By Counterforce Health Team in Lamzede (velmanase alfa-tycv) —

Getting Lamzede (Velmanase Alfa-tycv) Covered by Aetna CVS Health in Pennsylvania: ICD-10, HCPCS Codes, and Appeals Guide

Answer Box: Getting Lamzede Covered by Aetna CVS Health in Pennsylvania

Lamzede (velmanase alfa-tycv) requires prior authorization from Aetna CVS Health in Pennsylvania. Submit through Availity/Novologix with confirmed alpha-mannosidosis diagnosis (ICD-10: E77.1), genetic or enzyme testing, and specialist letter. Bill using HCPCS J0217 (1 unit = 1 mg). If denied, file internal appeals within plan deadlines, then use Pennsylvania's new external review program (50% overturn rate in 2024). First step: Have your metabolic specialist complete Aetna's specialty drug PA form via Availity portal.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit
  2. ICD-10 Mapping for Alpha-Mannosidosis
  3. HCPCS/J-Code and NDC Billing
  4. Clean Prior Authorization Anatomy
  5. Common Coding Pitfalls
  6. Verifying Codes with Aetna CVS Health
  7. Appeals Process in Pennsylvania
  8. Quick Audit Checklist
  9. FAQ

Coding Basics: Medical vs. Pharmacy Benefit Paths

Lamzede typically falls under the medical benefit for infusion therapies, though some Aetna plans route it through CVS Specialty under the pharmacy benefit. The coding approach differs slightly:

Medical Benefit Path:

  • Bill facility/physician using HCPCS J0217
  • Include NDC on claim line with appropriate modifiers
  • Prior authorization through Aetna medical management

Pharmacy Benefit Path:

  • CVS Specialty dispenses after PA approval
  • Still uses J0217 for billing but processed differently
  • PA through CVS Caremark pharmacy team
Tip: Check the patient's benefits summary or call the number on their ID card to confirm which benefit covers specialty infusions.

ICD-10 Mapping for Alpha-Mannosidosis

Primary Diagnosis Code

E77.1 – Defects in glycoprotein degradation is the standard ICD-10 code for alpha-mannosidosis due to MAN2B1 deficiency, as recognized by Orphanet and used in payer policies.

Supporting Documentation Language

When documenting for medical necessity, include these key phrases in clinical notes:

  • "Alpha-mannosidosis confirmed by MAN2B1 pathogenic variants"
  • "Documented deficiency of alpha-mannosidase activity in leukocytes"
  • "Non-CNS manifestations including [specific symptoms]"
  • "Progressive multisystem lysosomal storage disorder"

Additional ICD-10 Codes for Manifestations

Consider adding secondary codes to reflect disease burden:

  • F70-F79: Intellectual disabilities
  • H90-H94: Hearing loss
  • M87-M89: Skeletal dysplasia manifestations
  • R50-R69: Recurrent infections/immunodeficiency symptoms

HCPCS/J-Code and NDC Billing

Core Billing Information

Element Details Notes
HCPCS Code J0217 1 unit = 1 mg velmanase alfa-tycv
Vial Strength 10 mg single-dose Bill units based on patient weight
Dosing 1 mg/kg IV weekly Round to nearest mg per protocol
NDC Format 11-digit (5-4-2) Must match product labeling

Units Calculation Examples

  • 25 kg patient: 25 mg weekly = 25 units of J0217
  • 70 kg patient: 70 mg weekly = 70 units of J0217
  • 110+ kg patient: May require PA for doses >110 mg weekly
Note: Many payers set maximum billing limits at 110 units (110 mg) per week. Larger patients may need dose justification.

NDC and Modifier Requirements

Include on each claim line:

  • NDC number from the 10 mg vial packaging
  • Unit of measure (typically "UN" for units or "ML" depending on payer)
  • Quantity dispensed/administered
  • N4 qualifier if required by payer

For drug wastage, use appropriate modifiers:

  • JW: Drug amount discarded/not administered
  • JZ: Zero drug amount discarded/not administered

Clean Prior Authorization Anatomy

Essential PA Components

Patient Information Section:

  • Full name, DOB, Aetna member ID
  • Group number and plan type
  • Contact information for follow-up

Clinical Documentation:

  • Diagnosis: "Alpha-mannosidosis (E77.1) confirmed by [genetic testing/enzyme assay]"
  • Test Results: Include lab reports showing MAN2B1 variants or enzyme deficiency
  • Symptom Profile: Document non-CNS manifestations being targeted
  • Prior Treatments: List supportive care measures tried

Prescriber Attestation:

  • Specialty (metabolic genetics, pediatric neurology, etc.)
  • Statement of medical necessity
  • Confirmation of FDA-approved indication use
  • Monitoring plan for infusion reactions
From our advocates: We've seen the strongest PA approvals when genetic test results and a detailed specialist letter are submitted together in the initial request. This avoids the back-and-forth that can delay approval by weeks.

