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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

Alglucerase (Ceredase®) and Imiglucerase (Cerezyme®) are indicated for long-term enzyme replacement therapy for patients with a confirmed diagnosis of Type I Gaucher disease that results in one or more of the following conditions: moderate to severe anemia, thrombocytopenia with bleeding tendency, bone disease, significant 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.

IDENTIFICATION

Generic Name:  Alglucerase
Brand Name: Ceredase®

Generic Name: Imiglucerase
Brand Name: Cerezyme®

Generic Name: Velaglucerase
Brand Name: Vpriv™

Generic Name: Taliglucerase alfa
Brand Name: Elelyso™

Alglucerase (Ceredase®) and imiglucerase (Cerezyme®) are 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.

None

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.

Facts and Comparisons, January 1999

Genzyme Information Specialist (demographics)

Velaglucerase (VPRIV™) Prescribing Information

Taliglucerase alfa (Eleyso™) Prescribing Information

The code(s) listed below are ONLY covered if the procedure is performed according to the "Policy" section of this document. 

Covered Codes