Pegvaliase-pqpz (Palynziq)

Covered for adults (≥18) with PKU who have uncontrolled blood phenylalanine >600 µmol/L despite a phenylalanine‑restricted diet and/or prior sapropterin (no off‑label uses approved). Initial approval is 40 weeks with the first dose given under HCP supervision and prescription of auto‑injectable epinephrine; therapy must follow the specified induction/titration (start 2.5 mg weekly → titrate to 20 mg/day, may escalate to 40 mg/day after 24 weeks if <20% reduction), reauthorization (12 months) requires ≥20% reduction from baseline or current ≤600 µmol/L, and treatment must be stopped if response criteria are not met after 16 weeks at 40 mg, with documentation of age, diagnosis, baseline and follow‑up Phe levels, prior treatments, and dosing records required.

"Palynziq is indicated to reduce blood phenylalanine concentrations in adult patients with phenylketonuria (PKU) who have uncontrolled blood phenylalanine concentrations greater than 600 micromol/L ..."