By Counterforce Health Team in Pombiliti (cipaglucosidase alfa-atga) —

Get Pombiliti Covered by Blue Cross Blue Shield in New York: ICD-10, J-Code, and Billing Guide

Answer Box: Blue Cross Blue Shield plans in New York require prior authorization for Pombiliti (cipaglucosidase alfa) using specific coding requirements. Use ICD-10 code E74.02 for Pompe disease, HCPCS J1203 for billing (per 5 mg), and submit via your BCBS provider portal with complete diagnostic documentation. If denied, you have 4 months to file an external appeal through New York's Department of Financial Services. First step: Gather enzyme assay results and genetic testing to support your PA request today.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit
  2. ICD-10 Mapping for Late-Onset Pompe Disease
  3. Product Coding: HCPCS, J-Code, and NDC
  4. Clean Request Anatomy
  5. Frequent Coding Pitfalls
  6. Verification with Blue Cross Blue Shield
  7. Quick Audit Checklist
  8. Appeals Process in New York
  9. FAQ

Coding Basics: Medical vs. Pharmacy Benefit

Pombiliti (cipaglucosidase alfa-atga) is an infused enzyme replacement therapy that falls under your medical benefit, not pharmacy benefit. This distinction affects how you code, bill, and get approval from Blue Cross Blue Shield in New York.

Medical Benefit Coverage:

  • Physician-administered infusions at hospitals, infusion centers, or clinics
  • Billed using HCPCS J-codes and CPT administration codes
  • Requires prior authorization through BCBS medical management
  • Subject to medical necessity criteria and specialist requirements

Key Difference: The oral companion drug Opfolda (miglustat) may sometimes fall under pharmacy benefit depending on your specific BCBS plan, but Pombiliti infusions are always medical benefit.

Tip: Check your BCBS plan's Summary of Benefits to confirm whether specialty infusions require referrals to specific in-network facilities.

ICD-10 Mapping for Late-Onset Pompe Disease

The primary diagnosis code for late-onset Pompe disease is E74.02 (Pompe disease). This code covers all forms of Pompe disease, including late-onset presentations.

Documentation Requirements for E74.02

Your medical records must include:

Essential Clinical Documentation:

  • Progressive proximal muscle weakness (limb-girdle, trunk involvement)
  • Elevated creatine kinase (CK), AST, or ALT levels
  • Decreased acid alpha-glucosidase enzyme activity
  • Genetic confirmation via GAA gene sequencing (when performed)

Supporting Clinical Features:

  • Exercise intolerance or respiratory insufficiency
  • Scapular winging
  • Family history of Pompe disease
  • Exclusion of other muscle disorders
Clinician Corner: Document the timeline of symptom progression and impact on daily activities. BCBS reviewers look for clear evidence that symptoms are consistent with late-onset Pompe disease rather than other neuromuscular conditions.

Avoid These Common Coding Errors:

  • Using E74.00 (glycogen storage disease, unspecified) instead of E74.02
  • Failing to document enzyme deficiency confirmation
  • Missing genetic testing results when available

Product Coding: HCPCS, J-Code, and NDC

HCPCS J-Code: J1203

Pombiliti is billed using J1203 ("Injection, cipaglucosidase alfa-atga, POMBILITI, per 5 mg"), effective April 1, 2024.

Dosing and Unit Calculations:

  • Standard dose: 20 mg/kg actual body weight every 2 weeks
  • Each vial contains 105 mg
  • Billing units = total mg administered ÷ 5 mg per unit

Example Calculation:

  • 70 kg patient = 1,400 mg dose
  • Vials needed: 1,400 mg ÷ 105 mg = 13.33 → 14 vials (round up)
  • Billing units: 1,400 mg ÷ 5 mg = 280 units of J1203

NDC Reporting Requirements

Blue Cross Blue Shield requires 11-digit NDC codes on medical benefit claims for physician-administered drugs.

NDC Format on Claims:

  • Enter "N4" + NDC + unit of measure + quantity
  • Example: N471904020001UN280 (verify actual NDC from vial)
  • Report in Box 24A shaded area on CMS-1500 forms

Billing Modifiers

JW Modifier (Drug Wastage): Use when vials are discarded due to single-use requirements or dosing calculations.

  • Example: Patient needs 1,400 mg, uses 14 vials (1,470 mg total)
  • Bill: 280 units J1203 + 14 units J1203-JW for waste

Clean Request Anatomy

Prior Authorization Request Components

Patient Information Section:

  • BCBS member ID and group number
  • Patient weight (for dosing calculations)
  • Primary diagnosis: E74.02 (Pompe disease)

Clinical Documentation:

  • Enzyme assay results showing GAA deficiency
  • Genetic testing confirmation (GAA gene mutations)
  • Documentation of inadequate response to previous enzyme replacement therapy
  • Specialist consultation notes (neurologist, geneticist, or metabolic specialist)

Treatment Plan:

  • Pombiliti 20 mg/kg IV every 2 weeks
  • Combination with Opfolda (miglustat) oral therapy
  • Infusion site and administration plan
  • Monitoring schedule for hypersensitivity reactions
Example PA Request Line: "Request prior authorization for Pombiliti (J1203) for 70-year-old patient with genetically confirmed late-onset Pompe disease (E74.02). GAA enzyme activity 0.8% of normal. Failed to improve on 18 months of Lumizyme therapy with continued 6-minute walk test decline. Prescribed by Dr. Smith, neurologist, for IV infusion every 2 weeks at certified infusion center."

