Pharmacy Prior Authorization Criteria

Nexviazyme (avalglucosidase alfa-ngpt)

Indications for Prior Authorization

Nexviazyme (avalglucosidase alfa-ngpt)

• For diagnosis of Pompe Disease
  Indicated for the treatment of patients 1 year of age and older with late-onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency).

Criteria

Nexviazyme

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)

• Diagnosis of late-onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency) as confirmed by one of the following: [2, 3]
• Absence or deficiency (less than 40% of the lab specific normal mean) of GAA enzyme activity in lymphocytes, fibroblasts, or muscle tissues as confirmed by an enzymatic assay
OR
• Molecular genetic testing confirms mutations in the GAA gene
AND
• Presence of clinical signs and symptoms of the disease (e.g., respiratory distress, skeletal muscle weakness, etc.) [A]
AND
• Patient is 1 year of age or older

Nexviazyme

Prior Authorization (Reauthorization)

Length of Approval: 24 Month(s)

• Patient demonstrates positive clinical response to therapy.

P & T Revisions

2024-09-02, 2024-01-15, 2023-10-26, 2023-10-03, 2022-09-07, 2022-02-03, 2021-11-04, 2021-10-05

References

1. Nexviazyme Prescribing Information. Genzyme Corporation. Cambridge, MA. September 2023.
2. Barba-Romero MA, Barrot E, Bautista-Lorite J, et al. Clinical guidelines for late-onset Pompe disease. Rev Neurol 2012; 54 (8): 497-507.
3. Kishnani PS, Steiner RD, Bali D, et al. Pompe disease diagnosis and management guideline. Genet Med. May 2006; 8(5): 267–288.

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End Notes

1. Consensus recommendation based on current clinical guidelines indicate that treatment should be started in patients when they become symptomatic and/or show signs of disease progression [2, 3].

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Revision History

• 2024-09-02: 2024 Annual Review. No criteria changes.
• 2024-01-15: Update guideline
• 2023-10-26: Program update to standard reauthorization language. No changes to clinical intent.
• 2023-10-03: Annual review: Updated reauthorization approval length to 24 months. Updated references.
• 2022-09-07: Annual review: No criteria changes.
• 2022-02-03: Added embedded step through Lumizyme.
• 2021-11-04: updated criteria
• 2021-10-05: New Program for Nexviazyme

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