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Coverage criteria policies

Mucopolysaccharidoses (MPS) drug therapy – Minnesota Health Care Programs

These services may or may not be covered by all HealthPartners plans. Please see your plan documents for your own coverage information. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

Administrative Process

Enzyme replacement therapy for mucopolysaccharidoses (MPS) requires prior authorization from HealthPartners Pharmacy Administration.

Coverage

Mepsevii

Initial authorization criteria:

  1. Patient must be aged 5 months or older; and,
  2. Patient must be prescribed Mepsevii by, or in consultation with, a metabolic or genetic specialist; and,
  3. Patient must have a documented diagnosis of mucopolysaccharidoses VII (MPS VII, also known as Sly Syndrome) confirmed by both of the following:
    1. Beta-glucuronidase enzyme deficiency in peripheral blood leukocytes; and,
    2. Detection of pathogenic mutations in the GUSB gene by molecular genetic testing; and,
  4. Patient must have documented baseline value for at least one of the following:
    1. 6 minute walk test (6MWT) and/or motor function [e.g., Bruininks-Oseretsky Test of Motor Proficiency (BOT-2)]; or,
    2. Liver and/or spleen volume; or,
    3. Urinary excretion of glycosaminoglycans (GAGs) such as chondroitin sulfate and dermatan sulfate; or,
    4. Skeletal involvement; or,
    5. Pulmonary function tests

Reauthorization criteria:

  1. Patient has absence of unacceptable toxicity from the drug, including any of the following: anaphylaxis and severe allergic reactions, etc.; and,
  2. Patient must have demonstrated response to therapy compared to pretreatment baseline in at least one of the following:
    1. Stability or improvement in 6MWT and/or motor function; or,
    2. Reduction in liver and/or spleen volume; or,
    3. Reduction in urinary excretion of glycosaminoglycans (GAGs); or,
    4. Stability of skeletal disease; or,
    5. Stability or improvement in pulmonary function tests

Quantity limit:

46 vials per 14 days

Vimizim

Authorization criteria:

  1. Patient must be aged 5 months or older; and,
  2. Patient has a diagnosis of Mucopolysaccharidoses type IVA (MPS IVA; Morquio A syndrome)

Quantity limit:

Dosing will be limited to 2 mg/kg/week. Approval will include a quantity sufficient for a 34-day supply. Patient’s weight in kilograms must be supplied at time of prior authorization request and for any subsequent dose increases.

Aldurazyme, Elaprase, Naglazyme

Enzyme replacement therapy for Aldurazyme, Elaprase, or Naglazyme is generally covered when:

  1. Prescribed by a specialist; and,
  2. Prescribed for an FDA-approved indication using an approved regimen as below. A current patient weight is required for all requests; and,
  3. The patient and/or guardian has attested that they will adhere to the treatment plan.

Initial approvals will be for twelve months.

Annual reauthorizations will require medical chart documentation that the patient has been seen within the past 12 months and that markers of disease such as pulmonary function, spleen volume, walking or stair-climbing capacity are improved by therapy.

Indications and regimens that are covered

Aldurazyme (laronidase) is generally covered when prescribed for patients with Hurler and Hurler-Scheie forms of MPS I and for patients with the Scheie form who have moderate to severe symptoms. The approved regimen is 0.58 mg/kg of body weight administered once weekly as an intravenous (IV) infusion.

Elaprase (idursulfase) is indicated for patients with Hunter syndrome (MPS II). The approved regimen is 0.5 mg per kg of body weight administered once every week as an intravenous infusion.

Naglazyme (galsulfase) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for patients with MPS VI (Maroteaux-Lamy syndrome). The approved dosage regimen is 1 mg per kg of body weight administered once weekly as an intravenous infusion.

Mepsevii (vestronidase alfa-vjbk is indicated in pediatric and adult patients for the treatment of Mucopolysaccharidoses VII (MPS VII, Sly syndrome). The approved regimen is 4 mg/kg of body weight administered every two weeks as an intravenous (IV) infusion.

