By Counterforce Health Team in Kanuma (sebelipase alfa) —

Complete Guide to Getting Kanuma (Sebelipase Alfa) Covered by Blue Cross Blue Shield in Florida: ICD-10, J-Codes, and Appeals Process

Answer Box: Getting Kanuma Covered by Florida Blue

Blue Cross Blue Shield of Florida (Florida Blue) requires prior authorization for Kanuma (sebelipase alfa) with strict diagnostic and clinical criteria. The fastest path to approval involves three key steps: (1) Confirm LAL-D diagnosis with genetic testing or enzyme assay documentation, (2) Submit prior authorization with baseline labs and weight-based dosing calculations, and (3) Use correct billing codes—ICD-10 E75.5, HCPCS J2840, and proper NDC identification. Start today by gathering diagnostic confirmation and contacting Florida Blue's provider services at their prior authorization portal to obtain current PA forms and submission requirements.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit Paths
  2. ICD-10 Mapping for LAL-D
  3. Product Coding: HCPCS, J-Codes, and NDC
  4. Clean Request Anatomy
  5. Frequent Coding Pitfalls
  6. Verification with Florida Blue Resources
  7. Appeals Process for Denials
  8. Quick Audit Checklist

Coding Basics: Medical vs. Pharmacy Benefit Paths

Kanuma (sebelipase alfa) is administered as an intravenous infusion, which typically falls under the medical benefit rather than pharmacy benefit. This distinction is crucial for proper billing and prior authorization.

Medical Benefit Coverage:

  • Requires provider administration in clinical settings
  • Uses HCPCS J-codes for billing
  • Subject to medical necessity review
  • Often requires prior authorization through medical management

Key Documentation Requirements:

  • Confirmed LAL-D diagnosis via genetic testing or enzyme assay
  • Weight-based dosing calculations
  • Clinical justification for frequency and dose escalation
  • Infusion site and administration details
Tip: Contact Florida Blue's provider services to confirm whether your specific plan processes Kanuma under medical or pharmacy benefits, as some plans may have unique pathways.

ICD-10 Mapping for LAL-D

The primary ICD-10-CM code for all forms of lysosomal acid lipase deficiency is E75.5 (Other lipid storage disorders). This single code covers:

  • Lysosomal acid lipase deficiency (LAL-D)
  • Wolman disease (severe, infantile form)
  • Cholesteryl ester storage disease (CESD, later-onset form)

Documentation Requirements for E75.5:

  • Laboratory confirmation of LAL enzyme deficiency in dried blood spots, leukocytes, or fibroblasts
  • Genetic testing showing pathogenic LIPA gene variants
  • Clinical presentation supporting LAL-D spectrum disorder

Supporting Clinical Documentation:

  • Hepatomegaly and liver dysfunction (elevated ALT, AST)
  • Splenomegaly
  • Dyslipidemia (elevated LDL-C, triglycerides; low HDL-C)
  • Gastrointestinal symptoms (malabsorption, steatorrhea)
  • Adrenal calcifications (particularly in Wolman disease)
Note: While the ICD-10 code is the same for all LAL-D forms, clearly specify "Wolman disease" or "cholesteryl ester storage disease" in clinical documentation to support severity and treatment urgency.

Product Coding: HCPCS, J-Codes, and NDC

Primary Billing Codes:

  • HCPCS J-Code: J2840 (injection, sebelipase alfa, 1 mg)
  • NDC: 25682-001-01 (verify current NDC in your purchase records)
  • Billing Unit: 1 unit = 1 mg of sebelipase alfa

Dose Calculations and Vial Requirements:

Patient Population Dose Frequency Calculation Example (25 kg patient)
Pediatric/Adult LAL-D 1 mg/kg Every 2 weeks 25 mg = 2 vials (round up from 1.25)
Dose escalation 3 mg/kg Every 2 weeks 75 mg = 4 vials (round up from 3.75)
Infants (<6 months) 1-5 mg/kg Weekly Variable based on response

Vial Information:

  • Each vial contains 20 mg/10 mL
  • Single-use vials; discard unused portions
  • Calculate total vials needed: (Patient weight × dose) ÷ 20 mg per vial (round up)

Administration Requirements:

  • Minimum 2-hour infusion time
  • 0.2 micron low-protein-binding filter required
  • Dilution to final concentration 0.1-1.5 mg/mL

Clean Request Anatomy

Example Prior Authorization Request:

Patient: [Name], DOB: [Date], Weight: 25 kg
Diagnosis: E75.5 - Lysosomal acid lipase deficiency (Wolman disease)
Requested Treatment: Kanuma (sebelipase alfa) J2840
Dose: 1 mg/kg (25 mg) IV every 2 weeks
Vials Required: 2 vials per infusion
NDC: 25682-001-01
Site of Care: [Infusion center/hospital outpatient]

Supporting Documentation:
- LAL enzyme assay results showing deficiency
- Genetic testing confirming LIPA pathogenic variants
- Baseline labs: ALT 150 U/L, AST 120 U/L, LDL-C 280 mg/dL
- Clinical notes documenting hepatomegaly and failure to thrive

Required Elements for Clean Requests:

  1. Current patient weight and age
  2. Confirmed LAL-D diagnosis with test results
  3. Baseline clinical and laboratory data
  4. Dosing rationale and escalation criteria
  5. Administration site and schedule
  6. Provider attestation of medical necessity

Frequent Coding Pitfalls

Common Errors That Trigger Denials:

Pitfall Problem Solution
Incorrect units Billing in vials instead of mg Always bill J2840 in 1 mg units
Outdated weight Using old weight for dose calculation Update weight at each infusion
Missing escalation rationale Requesting higher doses without justification Document suboptimal response criteria
Wrong NDC Using incorrect or outdated NDC numbers Verify current NDC with pharmacy
Incomplete diagnosis Coding E75.5 without supporting documentation Include enzyme/genetic test results

Unit Conversion Mistakes:

  • Wrong: Billing 2 units for 2 vials used
  • Right: Billing 40 units for 40 mg administered (2 × 20 mg vials)

Documentation Gaps:

  • Failing to document dose escalation criteria
  • Missing baseline liver function tests
  • Inadequate clinical justification for frequency
  • Lack of specialist confirmation
Counterforce Health specializes in turning insurance denials into targeted, evidence-backed appeals by identifying the specific denial basis and drafting point-by-point rebuttals aligned to each plan's own rules. Their platform helps clinicians and patients navigate complex prior authorization requirements for specialty medications like Kanuma.

