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Idursulfase (Elaprase)

Section: Injections
Effective Date: June 01, 2019
Revised Date: May 31, 2019


Hunter syndrome (Mucopolysaccharidosis II, MPS II) is a rare, inherited disease which can lead to premature death. Hunter syndrome is a disease in which the person's body is defective in producing the chemical iduronate-2-sulfatase, which is needed to adequately break down complex sugars produced in the body. Hunter syndrome usually becomes apparent in children one to three years of age. Symptoms include growth delay, joint stiffness, and coarsening of facial features. In severe cases, patients experience respiratory and cardiac problems, enlargement of the liver and spleen, neurological deficits, and death.


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 website.

Idursulfase (Elaprase) may be considered medically necessary for use in individuals five (5) years old and older with Hunter syndrome (Mucopolysaccharidosis II, MPS II).

The use of idursulfase (Elaprase) for any indication other than Hunter syndrome is considered experimental/investigational, and therefore, not covered. The safety and efficacy for any other indication has not been established.

Procedure Codes


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 may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes




  1.  Jones S, Parini R, Harmatz P et al. The effect of idursulfase on growth in   patients with Hunter syndrome: Data from the Hunter Outcome Survey (HOS). Molecular Genetics and Metabolism. 2013;109(1):41-8. 
  2. Sohn Y, Cho S, Park S, et al. Phase I/II clinical trial of enzyme   replacement  therapy with idursulfase beta in patients with mucopolysaccharidosis II (Hunter Syndrome). Orphanet Journal of Rare Diseases. 2013;8:42. 
  3. Barbier A, Bielefeld B, Whiteman M, et al. The relationship between anti-idursulfase antibody status and safety and efficacy outcomes in attenuated mucopolysaccharidosis II patients aged 5 years and older treated with intravenous idursulfase. Molecular Genetics and Metabolism. 2013;110(3):303-10. 
  4. Żuber, Z, Świątkowska A, Jurecka A, et al. The Effect of Recombinant  Human Iduronate-2- Sulfatase (Idursulfase) on Growth in Young Patients with Mucopolysaccharidosis Type II. PLoS One. 2014; 9(1):e85074.
  5. Pano A, Barbier A, Bielefeld B, et al. Immunogenicity of idursulfase and clinical outcomes in very young patients (16 months to 7.5 years) with mucopolysaccharidosis II (Hunter syndrome). Orphanet Journal of Rare Diseases. 2015;10: 50.
  6. Muenzer J, Hendriksz C, Fan Z, et al. A phase I/II study of intrathecal idursulfase-IT in children with severe mucopolysaccharidosis II. Genetic in Medicine. 2015;36. 
  7. Tomanin R, Zanetti A, D’Avanzo F, et al. Clinical efficacy of Enzyme Replacement Therapy in paediatric Hunter patients, an independent study of 3.5 years. Orphanet Journal of Rare Diseases. 2014;9:129. 
  8. Giugliani R, Villarreal M, Valdez C, et al. Guidelines for diagnosis and treatment of Hunter Syndrome for clinicians in Latin America. Genetics and Molecular Biology. 2014;37(2): 315–329. 
  9. Giugliani R, Hwu W, Szymanska A, et al. A multicenter, open-label study evaluating safety and clinical outcomes in children (1.4–7.5 years) with Hunter syndrome receiving idursulfase enzyme replacement therapy. Genetics in Medicine. 2014;16(6):435–441. 
  10. ELAPRASE® (idursulfase) [package insert]. Shire Human Genetic Therapies, Inc. Lexington, MA. 2006, Revised 06/2013. 
  11. Sestito S, Ceravolo F, Grisolia M, et al. Profile of idursulfase for the treatment of Hunter syndrome. Research and Reports in Endocrine Disorders. 2015;5:79-90. 
  12. Guffon N, Heron B, Chabrol B, et al. Diagnosis, quality of life, and treatment of patients with Hunter syndrome in the French healthcare system: A retrospective observational study. Orphanet Journal of Rare Diseases. 2015;10:43. 
  13. Noh H, Lee JI. Current and potential therapeutic strategies for mucopolysaccharidoses. J Clin Pharm Ther. 2014;39(3):215-224. 
  14. Ceravolo F, Mascaro I, Sestito S, et al. Home treatment in paediatric patients with Hunter syndrome:the first Italian experience. Italian Journal of Pediatrics.2013;1-3. 
  15. Burton Bk and Whiteman AH. Incidence and timing of infusion-related reactions in patients with mucopolysaccharidosis type II (Hunter syndrome) on idursulfase therapy in the real-world setting: A perspective from the Hunter Outcome Survey (HOS). Mol Gen and Met. 2011;113-120.
  16. Polinski JM, Kowal MK, Gagnon M, et al. Home infusion: safe clinically effective, patient preferred, and cost saving. Healthcare. 2016. 
  17. ASHP Guidelines on Home Infusion Pharmacy Services, 2013. Accessed September 2, 2017. 
  18. MCG™ Care Guidelines, 22nd edition, 2018, Home Infusion Therapy, CMT: CMT-0009(SR). 
  19. MICROMEDEX®SOLUTIONS Compendia. 2017. Idursulfase. 
  20. Clinical Pharmacology Compendia. [database online]. Tampa FL: Gold Standard, Inc. Idursulfase.