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    v1.0.31
    OpenPayerPolicy UpdatesPharmacy & Specialty DrugsBaylor Scott & White Brineura Coverage | OpenPayer
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    CurrentBaylor Scott & White Health PlanPolicy 238

    Cerliponase alfa (Brineura) Coverage

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    Prior authorization and medical necessity review for cerliponase alfa administered for neuronal ceroid lipofuscinosis type 2 (CLN2), across lines of business subject to applicable benefit plan and federal or state requirements.

    Policy Summary
    PayerBaylor Scott & White Health Plan
    PolicyCerliponase alfa (Brineura) Coverage
    Policy CodePolicy 238
    Change TypeMaterial policy update
    Effective DateOct 1, 2026
    Last ReviewSep 24, 2026
    Next Review DateSep 24, 2027
    Key ActionSubmit prior authorization documentation showing that all policy criteria for cerliponase alfa are met.
    SourceLink
    POLICY UPDATE CHANGES

    Supporting documentation is now required with coverage requests.

    Universal and renewal criteria, indication, dosing, FDA-labeled contraindication, and experimental/investigational language were updated.

    Medicare NCD/LCD and InterQual language was clarified, while Medicare and Medicaid plan instructions remain in the policy.

    RequiredPrior authorization
    CLN2Covered indication
    3–6CLN2 score

    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.

    ≥37 wk
    Minimum age
    ≥2.5 kgMinimum weight
    ExcludedOther indications

    Coverage Criteria

    Universal Criteria Applied To All Requests

    BSWHP may consider cerliponase alfa (Brineura®) medically necessary when documentation is submitted showing ALL of the following criteria are met:

    ALL of the following

    • ONE of the following

      • Member has a deficiency of tripeptidyl peptidase 1 (TPP1) in leukocytes or fibroblasts.
      • Member has biallelic pathogenic or likely pathogenic variants in the TPP1 gene.
    • Member has a combined score of 3–6 on the motor and language domains of the CLN2 clinical rating scale (CRS), with a score of at least 1 in each of the 2 domains.
    • Cerliponase alfa will be administered by, or under the direction of, a physician knowledgeable in intraventricular administration and management of hypersensitivities, including anaphylaxis.
    • Cerliponase alfa will be dosed and administered according to FDA-approved labeling.

    Exclusions and Contraindications

    Coverage requires that NONE of the following exclusions or contraindications apply:

    NONE of the following

    • Member is less than 37 weeks post-menstrual age (gestational age at birth plus post-natal age).
    • Member weighs less than 2.5 kg.
    • Member has a sign or symptom of acute or unresolved localized infection on or around the device insertion site, such as cellulitis or abscess.
    • Member has a suspected or confirmed central nervous system (CNS) infection, such as cloudy cerebrospinal fluid (CSF), positive CSF Gram stain, or meningitis.
    • Member has an acute intraventricular access device-related complication, such as leakage, device failure, or extravasation of fluid.

    Cerliponase alfa is not covered when the applicable medical-necessity criteria are not met or when an exclusion or contraindication applies. Prior authorization is required, and requests are reviewed by both a clinical pharmacist and a medical director.

    BSWHP considers cerliponase alfa (Brineura®) for indications other than the treatment of neuronal ceroid lipofuscinosis type 2 (CLN2) to be experimental and investigational because the effectiveness of this strategy has not been established.

    Initial Therapy

    Initial Therapy

    Initial treatment is covered when all universal diagnostic, clinical, administration, dosing, and exclusion criteria are satisfied.

    Initial therapy criteria

    • Diagnostic confirmation

      • CLN2 is documented by deficiency of TPP1 in leukocytes or fibroblasts.
      • CLN2 is documented by biallelic pathogenic or likely pathogenic variants in the TPP1 gene.

    • Clinical, administration, and dosing requirements

      • The combined score on the motor and language domains of the CLN2 clinical rating scale is 3–6, with at least 1 point in each domain.3–6; at least 1 in each domain

    Continuation Therapy

    Continuation Therapy

    Continuation therapy is covered when ALL of the following renewal criteria are met.

    Continuation therapy

    • The member continues to meet all universal criteria.

    • Efficacy evidence

      • There has been no loss of ambulation.
      • The member's loss of ambulation has slowed from baseline.

    Renewal Requests

    Renewal requests require all universal criteria, documented efficacy, and manageable adverse effects.

    Renewal request criteria

    Coding and Clinical Thresholds

    HCPCS CodesHCPCS
    J0567Injection, cerliponase alfa, 1 mg
    ICD-10 CodesICD-10
    E75.4Neuronal ceroid lipofuscinosis
    Combined CLN2 clinical rating scale score
    Required combined score3–6 on the motor and language domains of the CLN2 clinical rating scale
    Motor domain minimumAt least 1 point
    Language domain minimumAt least 1 point
    Post-menstrual age
    Minimum post-menstrual ageAt least 37 weeks
    DefinitionGestational age at birth plus post-natal age

    Provider Submission Requirements

    Prior Authorization

    Prior Authorization Required

    Prior authorization is required for cerliponase alfa (Brineura®). Before submitting a request, review the member's Evidence of Coverage (EOC) or Summary Plan Description (SPD); Medicare and Medicaid coverage requirements may also apply.

    • For Medicare plans, check applicable NCD or LCD criteria; if none apply, use the criteria below.
    • For Medicaid plans, confirm coverage under the Texas Medicaid Provider Procedures Manual (TMHP/TMPPM). Texas Mandate HB154 applies to Medicaid plans.
    Documentation Required

    Initial Request Documentation

    For an initial request, submit documentation that the member has CLN2 and that every universal medical-necessity criterion is satisfied.