Frequent Coding Pitfalls

Unit Conversion Errors

  • Wrong: Billing by vial count instead of mg
  • Right: Calculate total mg administered ÷ 5 mg per billing unit

Missing Documentation

  • Wrong: Submitting PA without enzyme assay or genetic testing
  • Right: Include all diagnostic confirmation before requesting authorization

Incorrect Benefit Classification

  • Wrong: Trying to process Pombiliti through pharmacy benefit
  • Right: Always bill under medical benefit with J-codes

Administration Coding Mistakes

  • Wrong: Using basic injection codes (96372)
  • Right: Use appropriate infusion codes (96365, 96413) for 4-hour infusions

Verification with Blue Cross Blue Shield

Check Current Requirements

Provider Portal Steps:

  1. Log into your BCBS provider portal
  2. Navigate to "Prior Authorization" or "Utilization Management"
  3. Search for "Pombiliti" or "cipaglucosidase alfa"
  4. Download current clinical criteria document

Key Verification Points:

  • Confirm J1203 is recognized (effective April 2024)
  • Check if step therapy requirements apply
  • Verify in-network infusion site requirements
  • Review quantity limits or dosing restrictions

Contact Information for Verification

BCBS Provider Services:

  • Call the provider services number on your contract
  • Have NPI, tax ID, and patient member ID ready
  • Ask specifically about "specialty infusion prior authorization requirements"

Quick Audit Checklist

Before Submitting Your Request:

□ Diagnosis: E74.02 documented with supporting lab/genetic evidence
□ Product: J1203 with correct mg-to-unit conversion
□ NDC: 11-digit code properly formatted with N4 prefix
□ Dosing: 20 mg/kg calculation documented with patient weight
□ Specialist: Neurologist, geneticist, or metabolic specialist involved
□ Prior Therapy: Documentation of inadequate response to other ERTs
□ Site of Care: Confirmed in-network infusion facility
□ Combination Therapy: Opfolda prescription included in treatment plan
□ Modifiers: JW modifier calculated for any drug waste

Appeals Process in New York

If Blue Cross Blue Shield denies your Pombiliti request, New York offers strong appeal rights through the Department of Financial Services.

Internal Appeal (First Step)

  • Timeline: Submit within 180 days of denial
  • Method: BCBS member portal or written request
  • Documentation: Include additional clinical evidence, peer-reviewed studies
  • Decision: BCBS has 30 days to respond (15 days for urgent)

External Appeal (Final Step)

  • Eligibility: After final adverse determination from BCBS
  • Timeline: 4 months to file with New York DFS
  • Cost: Free to patients
  • Decision: Independent medical experts review within 30 days
  • Binding: BCBS must cover if appeal succeeds

New York DFS Contact:

  • Website: www.dfs.ny.gov
  • Phone: 1-800-400-8882
  • Forms: Download external appeal application online
From our advocates: We've seen Pompe disease appeals succeed when families include peer-reviewed studies showing Pombiliti's benefits for patients who plateau on traditional enzyme replacement therapy. The key is demonstrating that continued decline on current therapy makes Pombiliti medically necessary, not just preferred.

Consumer Assistance

Community Health Advocates (CHA):

  • Phone: 888-614-5400
  • Free help filing appeals and understanding your rights
  • Assistance with gathering medical documentation

FAQ

How long does Blue Cross Blue Shield prior authorization take in New York? Standard PA requests take 15 business days. Urgent requests (when delay could harm patient health) must be decided within 72 hours.

What if Pombiliti is non-formulary on my BCBS plan? File a formulary exception request with medical necessity documentation. Include evidence that formulary alternatives (Lumizyme, Nexviazyme) have failed or are contraindicated.

Can I request expedited appeal if denied? Yes, if your doctor certifies that delay would seriously jeopardize your health. New York requires expedited appeals to be decided within 72 hours.

Does step therapy apply if I've tried other ERTs outside New York? Yes, prior therapy documentation from any state counts toward step therapy requirements. Include medical records showing dates, dosages, and outcomes.

What happens if my infusion center isn't in-network? Request an out-of-network exception based on medical necessity and lack of in-network alternatives. Include distance/travel burden documentation.

How do I find BCBS-approved infusion centers in New York? Use your BCBS provider directory online or call member services. Search for "infusion centers" or "specialty pharmacies" in your area.

About Counterforce Health

Counterforce Health helps patients, clinicians, and specialty pharmacies turn insurance denials into successful appeals by providing evidence-backed, payer-specific strategies. Our platform analyzes denial letters and drafts targeted rebuttals using FDA labeling, peer-reviewed studies, and specialty guidelines aligned to each plan's own coverage criteria.

Whether you're facing a Blue Cross Blue Shield denial for Pombiliti or need help with other specialty drug appeals, Counterforce Health provides the documentation and strategic guidance needed to improve approval rates and reduce administrative burden on clinical teams.

Sources & Further Reading

Medical Disclaimer: This information is for educational purposes only and does not constitute medical advice. Always consult with qualified healthcare providers and verify current insurance policies before making treatment decisions. Coverage policies and coding requirements may change - confirm current requirements with your specific Blue Cross Blue Shield plan and healthcare providers.

Powered by Counterforce Health—AI that turns drug denials into evidence-based appeals patients and clinicians can submit today.