CurrentAvMedPolicy Pub. 100-2

Kanuma (sebelipase alfa) prior authorization for LAL deficiency

Form and clinical criteria governing prior authorization and step-edit requests for Kanuma (sebelipase alfa) provided via medical benefit; applies to prescribers submitting requests to AvMed for covered members.

Policy Summary

PayerAvMed

PolicyKanuma (sebelipase alfa) prior authorization for LAL deficiency

Policy CodePolicy Pub. 100-2

Change TypeNo material change

Effective Date

Next Review Date

Key ActionPrescriber must sign the prior authorization form and submit required supporting documentation to AvMed.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsInitial Authorization

6 monthsReauthorization

J2840HCPCS

≥1 moMin Age

Coverage Criteria for Kanuma (sebelipase alfa)

inv-01: Initial Therapy — Covered when ALL of the following are met

Policy Summary

PayerAvMed

PolicyKanuma (sebelipase alfa) prior authorization for LAL deficiency

Policy CodePolicy Pub. 100-2

Change TypeNo material change

Effective Date

Next Review Date

Key ActionPrescriber must sign the prior authorization form and submit required supporting documentation to AvMed.

SourceLink

On This Page

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Initial Authorization criteria: Member is 1 month of age or older AND has a diagnosis of Lysosomal Acid Lipase (LAL) deficiency AND diagnosis confirmed by ONE of: biallelic pathogenic variants in LIPA OR deficient LAL enzyme activity in peripheral blood leukocytes, fibroblasts, or dried blood spots AND member has at least ONE clinical manifestation (hyperlipidemia despite appropriate lipid therapy, hepatomegaly, splenomegaly, steatosis, or elevated serum transaminases) AND dose does not exceed specified dosing limits AND member's current weight is documented

All listed items must be met; supporting documentation required

inv-02: Continuation / Reauthorization — Covered when ALL of the following are met

Covered when ALL of the following are met:

Reauthorization criteria: Member must continue to meet all initial authorization criteria AND must meet ONE of: demonstrated positive response to therapy (improvement in weight-for-age z-scores for growth failure, improvement in LDL, HDL, triglycerides, or AST/ALT) OR for dose escalation must have suboptimal clinical response evidenced by poor growth, deteriorating biochemical markers (ALT/AST) or lipid parameters (LDL-c, TG), or persistent/worsening organomegaly

Documentation of response or rationale for dose escalation required; reauthorization interval is 6 months

Initial Therapy and Dosing Criteria

inv-13: Initial Therapy and Dosing — Initial dosing and criteria

Initial dosing and criteria:

Initial dosing and criteria: Recommended dosing: 1 mg/kg every other week for infants, children, adolescents, and adults; may increase to a maximum of 3 mg/kg every other week for members with suboptimal response. For infants with rapidly progressive disease presenting within the first 6 months of life: 1 mg/kg once weekly, may increase to 3 mg/kg once weekly if response is not optimal, and may further increase to a maximum of 5 mg/kg once weekly if response remains suboptimal. Initial authorization duration: 6 months. Member must meet diagnostic and clinical manifestation criteria and member's current weight must be documented.

Dose escalation requires documented suboptimal response and supporting documentation

Reauthorization / Continuation Criteria

inv-14: Reauthorization — Reauthorization requires continued meeting of initial criteria plus evidence of clinical benefit or justification for dose escalation.

Reauthorization requires continued meeting of initial criteria plus evidence of clinical benefit or justification for dose escalation:

Reauthorization nodes: Must continue to meet all initial authorization criteria AND have at least ONE objective sign of improvement (improvement in weight-for-age z-scores for members with growth failure, improvement in LDL, HDL, triglycerides, or improvement in AST/ALT) OR, for dose escalation, documentation of suboptimal clinical response defined by at least ONE of: poor growth, deteriorating biochemical markers (e.g., ALT, AST), deteriorating lipid parameters (e.g., LDL-c, TG), or persistent/worsening organomegaly. Member's current weight must be documented.

Reauthorization interval is 6 months; documentation of response or rationale for dose escalation required

Coding and Dosing Information

Covered HCPCS CodesHCPCSCovered

J2840

Kanuma (sebelipase alfa) solution for IV infusion

Dose limits

Typical dosing (older infants, children, adolescents, adults)1 mg/kg every other week; may be increased to a maximum of 3 mg/kg every other week for suboptimal response.

Infants with rapidly progressive disease (presentation within first 6 months of life)Start 1 mg/kg once weekly; may increase to 3 mg/kg once weekly if response is suboptimal.

Refractory infants dosing escalationIf response remains not optimal after escalation to 3 mg/kg weekly, may further increase to a maximum of 5 mg/kg once weekly.

