Notification



Notification Issue Date:



Medical Policy Bulletin


Title:Pegademase bovine (Adagen®)

Policy #:08.01.26

This policy is applicable to the Company’s commercial products only. Policies that are applicable to the Company’s Medicare Advantage products are accessible via a separate Medicare Advantage policy database.


The Company makes decisions on coverage based on Policy Bulletins, benefit plan documents, and the member’s medical history and condition. Benefits may vary based on contract, and individual member benefits must be verified. The Company determines medical necessity only if the benefit exists and no contract exclusions are applicable.

When services can be administered in various settings, the Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.

This Medical Policy Bulletin document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy Bulletin will be reviewed regularly and be updated as scientific and medical literature becomes available. For more information on how Medical Policy Bulletins are developed, go to the About This Site section of this Medical Policy Web site.



Policy

Coverage is subject to the terms, conditions, and limitations of the member's contract.

MEDICALLY NECESSARY

Pegademase bovine (Adagen®) is considered medically necessary and, therefore, covered for individuals with adenosine deaminase deficiency with severe combined immunodeficiency (SCID) who are not candidates for, or who have failed, bone marrow transplantation.

EXPERIMENTAL/INVESTIGATIONAL

All other uses of pegademase bovine (Adagen®) are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the Company medical policy on off-label coverage for prescription drugs and biologics. Uses of pegademase bovine (Adagen®) that are not supported in either this medical policy or the off-label coverage policy are considered experimental/investigational and, therefore, not covered.

REQUIRED DOCUMENTATION

The individual's medical record must reflect the medical necessity for the care provided. These medical records may include, but are not limited to: records from the professional provider's office, hospital, nursing home, home health agencies, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider. All documentation is to be available to the Company upon request. Failure to produce the requested information may result in a denial for the drug.
Guidelines

In accordance with the FDA-approved prescribing information, pegademase bovine (Adagen®) is recommended for use in infants from birth or in children of any age at the time of diagnosis. Pegademase bovine (Adagen®) is not intended as a replacement for human leukocyte antigen (HLA) identical bone marrow transplant therapy. Pegademase bovine (Adagen®) is also not intended to replace continued close medical supervision and the initiation of appropriate diagnostic tests and therapy as indicated for intercurrent illnesses.

BENEFIT APPLICATION

Subject to the terms and conditions of the applicable benefit contract, pegademase bovine (Adagen®) is covered under the medical benefits of the Company’s products when the medical necessity criteria listed in this medical policy are met.

US FOOD AND DRUG ADMINISTRATION (FDA) STATUS

Pegademase bovine (Adagen®) was approved by the FDA on March 21, 1990 for for enzyme replacement therapy for adenosine deaminase (ADA) deficiency in individuals with severe combined immunodeficiency (SCID) disease who are not suitable candidates for, or who have failed, bone marrow transplantation.

Description

Severe combined immunodeficiency (SCID) is an inherited heterogeneous group of disorders that affects the development and function of T-cells and B-cells. The classic symptoms of SCID are recurrent severe infections, failure to thrive, and chronic diarrhea. SCID is usually fatal within the first year of life if the underlying defect is not corrected.

Pegademase bovine (Adagen®) was approved by the US Food and Drug Administration (FDA) as an orphan drug (a drug used to treat, prevent, or diagnose a rare disease) for enzyme replacement therapy for adenosine deaminase (ADA) deficiency in individuals with SCID who are not suitable candidates for, or who have failed, bone marrow transplantation. Adenosine deaminase deficiency is an autosomal recessive type of SCID. It accounts for approximately 15 percent of SCID cases. In lymphocytes, adenosine deaminase is responsible for breaking down deoxyadenosine, a harmful molecule generated from the breakdown of DNA. A deficiency in adenosine deaminase leads to a buildup of deoxyadenosine, killing the lymphocytes. Immature lymphocytes in the thymus are especially vulnerable to the toxic buildup and die before reaching maturity, preventing them from helping to fight infections.

There may be additional indications contained in the Policy section of this document due to evaluation of criteria highlighted in the Company’s off-label policy, and/or review of clinical guidelines issued by leading professional organizations and government entities.
References


Hoffman R, Benz E, Silberstein L. Congenital disorders of lymphocyte function. In:Pai S, Notarangelo L,eds. Hematology: Basic Principles and Practices. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013.

Micromedex® Healthcare Series [Internet database]. DRUGDEX® Evaluations. Pegademase bovine (Adagen®). Greenwood Village, CO: Thomson Micromedex. 09/25/2015. Available at:
http://www.micromedexsolutions.com/micromedex2/librarian. Accessed October 4, 2016.

Pegademase bovine (Adagen®). Sigma Tau Pharmaceuticals, Inc, Gaithersburg, MD.
06/2015. Available at: http://www.adagen.com/pdf/AdagenPI_Jun2014.pdf . Accessed October 4, 2016.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Drug details: Pegademase bovine (Adagen®). [FDA Web site]. 06/05/2014. Available at: http://www.accessdata.fda.gov/drugsatfda_docs/label/2014/019818s053lbl.pdf. Accessed October 4, 2016.

US National Library of Medicine. Adenosine deaminase deficiency. Genetics Home Reference. http://ghr.nlm.nih.gov/condition/adenosine-deaminase-deficiency. October 5, 2015. Accessed October 4, 2016.





Coding

Inclusion of a code in this table does not imply reimbursement. Eligibility, benefits, limitations, exclusions, precertification/referral requirements, provider contracts, and Company policies apply.

The codes listed below are updated on a regular basis, in accordance with nationally accepted coding guidelines. Therefore, this policy applies to any and all future applicable coding changes, revisions, or updates.

In order to ensure optimal reimbursement, all health care services, devices, and pharmaceuticals should be reported using the billing codes and modifiers that most accurately represent the services rendered, unless otherwise directed by the Company.

The Coding Table lists any CPT, ICD-9, ICD-10, and HCPCS billing codes related only to the specific policy in which they appear.

CPT Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD - 10 Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD -10 Diagnosis Code Number(s)

D81.3 Adenosine deaminase [ADA] deficiency


HCPCS Level II Code Number(s)

J2504 Injection, pegademase bovine, 25 IU


Revenue Code Number(s)

N/A

Coding and Billing Requirements


Cross References


Policy History

Revisions from 08.01.26
06/06/2018This policy has been reissued in accordance with the Company's annual review process.


Effective 10/05/2017 this policy has been updated to the new policy template format.


Version Effective Date: 01/01/2016
Version Issued Date: 12/31/2015
Version Reissued Date: 06/15/2018

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