MedicaidCoverageMedium impact
Medical Therapies for Enzyme Deficiencies (for Pennsylvania Only)
UnitedHealthcare·PA · Genetics, Pediatrics, Neurology +1 more·Medical Benefit Drug Policy
Effective date
Nov 1, 2026
We identified it
Oct 3, 2026
Summary
UnitedHealthcare Community Plan of Pennsylvania added two new enzyme replacement therapies (Avlayah for Hunter syndrome/MPS II and Loargys for hyperargininemia) to its Medical Therapies for Enzyme Deficiencies policy, effective November 1, 2026. The policy also relaxed initial therapy requirements for four existing therapies (Aldurazyme, Elaprase, Kanuma, Lumizyme) by removing the requirement to document clinical signs/symptoms at treatment initiation, and added new billing codes J1757, J1818, and ICD-10 codes E76.1 and E27.21.
Action Required
By November 1, 2026: (1) Billing team must add HCPCS codes J1757 (Avlayah) and J1818 (Loargys) to the billing system with corresponding ICD-10 diagnosis codes E76.1 (MPS II/Hunter syndrome) and E27.21 (hyperargininemia). (2) Update prior authorization workflows to require documentation of enzyme deficiency confirmation (genetic testing or enzyme activity measurement) and clinical presentation for initial Avlayah and Loargys authorizations, with 12-month authorization limits. (3) For continuation therapy, require documentation of prior treatment and positive clinical response per policy criteria. (4) Update billing software to enforce the mutual exclusivity rule: Elaprase and Avlayah cannot be billed together for MPS II treatment. (5) For existing therapies (Aldurazyme, Elaprase, Kanuma, Lumizyme), update authorization criteria templates to remove the requirement for documented clinical signs/symptoms at initial therapy approval. (6) Providers must document dosing compliance with FDA-approved labeling, compendia, or peer-reviewed literature for all covered enzyme therapies. Failure to obtain proper prior authorization will result in claim denials. This applies only to Pennsylvania Medicaid members.