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

Cigna's Coverage Criteria for Pombiliti (cipaglucosidase alfa-atga) in Michigan: What Counts as "Medically Necessary"?

Answer Box: Getting Pombiliti Covered by Cigna in Michigan

Cigna requires prior authorization for Pombiliti + Opfolda combination therapy for late-onset Pompe disease patients ≥18 years, weighing >40 kg, who show no improvement in forced vital capacity (FVC) or 6-minute walk test after ≥1 year on Lumizyme or Nexviazyme. Submit documentation through Express Scripts/Cigna portal with specialist prescription, diagnostic confirmation (GAA deficiency), and objective evidence of ERT failure. If denied, Michigan patients have 127 days for external review through DIFS. First step today: Gather your prior ERT records showing lack of improvement and contact your specialist to initiate the PA request.

Table of Contents

  1. Policy Overview
  2. Indication Requirements
  3. Step Therapy & Exceptions
  4. Required Diagnostics
  5. Site of Care Requirements
  6. Evidence to Support Medical Necessity
  7. Common Denial Reasons & Fixes
  8. Appeals Process in Michigan
  9. Coverage at a Glance
  10. FAQ

Policy Overview

Cigna manages specialty medications like Pombiliti through its specialty pharmacy partner Express Scripts and Accredo. The coverage criteria apply across Cigna's commercial plans, including HMO, PPO, and POS products in Michigan. Medicare Advantage and Medicaid plans may have similar but distinct requirements.

Where to Find Official Documentation:

These policies are updated regularly, with the most recent revisions effective January 2026.

Indication Requirements

FDA-Approved Indication

Pombiliti (cipaglucosidase alfa-atga) in combination with Opfolda (miglustat) is FDA-approved for adult patients with late-onset Pompe disease who weigh at least 40 kg and are not improving on current enzyme replacement therapy.

Cigna's Coverage Criteria

All of the following must be met:

  • Age: ≥18 years old
  • Weight: >40 kg (88 pounds)
  • Diagnosis: Confirmed late-onset Pompe disease (acid alpha-glucosidase deficiency)
  • Combination requirement: Pombiliti must be used with Opfolda
  • Prescriber: By or in consultation with a specialist (geneticist, neurologist, or metabolic specialist)
Note: The combination cannot be split—both medications must be prescribed together and cannot be used with other GAA enzyme replacement therapies.

Step Therapy & Exceptions

Required Prior ERT Trial

Cigna requires documented failure of at least one year of treatment with either:

  • Lumizyme (alglucosidase alfa), OR
  • Nexviazyme (avalglucosidase alfa-ngpt)

Defining "No Improvement"

Patients must show lack of improvement in objective measures after ≥1 year of prior ERT, specifically:

  • Forced Vital Capacity (FVC): No improvement in percent predicted sitting FVC
  • Six-Minute Walk Test (6MWT): No improvement in walking distance

Medical Exception Pathways

Step therapy may be bypassed if:

  • Severe hypersensitivity reactions to prior ERT
  • Documented contraindications to Lumizyme or Nexviazyme
  • Clinical deterioration despite adequate trial duration

When requesting an exception, provide detailed documentation of adverse events or contraindications with supporting medical records.

Required Diagnostics

Diagnostic Confirmation

Laboratory Evidence Required:

  • Enzyme testing: Reduced GAA activity via dried blood spot (DBS) assay, confirmed by leukocyte or fibroblast testing
  • Genetic testing: Biallelic pathogenic variants in the GAA gene (e.g., c.-32-13T>G common in LOPD)

Functional Assessments

Baseline Documentation Needed:

  • Sitting FVC ≥30% predicted (required for eligibility)
  • 6MWT distance (baseline measurement essential)
  • Documentation of progressive muscle weakness
  • Evidence of respiratory involvement

Supporting Tests

While not always required, these may strengthen your case:

  • Muscle MRI showing paravertebral involvement
  • EMG demonstrating myopathic changes
  • Mildly elevated CK levels
  • Muscle biopsy (if performed) showing glycogen accumulation
Tip: Ensure all diagnostic tests are from CLIA-certified laboratories and include reference ranges for enzyme activity levels.

Site of Care Requirements

Cigna prioritizes the least intensive medically necessary site of care for infusion therapy. For Pombiliti:

Preferred Settings (in order)

  1. Home infusion (when medically appropriate)
  2. Ambulatory infusion centers
  3. Physician office infusion
  4. Hospital outpatient (only if medically necessary)

Hospital Coverage Criteria

Hospital-based infusion is covered only if:

  • FDA-approved site restrictions exist
  • Documented adverse events unmanageable by premedication
  • Significant comorbidities (cardiopulmonary issues) preclude less intensive sites

Accredo's home infusion program reports a 4.5% reaction rate with only 0.35% requiring advanced care, potentially offering 40-60% cost savings versus outpatient settings.

