Loading...

    Explore
    • Policy Monitoring
    • Live Policy Feed
    • Billing Codes
    • NPI Medicare & Medicaid
    • Healthcare Providers
    • Health Insurance Payers
    • States
    • Service Lines
    • Taxonomy Codes
    Lookup tools
    • Billing Code Lookup
    • CPT Modifier Lookup
    • Provider Taxonomy Code Lookup
    • Healthcare Provider & Facility Lookup
    • Health Insurance Payer Lookup
    • Payer Contact Directory

    Policy & Benchmark Tools

    • Prior Authorization Lookup
    • Coverage Criteria Lookup
    • Timely Filing Lookup
    • Billing Code Reimbursement Rates
    • Payer Comparison Dashboard
    • Facility Comparison Dashboard
    • All tools

    Company

    • About OpenPayer
    • Data Sources
    • Trek Health
    OpenPayerby Trek Health
    LinkedInYouTube

    © 2026 Trek Health. All rights reserved.

    OpenPayer is built and maintained by Trek Health.

    v1.0.28
    OpenPayerPolicy UpdatesNeurology/NeurosurgeryUnitedHealthcare Kebilidi Coverage Update — Prior …
    Ask Trek AI about this page
    CurrentUnitedHealthcarePolicy CS2026D0136D

    Kebilidi (eladocagene exuparvovec-tneq) — Coverage Criteria for AADC Deficiency

    Customize your policy alerts

    Sign up for UnitedHealthcare Policy CS2026D0136D alerts

    Get alerted when Policy CS2026D0136D changes without checking for updates manually.

    Monitor payer policy activity

    Receive email alerts as soon as relevant payer policies are published or updated.

    This policy governs medical benefit coverage criteria for Kebilidi (a one-time gene therapy) to treat aromatic L‑amino acid decarboxylase (AADC) deficiency and applies to UnitedHealthcare members except where state-specific exceptions are noted.

    Policy Summary
    PayerUnitedHealthcare
    PolicyKebilidi (eladocagene exuparvovec-tneq) — Coverage Criteria for AADC Deficiency
    Policy CodePolicy CS2026D0136D
    Change TypeRevised clinical confirmation and ambulation language
    Effective DateAug 1, 2026
    Last ReviewAug 1, 2026
    Next Review DateN/A
    Key ActionObtain prior authorization and submit medical records documenting genetically confirmed AADC deficiency with required clinical and laboratory evidence.
    SourceLink
    POLICY UPDATE CHANGES

    Replaced wording requiring 'submission of medical records documenting a diagnosis of AADC deficiency and presence of biallelic mutations in the dopa decarboxylase (DDC) gene' with 'submission of medical records documenting a genetically confirmed AADC deficiency due to biallelic mutations in the dopa decarboxylase (DDC) gene'.

    Clarified ambulation requirement from 'The patient is unable to ambulate independently' to 'the patient is unable to ambulate independently with or without an assistive device such as cane or walker'.

    one-timetreatment allowed per lifetime
    ≤60dauthorization duration

    OpenPayer is powered by Trek Health's payer performance platform. Trek continuously ingests, validates, and normalizes Transparency in Coverage data alongside payer policies and other commercial payer data to create a structured payer intelligence foundation. OpenPayer uses this foundation to deliver personalized search results, dynamically generated policy pages, and tailored policy monitoring based on each user's payers, specialties, billing codes, and areas of interest. The same intelligence powers broader payer performance workflows, including reimbursement benchmarking, contract evaluation, payer negotiations, and financial decision-making.

    1.8e11pivotal trial total dose
    n=13Phase 2 participants
    E70.81ICD-10 code

    Coverage Criteria for Kebilidi (eladocagene exuparvovec-tneq)

    Initial therapy — coverage criteria

    Covered when ALL of the following are met:

