Prior Auth Protocol

FDA Approved Indications:

For patients with Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I (MPS I) and for patients with Scheie form who have moderate to severe symptoms. The risks and benefits of treating mildly affected patients with the Scheie form have not been established. Aldurazyme has been shown to improve pulmonary function and walking capacity. Aldurazyme has not been evaluated for effects on the central nervous system manifestations of the disorder.

Health Net Approved Indications and Usage Guidelines:

Patient with MPS IH (Hurler disease), MPS IH/S (Hurler-Scheie disease), or moderate to severe MPS IS (Scheie disease) with enzymatic and/or mutational evidence of alpha-iduronidase deficiency

AND

OR

Coverage is Not Authorized For:

Non-FDA approved indications, which are not listed in the Health Net Approved Indications and usage guidelines section unless there is sufficient documentation of efficacy and safety in the published literature.

General Information:

Aldurazyme has been shown to improve pulmonary function and walking capacity in patients 6 years and older, a median difference of 2% in forced vital capacity (FVC) and 39 meters in 6 minute walk time (6MWT). In patients under 6 years of age, a decrease in gylcosaminoglycan (GAG) levels was maintained throughout 52 weeks.
The benefit of Aldurazyme in other forms of MPS such as MPS II (Hunter's syndrome), MPS IIIA, IIIB, IIIC, and IIID (the Sanfilippo syndromes), MPS IV (Morquio's syndrome), and MPS VI (Maroteaux-Lamy disease) is unknown.
The benefit of Aldurazyme for the treatment of other symptoms of MPS-I such as coarse faces, corneal clouding, organomegaly, bony abnormalities, hernias, short stature, mental retardation, valvular heart disease, increased intracranial pressure, and hearing loss is unknown.

Therapeutic Alternatives:

Drug	Dosing Regimen	Dose Limit/ Maximum Dose
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* Requires Prior Authorization

Recommended Dosing Regimen and Authorization Limit:

Drug	Dosing Regimen	Authorization Limit
Aldurazyme	0.58 mg/kg IV infusion once weekly	Medical: 6 months or renewal date, whichever is longer For continued authorization, documentation of clinical response (2-3% increase in % predicted FVC, and/or >30 meter increase in 6MWT is required and/or decrease in GAG from baseline.)

Product Availability:

Vial: 2.9 mg/5 ml

References:

Aldurazyme [Prescribing Information] Novato, CA: Biomarin Pharmaceutical Inc; April 2013.
Wraith JE, Clarke LA, Beck M, et al. Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human alpha-L-iduronidase (laronidase). J Peds. 2004;144(5):581-8.
Aldurazyme. In: DrugPoints^R System [Internet database]. Greenwood Village, Colo: Thomson Healthcare. Updated periodically. Accessed June 1, 2015.
Aldurazyme. American Hospital Formulary Service Drug Information. Available at: http://www.medicinescomplete.com/mc/ahfs/current/. Accessed June 1, 2015.
Clinical Pharmacology Web site. Available at: http://clinicalpharmacology-ip.com/. Accessed June 1, 2015.