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Aetna Medicare
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Pharmacy Clinical Policy Bulletins
Aetna Medicare Prescription Drug Plan
Subject: Metabolic Modifiers

Status Drug PR-B/D PR PR-QL PR-AL ST M EX‡ TOC§
C Aldurazyme®  (laronidase)              
C Ammonul®  (sodium benzoate & sodium phenylacetate)              
C Cystadane®  (betaine)              
C Fabrazyme®  (agalsidase beta)              
C Hectorol®  (doxercalciferol)              
C levocarnitine              
C Naglazyme®  (galsulfase)              
NC Buphenyl®  (sodium phenylbutyrate)           X  
NC Carnitor®  (levocarnitine)           X  
NC Orfadin®  (nitisinone)   X       X  


Policy:

  1. Precertification Criteria

    2. Under some plans, including plans that use an open or closed formulary, Orfadin is subject to precertification.  If precertification requirements apply Aetna considers Orfadin to be medically necessary for those members who meet the following precertification criterion:

    A. Member has documented diagnosis of hereditary tyrosinemia type 1 (HT-1)


  2. Medical Exception Criteria

    3. Buphenyl, Carnitor, and Orfadin are currently Not Covered Part D drugs under the Aetna Medicare Prescription Drug Plan.* Therefore, they are excluded from coverage for members enrolled in prescription drug benefits plans that use a closed formulary, unless a medical exception is granted.  Aetna considers Buphenyl, Carnitor, and Orfadin to be medically necessary for those members who meet the criteria specified below:

    For Buphenyl
    A. Member has a documented diagnosis of neonatal-onset or late-onset urea cycle disorders involving deficiencies of carbamylphosphate synthetase, argininosuccinic acid synthetase, or ornithine transcarbamylase.

    For Carnitor

    A.  A documented:

    • Contraindication to the preferred alternative agent - levocarnitine -  indicated for the member's condition OR
    • Intolerance to the preferred alternative agent - levocarnitine -  indicated for the member's condition OR
    • Allergy to the preferred alternative agent - levocarnitine -  indicated for the member's condition OR
    • Failure of an adequate trial of one month of the preferred alternative agent - levocarnitine - indicated for the member's condition

    For Orfadin

    A.  Member has documented diagnosis of hereditary tyrosinemia type 1 (HT-1)



* Coverage is provided through a Medicare Prescription Drug Plan Sponsor with a Medicare contract and benefits, limitations, service areas and premiums are subject to change on January 1 of each year.

Place of Service:

