plan structure. Coverage determinations are made case-by-case, with most commercial plans routing Xenpozyme through the medical benefit due to its infusion requirements.

Tip: Verify your specific benefit pathway before submitting claims. Contact UnitedHealthcare member services to confirm whether Xenpozyme processes under medical or pharmacy benefits for your plan.

ICD-10 Coding for ASMD Diagnosis

Proper ICD-10 coding is essential for Xenpozyme approval, as UnitedHealthcare requires specific documentation of acid sphingomyelinase deficiency (ASMD) diagnosis.

Primary ICD-10 Codes

ASMD Type ICD-10 Code Description Clinical Presentation
Type A E75.240 Niemann-Pick disease type A Infantile neurovisceral ASMD
Type A/B E75.244 Niemann-Pick disease type A/B Chronic neurovisceral ASMD
Type B E75.241 Niemann-Pick disease type B Chronic visceral ASMD
Type D E75.243 Niemann-Pick disease type D Nova Scotia variant

Documentation Requirements

Your medical records must clearly specify:

  • Disease type (A, A/B, B, or D) based on clinical presentation
  • Diagnostic confirmation through biochemical assay showing decreased acid sphingomyelinase activity (<10% of normal) in white blood cells or skin fibroblasts
  • Genetic testing revealing two pathogenic variants in the SMPD1 gene
  • Clinical manifestations supporting the specific ASMD subtype

All listed codes are billable and suitable for reimbursement purposes. Ensure your documentation aligns with the selected code to support medical necessity for Xenpozyme therapy.

HCPCS J-Code and NDC Billing

Xenpozyme received a permanent J-code effective April 1, 2023, replacing any temporary codes used previously. Claims filed with temporary J or C codes after this date are invalid.

Billing Codes and Units

HCPCS J-Code: Permanent J-code assigned (verify current code with UnitedHealthcare) NDC Numbers:

  • 4 mg/mL vial: WAC $1,515.25
  • 20 mg/5 mL vial: WAC $7,576.23

Dosing Calculations

Xenpozyme follows a specific dosing protocol:

Induction Phase (Weeks 0-14): Escalating doses from 0.3 mg/kg to 3 mg/kg Maintenance Phase (Week 16+): 3 mg/kg every two weeks

Weight-Based Calculations: Use actual body weight for patients with BMI ≤ 30. For higher BMI patients, adjusted calculations may apply per UnitedHealthcare guidelines.

Required Modifiers

JW Modifier: Required by Medicare and some commercial payers for discarded drug portions. Apply to any unused drug that cannot be stored or administered to another patient.

Site of Care Considerations: UnitedHealthcare may restrict coverage based on place of service. Non-hospital facility-based locations are typically preferred per utilization management policies.

Clean Prior Authorization Request

A complete prior authorization submission significantly improves your approval chances with UnitedHealthcare.

Essential Components

Patient Information:

  • Complete demographics and UnitedHealthcare member ID
  • Primary and specialist physician information
  • Preferred infusion site details

Clinical Documentation:

  • ASMD diagnosis confirmation with proper ICD-10 coding
  • Genetic testing results showing SMPD1 mutations
  • Biochemical assay results demonstrating enzyme deficiency
  • Baseline imaging (spleen MRI preferred)
  • Pulmonary function tests and liver function studies

Treatment Justification:

  • Medical necessity statement from metabolic disease specialist
  • Documentation that patient meets FDA labeling criteria
  • Dosing rationale based on patient weight and disease severity
  • Monitoring plan for infusion reactions and treatment response

Submission Process

Submit requests to OptumRx at 1-833-547-2030 with complete supporting documentation. Standard reviews are completed within 72 hours, while expedited reviews for urgent cases are completed within 24 hours.

Common Coding Pitfalls

Avoid these frequent errors that lead to UnitedHealthcare denials:

Unit Conversion Mistakes

Problem: Incorrect dosing calculations or unit conversions Solution: Double-check weight-based calculations using actual body weight. For a 70 kg patient on maintenance dosing (3 mg/kg), the total dose is 210 mg per infusion.

Mismatched ICD-10 Codes

Problem: Using generic or incorrect diagnostic codes Solution: Ensure your ICD-10 code matches the specific ASMD type documented in clinical notes. E75.240 for Type A, E75.241 for Type B, etc.

Missing Start Dates

Problem: Incomplete treatment timeline documentation Solution: Clearly document when Xenpozyme therapy is medically necessary to begin, including any delays that could impact patient health.

Site of Care Exclusions

Problem: Requesting administration at non-approved facilities Solution: Verify infusion center is in UnitedHealthcare's network and meets their site-of-care requirements before submitting.