Verification with Florida Blue Resources

Essential Verification Steps:

  1. Check Current PA Requirements: Visit the Florida Blue provider portal to download current prior authorization forms and criteria.
  2. Confirm Formulary Status: Review Florida Blue's specialty drug list to understand Kanuma's tier placement and any step therapy requirements.
  3. Validate Codes: Cross-reference J2840 and NDC numbers with Florida Blue's billing guidelines.
  4. Verify Submission Methods: Confirm whether to submit via portal, fax, or mail, and obtain current contact information.

Key Florida Blue Resources:

Pre-Submission Checklist:

  • Current PA form completed
  • All required clinical documentation attached
  • Correct billing codes verified
  • Patient weight and dosing calculations confirmed
  • Provider attestation signed and dated

Appeals Process for Denials

If Florida Blue denies your Kanuma prior authorization, you have specific appeal rights under Florida law:

Internal Appeal Process:

  • Deadline: 180 days from denial notice
  • Standard Review: 30 days for future treatments
  • Expedited Review: 24 hours for urgent cases
  • Required: Denial letter, medical justification, specialist support

External Review (Final Step):

  • Deadline: 4 months after final internal denial
  • Process: Florida Department of Financial Services coordinates independent medical review
  • Contact: 1-877-693-5236 or Florida DFS Consumer Services
  • Outcome: Binding decision that insurers must follow

Strengthening Your Appeal:

  1. Include detailed medical necessity letter from LAL-D specialist
  2. Cite FDA labeling and clinical guidelines
  3. Document failed alternative treatments or contraindications
  4. Provide evidence of clinical deterioration without treatment
  5. Reference peer-reviewed literature supporting Kanuma use
From our advocates: We've seen appeals succeed when providers clearly document the progressive nature of LAL-D and the lack of alternative treatments. One effective approach is creating a timeline showing clinical deterioration before Kanuma and improvement after initiation, with specific lab values and imaging results. This composite strategy has helped overturn denials by demonstrating clear medical necessity.

Expedited Appeals for Urgent Cases:

  • Required for life-threatening conditions
  • Must include physician declaration of urgency
  • Decision within 24-72 hours
  • Can proceed to external review simultaneously

Quick Audit Checklist

Pre-Submission Review:

Diagnosis & Documentation:

  • E75.5 coded with supporting LAL enzyme or genetic testing
  • Clinical notes clearly describe LAL-D symptoms and severity
  • Baseline labs include liver function and lipid panel

Coding & Billing:

  • J2840 used for Kanuma billing
  • Units calculated correctly (1 unit = 1 mg)
  • Current NDC verified with pharmacy
  • Patient weight and dose calculations accurate

Prior Authorization:

  • Current Florida Blue PA form completed
  • All required attachments included
  • Provider attestation signed and dated
  • Submission method confirmed (portal/fax/mail)

Clinical Justification:

  • Medical necessity clearly articulated
  • Dose escalation rationale provided if applicable
  • Alternative treatments addressed
  • Monitoring plan outlined

Appeals Preparation:

  • Denial reasons identified and addressed
  • Specialist support letter obtained
  • Additional clinical evidence gathered
  • Appeal deadlines noted (180 days internal, 4 months external)

Frequently Asked Questions

Q: How long does Florida Blue prior authorization take for Kanuma? A: Standard prior authorization typically takes 15-30 business days. Expedited reviews for urgent cases can be completed within 24-72 hours with proper clinical justification.

Q: What if Kanuma is non-formulary on my Florida Blue plan? A: You can request a formulary exception by demonstrating medical necessity and lack of formulary alternatives. Include documentation of failed treatments or contraindications to covered options.

Q: Can I request an expedited appeal for Kanuma denial? A: Yes, if delays in treatment would endanger the patient's health. Include a physician's declaration of urgency and clinical documentation supporting the immediate need for treatment.

Q: Does step therapy apply to Kanuma in Florida? A: Step therapy requirements vary by specific Florida Blue plan. Since Kanuma is the only FDA-approved treatment for LAL-D, step therapy typically doesn't apply, but confirm with your specific plan.

Q: What happens if my external review is denied? A: External review decisions are final and binding. Consider manufacturer patient assistance programs, clinical trials, or seeking care at specialized centers with different payer contracts.

When to Escalate

Contact Florida's insurance regulators if you encounter systematic issues with Florida Blue's prior authorization or appeals process:

Florida Department of Financial Services

Escalation Triggers:

  • Failure to respond within required timeframes
  • Requests for inappropriate documentation
  • Denial of expedited review for urgent cases
  • Procedural violations in appeals process

Counterforce Health provides specialized support for complex prior authorization and appeals processes, helping patients and providers navigate insurance denials with evidence-based strategies tailored to specific payer requirements.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Coverage policies and procedures may vary by specific Florida Blue plan and change over time. Always consult with your healthcare provider and insurance plan for the most current requirements and procedures. For personalized assistance with prior authorization and appeals, consider consulting with healthcare coverage specialists or patient advocacy services.

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