Evidence to Support Medical Necessity

Clinical Guidelines to Reference

  • FDA Prescribing Information: Pombiliti + Opfolda official labeling
  • PROPEL Trial Data: Phase 3 study showing 3% improvement in sitting FVC
  • Pompe Disease Guidelines: International consensus recommendations

Key Evidence Points

For Medical Necessity Letter:

  1. Problem Statement: Late-onset Pompe disease with progressive weakness/respiratory decline
  2. Prior Treatments: Detailed history of ERT failure with objective measurements
  3. Clinical Rationale: Why Pombiliti + Opfolda is appropriate next step
  4. Monitoring Plan: How response will be assessed (FVC, 6MWT, functional scales)

Documentation Best Practices

  • Include ICD-10 code E74.02 (Pompe disease)
  • Reference specific policy numbers (IP_0591, IP_0598)
  • Attach lab reports with clear reference ranges
  • Provide timeline of prior ERT with response measurements

Common Denial Reasons & Fixes

Denial Reason How to Fix Documentation Needed
Diagnosis not confirmed Submit enzyme/genetic testing GAA activity results, genetic report
Insufficient prior ERT trial Document ≥1 year treatment history Pharmacy records, clinic notes, objective measures
Missing specialist involvement Get specialist consultation Referral letter, specialist prescription
Concurrent ERT use Confirm no other GAA therapies Current medication list, pharmacy records
Weight <40 kg Document current weight Recent clinic visit notes
Lack of objective decline Provide FVC/6MWT data Pulmonary function tests, walk test results
From our advocates: "We've seen denials overturned when families provided a clear timeline showing declining FVC percentages over 12+ months of prior ERT, even when the absolute numbers seemed stable. The trend matters more than single measurements."

Appeals Process in Michigan

Internal Appeals with Cigna

First Level:

  • Submit within 180 days of denial
  • Decision within 30 days (standard) or 72 hours (expedited)
  • Include new clinical evidence or policy interpretation

Second Level:

  • Available if first appeal denied
  • Independent medical review by Cigna
  • Same timeframes as first level

Michigan External Review

If internal appeals are exhausted, Michigan offers external review through the Department of Insurance and Financial Services (DIFS).

Key Details:

  • Deadline: 127 days after final internal denial
  • Process: DIFS External Review Request (verify current form)
  • Timeline: 60 days maximum (often faster)
  • Expedited: 72 hours with physician urgency letter

Contact DIFS:

  • Phone: 877-999-6442
  • Online: Submit through DIFS portal
  • The decision is binding on Cigna

Coverage at a Glance

Requirement Details Source
Prior Authorization Required IP_0591
Age Limit ≥18 years FDA Label
Weight Requirement >40 kg FDA Label
Step Therapy ≥1 year Lumizyme or Nexviazyme IP_0591
Combination Required Must use with Opfolda IP_0598
Specialist Required Geneticist, neurologist, or metabolic specialist IP_0591
Approval Duration 1 year initial IP_0591
Site of Care Least intensive appropriate PH_1605

FAQ

Q: How long does Cigna prior authorization take for Pombiliti? A: Standard PA decisions are made within 15 business days. Expedited requests (for urgent medical need) are decided within 24-72 hours.

Q: What if Pombiliti is not on Cigna's formulary? A: Pombiliti requires prior authorization regardless of formulary status. Non-formulary medications may have higher copays but can still be covered if medically necessary.

Q: Can I get an expedited appeal in Michigan? A: Yes, both Cigna internal appeals and Michigan external reviews offer expedited processes (72 hours) with physician documentation of urgency.

Q: Does step therapy apply if I failed ERT in another state? A: Yes, prior ERT failure documented anywhere counts toward step therapy requirements, as long as adequate trial duration and objective measures are documented.

Q: What's the difference between Pombiliti and other Pompe treatments? A: Pombiliti is a next-generation ERT designed for patients not responding to current therapies (Lumizyme, Nexviazyme). It requires combination use with Opfolda.

Q: How much does Pombiliti cost without insurance? A: The combination therapy costs approximately $650,000 annually at list price. Amicus Assist offers patient support programs.

Counterforce Health specializes in turning insurance denials into successful appeals for complex medications like Pombiliti. Our platform analyzes denial letters, identifies the specific coverage gaps, and generates evidence-backed appeals that address payer requirements point-by-point. For patients and clinicians navigating Pompe disease coverage challenges, we provide the documentation strategies and clinical evidence needed to meet Cigna's medical necessity criteria.

Getting Pombiliti covered requires careful attention to Cigna's specific requirements, particularly around prior ERT failure documentation and combination therapy protocols. With proper preparation and understanding of Counterforce Health's systematic approach to coverage challenges, most medically appropriate requests can achieve approval through either initial authorization or the appeals process.

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage policies change frequently—verify current requirements with Cigna and consult your healthcare provider for medical decisions. For assistance with Michigan insurance issues, contact DIFS at 877-999-6442.

Sources & Further Reading:

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