|Mountain State Medical Policy Bulletin|
|Topic:||Agalsidase beta (Fabrazyme®)|
|Effective Date:||August 1, 2005|
|Issued Date:||March 29, 2010|
|Date Last Reviewed:||09/2009|
General Policy Guidelines
Indications and Limitations of Coverage
Fabrazyme® (agalsidase beta) is indicated for use in patients with Fabry disease.
The recommended dosage of agalsidase beta is 1.0 mg/kg body weight infused every two weeks as an IV infusion.
The initial IV infusion rate should be no more than 0.25 mg/min (15 mg/hr). The infusion rate may be slowed in the event of infusion-associated reactions. After patient tolerance to the infusion is well established the infusion rate may be increased in increments of 0.05 to 0.08 mg/min (increments of 3 to 5 mg/hr) each subsequent infusion.
The use of agalsidase beta for any indication other than Fabry disease is considered experimental/investigational, and therefore, not covered. A participating, preferred, or network provider can bill the member for the non-covered service.
Coverage for agalsidase beta is determined according to individual or group customer benefits. Agalsidase beta is not reimbursable under the prescription drug benefit.
Fabry disease is a rare, inherited metabolic disorder caused by the deficiency of the essential enzyme alpha-galactosidase. Without this enzyme patients are unable to break down a naturally occurring fatty substance in their body called globotriaosylceramide (GL-3), which then accumulates in cells within the blood vessels of the heart, kidneys, brain and other vital organs. The progressive build-up of this substance puts patients at risk for stroke, heart attack, kidney damage and debilitating pain. Most patients develop kidney failure during adulthood, and severe organ complications typically lead to death around age 50.
Fabry disease is estimated to affect about 5,000 people worldwide. The disease is significantly more prevalent among men than women. It affects all ethnic groups.
Agalsidase beta reduces globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types.
Under the Federal Employee Program, all services that utilize FDA-approved drugs, devices, or biological products are eligible when intended for the treatment of a serious life-threatening condition and when medically necessary and appropriate for the patient’s condition.
Managed Care POS Guidelines
Fabrazyme® (agalsidase beta)[package insert]. Genzyme Corporation; Cambridge, MA, 01/2009.
Enzyme Replacement Therapy Improves Function of C-, Aδ-, and Aβ- Nerve Fibers in Fabry Neuropathy, Neurology, 2004, 62: 1066-1072
Banikazemi M, Bultas J, Waldek S, et al. Agalsidase - beta therapy for advanced Fabry disease. Ann Intern Med. 2007;146:77-86.
Wrath JE, Tylki-Szymanska A, Guffon N, et al. Safety and efficacy of enzyme replacement therapy with Agalsidase Beta: an international, open-label study in pediatric patients with Fabry disease. J Pediatr. 2008;152:563-70.
Breunig F, Wanner C. Update on Fabry disease: kidney involvement, renal progression and enzyme replacement therapy. J Nephrol. 2008;21:32-37.
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