ph-0006
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Aldurazyme® (laronidase)

Policy Number: PH-0006

 

Intravenous

 

Last Review Date: 02/01/2022

Date of Origin: 11/28/2011

Dates Reviewed: 12/2011, 02/2013, 02/2014, 12/2014, 10/2015, 10/2016, 10/2017, 10/2018, 02/2020, 02/2021, 02/2022

FOR PEEHIP Members Only -Coverage excludes the provider-administered medication(s) outlined in this drug policy from being accessed through a specialty pharmacy. It must be obtained through buy and bill.

  1. Length of Authorization

Coverage will be provided for 12 months and may be renewed.

  1. Dosing Limits

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

  • Aldurazyme 2.9 mg vial: 92 vials every 28 days

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

  • 667 billable units every 7 days

Submission of medical records related to the medical necessity criteria is REQUIRED on all requests for authorizations. Records will be reviewed at the time of submission. Please provide documentation via direct upload through the PA web portal or by fax.

  1. Initial Approval Criteria 1-6

Depending on member benefits, additional criteria may apply for coverage of this drug in an outpatient facility setting. Verify any Site of Service requirements with the member’s plan and refer to the Voluntary Site of Service Policy or the Mandatory Site of Service Policy for additional information.

          Coverage is provided in the following conditions:

  • Patient is at least 6 months of age; AND
  • Patient has absence of severe cognitive impairment; AND
  • Documented baseline value for urinary glycosaminoglycan (uGAG) has been obtained; AND
  • Documented baseline age-appropriate values for one or more of the following have been obtained:
    • Patients 6 years of age or greater: percent predicted forced vital capacity (FVC), 6-minute walk test (6-MWT), joint range of motion, left ventricular hypertrophy, growth, and/or quality of life (CHAQ/HAQ/MPS HAQ); OR
    • Patients 6 months to less than 6 years of age: cardiac status, upper airway obstruction during sleep, growth velocity, mental development, FVC, and/or 6-MWT; AND

**NOTE: For very young patients in which FVC or 6-MWT are not suitable for measuring, requests will be reviewed on a case-by case basis.

Mucopolysaccharidosis I (MPS I) Ф 1,2

  • Patient has a definitive diagnosis of MPS I as confirmed by one of the following:
    • Detection of biallelic pathogenic mutations in the IDUA gene by molecular genetic testing; OR
    • Detection of deficient activity of the lysosomal enzyme α-L-iduronidase (IDUA); AND
  • Patient has one of the following diagnoses:
    • Hurler (severe) or Hurler-Scheie (attenuated) forms of disease; OR
    • Scheie (attenuated) form of disease with moderate to severe symptoms

FDA approved indication(s); Compendia recommended indication(s); Ф Orphan Drug

  1. Renewal Criteria 1,2,5,6

Coverage can be renewed based on the following criteria:

  • Patient continues to meet indication-specific relevant criteria identified in section III; AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: anaphylaxis and severe hypersensitivity reactions, acute respiratory complications, acute cardiorespiratory failure, severe infusion reactions, etc.; AND
  • Patient does not have progressive/irreversible severe cognitive impairment; AND
  • Patient has a documented reduction in uGAG levels compared to pretreatment baseline; AND
  • Patient has demonstrated a beneficial response to therapy compared to pretreatment age-appropriate baseline values in one or more of the following:
    • Patients 6 years of age or greater: stability or improvement in percent predicted FVC and/or 6-MWT, increased joint range of motion, decreased left ventricular hypertrophy, improved growth, and/or improved quality of life (clinically meaningful change in the CHAQ/HAQ/MPS HAQ disability index); OR
    • Patients 6 months to less than 6 years of age: stability or improvement in cardiac status, upper airway obstruction during sleep, growth velocity, mental development, FVC and/or 6-MWT
  1. Dosage/Administration 1

Indication

Dose

Mucopolysaccharidosis I (MPS I)

0.58 mg/kg of body weight administered once weekly as an intravenous infusion

  1. Billing Code/Availability Information

HCPCS Code:

  • J1931 – Injection, laronidase, 0.1 mg; 1 billable unit = 0.1 mg

NDC:

  • Aldurazyme 2.9 mg/5 mL single-dose vial: 58468-0070-xx
  1. References
  1. Aldurazyme [package insert].  Cambridge, MA; Genzyme Corporation.; December 2019.   Accessed January 2022.
  2. Clark LA. Mucopolysaccharidosis Type I. GeneReviews®. www.ncbi.nlm.nih.gov/books/NBK1162/. Initial Posting: October 31, 2002; Last Update: February 25, 2021. Accessed on January 19, 2022.
  3. Muenzer J, Wraith JE, Clarke LA; International Consensus Panel on Management and Treatment of Mucopolysaccharidosis I. Mucopolysaccharidosis I: management and treatment guidelines. Pediatrics. 2009 Jan; 123(1):19-29. doi: 10.1542/peds.2008-0416.
  4. Martins AM, Dualibi AP, Norato D, et al. 1.Guidelines for the Management of Mucopolysaccharidosis Type I. JPeds. 155 (4): S32 - S46.
  5. Clarke LA, Wraith JE, Beck M, et al. Long-term efficacy and safety of laronidase in the treatment of mucopolysaccharidosis I. Pediatrics. 2009 Jan;123(1):229-40. doi: 10.1542/peds.2007-3847.
  6. Wraith JE, Beck M, Lane R, et al. Enzyme replacement therapy in patients who have mucopolysaccharidosis I and are younger than 5 years: results of a multinational study of recombinant human alpha-L-iduronidase (laronidase). Pediatrics. 2007 Jul;120(1):e37-46. Epub 2007 Jun 4.

Appendix 1 – Covered Diagnosis Codes

ICD-10

ICD-10 Description

E76.01

Hurler's syndrome

E76.02

Hurler-Scheie syndrome

E76.03

Scheie's syndrome

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), Local Coverage Determinations (LCDs), and Local Coverage Articles (LCAs) may exist and compliance with these policies is required where applicable. They can be found at: https://www.cms.gov/medicare-coverage-database/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/LCA): 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

 

 

 

ALDURAZYME® (laronidase) Prior Auth Criteria
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