ph-0006
print Print

Aldurazyme (laronidase)

Policy Number: PH-0006

 

(Intravenous)

 

Last Review Date: 02/04/2020

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

  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
  1. Initial Approval Criteria1,2,3,4,5,6

           Coverage is provided in the following conditions:

           Universal Criteria

  • Patient is 6 months of age or older; AND

    Mucopolysaccharidosis I (MPS I)

  • Patient has a definitive diagnosis of MPS I 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
  • Diagnosis of Hurler (severe) or Hurler-Scheie (attenuated) forms of disease OR
  • Diagnosis of Scheie (attenuated) form of disease with moderate to severe symptoms; AND
  • Patient has absence of severe cognitive impairment; AND
  • Documented baseline value for urinary glycosaminoglycan (uGAG) ; AND
  • Documented baseline values for one or more of the following:
    • Patients 6 years or greater: percent predicted forced vital capacity (FVC), 6-minute walk test, joint range of motion, left ventricular hypertrophy, growth, quality of life (CHAQ/HAQ/MPS HAQ); OR
    • Patients 6 months to less than 6 years: cardiac status, upper airway obstruction during sleep, growth velocity, mental development, FVC, and/or 6-minute walk test

FDA approved indication(s)

  1. Renewal Criteria1,2,5,6

Authorizations can be renewed based on 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: 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 baseline in one or more of the following:
    • Patients 6 years or greater: stability or improvement in percent predicted FVC and/or 6-minute walk test, increased joint range of motion, decreased left ventricular hypertrophy, improved growth, improved quality of life (clinically meaningful change in the CHAQ/HAQ/MPS HAQ disability index); OR
    • Patients 6 months to less than 6 years: stability or improvement in cardiac status, upper airway obstruction during sleep, growth velocity, mental development, FVC and/or 6-minute walk test
  1. Dosage/Administration1,2,5,6

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 December 2020.
  2. Clark LA. Mucopolysaccharidosis Type I. GeneReviews®. www.ncbi.nlm.nih.gov/books/NBK1162/. Initial Posting: October 31, 2002; Last Update: February 11, 2016. Accessed on August 24, 2018.
  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 Articles 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/Article): 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
Proprietary Information. Restricted Access – Do not disseminate or copy without approval.
©2020, Magellan Rx Management

White MRx.PNG