By Counterforce Health Team in Carbaglu (carglumic acid) —

How to Get Carbaglu (Carglumic Acid) Covered by UnitedHealthcare in New York: Complete PA Guide with Appeals Process

Answer Box: Getting Carbaglu Covered by UnitedHealthcare in New York

UnitedHealthcare requires prior authorization for Carbaglu (carglumic acid) with 12-month approval periods. Key requirements: genetic confirmation of NAGS deficiency, elevated ammonia levels, and specialist documentation. If denied, New York residents can file internal appeals (180 days) then external review through NY Department of Financial Services (4 months). Start today: Gather genetic testing results, ammonia labs, and request metabolic specialist consultation. Submit PA via UnitedHealthcare Provider Portal.

Quick steps: 1) Collect genetic/lab evidence, 2) Get specialist letter, 3) Submit complete PA request, 4) Appeal if denied with full documentation.

Table of Contents

Coverage Requirements at a Glance

Requirement What It Means Where to Find It Source
Prior Authorization Required for all prescriptions UHC Provider Portal UHC PA Policy
Formulary Status Covered for approved diagnoses Plan formulary documents OptumRx Formulary
Step Therapy Not required for NAGS deficiency UHC clinical guidelines UHC PA Policy
Approved Diagnoses NAGS deficiency, PA, MMA only ICD-10: E72.2 FDA Label
Authorization Period 12 months initial and renewal UHC policy documents UHC PA Policy

Step-by-Step: Fastest Path to Approval

1. Gather Essential Documentation (Patient/Family)

Who: Patient or caregiver
Timeline: 1-2 weeks
Documents needed:

  • Genetic testing results confirming NAGS gene mutations
  • Recent plasma ammonia level reports
  • Insurance card and member ID
  • Complete medication history

2. Schedule Metabolic Specialist Consultation (Clinic)

Who: Primary care or referring physician
Timeline: 2-4 weeks for appointment
Action: Request consultation with geneticist or metabolic specialist familiar with urea cycle disorders. Ensure specialist can provide detailed medical necessity letter.

3. Collect Clinical Evidence (Specialist)

Who: Metabolic specialist
Timeline: During consultation visit
Required documentation:

  • Enzyme assay or genetic confirmation
  • Elevated plasma ammonia levels
  • Low citrulline and arginine levels
  • Absence of urinary orotic acid

4. Submit Prior Authorization (Clinic Staff)

Who: Prescribing physician's office
Timeline: Same day submission recommended
How: UnitedHealthcare Provider Portal
Include: All documentation from steps 1-3, ICD-10 code E72.2

5. Track Authorization Status (Clinic/Patient)

Timeline: 2-4 weeks for decision
Action: Monitor via UHC provider portal or call member services
Expected outcome: 12-month approval for confirmed NAGS deficiency

6. Appeal if Denied (Patient/Clinic)

Timeline: Within 180 days of denial
Process: Internal appeal first, then New York external review if needed
Resources: NY DFS External Appeal Portal

ICD-10 Coding and Documentation

Primary diagnosis code for NAGS deficiency: E72.2 ("Disorders of urea cycle metabolism")

Documentation Requirements for Medical Necessity

Essential clinical evidence includes:

  • Genetic confirmation: NAGS gene sequencing results or enzyme assay demonstrating deficiency
  • Biochemical evidence: Elevated plasma ammonia levels above age-appropriate reference ranges
  • Metabolic profile: Low citrulline and arginine levels, absence of urinary orotic acid
  • Specialist assessment: Consultation notes from geneticist or metabolic specialist
Tip: The Office of Inspector General has noted inappropriate denials for rare disease medications. Complete metabolic documentation significantly improves approval rates.

HCPCS, J-Codes, and NDC Information

Medical Benefit Coding

  • HCPCS J-Code: J7518 ("Carglumic acid, oral, 100 mg")
  • Billing units: Each unit = 100 mg (round up to nearest 100 mg)
  • Example: 2000 mg daily dose = 20 units of J7518

Pharmacy Benefit Coding

  • NDC codes:
    • 52276-312-05: Bottle of 5 tablets (200 mg each)
    • 52276-312-60: Bottle of 60 tablets (200 mg each)

Dosing Calculations

FDA-approved dosing for NAGS deficiency:

  • Acute phase: 100-250 mg/kg/day divided in 2-4 doses
  • Maintenance: Individualized based on ammonia levels

Example calculation for 20 kg patient:

  • Daily dose: 20 kg × 100 mg/kg = 2000 mg
  • Tablets needed: 2000 mg ÷ 200 mg/tablet = 10 tablets daily
  • HCPCS units: 2000 mg ÷ 100 mg/unit = 20 units J7518

Common Denial Reasons & How to Fix Them

Denial Reason How to Overturn Required Documentation
Missing genetic confirmation Submit complete gene sequencing NAGS gene mutation analysis results
Incomplete ammonia documentation Provide serial ammonia levels Multiple plasma ammonia lab reports
Inadequate specialist notes Request detailed medical necessity letter Metabolic specialist consultation with rationale
"Not medically necessary" Reference FDA approval and guidelines FDA labeling, specialist letter citing guidelines
Incorrect diagnosis codes Verify ICD-10 coding Use E72.2 for NAGS deficiency
Missing prior therapy history Document treatment attempts Chart notes showing failed/contraindicated alternatives
Note: UnitedHealthcare's Medicare Advantage plans show approximately 9% prior authorization denial rates, above peer averages. Thorough documentation is essential for approval.

