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Galsulfase (Naglazyme)

Section: Injections
Effective Date: August 01, 2019
Revised Date: July 23, 2019

Description

Galsulfase (Naglazyme) is a human enzyme produced by recombinant DNA technology in a Chinese hamster ovary. Galsulfase (Naglazyme) is an orphan drug used to treat the inherited metabolic disorder mucopolysaccharidosis VI (MPS VI or Maroteaux-Lamy Syndrome). Mucopolysaccharidosis VI is caused by a lack of the enzyme arylsulfatase B that normally breaks down certain carbohydrates known as glycosaminoglycans. MPS VI causes widespread cumulative organ and tissue damage.

Criteria

Coverage is subject to the specific terms of the member's benefit plan. 

Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website. 

 

Food and Drug Administration (FDA) Indication

Galsulfase (Naglazyme) may be considered medically necessary for the treatment of mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome). 

The use of galsulfase (Naglazyme) for any other indication, is considered experimental/investigational and therefore, non-covered, due to lack of supporting published peer reviewed literature.

Procedure Code

J1458

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes

Covered Diagnosis Codes for Procedure Code J1458        

E76.29   E76.3

Links

I-140

  1. Brunelli MJ, Atallah ÁN, da Silva EM. Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI. Cochrane Database Syst Rev. 2016(3):CD009806. 
  2. Naglazyme [package insert]. Novato. CA: BioMarin; 2005. Revised 3/2013. 
  3. Giugliani R, Lampe C, Harmatz P, et al. Natural history and galsulfase treatment in mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome)--10-year follow-up of patients who previously participated in an MPS VI Survey Study. Am J Med Genet. Part A. August 2014;164A(8):1953-1964. 
  4. US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Galsulfase (Naglazyme®) Product approval information, approval letter 125117, 05/2005. 
  5. Harmatz PR, Garcia P, Guffon N, Randolph LM, Shediac R, et al. Galsulfase (Naglazyme®) therapy in infants with mucopolysaccharidosis VI. J Inherit Metab Dis. 2014;37:277–287. 
  6. Furujo M, Kosuga M, Okuyama T. Enzyme replacement therapy attenuates disease progression in two Japanese siblings with mucopolysaccharidosis type VI: 10-Year follow up. Mol Genet Metab Rep. 2017;13: 69–75. 
  7. Clinical Pharmacology Compendia. 2019 Tampa FL: Gold Standard, Inc. Galsulfase. 
  8. ASHP Guidelines on Home Infusion Pharmacy Services, 2013. 
  9. MCG™ Care Guidelines, 22nd edition, 2018, Home Infusion Therapy, CMT: CMT-0009(SR). 
  10. Polinski JM, Kowal MK, Gagnon M, et al. Home infusion: safe clinically effective, patient preferred, and cost saving. Healthcare. 2016.