By Counterforce Health Team in Carbaglu (carglumic acid) —

How to Get Carbaglu (Carglumic Acid) Covered by Aetna CVS Health in Ohio: ICD-10, HCPCS, and Prior Authorization Guide

Answer Box: Getting Carbaglu Covered by Aetna CVS Health in Ohio

Fastest path to approval: Submit prior authorization with genetic/biochemical confirmation of NAGS deficiency (ICD-10: E72.20), elevated ammonia labs, and specialist documentation. Use HCPCS J7518 for medical benefit billing. If denied, file internal appeal within 65 days, then external review through Ohio Department of Insurance within 180 days. Start today: Gather genetic test results and baseline ammonia levels before submitting PA request via Aetna provider portal.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit Paths
  2. ICD-10 Mapping for NAGS Deficiency
  3. Product Coding: HCPCS, J-Codes, and NDCs
  4. Clean Prior Authorization Request
  5. Frequent Coding Pitfalls
  6. Verification with Aetna CVS Health
  7. Appeals Process in Ohio
  8. Quick Audit Checklist

Coding Basics: Medical vs. Pharmacy Benefit Paths

Carbaglu (carglumic acid) can be processed under different benefit structures depending on your Aetna CVS Health plan and how it's prescribed. Understanding the right pathway helps ensure faster approval and proper reimbursement.

Medical Benefit Path:

  • Uses HCPCS J-code J7518 (Carglumic acid, oral, 100 mg)
  • Typically for acute/inpatient settings or when prescribed by specialists
  • May require additional documentation of administration details
  • Often processed through medical claims, not pharmacy benefits

Pharmacy Benefit Path:

  • Uses NDC codes (52276-312-05 or 52276-312-60)
  • Standard outpatient prescription processing
  • Handled by CVS Specialty Pharmacy for most Aetna plans
  • Requires prior authorization regardless of pathway

Most Aetna CVS Health plans process Carbaglu through the specialty pharmacy benefit, but confirming your specific plan's preferred pathway can save time during the approval process.

ICD-10 Mapping for NAGS Deficiency

Accurate diagnosis coding is crucial for Carbaglu approval, as Aetna CVS Health requires clear documentation of N-acetylglutamate synthase (NAGS) deficiency.

Primary ICD-10 Code

E72.20 - Disorder of urea cycle metabolism, unspecified This is the most appropriate code for NAGS deficiency, as there's no specific ICD-10 code exclusively for this rare condition.

Supporting Codes

  • E72.8 - Other specified disorders of amino-acid metabolism
  • E72.4 - Disorders of ornithine metabolism (if clinical presentation warrants)

Documentation Words That Support Coding

When submitting clinical notes, include these specific terms to strengthen your coding justification:

  • "N-acetylglutamate synthase deficiency"
  • "NAGS deficiency"
  • "Hyperammonemia"
  • "Urea cycle disorder"
  • "Elevated plasma ammonia"
  • "Genetic confirmation of NAGS mutations"
Tip: Always include the genetic or biochemical test results that confirm NAGS deficiency. Aetna CVS Health frequently denies requests lacking this definitive diagnostic evidence.

Product Coding: HCPCS, J-Codes, and NDCs

HCPCS J-Code Details

J7518 - Carglumic acid, oral, 100 mg

  • Used for medical benefit billing
  • Represents 100 mg units (not per tablet)
  • Carbaglu tablets are 200 mg each, so one tablet = 2 units of J7518

NDC Codes

Primary NDCs:

  • 52276-312-05 - 200 mg tablets, 5-count bottle
  • 52276-312-60 - 200 mg tablets, 60-count bottle

Unit Conversion Math

Common dosing scenarios and their J7518 unit calculations:

  • 400 mg daily (2 tablets) = 4 units of J7518 per day
  • 600 mg daily (3 tablets) = 6 units of J7518 per day
  • 30-day supply at 400 mg daily = 120 units of J7518

When submitting claims, ensure your unit calculations match the prescribed dose and duration.

Clean Prior Authorization Request

Counterforce Health specializes in turning insurance denials into successful appeals by crafting evidence-backed prior authorization requests that align with each payer's specific requirements. Their platform identifies the exact denial basis and creates targeted rebuttals using the right clinical evidence and payer-specific workflows.

Required Documentation Checklist

Diagnostic Evidence:

  • Genetic test results confirming NAGS gene mutations
  • Biochemical testing showing elevated ammonia levels
  • Clinical notes describing hyperammonemia episodes
  • ICD-10 code E72.20 with supporting narrative

Prescription Details:

  • Exact dosing (mg per day)
  • Duration of therapy requested
  • Prescriber information and specialty credentials
  • Site of care (outpatient)

Clinical Justification:

  • Medical necessity statement
  • Treatment goals and monitoring plan
  • Documentation of symptom severity
  • Risk assessment without treatment

Sample PA Request Structure

Patient: [Name], DOB [Date]
Diagnosis: NAGS deficiency (ICD-10: E72.20)
Medication: Carbaglu (carglumic acid) 200mg tablets
Dosing: [X] mg daily, divided doses
Duration: 12 months
HCPCS: J7518 (if medical benefit)
NDC: 52276-312-60

Clinical Summary:
Patient has genetically confirmed NAGS deficiency with recurrent hyperammonemia episodes. Baseline ammonia: [value] μmol/L (normal <50). Carbaglu is FDA-approved and medically necessary to prevent life-threatening hyperammonemic crises.

