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Imiglucerase (Cerezyme®) is an analogue of the human enzyme beta-glucocerebrosidase produced by recombinant DNA technology using mammalian cell cultures. Velaglucerase (Vpriv™®) is produced by gene activation technology in a human fibroblast cell line. Imiglucerase (Cerezyme®), taliglucerase alfa (Elelyso™), and velaglucerase (Vpriv™®) catalyze the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide as part of the normal degradation pathway for membrane lipids. Gaucher disease is a chronic congenital disorder of lipid metabolism caused by a deficiency of the enzyme beta-glucocerebrosidase. Fatty substances called glycospingolipids accumulate in the reticuloendothelial cells (phagocytic cells). Accumulation in splenic macrophages and in the Kupffer cells of the liver is associated with enlargement of these organs. The resulting hypersplenism produces progressive anemia and thrombocytopenia. Accumulation of glucocerebrosidase in bone marrow is associated with osteopenia, lytic lesions, pathologic fractures, chronic bone pain, acute episodes of excruciating "bone crisis", bone infarcts, and osteonecrosis.
There are three clinical subtypes of the disease. Type I (non-neuropathic) includes 99% of the cases and is associated with enlarged liver and spleen, increased skin pigmentation, and painful bone lesions. It is estimated that there are about 1 in 200,000 Type I Gaucher patients with about 50% of those being symptomatic. This is approximately equal to 10,000 to 15,000 Type I Gaucher patients. Type II is characterized by neurologic symptoms including oculomotor apraxia, strabismus, and hypertonicity. These occur, usually in the first year of life, with death following within the first 18 months of life. Type III is similar to Type II but onset of symptoms is much later and the course is longer.
FDA APPROVED INDICATIONS
Imiglucerase (Cerezyme®) is indicated for long-term enzyme replacement therapy for pediatric and adult patients with a confirmed diagnosis of Type I Gaucher disease that results in one or more of the following conditions: anemia, thrombocytopenia, bone disease, hepatomegaly or splenomegaly.
Velaglucerase (Vpriv™®) is indicated for long-term enzyme replacement therapy for pediatric and adult patients with type 1 Gaucher disease.
Taliglucerase alfa (Elelyso™) is indicated for the long-term enzyme replacement therapy for adults with a confirmed diagnosis of Type 1 Gaucher disease.
Generic Name: Imiglucerase
Generic Name: Velaglucerase
Generic Name: Taliglucerase alfa
Prior authorization is required.
Imiglucerase (Cerezyme®) is considered medically necessary for patients with Type I Gaucher disease with one or more of the following conditions: moderate to severe anemia, thrombocytopenia with bleeding tendency, bone disease, significant hepatomegaly, or splenomegaly.
Velaglucerase (Vpriv®™) is considered medically necessary for pediatric and adult patients with type 1 Gaucher disease.
Taliglucerase alfa (Eleyso™) is considered medically necessary for adult patients with type 1 Gaucher disease.
Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Nervous/Mental Conditions, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:
A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and
B. appropriate with regard to standards of good medical practice; and
C. not solely for the convenience of the Member, his or her Provider; and
D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.
For the definition of Medically Necessary, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.
Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
11/1999: Approved by Pharmacy & Therapeutics Committee (P & T)
4/16/2001: Managed Care Requirements deleted
1/30/2002: Prior authorization added
4/18/2002: Type of Service and Place of Service deleted
11/6/2002: Nova Factor new telephone and fax numbers added. Telephone # 1-800-235-8498 and fax # 1-888-355-6652 deleted
10/14/2004: Code Reference section reviewed, no changes
09/06/2006: FDA Approved Indications and Policy sections revised. Accredo information updated. Ceredase® Prescribing Information (added 09-06-2006); Cerezyme® Prescribing Information (added 09-06-2006)
9/12/2006: Coding Updated. CPT/HCPCS 2006 revisions added to policy
8/17/2007: Policy reviewed, no changes
12/17/2007: Coding updated. CPT4/HCPCS 2008 revisions added to policy.
01/01/2009: Accredo preferred provider information removed. BCBSMS information added.
03/15/2010: Title changed from Alglucerase (Ceredase®) and Imiglucerase (Cerezyme®) to Glucocerebrosidase Enzyme Replenishers; FDA Approved Indications and Policy sections revised with addition of Velaglucerase (VPRIV™); Prescribing information for VPRIV™ added. Added HCPCS Code J3490 to Covered Codes Table for VPRIV™ and removed deleted HCPCS Code S0147.
02/28/2011: Added new HCPCS codes J3385 and J1786 to the Code Reference section.
01/02/2013: Policy description and policy statement updated to add coverage guidelines for Taliglucerase alfa (Eleyso™), which is considered medically necessary for adult patients with type 1 Gaucher disease. Removed J1785 from the Code Reference section as this code has been deleted. Added J3590 to the Code Reference section.
04/01/2014: Policy updated to remove Alglucerase (Ceredase®) from the policy description and the first medically necessary policy statement. Removed HCPCS code J0205 from the Code Reference section.
08/26/2015: Medical policy revised to add ICD-10 codes. Added HCPCS code J3060 to the Code Reference section.
02/02/2016: Approved by Pharmacy & Therapeutics (P&T) Committee. Policy guidelines updated to add medically necessary and investigative definitions.
Drug Information Handbook 1997-98
Facts and Comparisons, January 1999
Genzyme Information Specialist (demographics)
Velaglucerase (VPRIV®™) Prescribing Information
Taliglucerase alfa (Eleyso™) Prescribing Information
This may not be a comprehensive list of procedure codes applicable to this policy.
The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.