ph-0277
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Kanuma (sebelipase alfa)

Policy Number: PH-0277

(Intravenous)

 

Last Review Date: 02/04/2020

Date of Origin: 05/31/2016

Dates Reviewed: 05/2016, 04/2017, 04/2018, 02/2019, 02/2020

  1. Length of Authorization

Initial coverage will be provided for 6 months and may be renewed annually thereafter.

  1. Dosing Limits

A.  Quantity Limit (max daily dose) [NDC unit]:

  • Kanuma 20 mg/10 mL single-use vials: 68 vials per 28 day supply

B.  Max Units (per dose and over time) [HCPCS Unit]:

  • 340 billable units once weekly
  1. Initial Approval Criteria 1-6

Coverage is provided in the following conditions:

Universal Criteria

  • Patient is at least 1 month old

Lysosomal Acid Lipase (LAL) deficiency †

  • Diagnosis has been confirmed by either biallelic pathogenic variants in LIPA or deficient LAL enzyme activity in peripheral blood leukocytes, fibroblasts, or dried blood spots; AND

FDA Approved Indication(s)

  1. Renewal Criteria 1-6

Coverage can be renewed based upon the following criteria:

  • Patient continues to meet universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include:  hypersensitivity reactions (anaphylaxis, abdominal pain, fever, chills, pruritus, rash, vomiting), etc. ; AND
  • Treatment has resulted in clinical benefit as evidenced in one or more of the following:
  • Improvement in weight-for-age z-scores for patients exhibiting growth failure
  • Improvement in LDL
  • Improvement in HDL
  • Improvement in triglycerides
  • Improvement of AST or ALT
  1. Dosage/Administration1

Indication

Dose

LAL deficiency

Pediatric & Adult patients:

  • 1 mg/kg administered once every other week as an IV infusion

Rapidly progressive disease presenting within the first 6 months of life:

  • 1 mg/kg administered once weekly as an IV infusion
  • May increase to 3 mg/kg once weekly for patients who do not achieve an optimal clinical response
  1. Billing Code/Availability Information

HCPCS code:

J2840 - Injection, sebelipase alfa, 1 mg: 1 billable unit = 1 mg

NDC(s):

Kanuma 20 mg/10 mL single-use vials: 25682-0007-xx

  1. References
  1. Kanuma [package insert]. Cheshire, CT; Alexion Pharmaceuticals, Inc; December 2015. Accessed January 2020.
  2. Porto AF. Lysosomal acid lipase deficiency: diagnosis and treatment of Wolman and Cholesteryl Ester Storage Diseases. Pediatr Endocrinol Rev. 2014 Sep;12 Suppl 1:125-32.
  3. Zhang B, Port AF. Cholesteryl ester storage disease: protean presentations of lysosomal acid lipase deficiency. Pediatr Gastroenterol Nutr. 2013;56(6):682. 
  4. Reiner Z, Guardamagna O, Nair D, et al. Lysosomal acid lipase deficiency--an under-recognized cause of dyslipidaemia and liver dysfunction. Atherosclerosis. 2014 Jul;235(1):21-30. doi: 10.1016/j.atherosclerosis.2014.04.003.
  5. Hamilton J, Jones I, Srivastava R. A new method for the measurement of lysosomal acid lipase in dried blood spots using the inhibitor Lalistat 2. Clin Chim Acta. 2012 Aug 16;413(15-16):1207-10. doi: 10.1016/j.cca.2012.03.019.
  6. Burton BK, Balwani M, Feillet F, et al. A Phase 3 Trial of Sebelipase Alfa in Lysosomal Acid Lipase Deficiency. 2015 Sep 10;373(11):1010-20. doi: 10.1056/NEJMoa1501365.

Appendix 1 – Covered Diagnosis Codes

ICD-10

ICD-10 Description

E75.5

Other lipid storage disorders

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determination (NCD) and Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. They can be found at: http://www.cms.gov/medicare-coverage-database/search/advanced-search.aspx. Additional indications may be covered at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD): N/A

Medicare Part B Administrative Contractor (MAC) Jurisdictions

Jurisdiction

Applicable State/US Territory

Contractor

E (1)

CA, HI, NV, AS, GU, CNMI

Noridian Healthcare Solutions, LLC

F (2 & 3)

AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ

Noridian Healthcare Solutions, LLC

5

KS, NE, IA, MO

Wisconsin Physicians Service Insurance Corp (WPS)

6

MN, WI, IL

National Government Services, Inc. (NGS)

H (4 & 7)

LA, AR, MS, TX, OK, CO, NM

Novitas Solutions, Inc.

8

MI, IN

Wisconsin Physicians Service Insurance Corp (WPS)

N (9)

FL, PR, VI

First Coast Service Options, Inc.

J (10)

TN, GA, AL

Palmetto GBA, LLC

M (11)

NC, SC, WV, VA (excluding below)

Palmetto GBA, LLC

L (12)

DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA)

Novitas Solutions, Inc.

K (13 & 14)

NY, CT, MA, RI, VT, ME, NH

National Government Services, Inc. (NGS)

15

KY, OH

CGS Administrators, LLC

 

 

KANUMA™ (sebelipase alfa) Prior Auth Criteria
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