    • Document CLN2 by either TPP1 deficiency in leukocytes or fibroblasts, or biallelic pathogenic or likely pathogenic TPP1 gene variants.

    Clinical Background

    Late infantile CLN2 disease, also known as neuronal ceroid lipofuscinosis type 2, is a rare inherited lysosomal storage disorder caused by deficiency of tripeptidyl peptidase-1 (TPP1). The deficiency leads to progressive motor decline; typical early manifestations include language delay, recurrent seizures, and ataxia. Cerliponase alfa is an intraventricular TPP1 replacement therapy intended to slow decline in motor function and loss of ambulation in children with CLN2.

    Definitions

    CLN2
    Full nameNeuronal ceroid lipofuscinosis type 2
    Accepted diagnostic documentationDeficiency of tripeptidyl peptidase 1 (TPP1) in leukocytes or fibroblasts
    Alternative diagnostic documentationBiallelic pathogenic or likely pathogenic variants in the TPP1 gene
    CLN2 clinical rating scale
    DefinitionThe sum of the motor and language domain scores used to assess CLN2 disease progression
    Required combined score3–6
    Required domain scores
    Policy Summary
    PayerBaylor Scott & White Health Plan
    PolicyCerliponase alfa (Brineura) Coverage
    Policy CodePolicy 238
    Change TypeMaterial policy update
    Effective DateOct 1, 2026
    Last ReviewSep 24, 2026
    Next Review DateSep 24, 2027
    Key ActionSubmit prior authorization documentation showing that all policy criteria for cerliponase alfa are met.
    SourceLink
    On This Page
    Coverage CriteriaInitial TherapyContinuation TherapyCoding and Clinical ThresholdsProvider Submission RequirementsClinical BackgroundDefinitions
    Member has a ventriculoperitoneal shunt.
  1. Member has another FDA-labeled contraindication to cerliponase alfa.
  2. Cerliponase alfa is administered by, or under the direction of, a physician knowledgeable in intraventricular administration and management of hypersensitivities, including anaphylaxis.
  3. Cerliponase alfa is dosed and administered according to FDA-approved labeling.

  4. Exclusion and contraindication requirements

    • The member is at least 37 weeks post-menstrual age and weighs at least 2.5 kg.At least 37 weeks post-menstrual age; at least 2.5 kg
    • There is no acute or unresolved localized infection on or around the device insertion site.
    • There is no suspected or confirmed CNS infection.
    • There are no acute intraventricular access device-related complications.
    • The member does not have a ventriculoperitoneal shunt or another FDA-labeled contraindication to cerliponase alfa.
    • The member continues to meet all universal criteria.

    • Documented efficacy

      • There has been no loss of ambulation.
      • The member's loss of ambulation has slowed from baseline.
    • Adverse effects are manageable.
    Weight
    Minimum weightAt least 2.5 kg
  5. Submit the CLN2 clinical rating scale motor-and-language combined score of 3–6, with a score of at least 1 in each domain.
  6. Identify the physician responsible for, or directing, intraventricular administration and management of hypersensitivities, including anaphylaxis.
  7. Confirm FDA-labeled dosing and administration, and document absence of listed contraindications or exclusions, including prohibited age or weight, infection, device complications, ventriculoperitoneal shunt, or other FDA-labeled contraindications.
    8. Documentation Required

    Renewal Documentation

    Renewal requests must demonstrate that the member continues to qualify and is benefiting from treatment without unacceptable toxicity.

    • Confirm that all universal criteria remain satisfied.
    • Document efficacy by showing no loss of ambulation or that loss of ambulation has slowed from baseline.
    • Document that adverse effects remain manageable.
    Denial Risk

    Failure to Meet Universal Criteria

    A request may be denied when any universal coverage criterion is not met or is not adequately documented.

    • No qualifying CLN2 diagnosis supported by TPP1 deficiency or biallelic pathogenic or likely pathogenic TPP1 variants.
    • CLN2 clinical rating scale motor-and-language combined score is outside 3–6 or either domain scores less than 1.
    • Administration is not by or under the direction of a physician knowledgeable in intraventricular administration and hypersensitivity management, or dosing does not follow FDA-approved labeling.
    • Any exclusion or contraindication is present, including age less than 37 weeks post-menstrual age, weight less than 2.5 kg, acute or unresolved insertion-site infection, suspected or confirmed CNS infection, acute intraventricular access-device complication, ventriculoperitoneal shunt, or another FDA-labeled contraindication.
    Denial Risk

    Insufficient Renewal Response

    A renewal request may be denied when the response does not adequately establish continued efficacy or tolerability.

    • Insufficient documentation that ambulation has been maintained or that loss of ambulation has slowed from baseline.
    • Adverse effects are not documented as manageable or are unacceptable.
    Denial Risk

    Uncovered Indication

    Coverage is limited to the covered CLN2 indication. BSWHP considers cerliponase alfa (Brineura®) for all other indications experimental and investigational because effectiveness has not been established.

    • Requests for treatment outside neuronal ceroid lipofuscinosis type 2 (CLN2) may be denied as an uncovered indication.
    • All requests are reviewed by both a clinical pharmacist and a medical director.
    At least 1 point in each of the motor and language domains
    Post-menstrual age
    DefinitionGestational age at birth plus post-natal age