Currentblue cross blue shield - louisianaPolicy # 00771

Pharmacotherapy for Pompe Disease

Defines coverage eligibility, medical necessity, initial and continuation authorization criteria, dosing limits, and exclusions for alglucosidase alfa (Lumizyme), avalglucosidase alfa-ngpt (Nexviazyme), cipaglucosidase alfa-atga (Pombiliti) and miglustat (Opfolda) for treatment of Pompe disease for members of Blue Cross and Blue Shield of Louisiana.

Policy Summary

Payerblue cross blue shield - louisiana

PolicyPharmacotherapy for Pompe Disease

Policy CodePolicy Policy # 00771

Change TypeCoverage additions and criteria updates (added Pombiliti/Opfolda; updated Nexviazyme; added Lumizyme; coding changes)

Effective DateMar 1, 2026

Next Review DateDec 1, 2026

Key ActionObtain prior authorization and submit required diagnostic documentation (GAA enzyme deficiency or 2 GAA gene mutations, baseline FVC and 6MWT when appropriate) and attest to monitoring/contraindication requirements per the drug-specific criteria.

SourceLink

POLICY UPDATE CHANGES

Added Pombiliti and Opfolda with relevant criteria, background information, and rationale.

Updated Nexviazyme criteria to include Pombiliti as an additional ERT option.

Removed Nexviazyme criterion requiring that the patient has not failed therapy with Lumizyme.

Added coverage criteria for Opfolda for the diagnosis of Niemann-Pick Disease type C.

Added Lumizyme to the policy with relevant criteria, background information and rationale.

Deleted HCPCS codes J3490, J3590, C9399 from policy coding effective 05/01/2025.

Added HCPCS code J0221 effective 01/01/2026.

4Drugs addressed

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6 monthsInitial auth period

Coverage Summary

Scope: This policy defines coverage eligibility, medical necessity, initial and continuation authorization criteria, dosing limits, and exclusions for enzyme replacement and adjunctive therapies for Pompe disease — specifically Lumizyme (alglucosidase alfa), Nexviazyme (avalglucosidase alfa‑ngpt), Pombiliti (cipaglucosidase alfa‑atga) used in combination with Opfolda (miglustat). Coverage stance: mixed — therapies are covered when the policy’s drug‑specific clinical criteria are met and are considered investigational or not medically necessary when criteria are not met. Primary indications: Pompe disease (infantile‑onset and late‑onset) for the ERTs; Opfolda additionally has an approved alternative indication for Niemann‑Pick disease type C when criteria are met. Effective date: 2026-03-01. Last review: 2025-12-10; next review scheduled 2026-12-01.

Policy quick facts

Policy numberPolicy # 00771

Current effective date03/01/2026

Last review12/10/2025

Next review12/01/2026

Named therapies referenced4 (Lumizyme, Nexviazyme, Pombiliti, Opfolda)

Typical initial authorization period6 months (Pombiliti and Opfolda initial authorizations)

PayerBlue Cross and Blue Shield of Louisiana