Attached: Genetic test report, ammonia labs, specialist consultation notes

Frequent Coding Pitfalls

Unit Conversion Errors

Problem: Submitting J7518 units based on tablet count instead of mg dosage Fix: Always calculate units based on 100 mg increments (one 200mg tablet = 2 units)

Mismatched Benefit Pathways

Problem: Using pharmacy NDC codes when plan requires medical benefit billing Fix: Verify with Aetna CVS Health whether your specific plan processes Carbaglu under medical or pharmacy benefits

Missing Diagnosis Linkage

Problem: Submitting codes without clear connection between ICD-10 and medication request Fix: Include narrative explanation linking NAGS deficiency diagnosis to Carbaglu medical necessity

Incomplete Start Date Documentation

Problem: PA requests without clear therapy initiation timeline Fix: Specify requested start date and provide clinical justification for timing

Wrong Specialty Pharmacy Designation

Problem: Assuming all Aetna plans use CVS Specialty Pharmacy Fix: Confirm your plan's designated specialty pharmacy before submitting

Verification with Aetna CVS Health

Before submitting your prior authorization, verify these key details:

Plan-Specific Requirements

Contact: Aetna provider services at 1-800-624-0756 Verify:

  • Current formulary status of Carbaglu
  • Required prior authorization forms
  • Preferred submission method (portal vs. fax)
  • Medical vs. pharmacy benefit designation

Current Coding Confirmation

Resources:

Submission Portal Access

Ensure you're using the current Aetna provider portal. Outdated submission methods are a common cause of processing delays.

Appeals Process in Ohio

If your Carbaglu prior authorization is denied, Ohio provides specific protections and timelines for appeals.

Internal Appeal (Aetna CVS Health)

Timeline: 65 days from denial notice (Medicare); 60 days (Medicaid) Decision timeframe:

  • Standard: 7 days (prescription drugs)
  • Expedited: 72 hours (urgent medical need)

How to file:

  • Online through Aetna member/provider portal
  • Fax: 1-855-330-1716 (verify current number)
  • Include all original documentation plus additional evidence

External Review (Ohio Department of Insurance)

Eligibility: After internal appeal denial Timeline to request: 180 days from final internal denial Decision timeframe:

  • Standard: 30 days
  • Expedited: 72 hours

Contact: Ohio Department of Insurance Consumer Services Phone: 1-800-686-1526

Note: Self-funded employer plans may follow federal ERISA rules instead of Ohio's external review process, but many voluntarily provide similar protections.

When appealing Carbaglu denials, Counterforce Health can help identify the specific denial basis—whether it's missing PA criteria, step therapy requirements, or "not medically necessary" determinations—and craft point-by-point rebuttals using FDA labeling, peer-reviewed studies, and specialty guidelines that align with Aetna's own coverage policies.

Common Denial Reasons & Solutions

Denial Reason Solution Required Documentation
Missing genetic confirmation Submit molecular test results NAGS gene mutation report
Insufficient lab evidence Provide ammonia levels Baseline and follow-up labs
Incomplete clinical notes Add detailed symptom history Specialist consultation notes
Wrong benefit pathway Resubmit with correct codes Verify medical vs. pharmacy benefit
Administrative errors Correct and resubmit Complete forms with signatures

Quick Audit Checklist

Before submitting your Carbaglu prior authorization to Aetna CVS Health:

Diagnostic Coding:

  • ICD-10 E72.20 included and documented
  • Genetic/biochemical test results attached
  • Clinical narrative supports coding choice

Product Coding:

  • Correct HCPCS (J7518) or NDC codes used
  • Unit calculations verified (100 mg = 1 unit J7518)
  • Dosing and duration clearly specified

Documentation:

  • All required forms completed and signed
  • Medical necessity clearly articulated
  • Prescriber credentials and specialty noted
  • Patient demographics and insurance info current

Submission:

  • Correct portal/fax number confirmed
  • All attachments included
  • Follow-up plan established

From Our Advocates

"We've seen Carbaglu approvals go much smoother when providers include a simple timeline in their PA request—diagnosis date, first ammonia crisis, genetic testing completion, and proposed treatment start. This narrative helps medical reviewers understand the urgency and appropriateness of therapy, especially for a rare condition many may not encounter regularly."

FAQ

How long does Aetna CVS Health prior authorization take for Carbaglu in Ohio? Standard PA decisions typically take 30-45 days, but expedited reviews for urgent medical situations can be completed within 72 hours.

What if Carbaglu is non-formulary on my Aetna plan? You can request a formulary exception with medical necessity documentation. NAGS deficiency is a rare condition with limited treatment options, which often supports exception approval.

Can I request an expedited appeal if Carbaglu is denied? Yes, if delays would seriously endanger your health. Document the medical urgency and request expedited review, which Ohio requires to be completed within 72 hours.

Does step therapy apply to Carbaglu for NAGS deficiency? Step therapy requirements are generally not enforced for rare diseases like NAGS deficiency, as Carbaglu is the only FDA-approved treatment for this condition.

What happens if my employer plan is self-funded? Self-funded plans follow federal ERISA rules rather than Ohio state regulations, but many provide similar appeal processes. Check your plan documents or contact HR for specific procedures.

Sources & Further Reading

Disclaimer: This information is for educational purposes and does not constitute medical or legal advice. Coverage decisions depend on individual plan terms and medical circumstances. Always consult with your healthcare provider and insurance plan for specific guidance. For assistance with complex appeals, contact the Ohio Department of Insurance Consumer Services at 1-800-686-1526.

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