Common Coding Pitfalls

Unit Conversion Errors

Wrong: Billing 10 units because there's one 10 mg vial Right: Billing actual mg administered (e.g., 40 units for 40 kg patient)

Mismatched Diagnosis Codes

Wrong: Using general "metabolic disorder" codes Right: Specific E77.1 with supporting documentation

Missing Start Dates

Always include:

  • Date of first infusion
  • Treatment start rationale
  • Expected duration of therapy

Incomplete NDC Information

Ensure NDC matches exactly:

  • Product packaging NDC
  • Correct 11-digit format
  • Appropriate unit of measure

Verifying Codes with Aetna CVS Health

Pre-Submission Verification

  1. Check Formulary Status
  2. Confirm PA Requirements
  3. Validate Coding
    • Cross-reference J0217 in payer fee schedules
    • Confirm NDC acceptance in system
    • Check for any billing edits or restrictions

Real-Time Verification Tools

  • Availity Portal: Real-time PA status checking
  • CVS Specialty: Benefits investigation services
  • Aetna Provider Services: Phone verification (number on ID card)

Appeals Process in Pennsylvania

Internal Appeals with Aetna CVS Health

First Level - Standard Appeal:

  • Deadline: Typically 180 days from denial
  • Timeline: 30-45 days for decision
  • Submission: Via Availity portal or fax to PA department

Expedited Appeals:

  • Timeline: 72 hours for urgent cases
  • Criteria: Delay would seriously jeopardize health
  • Documentation: Physician urgency statement required

Pennsylvania External Review (2024)

Pennsylvania's new Independent External Review Program offers significant hope for denied specialty drug appeals. In 2024, about 50% of appealed denials were overturned.

Eligibility Requirements:

  • Must complete Aetna's internal appeal process first
  • Receive Final Adverse Benefit Determination letter
  • Apply within 4 months of final denial
  • Plan must be fully insured (not self-funded)

Timeline:

  • Standard Review: 45 days from IRO assignment
  • Expedited Review: 72 hours for urgent cases
  • Additional Evidence: 15 days after IRO assignment to submit extra documentation

How to File:

Tip: The Pennsylvania Insurance Department Consumer Services Bureau (1-877-881-6388) can help guide you through the external review process.

Quick Audit Checklist

Before Submitting PA:

  • Confirmed alpha-mannosidosis diagnosis with genetic/enzyme testing
  • ICD-10 E77.1 properly documented in medical records
  • Specialist prescriber (metabolic genetics, neurology, etc.)
  • Prior supportive treatments documented
  • Medical necessity letter addresses non-CNS manifestations

Before Billing:

  • J0217 units match actual mg administered (not vials used)
  • NDC matches 10 mg vial product exactly
  • 11-digit NDC format correct
  • Appropriate modifiers for wastage (JW/JZ)
  • E77.1 as primary diagnosis on claim

Before Appeals:

  • All denial letters collected and reviewed
  • Specific denial reasons identified
  • Additional clinical evidence gathered
  • Appeal deadline calculated and noted
  • Urgency criteria evaluated for expedited process

FAQ

How long does Aetna CVS Health PA take for Lamzede in Pennsylvania? Aetna reports over 95% of eligible prior authorizations are approved within 24 hours when complete documentation is submitted. Complex cases may take 24-72 hours.

What if Lamzede is non-formulary on my Aetna plan? Request a formulary exception through the same PA process. Document that formulary alternatives are not effective for alpha-mannosidosis or would cause adverse effects.

Can I request an expedited appeal if treatment is urgent? Yes, if delay would seriously jeopardize health or ability to regain function. Include physician statement of urgency and request expedited review (72-hour timeline).

Does step therapy apply to ultra-rare diseases like alpha-mannosidosis? Many payers waive step therapy for orphan diseases with no alternatives. Document that Lamzede is the only FDA-approved therapy for alpha-mannosidosis.

What happens if my employer plan is self-funded? Self-funded plans typically follow federal external review rules rather than Pennsylvania's state program. Check your plan documents or contact HR for specific appeal rights.

How do I find out if my Aetna plan covers the medical or pharmacy benefit? Check your benefits summary or call the member services number on your ID card. CVS Specialty can also perform a benefits investigation to determine coverage pathway.

About Counterforce Health

Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into successful appeals for prescription drugs. Our platform analyzes denial letters and plan policies to create targeted, evidence-backed appeals that address each payer's specific requirements—helping families get life-changing medications like Lamzede covered.

When navigating complex prior authorizations and appeals, having the right documentation and strategy can make the difference between approval and ongoing denials. Counterforce Health's system pulls together FDA labeling, peer-reviewed studies, and payer-specific criteria to build compelling cases for medical necessity.

Disclaimer: This guide provides general information about insurance coverage and appeals processes. It is not medical advice and should not replace consultation with your healthcare provider or insurance professional. Coverage policies vary by plan and change frequently. Always verify current requirements with your specific Aetna CVS Health plan and consult with qualified professionals for your individual situation.

For additional help with insurance appeals in Pennsylvania, contact the Pennsylvania Insurance Department Consumer Services Bureau at 1-877-881-6388.

Sources & Further Reading

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