    Main coverage criteria: Submission of medical records documenting genetically confirmed AADC deficiency due to biallelic mutations in the DDC gene; presence of one or more typical clinical characteristics associated with AADC deficiency (e.g., hypotonia, oculogyric crises, dystonia, hypokinesia, autonomic dysfunction, developmental delay); decreased AADC enzyme activity in plasma per current laboratory standards; skull maturity confirmed by neuroimaging sufficient for stereotactic neurosurgical administration of Kebilidi; persistent symptoms of AADC deficiency despite use of standard medical therapy (e.g., dopamine agonists, monoamine oxidase inhibitors, pyridoxine/other forms of vitamin B6); patient is unable to ambulate independently with or without an assistive device such as cane or walker; anti-AAV2 antibody titer not higher than 1:1200 and not >1 optical density by ELISA; prescribed by a neurologist or neurosurgeon; patient has not previously received Kebilidi or other gene therapy for AADC deficiency in their lifetime; dosing consistent with FDA‑approved labeling; authorization limited to one treatment per lifetime and for no longer than 60 days from approval.

    All conditions must be satisfied.

    Coverage excludes patients with an anti‑adeno‑associated virus, serotype 2 (anti‑AAV2) antibody titer that is higher than 1:1200 or an ELISA result that is > 1 optical density (OD) value. Patients who have previously received Kebilidi or any other gene therapy for AADC deficiency are not eligible for coverage. Authorization is limited to one treatment per lifetime and for no longer than 60 days from approval. Dosing must be consistent with the FDA‑approved labeling and the therapy must be prescribed by a neurologist or neurosurgeon.

    Kebilidi (eladocagene exuparvovec‑tneq) is indicated for treatment of aromatic L‑amino acid decarboxylase (AADC) deficiency under the U.S. Food and Drug Administration’s accelerated approval pathway. The approval was based on change from baseline in gross motor milestone achievement at 48 weeks post‑treatment. Continued approval may be contingent on verification and description of clinical benefit in a confirmatory clinical trial.

    Coding and Test Thresholds

    Applicable HCPCS CodesHCPCS
    C9399Unclassified drugs or biologicals
    J3590Unclassified biologics
    Diagnosis CodesICD-10
    E70.81Aromatic L-amino acid decarboxylase (AADC) deficiency
    inv-06: Anti-AAV2 antibody titer — numeric threshold
    ThresholdAnti-AAV2 antibody titer not higher than 1:1200 or not >1 optical density by ELISA
    Test methodEnzyme-linked immunosorbent assay (ELISA) reported as titer or optical density (OD)
    Coverage implicationPatients with titer higher than 1:1200 or >1 OD by ELISA are excluded from coverage

    Provider Actions, Authorization, and Documentation

    Prior Authorization

    Prior authorization required — all coverage criteria must be met

    Prior authorization is required. Authorization will be issued only when the member meets all coverage criteria including genetically confirmed AADC deficiency due to biallelic DDC mutations, presence of typical clinical characteristics, decreased AADC enzyme activity in plasma, skull maturity for neurosurgical administration, persistent symptoms despite standard therapy, ambulation status, acceptable anti-AAV2 antibody titer, specialist prescription, no prior gene therapy, and dosing consistent with FDA labeling. Authorization is limited to one treatment per lifetime and for no longer than 60 days from approval.

    • Genetically confirmed AADC deficiency due to biallelic DDC mutations
    • Typical clinical characteristics (e.g., hypotonia, oculogyric crises, dystonia, hypokinesia, autonomic dysfunction, developmental delay)
    • Decreased AADC enzyme activity in plasma
    • Skull maturity confirmed by neuroimaging
    • Persistent symptoms despite standard medical therapy
    • Unable to ambulate independently with or without an assistive device
    • Anti-AAV2 antibody titer not higher than 1:1200 or not >1 OD by ELISA
    • Prescribed by a neurologist or neurosurgeon
    • No prior treatment with Kebilidi or other gene therapy for AADC deficiency

    Background and Clinical Context

    Aromatic L‑amino acid decarboxylase (AADC) deficiency is a rare, inherited neurologic disorder caused by biallelic pathogenic variants in the DDC gene leading to deficient AADC enzyme activity. The enzyme deficiency impairs synthesis of dopamine and serotonin and produces a characteristic clinical syndrome including hypotonia, oculogyric crises, dystonia, hypokinesia, autonomic dysfunction, and global developmental delay.

