CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Kebilidi (eladocagene exuparvovec-tneq) intraputaminal gene therapy — Coverage Criteria

Coverage policy for one-time intraputaminal administration of Kebilidi for treatment of aromatic L-amino acid decarboxylase (AADC) deficiency for Medicaid, Commercial, and Medicare-Medicaid Plan (MMP) members of Neighborhood Health Plan of Rhode Island.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyKebilidi (eladocagene exuparvovec-tneq) intraputaminal gene therapy — Coverage Criteria

Policy CodePolicy N/A

Change TypeNo material change

Effective DateMay 1, 2024

Next Review DateFeb 5, 2025

Key ActionPrior authorization is required with submission of supporting medical records demonstrating the patient meets all initial approval criteria.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

one doseauthorized doses

1.8x10^11 vgtotal dose

16 months–10 yrsage range

E70.81ICD-10

J3590HCPCS

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Coverage Criteria for Kebilidi (eladocagene exuparvovec-tneq)

Initial Therapy — Covered when ALL of the following are met.

Covered when ALL of the following are met:

Prescriber: Kebilidi is prescribed by or in consultation with a pediatric neurologist or geneticist.

Age: Patient is at least 16 months of age through 10 years of age.16 months-10 years

Diagnostic confirmation: Patient has a diagnosis of severe Aromatic L‑amino acid decarboxylase (AADC) deficiency established by EITHER biallelic pathogenic variants in the DDC gene identified by molecular genetic testing OR a CSF or plasma neurotransmitter profile consistent with AADC deficiency.

either molecular genetic confirmation OR supportive neurotransmitter profile satisfies this node

Enzyme activity: Patient has significantly reduced AADC enzyme activity in plasma.

Refractory disease: Patient is experiencing persistent neurological defects (e.g., autonomic dysfunction, hypotonia, dystonia, and other movement disorders) secondary to AADC deficiency despite standard medical therapy (e.g., dopamine agonists, monoamine oxidase inhibitor, pyridoxine, or other forms of vitamin B6) with stable dosages for at least 3 months prior to treatment.3 months stable therapy

Functional status: Patient is unable to ambulate independently.

Skull maturity: Patient has achieved skull maturity as assessed by neuroimaging.

Exclude other deficiencies: Patient does not have pyridoxine 5'-phosphate oxidase deficiency or tetrahydrobiopterin (BH4) deficiency.

No prior gene therapy: Patient has not received prior gene therapy.

Anti-AAV2 antibody: Patient must not have a baseline anti-AAV2 antibody titer above the established threshold for a positive result.below lab-defined positive threshold

Surgical candidacy: Patient does not have any contraindications that would preclude the surgical intraputaminal administration (e.g., significant brain structural abnormality).

Treatment center: Patient will undergo treatment at a manufacturer‑approved Qualified Treatment Center (QTC).

Coverage is excluded when the patient has a diagnosis of pyridoxine 5'-phosphate oxidase deficiency or tetrahydrobiopterin (BH4) deficiency.

Coverage is excluded for patients who have previously received gene therapy.

Authorization for Kebilidi is limited to a single dose; coverage cannot be renewed and renewal requests will not be authorized.

Coding and Eligibility Details

HCPCS codes providedHCPCS

J3590

Unclassified biologics

NDCNDC

52856-0601-xx

Kebilidi 5.6 × 10 11 vector genomes (vg) per mL - 2 mL single dose vial

Covered Diagnosis Codes (ICD-10)ICD-10Covered

E70.81

Aromatic L-amino acid decarboxylase deficiency

Age

Covered patient age rangePatient is at least 16 months of age through 10 years of age.

Minimum age16 months

Maximum age10 years

Anti-AAV2 antibody titer

Anti-AAV2 antibody titer requirementPatient must not have a baseline anti-AAV2 antibody titer above the established threshold for a positive result.

InterpretationTiter must be below the lab-defined positive threshold per the testing laboratory.

TimingBaseline (pre-treatment) measurement required.

Prior Authorization, Documentation, and Billing Actions

Prior Authorization

Prior authorization required

Prior authorization is required for J3590 with submission of supporting medical records and evidence that the patient meets all initial approval criteria.

Code: J3590 (Unclassified biologics)

Step Therapy

Step therapy exception for recent prior receipt

MMP members who previously received this medication within the past 365 days are exempt from Step Therapy requirements and should not be subject to step edits.

Documentation Required

Required documentation

Submission of medical records (chart notes) related to the medical necessity criteria is REQUIRED on all authorization requests; provide documentation via direct upload through the PA web portal or by fax.

Include documentation related to diagnosis, step therapy, and clinical markers (e.g., genetic and mutational testing) when applicable.

Denial Risk

Documentation submission required — risk of denial

Requests that do not include the required medical records (chart notes) supporting medical necessity, diagnosis, step therapy, and genetic/mutational testing documentation will be at risk for denial.

Records will be reviewed at time of submission.
Documentation should support initiation (diagnosis, step therapy status, genetic/mutational testing, clinical markers).

Definitions

Aromatic L-amino acid decarboxylase (AADC) deficiency

DefinitionA rare genetic disorder caused by biallelic pathogenic variants in the DDC gene leading to deficient AADC enzyme activity and characteristic neurotransmitter abnormalities and movement/autonomic dysfunction.

Typical manifestationsMovement disorders (dystonia, hypotonia), autonomic dysfunction, and developmental delay.

Confirmation methodsMolecular genetic testing showing biallelic DDC variants or CSF/plasma neurotransmitter profile consistent with AADC deficiency.