58468-4663-XX Cerezyme 400 UNIT SOLR (GENZYME)
Imiglucerase is a recombinant analog of the human enzyme β-glucocerebrosidase, a lysosomal glycoprotein enzyme, which normally catalyzes the hydrolysis of glucocerebroside to glucose and ceramide. Mutations of the gene encoding β-glucocerebrosidase prevent this breakdown and lead to accumulation of massive amounts of glucocerebrosides in cells of the macrophage-monocyte system. This condition is known as Gaucher Disease, the most common lysosomal storage disease.
Three types of Gaucher Disease are delineated by absence or presence and progression of neurologic involvement. Type I disease accounts for 99% of cases and is known as the chronic non-neuronopathic form. Its glucocerebrosidase storage is limited to phagocytes throughout the body although are seen primarily in the spleen and bone marrow. Type II or acute neuronopathic Gaucher Disease, is the infantile acute cerebral pattern of the disease dominated by progressive central nervous system disease. Type III disease combines types I and II, with systemic glucocerebrosidase storage throughout the body as well as progressive central nervous system disease. Of the three types, only Type I disease is treatable through recombinant enzyme replacement therapy.
Imiglucerase for the treatment of Gaucher Disease is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Imiglucerase for the treatment of other conditions/diseases is considered investigational.
Imiglucerase for the treatment of Gaucher Disease is considered medically appropriate if ALL the following criteria are met:
Diagnosis is confirmed by a beta-glucosidase leukocyte (BGL) enzyme test with significantly reduced or absent enzyme activity
Disease is classified as Type I (non-neuronopathic)
Individual is ANY ONE of the following
Pediatric (at least 2 years of age or older)
Adult (eighteen years of age or older) with symptoms of ANY ONE of the following:
Moderate-to-severe anemia [hemoglobin less than or equal to 11 g/dL (women) or 12 g/dL (men)]
Thrombocytopenia with bleeding tendency (platelet count less than or equal to 120,000/mm3)
Skeletal disease (e.g., lesions, remodeling defects and/or deformity of long bones, osteopenia/osteoporosis, etc.)
Moderate to severe hepatomegaly (liver size 1.25 or more times normal volume) OR Moderate to severe splenomegaly (spleen size 5 or more times normal volume)
Symptomatic disease (e.g. bone pain, fatigue, dyspnea, angina, abdominal distension, diminished quality of life, etc.)
Must be used as a single agent
Imiglucerase is considered medically appropriate for renewal if ALL of the following criteria are met:
Individual continues to meet initial approval criteria
Disease response as indicated by one or more of the following as compared to pretreatment baseline:
Improvement in symptoms (e.g. bone pain, fatigue, dyspnea, angina, abdominal distension, diminished quality of life, etc.)
Reduction in size of liver or spleen
Improvement in hemoglobin/anemia
Improvement in skeletal disease
Improvement in platelet counts
Absence of unacceptable toxicity from the drug including hypersensitivity reactions
|INDICATION(S)||DOSAGE & ADMINISTRATION|
|Type 1 Gaucher Disease||
Initial dosages range from 2.5 U/kg of body weight 3 times a week to 60 U/kg once every 2 weeks based on disease severity. Cerezyme is administered by intravenous infusion over 1–2 hours.Dosage adjustments should be made on an individual basis and may increase or decrease, based on achievement of therapeutic goals as assessed by routine comprehensive evaluations of the patient’s clinical manifestations.
LENGTH OF AUTHORIZATION
Coverage will be provided for 12 months and may be renewed
Refer to DOSAGE LIMITS below
APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS
BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.
For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).
Lexi-Comp, Inc. (2018). AHFS DI. Imiglucerase. Retrieved August 31, 2018 from Lexi-Comp Online with AHFS.
Mayo Medical Laboratories Test Catalog. (2018). Test ID: BGL Beta-glucosidase, leukocytes. Retrieved September 4, 2018 from https://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/8788.
MICROMEDEX Healthcare Series. Drugdex Drug Evaluations. (2018, May). Imiglucerase. Retrieved August 31, 2018 from MICROMEDEX Healthcare Series.
U. S. Food and Drug Administration. (2005, March). Center for Drug Evaluation and Research. Label and Approval History. Cerezyme® (imiglucerase for injection). Retrieved August 31, 2018 from https://www.accessdata.fda.gov/drugsatfda_docs/label/2005/20367s066lbl.pdf.
ORIGINAL EFFECTIVE DATE: 8/1/2004
MOST RECENT REVIEW DATE: 10/9/2018
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
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Maximum billable units per dose and over time by indication as a Medical Benefit