    Kebilidi is a one‑time gene replacement therapy delivered via a recombinant AAV2 vector to the putamen with the goal of restoring AADC enzyme expression and increasing dopamine production. Appropriate candidate selection requires genetically confirmed biallelic DDC mutations, documentation of decreased AADC enzyme activity per current laboratory standards, skull maturity confirmed by neuroimaging for stereotactic delivery, persistent symptoms despite standard medical therapy, and evaluation by a neurologist or neurosurgeon.

    Definitions

    inv-12: AADC deficiency — concise definition
    DefinitionA rare genetic neurological disorder caused by biallelic pathogenic variants in the DDC gene leading to deficiency of the aromatic L‑amino acid decarboxylase (AADC) enzyme, resulting in impaired dopamine and serotonin synthesis and characteristic movement, autonomic, behavioral, and developmental symptoms.
    Genetic basisBiallelic pathological variants in the dopa decarboxylase (DDC) gene
    Core biochemical effectDeficient AADC enzyme activity causing inability to synthesize dopamine and serotonin from L-DOPA and 5-HTP
    Typical clinical featuresMovement disorders (hypokinesia, dystonia, oculogyric crises), autonomic dysfunction, behavioral problems, and developmental delay
    inv-13: Kebilidi (eladocagene exuparvovec-tneq) — therapy definition
    Therapy type
    Policy Summary
    PayerUnitedHealthcare
    PolicyKebilidi (eladocagene exuparvovec-tneq) — Coverage Criteria for AADC Deficiency
    Policy CodePolicy CS2026D0136D
    Change TypeRevised clinical confirmation and ambulation language
    Effective DateAug 1, 2026
    Last ReviewAug 1, 2026
    Next Review DateN/A
    Key ActionObtain prior authorization and submit medical records documenting genetically confirmed AADC deficiency with required clinical and laboratory evidence.
    SourceLink
    On This Page
    Coverage Criteria for Kebilidi (eladocagene exuparvovec-tneq)Coding and Test ThresholdsProvider Actions, Authorization, and DocumentationBackground and Clinical ContextDefinitions
  1. Dosing per FDA approved labeling
  2. One treatment per lifetime; authorization ≤ 60 days
    3. Step Therapy

    Requirement to try standard medical therapy first

    Patient must have persistent symptoms of AADC deficiency despite trials of standard medical therapy (for example, dopamine agonists, monoamine oxidase inhibitors, pyridoxine/other forms of vitamin B6) before Kebilidi is considered.

    • First-line treatments include selective dopamine agonists, MAO-inhibitors, and pyridoxine
    • Multiple drug classes are often needed and a stepwise approach is recommended
    Documentation Required

    Required documentation to support prior authorization

    Submit medical records documenting genetically confirmed AADC deficiency due to biallelic mutations in the DDC gene, the presence of one or more typical clinical characteristics of AADC deficiency, and decreased AADC enzyme activity in plasma per current laboratory standards.

    • Genetic testing results showing biallelic DDC mutations
    • Clinical notes describing characteristic signs (e.g., hypotonia, oculogyric crises, dystonia, hypokinesia, autonomic dysfunction, developmental delay)
    • Laboratory report of decreased AADC enzyme activity in plasma
    • Neuroimaging confirming skull maturity for stereotactic neurosurgical administration
    Denial Risk

    Triggers for denial

    Denial may be issued if the patient previously received Kebilidi or any other gene therapy for AADC deficiency, if the anti-AAV2 antibody titer is higher than 1:1200 or >1 optical density by ELISA, if dosing is not in accordance with FDA labeling, or if the request exceeds one lifetime treatment or an authorization period longer than 60 days.

    • Prior gene therapy for AADC deficiency (including prior Kebilidi) excludes coverage
    • Anti-AAV2 antibody titer > 1:1200 or >1 OD by ELISA will trigger denial
    • Non‑FDA‑label dosing or requests for >1 lifetime treatment are not covered
    • Authorization requests exceeding 60 days may be denied
    One-time gene replacement therapy delivered via recombinant adeno-associated virus serotype 2 (AAV2)
    Active ingredientEladocagene exuparvovec-tneq
    Route and targetStereotactic intraputaminal administration to cells within the putamen
    Therapeutic goalDelivers a copy of the DDC gene to enable AADC enzyme expression and subsequent dopamine production