Criteria
Coverage is subject to the specific terms of the member's benefit plan.
The use of agalsidase beta (Fabrazyme) may be considered medically necessary when
ALL
of the following criteria are met:
-
The individual is two (2) years of age or older;
and
-
The prescriber is, or in consult with, a metabolic specialist, geneticist, cardiologist, or specialist in Fabry disease;
and
-
The individual will not be concurrently treated with Galafold (migalastat);
and
-
The individual must have a diagnosis of Fabry disease with the one of the following (as evidenced with submitted documentation):
-
In males assigned at birth:
-
Deficiency of less than 35% of mean normal alpha-galactosidase A (α-Gal A) enzyme activity;
and
-
Diagnosis is confirmed to be caused by a pathologic galactosidase alpha gene (GLA);
or
-
In females assigned at birth and males with a α-Gal A enzyme activity greater than 35%:
-
Diagnosis must be confirmed to be caused by a pathologic galactosidase alpha gene (GLA);
and
-
Baseline value for plasma or urinary globotriosylceramide (GL-3) levels greater than or equal to (≥)5 ng/mcL or GL-3 inclusions greater than or equal to (≥) 0.3 per kidney interstitial capillary (KIC) as measured in kidney biopsy;
and
-
The individual is experiencing one of the following symptoms:
-
Acroparesthesia (burning pain in the extremities);
or
-
Angiokeratomas (cutaneous vascular lesions);
or
-
Hypo- or anhidrosis (diminished perspiration);
or
-
Corneal and lenticular opacities;
or
-
Left ventricular hypertrophy (LVH), hypertrophic cardiomyopathy, or arrythmia of unknown etiology;
or
- Chronic kidney disease (CKD), multiple renal cysts, and/or proteinuria of unknown etiology.
Initial Authorization: Six (6) months
Reauthorization Criteria
Continuation of therapy with agalsidase beta (Fabrazyme) may be considered medically necessary when the following is met:
-
The individual must have a decreased Gb3 level or Cb3 inclusion per KIC level and experienced and maintained improvement in one of the following symptoms since starting treatment with requested product, as evidenced by medical documentation (e.g., chart notes) attached to the request (subject to clinical review):
-
Acroparesthesia (burning pain in the extremities);
or
-
Angiokeratomas (cutaneous vascular lesions);
or
-
Hypo- or anhidrosis (diminished perspiration);
or
-
Corneal and lenticular opacities;
or
-
Left ventricular hypertrophy (LVH), hypertrophic cardiomyopathy, or arrythmia of unknown etiology;
or
- Chronic kidney disease (CKD), multiple renal cysts, and/or proteinuria of unknown etiology.
Continuation Authorization: 12 months
Agalsidase beta (Fabrazyme) for all other indications not listed in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness cannot be established by the available published peer-reviewed literature.
Procedure Code
