By Counterforce Health Team in Kanuma (sebelipase alfa) —

How to Get Kanuma (Sebelipase Alfa) Covered by Cigna in Illinois: Coding, Appeals, and Authorization Guide

Answer Box: Getting Kanuma Covered by Cigna in Illinois

Kanuma (sebelipase alfa) requires prior authorization from Cigna in Illinois. The fastest path: 1) Submit PA request with LAL-D diagnosis (ICD-10: E75.5), genetic/enzyme test results, and clinical documentation to Cigna via fax (855-840-1678) or CoverMyMeds. 2) Use HCPCS code J2840 and NDC 25682-0007-01 for billing. 3) If denied, appeal within 180 days; external review available within 120 days of final denial. Expected timeline: 72 hours for urgent PA, 30 days for standard review.

Table of Contents

Coding Basics: Medical vs. Pharmacy Benefit

Kanuma (sebelipase alfa) is covered under the medical benefit, not pharmacy. This IV-administered enzyme replacement therapy requires provider administration, making it ineligible for standard pharmacy dispensing.

Key Coverage Points:

  • Medical benefit: All infusion therapies bill through medical claims
  • Specialty pharmacy: Accredo typically dispenses for Cigna patients
  • Prior authorization required: Clinical documentation must support LAL-D diagnosis
  • Site of care: Home infusion, physician office, or outpatient facility
Note: Unlike self-administered medications, provider-administered therapies like Kanuma bypass traditional pharmacy step therapy requirements but still need robust clinical justification.

ICD-10 Mapping for LAL-D

Primary diagnosis code: E75.5 (Other lipid storage disorders) covers lysosomal acid lipase deficiency and its variants, including Wolman disease and cholesteryl ester storage disease.

Documentation Requirements for E75.5:

  • Clinical symptoms: Hepatomegaly, splenomegaly, liver dysfunction, dyslipidemia
  • Laboratory evidence: Reduced LAL enzyme activity (dried blood spot or leukocyte testing)
  • Genetic confirmation: LIPA gene mutations when available
  • Imaging/histology: Liver findings showing microvesicular steatosis or fibrosis

Supporting codes may include:

  • K76.0 (Fatty liver, not elsewhere classified)
  • E78.2 (Mixed hyperlipidemia)
  • R16.0 (Hepatomegaly, not elsewhere classified)

Product Coding: HCPCS, NDC, and Units

Essential Billing Codes

Code Type Code Description Units
HCPCS J2840 Injection, sebelipase alfa 1 mg per unit
NDC 25682-0007-01 20 mg/10 mL vial Report as N425682000701
Revenue 0636 Drugs requiring detailed coding Per facility

Dosing and Units Calculation

Standard dosing: 1 mg/kg IV every other week

  • Pediatric escalation: Up to 3 mg/kg every other week
  • Infant dosing: 1-5 mg/kg weekly for severe cases

Units calculation example:

  • 25 kg patient × 1 mg/kg = 25 mg dose
  • Bill 25 units of J2840 (each unit = 1 mg)
  • Requires 2 vials (20 mg each), discard unused portion with JW modifier
Critical: Always bill by milligrams administered, not vials used. Each J2840 unit equals exactly 1 mg of sebelipase alfa.

Clean Request Anatomy

Complete Prior Authorization Submission

Patient Information:

  • Full name, DOB, Cigna member ID
  • Weight (kg) for dosing calculation
  • Diagnosis: LAL-D with ICD-10 E75.5

Clinical Documentation:

  • LAL enzyme activity results showing deficiency
  • Genetic testing (LIPA mutations) if available
  • Liver function tests (ALT, AST)
  • Lipid panel showing abnormalities
  • Clinical notes documenting symptoms

Prescriber Details:

  • NPI number and DEA (if applicable)
  • Facility information for administration
  • Preferred infusion site justification

Treatment Plan:

  • Exact dosing (mg/kg)
  • Administration schedule (every other week)
  • Monitoring plan (liver enzymes, lipids)
  • Expected clinical outcomes

Counterforce Health helps patients and clinicians streamline this process by automatically generating targeted, evidence-backed appeals that align with Cigna's specific requirements and timelines.