Appeals Process in New York

Internal Appeal with UnitedHealthcare

Deadline: 180 days from denial
How to file:

  • Online: UnitedHealthcare member portal
  • Mail: Address provided in denial letter
  • Fax: Number provided in denial letter

Required documents:

  • Original denial letter
  • Medical necessity letter from specialist
  • All supporting lab and genetic test results
  • Treatment history documentation

External Review through NY Department of Financial Services

When to use: After internal appeal denial
Deadline: 4 months from final adverse determination
Filing fee: $25 (waived for Medicaid or financial hardship)

How to file:

  1. Complete NY External Appeal Application
  2. Submit via DFS External Appeal Portal
  3. Include all denial letters and medical records

Timeline: 30 days standard, 72 hours for expedited urgent cases

From our advocates: We've seen NAGS deficiency cases succeed in New York external review when families include comprehensive genetic testing results and detailed specialist letters explaining why Carbaglu is the only FDA-approved treatment. Complete documentation makes the difference.

New York Consumer Protections

  • External reviewer decisions are binding on UnitedHealthcare
  • Success rates for well-documented rare disease appeals: 50-70%
  • No step therapy restrictions for rare diseases when specialist certifies medical necessity
  • Community Health Advocates provides free assistance: 888-614-5400

Clinician Corner: Medical Necessity Letters

Essential Elements for Approval

Patient identification: Name, DOB, member ID, diagnosis
Clinical presentation: Symptoms, ammonia levels, metabolic profile
Diagnostic confirmation: Genetic testing results, enzyme assays
Treatment rationale: Why Carbaglu is medically necessary
Alternative therapies: Prior treatments tried, outcomes, contraindications
Monitoring plan: Ammonia level tracking, dose adjustments

Key Clinical Guidelines to Reference

Costs and Patient Assistance

Typical costs: Cash prices exceed $200 per 200mg tablet; monthly costs vary by weight and dosing requirements.

Financial Assistance Options

  • Manufacturer support: Recordati Patient Assistance Program
  • Copay assistance: Available for eligible commercial insurance patients
  • Foundation grants: Ultra-rare disease foundations may provide support
  • New York state programs: Medicaid coverage for eligible residents

At Counterforce Health, we help patients navigate the complex prior authorization process for rare disease medications like Carbaglu. Our platform analyzes denial letters and creates evidence-based appeals that address specific payer criteria, increasing approval rates for families facing coverage challenges.

FAQ

How long does UnitedHealthcare prior authorization take for Carbaglu?
Typically 2-4 weeks for complete applications. Incomplete submissions often face delays of 4-6 weeks while additional documentation is requested.

What if Carbaglu is not on my UnitedHealthcare formulary?
Carbaglu is covered for approved diagnoses (NAGS deficiency, PA, MMA) even if not listed on standard formularies. Submit prior authorization with complete documentation.

Can I get expedited approval for urgent cases?
Yes, UnitedHealthcare offers expedited review for urgent medical needs, typically within 72 hours. Your specialist must certify the urgent medical necessity.

Does step therapy apply to Carbaglu in New York?
No, step therapy is not required for NAGS deficiency. New York law limits step therapy requirements for rare diseases when specialists certify medical necessity.

What happens if my internal appeal is denied?
New York residents can file external review through the Department of Financial Services within 4 months. The external reviewer's decision is binding on UnitedHealthcare.

How do I find a metabolic specialist in New York?
Contact major medical centers like NYU Langone, Mount Sinai, or Columbia Presbyterian. Many have dedicated metabolic/genetic disorder clinics.

Can I maintain coverage during the appeals process?
Yes, request "aid continuing" when filing appeals to maintain coverage during review, especially for marketplace plans through NY State of Health.

What if I move to New York from another state while on Carbaglu?
Contact UnitedHealthcare member services immediately to transfer your authorization. You may need to resubmit documentation under New York plan requirements.

Sources & Further Reading

For additional support with prior authorization appeals and coverage challenges, Counterforce Health provides specialized assistance for rare disease medications, helping families navigate complex insurance requirements and create compelling evidence-based appeals.

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Always consult with your healthcare provider and insurance plan for specific coverage decisions. Coverage policies may vary by plan type and can change over time.

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