Initial authorization durationInitial Authorization: 6 months.

Weight documentation requirementMember's current weight must be noted on the request form.

Provider Actions, Documentation & Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required for Kanuma (sebelipase alfa) billed with J2840. The prescribing physician must hand-sign and clearly print their name on the Medical Prior Authorization/Step-Edit Request form (preprinted stamps are not valid). Fax the completed form to 1-877-535-1391. For urgent reviews, contact AvMed Pre-Authorization Department if standard review timing would seriously jeopardize the member's life, health, or ability to regain maximum function.

Prior authorization required for Kanuma (sebelipase alfa) — HCPCS J2840
Fax completed, hand-signed form to 1-877-535-1391
Urgent review: contact AvMed Pre-Authorization Department

Denial Risk

Incomplete or Incorrect Documentation May Delay or Deny Authorization

Authorization can be delayed or denied if the request form or supporting documentation is incomplete, incorrect, or illegible. Ensure all contact information (including phone and fax numbers) is correct on the form. For Medicare members, applicable NCDs/LCDs and Medicare Benefit Policy Manual (Pub. 100-2, Chapter 15, §50) requirements must be followed where relevant.

Incomplete/incorrect/illegible forms may delay or deny authorization
Verify and include correct phone and fax numbers on the form
Comply with applicable NCDs/LCDs and Medicare policies for Medicare members

Documentation Required

Required Documentation

Provide all required documentation to support the diagnosis and clinical manifestations. The prescribing physician’s hand-signed form and printed name are required. Include documentation such as genetic testing results, enzyme assay reports, relevant laboratory values, imaging or physical exam findings (e.g., hepatomegaly, splenomegaly), weight and date obtained, and prior therapy information to support step-edit criteria.

Prescriber signature (hand-signed) and printed name required — stamps not valid
Documentation supporting diagnosis: biallelic pathogenic LIPA variants OR deficient LAL enzyme activity (blood leukocytes, fibroblasts, or dried blood spot)
Clinical manifestations supporting LAL deficiency (e.g., hepatomegaly, splenomegaly, steatosis, elevated transaminases, persistent hyperlipidemia)
Weight and date weight obtained (current weight must be noted)
Lab results, diagnostics, chart notes, and prior therapy records as applicable

Note

Samples, Prior Therapy Verification, and Administration Site

Use of drug samples to initiate therapy does not satisfy step-edit or prior authorization criteria. Previous therapies will be verified via pharmacy paid claims or submitted chart notes. Indicate the location/site of drug administration and include the administering location’s NPI or DEA number, or indicate if being provided by Specialty Pharmacy (PropriumRx).

Samples cannot be used to meet prior authorization or step-edit requirements
Previous therapies will be verified through pharmacy paid claims or chart notes
Provide administering site and NPI/DEA #, or specify Specialty Pharmacy (PropriumRx)

Requirement	Notes
Previous therapies must be documented via claims or chart notes	Previous therapies will be verified through pharmacy paid claims or submitted chart notes; use of samples to initiate therapy does not meet step edit/preauthorization criteria.

Site of Administration Requirements

Billing Rule

Document site of care and administering location identifiers

Specify the location/site of administration and provide the NPI or DEA number of the administering location on the form, or indicate that the medication will be provided by the specialty pharmacy PropriumRx.

Provide location/site of drug administration and NPI or DEA number of administering location
Or indicate Specialty Pharmacy - PropriumRx if applicable

Definitions and Diagnostic Confirmation

Diagnostic confirmation (LAL deficiency)

Diagnostic confirmation option 1Biallelic pathogenic variants in LIPA (genetic testing).

Diagnostic confirmation option 2Deficient LAL enzyme activity in peripheral blood leukocytes, fibroblasts, or dried blood spots (enzyme assay).

Age requirementMember is 1 month of age or older for authorization.

Supporting documentationGenetic or enzyme testing results must be provided with the request; all documentation (labs, diagnostics, chart notes) may be required to substantiate criteria.

Background: Lysosomal Acid Lipase Deficiency

Lysosomal Acid Lipase (LAL) deficiency is a genetic disorder leading to deficient LAL enzyme activity with multisystem effects, including hepatic involvement and dyslipidemia. For administration logistics, the form requires the location/site of drug administration and the NPI or DEA number of the administering location, or indication that the medication will be dispensed via Specialty Pharmacy - PropriumRx. For purposes of prior authorization and step edits, use of samples to initiate therapy does not meet step edit/preauthorization criteria, and any previous therapies will be verified through pharmacy paid claims or submitted chart notes.

OpenPayer