Mepsevi (vestronidase alfa-vjbk) is considered medically necessary for members
meeting the following criteria (must meet all):
of mucopolysaccharidosis VII (Sly Syndrome) confirmed by one of the following:
repeated enzyme assay tests demonstrating a deficiency of beta-glucuronidase;
testing showing GUSB gene mutation.
clinical signs of lysosomal storage disease, including at least one of the
liver and spleen;
obstruction or pulmonary problems;
by or in consultation with a specialist with expertise in lysosomal storage
diseases (e.g. pediatric endocrinologist, pediatric geneticist);
not exceed 4mg/kg IV every 2 weeks.
Initial approval duration
is 6 months.
previously met initial approval criteria.
responding positively to therapy Some examples include improvement in:
Hepatomegaly or splenomegaly
of total urinary glycosaminoglycan (uGAG) excretion
climbing capacity as measured by the 3 Minute Stair Climb Test
is for a dose increase, new dose does not exceed 4mg/kg IV every 2 weeks.
Used In This BI:
Injection, vestronidase alfa-vjbk, 1mg