Sebelipase alfa (Kanuma)

Section: Injections
Effective Date: June 01, 2020
Revised Date: May 04, 2020
Last Reviewed: May 19, 2020


Sebelipase alfa (Kanuma) is a recombinant human lysosomal acid lipase (rhLAL). Lysosomal acid lipase is a lysosomal glycoprotein enzyme that catalyzes the hydrolysis of cholesteryl esters to free cholesterol and fatty acids and the hydrolysis of triglycerides to glycerol and free fatty acids.


Coverage is subject to the specific terms of the member's benefit plan.

Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the website.

Sebelipase alfa (Kanuma) ) may be considered medically necessary for the treatment of lysosomal acid lipase (LAL) [Wolman's disease, cholesteryl ester storage disease (CESD)] deficiency when the following criteria are met:

  • Deficiency of LAL in peripheral blood leukocytes, fibroblasts, or dried blood spots; or
  • Documented molecular genetic test revealing biallelic pathogenic variants (2) mutations in the LIPA gene; and
  • Individual does not have severe liver cirrhosis (e.g., Child-Pugh class C); and
  • Documentation of baseline weight for age Z-score for LDL, HDL, AST, ALT and/or triglycerides.

Reauthorization Criteria

Continuation of therapy with sebelipase alfa (Kanuma) may be considered medically necessary for a reauthorization period of 12 months when ALL of the following are met:

  • No unacceptable adverse effect, e.g., anaphylaxis; and
  • Treatment has resulted in a clinical benefit such as an improvement in weight for age Z-scores for individuals with growth failure, improved LDL, HDL, AST, ALT and/or triglycerides compared to previously submitted documentation.

Sebelipase Alfa (Kanuma) is considered experimental/investigational for any other indication and therefore non-covered. Scientific evidence does not support its use for any other indications.

Procedure Codes


NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes


Professional Statements and Societal Positions Guidelines

Not Applicable

ND Committee Review

Original Effective Date: July 1, 2019

Internal Medical Policy Committee 5-19-2020 Added additional criteria and reauthorization criteria



Current medical policy is to be used in determining a Member's contract benefits on the date that services are rendered. Contract language, including definitions and specific inclusions/exclusions, as well as state and federal law, must be considered in determining eligibility for coverage. Members must consult their applicable benefit plans or contact a Member Services representative for specific coverage information. Likewise, medical policy, which addresses the issue(s) in any specific case, should be considered before utilizing medical opinion in adjudication. Medical technology is constantly evolving and the Company reserves the right to review and update medical policy periodically.