UnitedHealthcare Verification Steps

Before submitting your prior authorization, verify these key elements through UnitedHealthcare resources:

Coverage Verification

  1. Check formulary status through the UnitedHealthcare provider portal
  2. Confirm prior authorization requirements for your specific plan type
  3. Verify preferred infusion sites in your geographic area
  4. Review any step therapy requirements or alternative medication trials needed

Documentation Cross-Check

Compare your submission against UnitedHealthcare's enzyme replacement therapy policy to ensure all requirements are addressed:

  • Genetic confirmation of ASMD
  • Specialist consultation documentation
  • Baseline clinical assessments
  • Appropriate dosing justification

Pre-Submission Audit Checklist

Review these items before submitting your Xenpozyme prior authorization:

Diagnosis & Coding:

  • Correct ICD-10 code selected (E75.240-E75.244)
  • Genetic testing results included
  • Biochemical assay documentation attached
  • Clinical notes support selected diagnostic code

Clinical Justification:

  • Medical necessity letter from specialist
  • FDA labeling criteria addressed
  • Dosing calculations verified
  • Monitoring plan documented

Administrative Details:

  • Complete patient demographics
  • UnitedHealthcare member ID verified
  • Preferred infusion site confirmed
  • All required forms completed

Supporting Documents:

  • Recent imaging studies (spleen assessment)
  • Laboratory results (liver function, lipids)
  • Pulmonary function tests
  • Previous treatment history documented

Appeals Process in Florida

If UnitedHealthcare denies your Xenpozyme prior authorization, Florida provides specific appeal rights and timelines.

Internal Appeal Process

Timeline: 180 days from denial date to file internal appeal Level 1 Decisions:

  • Standard appeals: 15 days
  • Expedited appeals: 72 hours (when delays could jeopardize health)

Submission Methods: Online portal, fax, or mail as specified in denial letter. Appeals can be filed by patient, provider, or authorized representative.

External Review Rights

Eligibility: Available after completing internal appeals or if UnitedHealthcare waives further review Timeline: 4 months from final internal denial to request external review Process: Contact Florida Department of Financial Services at 1-877-MY-FL-CFO (1-877-693-5236)

Binding Decision: External review decisions are legally binding and conducted by independent medical experts not affiliated with UnitedHealthcare.

State Regulatory Support

Florida's Division of Consumer Services provides assistance with insurance problems. Contact the Insurance Consumer Helpline at 1-877-MY-FL-CFO for help navigating the appeal process or if UnitedHealthcare fails to meet required response timelines.

From our advocates: "We've seen successful Xenpozyme appeals when families work closely with their metabolic specialist to document clear genetic confirmation and baseline disease measurements. The key is showing UnitedHealthcare that all their specific criteria are met with complete clinical evidence. This collaborative approach between patient, family, and medical team creates the strongest foundation for approval."

FAQ

How long does UnitedHealthcare prior authorization take for Xenpozyme in Florida? Standard prior authorization decisions are completed within 72 hours. Expedited reviews for urgent cases are completed within 24 hours when your provider attests that delays could seriously jeopardize your health.

What if Xenpozyme is non-formulary on my UnitedHealthcare plan? UnitedHealthcare may provide coverage for non-formulary medications when they're determined to be medically necessary. Submit a prior authorization with comprehensive clinical justification demonstrating medical necessity for ASMD treatment.

Can I request an expedited appeal if UnitedHealthcare denies coverage? Yes, expedited appeals are available when delays could seriously jeopardize your health. Your provider must attest to the urgent medical need, and decisions are made within 72 hours.

Does step therapy apply to Xenpozyme coverage? Since Xenpozyme is the only FDA-approved treatment for non-CNS ASMD manifestations, step therapy typically doesn't apply. However, UnitedHealthcare may require documentation that supportive care measures have been attempted.

What genetic testing is required for UnitedHealthcare approval? UnitedHealthcare requires either biochemical assay showing decreased acid sphingomyelinase activity (<10% of normal) in white blood cells or skin fibroblasts, OR genetic testing revealing two pathogenic variants in the SMPD1 gene.

How do I verify my infusion site is approved by UnitedHealthcare? Contact UnitedHealthcare member services or check the provider directory online. Some plans restrict coverage to specific infusion centers or require pre-approval for out-of-network facilities.

What happens if UnitedHealthcare doesn't respond within required timelines? If UnitedHealthcare fails to respond within statutory deadlines (72 hours for standard, 24 hours for expedited), contact the Florida Department of Financial Services at 1-877-MY-FL-CFO to file a complaint and ensure you receive full appeal rights.

About Counterforce Health: Counterforce Health specializes in helping patients, clinicians, and specialty pharmacies navigate complex prior authorization and appeals processes for specialty medications like Xenpozyme. Our platform transforms insurance denials into targeted, evidence-backed appeals by analyzing denial letters, plan policies, and clinical notes to identify specific denial reasons and draft point-by-point rebuttals aligned to each payer's requirements.

For Xenpozyme cases, Counterforce Health pulls the right citations from FDA labeling, peer-reviewed studies, and specialty guidelines while weaving in required clinical facts like genetic confirmation, baseline assessments, and treatment goals. The system produces letters that meet UnitedHealthcare's procedural requirements while tracking deadlines and required documentation, helping families get faster approvals with less back-and-forth.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage decisions vary by individual plan and clinical circumstances. Always consult with your healthcare provider and insurance company for guidance specific to your situation. For assistance with insurance problems in Florida, contact the Department of Financial Services Consumer Helpline at 1-877-MY-FL-CFO.

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