Medical Policy
Effective Date:03/01/2019 Title:Mepsevii (vestronidase alfa-vjbk)
Revision Date: Document:BI598:00
CPT Code(s):N/A
Public Statement

Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Mepsevii (vestronidase alfa-vjbk) requires prior authorization.

2)    Mepsevii is used to treat mucopolysaccharidosis VII (MPS VII, Sly syndrome).
Medical Statement

Mepsevi (vestronidase alfa-vjbk) is considered medically necessary for members meeting the following criteria (must meet all):

1)    Diagnosis of mucopolysaccharidosis VII (Sly Syndrome) confirmed by one of the following:

a.    Two repeated enzyme assay tests demonstrating a deficiency of beta-glucuronidase;

b.    One DNA testing showing GUSB gene mutation.

2)    Apparent clinical signs of lysosomal storage disease, including at least one of the following:

a.    Enlarged liver and spleen;

b.    Joint limitations;

c.    Airway obstruction or pulmonary problems;

d.    Limitations of mobility;

3)    Prescribed by or in consultation with a specialist with expertise in lysosomal storage diseases (e.g. pediatric endocrinologist, pediatric geneticist);

4)    Dose does not exceed 4mg/kg IV every 2 weeks.

 

Initial approval duration is 6 months.

 

Reauthorization Criteria:

1)    Member has previously met initial approval criteria.

2)    Member is responding positively to therapy Some examples include improvement in:

a.    6-minute walking distance

b.    Breathing difficulties

c.    Muscle weakness

d.    Vision or hearing problems

e.    Hepatomegaly or splenomegaly

f.     Reduction of total urinary glycosaminoglycan (uGAG) excretion

g.    Stair climbing capacity as measured by the 3 Minute Stair Climb Test

3)    If request is for a dose increase, new dose does not exceed 4mg/kg IV every 2 weeks.

 

 

Codes Used In This BI:

 

J3397 – Injection, vestronidase alfa-vjbk, 1mg
Limits
Intentially left empty
Reference

1)    Mepsevii Prescribing Information. Noato, CA: Ultragenyx Pharmaceutical Inc.; November 2017.

2)    Clinical Pharmacology. Accessed online January 14, 2019.
Application to Products
This policy applies to all health plans administered by QualChoice, both those insured by QualChoice and those that are self-funded by the sponsoring employer, unless there is indication in this policy otherwise or a stated exclusion in your medical plan booklet. Consult the individual plan sponsor Summary Plan Description (SPD) for self-insured plans or the specific Evidence of Coverage (EOC) for those plans insured by QualChoice. In the event of a discrepancy between this policy and a self-insured customer’s SPD or the specific QualChoice EOC, the SPD or EOC, as applicable, will prevail. State and federal mandates will be followed as they apply.
Changes: QualChoice reserves the right to alter, amend, change or supplement benefit interpretations as needed.