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Avalglucosidase Alfa-ngpt (Nexviazyme™)
Act
by Nov 1, 2026
Effective date
Dec 1, 2026
Identified
Sep 10, 2026
Summary
BlueCross BlueShield of Tennessee is establishing a new coverage policy for Avalglucosidase Alfa-ngpt (Nexviazyme™) for treatment of late-onset Pompe disease in patients 1 year and older. The policy requires prior authorization with specific documentation including enzyme assay/genetic testing confirmation and clinical symptom documentation. Treatment must be prescribed by metabolic disease or lysosomal storage disorder specialists, with 12-month authorization periods for both initial and continuation therapy.
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What to do
by Nov 1, 2026 · 7 steps
01Billing team must implement prior authorization workflow for Nexviazyme™ claims.
021) Update billing system to flag all Nexviazyme™ claims for mandatory prior auth review before submission.
032) Create prior auth checklist requiring: (a) enzyme assay or genetic testing documentation confirming GAA deficiency <40% of normal, (b) baseline clinical documentation of disease symptoms (proximal muscle weakness, respiratory insufficiency, or cardiomyopathy), and (c) prescriber specialty verification (metabolic disease or lysosomal storage disorder specialist).
043) For continuation therapy, ensure prior auth requests include documentation of positive response (improvement, stabilization, or slowing disease progression).
054) Train billing and authorization staff on 12-month authorization periods for both initial and reauthorization requests.
065) Update provider communication materials to notify metabolic specialists and neurology departments of new requirement.
07Claims submitted without prior authorization or incomplete documentation will be denied effective 12/1/26.
Original notice
Avalglucosidase Alfa-ngpt (Nexviazyme™)
bcbst.com · Always check the source before changing claims.