CurrentBlue Cross Blue Shield - North CarolinaPolicy N/A

Eladocagene exuparvovec-tneq (Kebilidi) intraputaminal infusion coverage criteria

Defines medical necessity, coverage limitations, and documentation requirements for eladocagene exuparvovec-tneq (Kebilidi) intraputaminal infusion for treatment of AADC deficiency for Blue Cross NC members.

Policy Summary

PayerBlue Cross Blue Shield - North Carolina

PolicyEladocagene exuparvovec-tneq (Kebilidi) intraputaminal infusion coverage criteria

Policy CodePolicy N/A

Change TypeCoding update (revenue codes added)

Effective Date4/1/2026

Next Review DateN/A

Key ActionObtain prior authorization with documentation demonstrating all medical necessity criteria and requested dose within FDA-labeled dosing.

SourceLink

POLICY UPDATE CHANGES

Added revenue codes 0891 and 0892 as applicable to the policy HCPCS code(s).

16 months–10 yrseligible patient age range

90 daysduration of approval

1one-time lifetime treatment

1.8e11 vgFDA recommended dose

C9399, J3490, J3590

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Coverage Criteria

(untitled)

Covered when ALL of the following are met:

ALL of the following

The patient is at least 16 months of age through 10 years of age;
The patient has a diagnosis of severe aromatic L-amino acid decarboxylase (AADC) deficiency (medical record documentation required);
Diagnosis confirmation
- Molecular genetic testing demonstrating biallelic mutations in the human DOPA decarboxylase (DDC) gene (medical record documentation required);
- Decreased plasma AADC enzyme activity (medical record documentation required);
The patient is experiencing persistent neurological defects (e.g., autonomic dysfunction, hypotonia, movement disorders [oculogyric crises, dystonia, hypokinesia], developmental delay) secondary to AADC deficiency despite standard medical therapy (e.g., dopamine agonists, monoamine oxidase inhibitors, pyridoxine, or other forms of vitamin B6) on stable dosages for at least 3 months prior to treatment with the requested agent (medical record documentation required);
The patient is unable to ambulate independently (with or without assistive device) (medical record documentation required);
The patient has achieved skull maturity as assessed by neuroimaging (medical record documentation required);
The patient does NOT have any other enzyme deficiencies, including pyridoxine 5'-phosphate oxidase or tetrahydrobiopterin (BH4) deficiency (medical record documentation required);
The patient does NOT have a baseline anti-adeno-associated virus, serotype 2 (anti-AAV2) antibody titer higher than 1:1200 or greater than 1 optical density value by enzyme-linked immunosorbent assay (medical record documentation required);
The patient does NOT have evidence of a clinically active infection (medical record documentation required);
The patient has NOT received any previous gene therapy, including the requested agent (medical record documentation required);

(untitled)

AND the following provider/dosing conditions must be met:

ALL of the following

The prescriber is a specialist in the area of the patient's diagnosis (e.g., pediatric neurologist) or has consulted with a specialist in the area of the patient's diagnosis (medical record documentation required);
The requested dose is within FDA labeled dosing for the requested indication, and the requested quantity does NOT exceed the maximum units allowed for the duration of approval (medical record documentation required).
Duration of Approval: 90 days (one-time treatment per lifetime).
FDA label dosing: Total recommended dose of 1.8 x 10^11 vector genomes (vg), administered as four intraputaminal infusions in a single stereotactic neurosurgical procedure (medical record documentation required).

Exclude other enzyme deficiencies: The patient must NOT have any other enzyme deficiencies (e.g., pyridoxine 5'-phosphate oxidase or tetrahydrobiopterin [BH4] deficiency). Medical record documentation confirming absence of these alternative diagnoses is required.

Not medically necessary when any of the following apply: prior receipt of any gene therapy (including the requested agent); presence of a clinically active infection; baseline anti-AAV2 antibody titer greater than 1:1200 or optical density above assay cutoff; or other contraindicating conditions per FDA labeling. Medical record documentation required for exclusions and prior treatments.

Coding and Billing

Applicable HCPCS CodesHCPCS

C9399	HCPCS code listed for eladocagene exuparvovec-tneq (Kebilidi) intraputaminal infusion
J3490	HCPCS code listed for eladocagene exuparvovec-tneq (Kebilidi) intraputaminal infusion
J3590	HCPCS code listed for eladocagene exuparvovec-tneq (Kebilidi) intraputaminal infusion

Applicable Revenue CodesRevenue

0891	Special Processed Drugs - FDA Approved Cell Therapy (applicable revenue code)
0892	Special Processed Drugs - FDA Approved Gene Therapy (applicable revenue code)

inv-07: Anti-AAV2 antibody titer — threshold and testing specification

ThresholdBaseline anti-AAV2 antibody titer not higher than 1:1200 or greater than 1 optical density (OD) value by enzyme-linked immunosorbent assay (ELISA)

Test methodEnzyme-linked immunosorbent assay (ELISA) measuring anti-AAV2 antibody optical density

Specimen timingBaseline (pre-treatment) measurement required prior to administration of gene therapy

DocumentationMedical record documentation of the quantitative titer/OD result required

Provider Actions and Requirements

Prior Authorization

Prior Authorization and Dosing/Quantity Limits

Prior authorization is required for coverage of the requested therapy. The requested dose must be within FDA-labeled dosing for the indication and the requested quantity must not exceed the maximum units allowed for the duration of approval. Duration of approval is a one-time treatment per lifetime (90 days). Distribution from a specialty pharmacy provider may be required when coverage is available — contact Blue Cross NC to coordinate therapy.

Affected HCPCS: C9399, J3490, J3590
Maximum units: 1 (see dosing table and FDA label)
Duration of approval: 90 days (one-time treatment per lifetime)

Documentation Required

Medical Record Documentation Required for Diagnosis Confirmation and Testing

Medical record documentation is required to confirm the diagnosis and to support that medical necessity criteria are met. Submit documentation demonstrating patient age, diagnosis of severe AADC deficiency, confirmation testing results, prior treatment history, functional status, skull maturity assessment, infection and antibody testing, prior gene therapy history, and prescriber specialty or consultation.

Background

Aromatic L-amino acid decarboxylase (AADC) deficiency is a rare, inherited disorder caused by biallelic mutations in the DDC gene leading to markedly reduced AADC enzyme activity. Affected children present with persistent neurological deficits such as hypotonia, autonomic dysfunction, movement disorders (for example oculogyric crises, dystonia, hypokinesia), and developmental delay. Diagnosis requires medical record documentation of severe AADC deficiency confirmed by molecular genetic testing demonstrating biallelic DDC mutations and decreased plasma AADC enzyme activity.

Definitions

inv-13: Aromatic L-amino acid decarboxylase (AADC) deficiency — diagnostic definition (biallelic DDC mutations and decreased plasma AADC activity)

Diagnostic definitionAromatic L-amino acid decarboxylase (AADC) deficiency confirmed by molecular genetic testing demonstrating biallelic mutations in the DDC gene and decreased plasma AADC enzyme activity

Required genetic testingMolecular genetic testing demonstrating biallelic mutations in the human DOPA decarboxylase (DDC) gene (medical record documentation required)

Enzyme activity measurementDecreased plasma AADC enzyme activity documented in the medical record (medical record documentation required)

Associated clinical featuresPersistent neurological defects (eg autonomic dysfunction, hypotonia, movement disorders, developmental delay) consistent with AADC deficiency

inv-14: Eladocagene exuparvovec-tneq dosing — FDA recommended dosing (total 1.8 x 10^11 vg as four intraputaminal infusions)