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

Pombiliti Coverage by Aetna CVS Health in Illinois: Decision Tree, Forms & Appeal Process

Answer Box: Getting Pombiliti Covered by Aetna CVS Health in Illinois

To get Pombiliti (cipaglucosidase alfa-atga) covered by Aetna CVS Health in Illinois, you must meet specific criteria: be 18+ years old, weigh ≥40 kg, have confirmed late-onset Pompe disease, and demonstrate you're not improving on current enzyme replacement therapy. Your first step today is to gather documentation of ERT failure (6-minute walk test results, pulmonary function tests, muscle strength assessments) and have your specialist submit a prior authorization through Aetna's Availity portal at least two weeks in advance. If denied, Illinois law gives you strong appeal rights with 4 months to file for external review through the Illinois Department of Insurance.

Table of Contents

  1. How to Use This Guide
  2. Eligibility Triage: Do You Qualify?
  3. If "Likely Eligible": Your Action Plan
  4. If "Possibly Eligible": Tests to Request
  5. If "Not Yet": Alternative Options
  6. If Denied: Illinois Appeal Process
  7. Coverage Requirements at a Glance
  8. Common Denial Reasons & Solutions
  9. Frequently Asked Questions
  10. Sources & Further Reading

How to Use This Guide

This decision tree helps patients and clinicians navigate Aetna CVS Health's prior authorization process for Pombiliti in Illinois. Work through each section sequentially—start with the eligibility triage, then follow the appropriate pathway based on your results.

Key contacts to save now:

  • Aetna Prior Authorization: 1-888-632-3862 (commercial) / 1-800-624-0756 (Medicare)
  • Specialty PA Fax: 1-888-267-3277
  • Illinois Department of Insurance: 1-877-527-9431

Eligibility Triage: Do You Qualify?

Answer these questions to determine your approval likelihood:

✅ Likely Eligible If ALL Apply:

  • Age 18+ years and weight ≥40 kg
  • Confirmed late-onset Pompe disease diagnosis (enzyme assay + genetic testing)
  • Currently on Lumizyme or Nexviazyme with documented lack of improvement
  • Specialist (metabolic/lysosomal storage disorder expert) involved in care
  • Will take Pombiliti with Opfolda (miglustat) combination therapy

⚠️ Possibly Eligible If SOME Apply:

  • Have Pompe disease but missing complete diagnostic confirmation
  • On ERT but improvement/decline documentation incomplete
  • Seeing a neurologist but not a metabolic specialist
  • Previous ERT trials but gaps in medical records

❌ Not Yet Eligible If ANY Apply:

  • Under 18 years old or weight <40 kg
  • No confirmed Pompe disease diagnosis
  • Never tried standard ERT (Lumizyme/Nexviazyme)
  • Currently improving on ERT with documented benefits

If "Likely Eligible": Your Action Plan

Document Checklist

Gather these items before submitting your prior authorization:

Required Clinical Documentation:

  • Diagnostic confirmation: GAA enzyme assay results AND genetic testing showing pathogenic GAA variants
  • ERT failure evidence: Serial assessments showing ≥10% decline or lack of improvement in:
    • 6-minute walk test results (6+ months apart)
    • Forced vital capacity (upright/supine positions)
    • Manual muscle testing or quantitative strength assessments
  • Current ERT details: Drug name, dose, duration (minimum 12-18 months), adherence records
  • Specialist attestation from metabolic disease or lysosomal storage disorder physician

Insurance Information:

  • Current Aetna member ID and group number
  • Prior authorization history for other Pompe treatments
  • Formulary tier verification for Pombiliti

Submission Process

Step 1: Your specialist completes the Aetna prior authorization form with all clinical documentation attached.

Step 2: Submit via Availity provider portal or fax to 1-888-267-3277 (specialty drugs).

Step 3: Aetna reviews within 30-45 days for standard requests, 72 hours for expedited.

Step 4: If approved, coordinate with CVS Specialty Pharmacy for delivery and infusion scheduling.

Tip: Submit at least two weeks before you need to start treatment. Aetna approves over 95% of eligible prior authorizations within 24 hours when complete documentation is provided.

If "Possibly Eligible": Tests to Request

Diagnostic Gaps to Address

Missing Pompe Diagnosis Confirmation:

  • Request GAA enzyme activity assay (blood-based screening)
  • Follow up with GAA gene sequencing for definitive diagnosis
  • Consider dried blood spot testing as rapid first-line option

Incomplete ERT Failure Documentation:

  • Schedule 6-minute walk test if not done in past 6 months
  • Request pulmonary function testing (FVC upright and supine)
  • Document current functional status and disability progression

Specialist Referral Needed:

  • Ask your neurologist for referral to metabolic disease specialist
  • Consider neuromuscular specialist familiar with lysosomal storage disorders

Timeline to Reapply

Plan for 3-6 months to gather complete documentation before resubmitting. Use this time to:

  • Complete missing diagnostic tests
  • Document ERT response over additional months
  • Establish care with appropriate specialist

If "Not Yet": Alternative Options

Continue Current ERT Optimization

Work with your care team to:

  • Optimize current Lumizyme or Nexviazyme dosing
  • Address any adherence barriers
  • Monitor for future decline that would qualify you for Pombiliti

Prepare for Future Exception Request

If you don't meet standard criteria but have compelling clinical circumstances:

  • Document any contraindications to standard ERT
  • Gather supporting literature for off-label or compassionate use
  • Consider clinical trial participation through ClinicalTrials.gov

If Denied: Illinois Appeal Process

Illinois provides robust appeal rights with specific timelines and processes.