Frequent Coding Pitfalls

Common Errors That Delay Approval

Weight-Based Dosing Mistakes:

  • Using outdated patient weights
  • Incorrect kg to mg conversion
  • Rounding doses instead of vial quantities

Billing Unit Confusion:

  • Billing per vial (20 units) instead of actual mg administered
  • Missing JW modifier for discarded drug
  • Incorrect NDC format (must be 11-digit HIPAA compliant)

Documentation Gaps:

  • Missing LAL enzyme activity results
  • Inadequate clinical symptom documentation
  • Unclear diagnosis coding (using non-specific lipid disorder codes)

Site of Care Issues:

  • Not justifying preferred infusion location
  • Missing medical necessity for higher-cost settings

Cigna Verification Resources

Official Channels for Code Verification

Provider Resources:

  • Cigna Provider Portal for current PA requirements
  • Express Scripts formulary lookup for coverage tier
  • Accredo specialty pharmacy for dispensing coordination

Key Verification Steps:

  1. Confirm PA requirement via Cigna provider portal
  2. Check formulary status for any step therapy requirements
  3. Verify NDC coverage in Cigna's drug database
  4. Review site-of-care restrictions for infusion therapies

Contact Information:

  • PA submissions: Fax 855-840-1678
  • Urgent requests: Phone 800-882-4462
  • Electronic submission: CoverMyMeds, EviCore ePA

Pre-Submission Audit Checklist

Essential Review Points

Clinical Documentation ✓

  • LAL-D diagnosis confirmed with enzyme/genetic testing
  • ICD-10 E75.5 documented in medical record
  • Clinical symptoms clearly described
  • Prior treatment attempts documented (if applicable)

Coding Accuracy ✓

  • HCPCS J2840 with correct unit calculation
  • NDC 25682-0007-01 in proper 11-digit format
  • Weight-based dosing calculation verified
  • JW modifier applied for discarded drug

Administrative Requirements ✓

  • Cigna PA form completed fully
  • Prescriber NPI and facility information included
  • Infusion site justification provided
  • All supporting documents attached

Submission Method ✓

  • Electronic submission via preferred portal
  • Fax backup with confirmation receipt
  • Urgent designation if clinically appropriate

Common Denial Reasons & Solutions

Denial Reason Solution Strategy Required Documentation
Insufficient LAL-D diagnosis Submit comprehensive testing results Enzyme activity assay, genetic testing, clinical notes
Dosing not per label Provide weight-based calculation Current weight, mg/kg justification, FDA label reference
Missing medical necessity Clinical letter with outcomes data Liver enzymes, lipid levels, symptom progression
Site of care not justified Medical necessity letter Contraindications to alternative sites, patient-specific factors
Prior therapy not documented Submit treatment history Records of supportive care attempts, outcomes

Strengthening Your Appeal

When facing denials, Counterforce Health's platform analyzes the specific denial reason and generates point-by-point rebuttals using Cigna's own policy language, FDA labeling, and peer-reviewed evidence for LAL-D treatment.

Appeals Process in Illinois

Internal Appeal Timeline

Step 1: File Internal Appeal

Step 2: Second-Level Review

  • Available if first appeal denied
  • Same 30-day timeline for PA-related appeals
  • Peer-to-peer review option available

External Review (Illinois-Specific)

Independent Review Organization (IRO)

  • Deadline: 120 days after final internal denial
  • Timeline: 45 days for standard review, 72 hours for expedited
  • Cost: No fee to patient (insurer pays IRO)
  • Binding: IRO decision compels coverage if favorable

Illinois Department of Insurance Resources:

Illinois-Specific: The 30-day external review request deadline is shorter than many states' 4-month window, so act promptly after final denial.

FAQ

How long does Cigna prior authorization take for Kanuma in Illinois? Standard PA reviews take up to 72 hours; urgent requests within 24 hours. If Cigna doesn't respond within these timeframes, the request is automatically approved.

What if Kanuma is non-formulary on my Cigna plan? Kanuma typically requires PA regardless of formulary status. For rare disease medications, step therapy is generally not applicable, but medical necessity must still be demonstrated.

Can I request an expedited appeal if my condition is rapidly progressing? Yes. Illinois law provides expedited external review (72 hours) for urgent medical situations. Document the clinical urgency in your appeal request.

Does step therapy apply to Kanuma in Illinois? Step therapy is typically waived for rare disease treatments like LAL-D since no therapeutic alternatives exist. Focus on confirming diagnosis and medical necessity.

What documentation strengthens a Kanuma appeal? Include LAL enzyme activity results, genetic testing, liver function trends, lipid profiles, clinical photos/imaging if available, and peer-reviewed literature on LAL-D outcomes.

How do I find Illinois-specific insurance appeal forms? Use Cigna's standard appeal forms, but Illinois law provides additional external review rights. The Illinois Department of Insurance website has state-specific guidance and forms.

From our advocates: "We've seen Kanuma appeals succeed when families provide comprehensive enzyme testing results alongside clear documentation of disease progression. The key is showing both definitive diagnosis and clinical need in language that matches the insurer's medical policy. This composite approach has helped streamline approvals across multiple rare disease cases."

Sources & Further Reading

Medical Disclaimer: This information is for educational purposes only and does not constitute medical advice. Always consult with qualified healthcare professionals for diagnosis, treatment decisions, and insurance coverage questions. Coverage policies and appeal processes may vary by specific plan and change over time. Verify current requirements with your insurer and healthcare team.

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