Skip to main content
HealthPartners

Coverage criteria policies

Pompe Disease Enzyme Replacement Therapy: Alglucosidase alfa (Lumizyme®)

These services may or may not be covered by your HealthPartners plan. Please see your plan documents for your specific coverage information. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

Administrative Process

Treatment with alglucosidase alfa requires prior authorization from Pharmacy Administration. The setting of drug administration will be reviewed as part of the prior authorization.

Note:

For all inquiries, transfer to the Medical Injectable Line (ext 26135).

Coverage

Enzyme replacement for Pompe disease is generally covered when:

    1. Prescribed by a specialist; and

    2. Prescribed for patients with Pompe disease (GAA deficiency). The approved regimen is 20 mg/kg of body weight administered every 2 weeks as an intravenous infusion. A current weight is required; and

    3. The patient and/or guardian has attested that they will adhere to the treatment plan; and

    4. For commercial products only (does not apply to Medicare or Minnesota Health Care Programs products), medication administration must occur at a clinic office or home-infusion setting unless medical necessity is met based on the criteria below, supported by medical documentation:

    1. The patient has experienced a severe or life-threatening reaction with previous infusions of the same or similar products; or,
    2. The patient has a medical condition that renders him or her unstable, exceptionally complex, immunocompromised or otherwise high-risk such that continued oversight in the current facility is required; or,
    3. There are no alternative settings available to the patient as a result of both of the following:
      1. The patient is unable to use home-infusion services as documented by the physician, social worker, or infusion provider; AND,
      2. The patient is unable to access alternative settings due to unreasonable distance [>30 miles] or other extenuating circumstances.

Initial approvals will be for twelve months.

Annual reauthorizations will require medical chart documentation that the patient has been seen within the past 12 months and that markers of disease are improved by therapy.

Definitions

Pompe disease is a progressive, multisystemic, debilitating, and often fatal neuromuscular disorder. It results from an inherited deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA) (also called acid maltase), which is responsible for the breakdown of glycogen to glucose. The result is intralysosomal accumulation of glycogen, primary in muscle cells, that leads to a progressive loss of muscle function.

Pompe disease is also referred to as:

  • Acid maltase deficiency (AMD)
  • Glycogen storage disease (GSD) type II
  • Glycogenosis type II
  • Acid alpha-glucosidase (GAA) deficiency
  • Lysosomal alpha-glucosidase deficiency

Lumizyme is a hydrolytic lysosomal glycogen-specific enzyme indicated for patients with Pompe disease (GAA deficiency).

Setting: The type of physical site where the drug is provided. Settings include inpatient hospital, outpatient hospital, clinic office, or home-infusion.

  • Outpatient Hospital sites have physicians and practitioners on-site and are the appropriate site to manage unstable patients and patients experiencing certain moderate to severe adverse events. Hospital settings are typically the highest-cost, most-intensive, and are the highest level settings.
  • Clinic offices are lower level settings which are not outpatient hospital settings that can manage some unstable patients and patients experiencing adverse events. Physicians may or may not be readily available.
  • Home-infusion is a lower level setting, and is performed by a licensed nurse supported by a licensed pharmacy who have expertise in administering complex medications in a patient’s home. Home infusion providers regularly manage mild to moderate adverse events, and are prepared to manage severe adverse events if needed.

If available, codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.

HCPCS

Codes

Description

J0221

Injection, alglucosidase alfa, (Lumizyme), 10 mg

NDC

Codes

Description

58468016001

Lumizyme single use 50 mg solution

58468016002

Lumizyme 10 vial package of 50mg

CPT Copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

Products

This information is for most, but not all, HealthPartners plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.

References

  1. Lumizyme Prescribing Information Genzyme Corporation, Inc., Cambridge, MA. August 2014.
  2. van der Ploeg AT, Clemens PR, Corzo D, et al., A randomized study of alglusidase alfa in late-onset Pompe’s disease. N Eng J Med 2010;362:1396-406.

Go to

Policy activity

  • 05/01/2007 - Date of origin
  • 01/01/2017 - Effective date
Review date
  • 11/2016
Revision date
  • 10/01/2016

Related content