Outpatient

The above policy is based on the following references:
  1. Olin BR, editor. Drugs Facts and Comparisons (electronic online version). St. Louis: J.B. Lippincott Company, 2006.
  2. USPDI Drug Information for the HealthCare Professional (online through Stat!Ref). Thomson MICROMEDEX, Greenwood Village, Colorado; 2006.
  3. McEvoy GK, editor. AHFS Drug Information (online through Stat!Ref). American Society of Health-Systems Pharmacists, Bethesda, Maryland; 2006.
  4. Medical Economics, Inc., PDR Electronic Library. Thomson Medical Economics, Montvale, NJ; 2003.
  5. Fick DM, Cooper JW, Wade WE, et al.  Updating the Beers criteria for potentially inappropriate medication use in older adults.  Arch Intern Med. 2003;163:2716-24.
  6. Zahn C, Sangl J, Bierman AS, et al.  Potentially inappropriate medication use in the community-dwelling elderly.  JAMA.  2001;286:2823-29.
  7. Klasco RK (Ed): DRUGDEX® System (electronic version). Thomson Micromedex, Greenwood Village, Colorado, USA. Available at: http://www.thomsonhc.com (cited: November 21, 2005).
  8. Kakkis ED, Muenzer J, Tiller GE, et al: Enzyme-replacement therapy in mucopolysaccharidosis I. N Engl J Med 2001; 344:182-188.
  9. Product Information: Ammonul®, sodium phenylacetate and sodium benzoate. Ucyclyd Pharma, Inc., Scottsdale, AZ, 2005.
  10. Tuchman M, Knopman DS, & Shih VE: Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Arch Neurol 1990b; 47:1134-1137.
  11. Product Information: Buphenyl(R), sodium phenylbutyrate. Ucyclyd Pharma, Hunt Valley, MD, 1996.
  12. Hurot JM, Cucherat M, Haugh M, et al: Effects of L-carnitine supplementation in maintenance hemodialysis patients: a systematic review. J Am Soc Nephrol 2002; 13(3):708-714.
  13. Ghidini O, Azzurro M, Vita G, et al: Evaluation of the therapeutic efficacy of L-carnitine in congestive heart failure. Int J Clin Pharmacol Ther Toxicol 1988; 26(4):217-220.
  14. Moretti S, Alesse E, Di Marzio L, et al: Effect of L-carnitine on human immunodeficiency virus-1 infection-associated apoptosis: a pilot study. Blood 1998; 91(10):3817-3824.
  15. Cecere A, Ciaramella F, Tancredi L, et al: Efficacy of L-carnitine in reducing hyperammonaemia and improving neuropsychological test performance in patients with hepatic cirrhosis: results of a randomised trial. Clin Drug Invest 2002; 22(Suppl 1):7-14.
  16. Matsumoto Y, Sato M, Ohashi H, et al: Effects of L-carnitine supplementation on cardiac morbidity in hemodialyzed patients. Am J Nephrol 2000; 20(3):201-207.
  17. Sloan RS, Kastan B, Rice SI, et al: Quality of life during and between hemodialysis treatments: role of L-carnitine supplementation. Am J Kidney Dis 1998; 32(2):265-272.
  18. Elisaf M, Bairaktari E, Katopodis K, et al: Effect of L-carnitine supplementation on lipid parameters in hemodialysis patients. Am J Nephrol 1998; 18(5):416-421.
  19. Dudman NPB, Guo X-W, Gordon RB et al: Human homocysteine catabolism: three major pathways and their relevance to development of arterial occlusive disease. J Nutrition 1996 (Suppl); 126:1295S-1300S, 1996.
  20. Abdelmalek MF, Angulo P, Jorgensen RA, et al: Betaine, a promising new agent for patients with nonalcoholic steatohepatitis: results of a pilot study. Am J Gastroenterol 2001; 96:2711-2717.
  21. Miglio F, Rovati LC, Santoro A, et al: Efficacy and safety of oral betaine glucuronate in non-alcoholic steatohepatitis. Arzneimmittelforschung Drug Res 2000; 50(8):722-727.
  22. Hurot JM, Cucherat M, Haugh M, et al: Effects of L-carnitine supplementation in maintenance hemodialysis patients: a systematic review. J Am Soc Nephrol 2002; 13(3):708-714.
  23. Ghidini O, Azzurro M, Vita G, et al: Evaluation of the therapeutic efficacy of L-carnitine in congestive heart failure. Int J Clin Pharmacol Ther Toxicol 1988; 26(4):217-220.
  24. Cecere A, Ciaramella F, Tancredi L, et al: Efficacy of L-carnitine in reducing hyperammonaemia and improving neuropsychological test performance in patients with hepatic cirrhosis: results of a randomised trial. Clin Drug Invest 2002; 22(Suppl 1):7-14.
  25. Matsumoto Y, Sato M, Ohashi H, et al: Effects of L-carnitine supplementation on cardiac morbidity in hemodialyzed patients. Am J Nephrol 2000; 20(3):201-207.
  26. Moe SM, Sprague SM, Adler S, et al: Two-year treatment with the calcimimetic AMG 073 in hemodialysis patients with secondary hyperparathyroidism (SHPT) (abstract SU-P0508). J Am Soc Nephrol 2002; 13:572A.
  27. Lindberg JS, Moe SM, Goodman WG, et al: The calcimimetic AMG 073 reduces parathyroid hormone and calcium x phosphorus in secondary hyperparathyroidism. Kidney Int 2003; 63:248-254.
  28. Quarles LD, Sherrard DJ, Adler S, et al: The calcimimetic AMG 073 as a potential treatment for secondary hyperparathyroidism of end-stage renal disease. J Am Soc Nephrol 2003; 14:575-583.
  29. Quarles LD, Spiegel DM, Curzi M, et al: The effects of one-year treatment with the calcimimetic AMG 073 on bone health in ESRD patients with secondary hyperparathyroidism (SHPT) (abstract SU-P0510). J Am Soc Nephrol 2002; 13:572A.
  30. Sorbera LA, Castaner RM, & Bayes M: Cinacalcet hydrochloride. Drugs Future 2002; 27(9):831-836.
  31. Product Information: Sensipar™, cinacalcet. Amgen, Thousand Oaks, CA, 2004.
  32. Block GA, Martin KJ, de Francisco ALM, et al: Cinacalcet for secondary hyperparathyroidism in patients receiving hemodialysis. N Engl J Med 2004; 350(15):1516-1525.
  33. Shoback DM, Bilezikian JP, Turner SA, et al: The calcimimetic cinacalcet normalizes serum calcium in subjects with primary hyperparathyroidism. J Clin Endocrinol Metab 2003; 88:5644-5649.
  34. Ros J, Vilaseca MA, Lambruschini N, et al: NTBC as palliative treatment in chronic tyrosinaemia type I. J Inher Metab Dis 1999; 22:665-666.
  35. Holme E & Lindstedt S: Nontransplant treatment of tyrosinemia. Clin Liver Dis 2000; 4(4):805-814.
  36. Product Information: Orfadin(R), nitisinone. Rare Disease Therapeutics Inc., Nashville, TN, 2002.
  37. Shuja SB & Raja RM: Severe hyperparathyroidism despite paricalcitol therapy: one-year follow-up. Adv Perit Dial 2003; 19:231-235.
  38. Pai AB, Lin S, Arruda JAL, et al: Long-term therapy with paricalcitol for secondary hyperparathyroidism in hemodialysis patients. Int J Artif Organs 2003; 26(6):484-490.
  39. Lindberg JS, Moore J, Martin KJ, et al: 19-nor safely and efectively reduces iPTH levels in hemodialysis patients (abstract), Annual Meeting of the Am Soc Nephrol, San Antonio, TX, 1997.
  40. Product Information: ZEMPLAR(R) Injection , paricalcitol. Abbott Laboratories, North Chicago, IL, 2005.
  41. Teng M, Wolf M, Lowrie E, et al: Survival of patients undergoing hemodialysis with paricalcitol or calcitriol therapy. N Engl J Med 2003; 349(5):446-456.
  42. Sprague SM, Llach F, Amdahl M, et al: Paricalcitol versus calcitriol in the treatment of secondary hyperparathyroidism. Kidney Internat 2003; 63:1483-1490.
  43. Clayman GL, Gonzalez HE, El-Naggar A, et al.: Parathyroid carcinoma: evaluation and interdisciplinary management. Cancer 100 (5): 900-5, 2004. 
  44. Strewler GJ: Medical approaches to primary hyperparathyroidism. Endocrinol Metab Clin North Am 29 (3): 523-39, vi, 2000.
  45. Collins MT, Skarulis MC, Bilezikian JP, et al.: Treatment of hypercalcemia secondary to parathyroid carcinoma with a novel calcimimetic agent. J Clin Endocrinol Metab 83 (4): 1083-8, 1998.
Property of Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

August 9, 2006
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