Highmark Commercial Medical Policy - Pennsylvania

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Medical Policy: I-188-001
Topic: Vestronidase Alfa (Mepsevii)
Section: Injections
Effective Date: August 6, 2018
Issue Date: August 6, 2018
Last Reviewed: July 2018

Vestronidase alpha-vjbk (MepseviiTM) is a recombinant human lysosomal beta glucuronidase enzyme replacement indicated in pediatric and adult individuals for the treatment of Mucopolysaccharidosis VII (MPS VII, Sly syndrome).

MPS VII is caused by pathogenic mutations in the GUSB gene. The GUSB gene is responsible for producing an enzyme called beta-glucuronidase which is involved in the breakdown of large moleules called glycosaminoglycans (GAGs).

This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical circumstances may warrant individual consideration, based on review of applicable medical records.

Policy Position Coverage is subject to the specific terms of the member’s benefit plan.

Vestronidase alpha (Mepsevi) may be considered medically necessary in infant, pediatric and adult individuals’ age five (5) months and older that meets All of the following:

  • Diagnosis of MPS VII (Sly syndrome); and
  • ANY of the following:
    • Molecular genetic confirmation of pathogenic mutations in the GUSB gene; or
    • Clinical signs and symptoms of MPS VII (may include but are not limited to skeletal abnormalities shown on x-ray, short stature, macrocephaly, hepatosplenomegaly, history of inguinal or umbilical hernia, decreased pediatric quality of life score, decreased forced vital capacity (FVC) or impaired six minute walk test appropriate for age) and the following abnormal lab values:
      • Decreased level of beta-glucuronidase activity in blood; and
      • Increased level of urinary GAGs.

Continuation of therapy after twelve (12) months will be considered medically necessary for individuals diagnosed with MPS VII when all of the following criteria are met:

  • Documentation of decreased urinary GAG level; and
  • Documentation of improvement of clinical signs and symptoms such as but not limited to improvement in the six minute walk test, pediatric quality of life or forced vital capacity (FVC).

The use of vestronidase alpha (Mepsevii) for any other indication is considered experimental/investigational and therefore, non-covered. There is a lack of clinical data to support its safety and efficacy in other conditions.

Procedure Codes

NOTE: Dosage recommendations per the FDA label.

Place of Service: Inpatient/Outpatient

Experimental/Investigational (E/I) services are not covered regardless of place of service.

The use of vestronidase alpha (Mepsevii) is typically an outpatient procedure which is only eligible for coverage as an inpatient procedure in special circumstances, including, but not limited to, the presence of a co-morbid condition that would require monitoring in a more controlled environment such as the inpatient setting.

The policy position applies to all commercial lines of business

Denial Statements

Services that do not meet the criteria of this policy will be considered experimental/investigational (E/I). A network provider can bill the member for the experimental/investigational service. The provider must give advance written notice informing the member that the service has been deemed E/I. The member must be provided with an estimate of the cost and the member must agree in writing to assume financial responsibility in advance of receiving the service. The signed agreement must be maintained in the provider’s records.


Medical policies do not constitute medical advice, nor are they intended to govern the practice of medicine. They are intended to reflect Highmark's reimbursement and coverage guidelines. Coverage for services may vary for individual members, based on the terms of the benefit contract.

Discrimination is Against the Law
The Claims Administrator/Insurer complies with applicable Federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex. The Claims Administrator/Insurer does not exclude people or treat them differently because of race, color, national origin, age, disability, or sex. The Claims Administrator/ Insurer:
  • Provides free aids and services to people with disabilities to communicate effectively with us, such as:
    • Qualified sign language interpreters
    • Written information in other formats (large print, audio, accessible electronic formats, other formats)
  • Provides free language services to people whose primary language is not English, such as:
    • Qualified interpreters
    • Information written in other languages
If you need these services, contact the Civil Rights Coordinator.

If you believe that the Claims Administrator/Insurer has failed to provide these services or discriminated in another way on the basis of race, color, national origin, age, disability, or sex, you can file a grievance with: Civil Rights Coordinator, P.O. Box 22492, Pittsburgh, PA 15222, Phone: 1-866-286-8295, TTY: 711, Fax: 412-544-2475, email: CivilRightsCoordinator@highmarkhealth.org. You can file a grievance in person or by mail, fax, or email. If you need help filing a grievance, the Civil Rights Coordinator is available to help you.

You can also file a civil rights complaint with the U.S. Department of Health and Human Services, Office for Civil Rights electronically through the Office for Civil Rights Complaint Portal, available at https://ocrportal.hhs.gov/ocr/portal/lobby.jsf, or by mail or phone at:

U.S. Department of Health and Human Services
200 Independence Avenue, SW
Room 509F, HHH Building
Washington, D.C. 20201
1-800-368-1019, 800-537-7697 (TDD)

Complaint forms are available at http://www.hhs.gov/ocr/office/file/index.html.

Insurance or benefit/claims administration may be provided by Highmark, Highmark Choice Company, Highmark Coverage Advantage, Highmark Health Insurance Company, First Priority Life Insurance Company, First Priority Health, Highmark Benefits Group, Highmark Select Resources, Highmark Senior Solutions Company or Highmark Senior Health Company, all of which are independent licensees of the Blue Cross and Blue Shield Association, an association of independent Blue Cross and Blue Shield plans.

Highmark retains the right to review and update its medical policy guidelines at its sole discretion. These guidelines are the proprietary information of Highmark. Any sale, copying or dissemination of the medical policies is prohibited; however, limited copying of medical policies is permitted for individual use.

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