Vimizim (elosulfase) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for patients with Mucopolysaccharidoses type IVA (MPS IVA: Morquio A syndrome). The FDA approved regimen is 2 mg per kg body weight administered once every week as an intravenous infusion over a minimum of 3.5 to 4.5 hours, based on infusion volume.

Indications that are not covered

Requests for use in indications that are not FDA-approved will be reviewed on a case by case basis for medical necessity.

Definitions

Mucopolysaccharidoses (MPS) is a term for several lysosomal storage disorders caused by the deficiency of enzymes required for the breakdown of glycosaminoglycans, also known as mucopolysaccharidoses. Enzyme replacement therapy is approved for patients with MPS I, MPS II, and MPS VI. MPS I or Hurler, Hurler-Scheie and Scheie syndromes result from a deficiency of alpha-L-iduronidase or laronidase.

Aldurazyme (laronidase) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for patients with Hurler and Hurler-Scheie forms of MPS I and for patients with the Scheie form who have moderate to severe symptoms. The risks and benefits of treating mildly affected patients with the Scheie form have not been established. Aldurazyme has been shown to improve pulmonary function and walking capacity. It has not been evaluated for effects on the central nervous system manifestations of the disorder.

MPS II or Hunter syndrome results from insufficient levels of iduronate sulfatase or idursulfase.

Elaprase (idursulfase) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for patients with Hunter syndrome (Mucopolysaccharidoses II, MPS II). ELAPRASE has been shown to improve walking capacity in patients 5 years and older. In patients 16 months to 5 years of age, no data are available to demonstrate improvement in disease-related symptoms or long term clinical outcome; however, treatment with ELAPRASE has reduced spleen volume similarly to that of adults and children 5 years of age and older. The safety and efficacy of ELAPRASE have not been established in pediatric patients less than 16 months of age (1).

Mepsevii (vestronidase alfa-vjbk) is a recombinant human lysosomal beta glucuronidase indicated in pediatric and adult patients for the treatment of Mucopolysaccharidoses VII (MPS VII, Sly syndrome).

MPS VI or Maroteaux-Lamy syndrome results from a deficiency of N-acetylgalactosamine-4-sulfate or galsulfase.

Naglazyme (galsulfase) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for patients with MPS VI (Maroteaux-Lamy syndrome). It has been shown to improve walking and stair-climbing capacity.

Vimizim (elosulfase) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for patients with Mucopolysaccharidoses type IVA (MPS IVA: Morquio A syndrome).

If available, codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.

HCPCS Codes

Codes

Description

C9399

Unclassified drugs or biologicals (Hospital Outpatient Use ONLY)

J1931

Injection, laronidase, 0.1 mg (Aldurazyme)

J1743

Injection, idursulfase, 1 mg (Elaprase)

J1458

Injection, galsulfase, 1 mg (Naglazyme)

J1322

Injection, elosulfase alfa, 1mg (Vimizim)

J3397

Injection, vestronidase alfa-vjbk, 1 mg (Mepsevii) (effective 1/1/2019)

J3590

Unclassified biologics (Use before 1/1/2019)

NDC Codes

Codes

Description

58468007001

Aldurazyme 2.9 MG/5ML SOLN

54092070001

Elaprase 6 MG/3ML SOLN

68135002001

Naglazyme 1 MG/ML SOLN

68135010001

Vimizim 5 MG/5ML SOLN

69794000101

Mepsevii 10 MG/5ML SOLN

CPT Copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

Products

This information is for most, but not all, HealthPartners plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.

References

  1. https://mn.gov/dhs/partners-and-providers/policies-procedures/minnesota-health-care-programs/provider/types/rx/pa-criteria/mepsevii.jsp

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Policy activity

  • 01/01/2017 - Date of origin
  • 10/01/2018 - Effective date
Review date
  • 11/2018
Revision date
  • 05/07/2018

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