CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Kanuma (sebelipase alfa) — Coverage Criteria for Lysosomal Acid Lipase (LAL) Deficiency

Covers medical benefit authorization, dosing, and renewal criteria for Kanuma (sebelipase alfa) for treatment of Lysosomal Acid Lipase (LAL) deficiency for Neighborhood Health Plan of Rhode Island members.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyKanuma (sebelipase alfa) — Coverage Criteria for LAL deficiency

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateDec 20, 2017

Next Review DateN/A

Key ActionObtain prior authorization with documentation of age ≥1 month, specialist prescriber, baseline labs, and confirmed diagnostic testing.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsLength of initial and renewal authorization

1→5 mg/kgDosing range described

J2840HCPCS code

E75.5Covered ICD-10

112/28dQuantity limit (20 mg vials)

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Coverage Criteria for Kanuma (sebelipase alfa)

Initial Approval

Covered when ALL of the following are met:

Initial Approval Criteria: Patient is at least 1 month of age

Initial Approval Criteria: Prescribing physician is a specialist in genetics and metabolism

Initial Approval Criteria: Weight, baseline liver function and baseline lipid panel are provided

Initial Approval Criteria: Diagnosis confirmed by either biallelic pathogenic LIPA variants OR deficient LAL enzyme activity in peripheral blood leukocytes, fibroblasts, or dried blood spots

Members who received this medication within the past 365 days are not subject to step therapy requirements.

Renewal Criteria

Renewal approval provided when ALL of the following are met:

Renewal - general: Patient continues to meet indication-specific relevant criteria identified in the Initial Approval section (e.g., concomitant therapy requirements, performance status)

Renewal - safety: Absence of unacceptable toxicity from the drug (examples include hypersensitivity reactions including anaphylaxis)

Renewal - benefit or escalation: Either treatment has resulted in clinical benefit as evidenced by one or more of: improvement in weight-for-age z-scores, improvement in LDL, improvement in HDL, improvement in triglycerides, or improvement of AST or ALT; OR dose escalation is clinically justified as defined in policy

Dose escalation definitions include criteria for pediatric/adult suboptimal response and for infants with rapidly progressive disease presenting within the first 6 months of life.

Dosage/Administration

Dosing rules recognized by policy:

Pediatric & adult standard dosing: 1 mg/kg IV once every other week; may increase to 3 mg/kg once every other week for patients who do not achieve an optimal clinical response to 1 mg/kg

Infant escalation - initial: Infants with rapidly progressive disease presenting within the first 6 months of life: 1 mg/kg IV once weekly

Infant escalation - subsequent: May increase to 3 mg/kg IV once weekly for suboptimal response; if still suboptimal at 3 mg/kg, may further increase to 5 mg/kg IV once weekly

For INTEGRITY (Medicare-Medicaid Plan) members, these coverage criteria apply only in the absence of a National Coverage Determination (NCD) or Local Coverage Determination (LCD). When an applicable NCD or LCD exists, that determination takes precedence; otherwise the plan will apply the criteria in this policy and consider individual requests using submitted clinical information.

This policy bases coverage on FDA-approved labeling and the supporting clinical evidence reviewed by the plan. Kanuma (sebelipase alfa) is FDA-indicated for treatment of Lysosomal Acid Lipase (LAL) deficiency; the plan’s criteria are designed to align use with that indication and with published trial evidence. Use outside the FDA indication or without the diagnostic confirmation required by the policy may be considered not medically necessary.

Provider Requirements and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required. Initial and renewal authorizations are provided for 6 months.

PA required prior to service
Initial authorization length: 6 months
Prescribing specialty required: genetics and metabolism

Step Therapy

Step Therapy Exemption

Members who have received this medication within the past 365 days are exempt from Step Therapy requirements.

Step therapy exemption: prior receipt within 365 days

Documentation Required

Documentation and Diagnostic Requirements

Provide documentation of patient age, weight, prescriber specialty, baseline labs, and diagnostic confirmation. Missing required documentation may lead to denial of the request.

Minimum age: ≥ 1 month
Prescriber specialty: genetics and metabolism
Required baseline labs: weight, baseline liver function tests (AST/ALT), baseline lipid panel (LDL, HDL, triglycerides)
Diagnostic confirmation: biallelic pathogenic LIPA variants or deficient LAL enzyme activity (peripheral blood leukocytes, fibroblasts, or dried blood spots)

Denial Risk

Incomplete Documentation May Result in Denial

Failure to provide age, specialty of prescriber, patient weight, baseline liver function tests, baseline lipid panel, and diagnostic confirmation (genetic testing or enzyme assay) increases risk of denial.

Denial risk if any of the following are missing: age, prescriber specialty, weight, baseline AST/ALT, baseline lipid panel, diagnostic confirmation (LIPA genetic testing or enzyme assay)
Renewal requires evidence of clinical benefit and absence of unacceptable toxicity

OpenPayer

Kanuma (sebelipase alfa) — Coverage Criteria for Lysosomal Acid Lipase (LAL) Deficiency

Coverage Criteria for Kanuma (sebelipase alfa)

Billing and Coding

Provider Requirements and Authorization

Definitions and Diagnostic Confirmation

Background