Internal Appeal (First Level)

Timeline: Must file within 180 days of denial notice Process: Submit appeal letter with additional clinical documentation to Aetna Decision Time: 15 business days for pre-service requests, 24 hours if expedited Contact: Use denial notice instructions or call member services

Peer-to-Peer Review

Request your specialist speak directly with Aetna's medical director:

  • Call 1-855-240-0535 for specialty drug peer-to-peer requests
  • Prepare clinical summary emphasizing ERT failure evidence
  • Reference Aetna's published criteria point-by-point

External Review (Illinois)

If internal appeal fails, Illinois law guarantees independent external review:

Timeline: Must file within 4 months of final adverse determination Process: Complete Illinois Department of Insurance external review form Submit to: Illinois Department of Insurance, 320 W. Washington St. 4th Floor, Springfield, IL 62767 Decision Time: 45 days standard, 72 hours expedited Cost: Free to patients (insurers pay review costs)

Important: Illinois has a shorter external review window than many states. Don't wait—file within 4 months of your final denial.

Coverage Requirements at a Glance

Requirement What It Means Where to Find Documentation Source
Age ≥18 years Adult patients only Birth certificate, ID Aetna Policy
Weight ≥40 kg Minimum body weight Recent clinic visit notes Aetna Policy
LOPD diagnosis Confirmed late-onset Pompe Enzyme assay + genetic testing Aetna Policy
ERT failure Not improving on current therapy Serial 6MWT, FVC, strength tests Aetna Policy
Specialist care Metabolic disease physician Referral letter, consultation notes Aetna Policy
Combination therapy Must use with Opfolda Prescription for both medications Aetna Policy

Common Denial Reasons & Solutions

Denial Reason How to Overturn Required Documentation
"Diagnosis not confirmed" Submit complete diagnostic workup GAA enzyme assay + genetic testing results
"No evidence of ERT failure" Provide serial objective assessments 6MWT, FVC, muscle strength over 6-12 months
"Not prescribed by specialist" Obtain specialist consultation Letter from metabolic disease physician
"Experimental/investigational" Reference FDA approval FDA approval letter and prescribing information
"Non-formulary drug" File formulary exception Clinical justification for medical necessity

Frequently Asked Questions

How long does Aetna prior authorization take in Illinois? Standard requests take 30-45 days, but over 95% of eligible requests are approved within 24 hours when complete documentation is provided. Expedited requests are processed within 72 hours.

What if Pombiliti is non-formulary on my plan? You can request a formulary exception by demonstrating medical necessity and that formulary alternatives are inappropriate for your condition. Submit via the specialty prior authorization process.

Can I request an expedited appeal in Illinois? Yes, if waiting for standard review could seriously jeopardize your health. Mark your appeal as "expedited" and have your physician document the urgency. Illinois requires decisions within 72 hours for expedited external reviews.

Does step therapy apply if I've tried ERT outside Illinois? Yes, ERT failure documentation from any state is acceptable as long as you have complete medical records showing lack of improvement on Lumizyme or Nexviazyme.

How much does Pombiliti cost without insurance? The combination of Pombiliti and Opfolda costs approximately $650,000 annually at list price. Amicus Therapeutics offers patient assistance programs that may help with copays and access.

What support is available during the appeal process? The Illinois Department of Insurance offers free consumer assistance at 1-877-527-9431. The Illinois Attorney General's Health Care Helpline (1-877-305-5145) can also help with complex cases.

About Counterforce Health

Counterforce Health helps patients, clinicians, and specialty pharmacies get prescription drugs approved by turning insurance denials into targeted, evidence-backed appeals. The platform analyzes denial letters and plan policies to create point-by-point rebuttals aligned with payers' own rules, pulling the right clinical evidence and citations to support medical necessity. For complex cases like Pombiliti approval, having expert support can make the difference between approval and prolonged delays in accessing life-changing treatment.

From our advocates: "We've seen many Pompe disease appeals succeed when families provide complete ERT failure documentation upfront. The key is showing objective decline in walking ability or lung function over time, not just a single test result. Specialists who document this clearly in their letters see much higher approval rates." This represents general best practices from multiple cases, not guaranteed outcomes.

Sources & Further Reading

This guide provides general information about insurance coverage and appeal processes. It is not medical advice and should not replace consultation with your healthcare provider or insurance company. Coverage policies may change, and individual circumstances vary. Always verify current requirements with your specific plan and consult the official sources linked above. For personalized assistance with complex appeals, consider working with organizations like Counterforce Health that specialize in insurance authorization support.

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