PALYNZIQ® (pegvaliase-pqpz) Injection is a daily at-home subcutaneous injection available in 3 dosage strengths1

PALYNZIQ is available in 3 different dosage strengths1:
2.5 mg /0.5 mL single-dose prefilled syringe (white)10 mg /0.5 mL single-dose prefilled syringe (green)20 mg/mL single-dose prefilled syringe (blue)
- PALYNZIQ is supplied in a prefilled syringe intended for use as a single at-home subcutaneous injection1
- PALYNZIQ should be refrigerated at 36ºF (2ºC) to 46ºF (8ºC) in the original carton to protect from light1
- PALYNZIQ can be safely stored at room temperature in the original carton for up to 30 days1
- On the carton, patients should record the date that they remove PALYNZIQ from the refrigerator
- Do not re-refrigerate once stored at room temperature
Recommended dosing regimen
- There are 3 phases to the recommended dosing regimen for PALYNZIQ: induction, titration, and maintenance1
The first injection must be performed under the supervision of a healthcare provider equipped to manage anaphylaxis; patients must be closely observed for at least 1 hour following injection. Prior to self-injection, the patient's competency with self-administration must be confirmed.1


Additional time may be required between dose escalations based on patient tolerability.1
Individualize treatment to the lowest effective and tolerated dosage. Consider increasing to a maximum of
Discontinue PALYNZIQ if either a ≥20% reduction from baseline or a blood Phe concentration
Therapeutic dosage is based on patient tolerability, blood Phe concentrations, and dietary Phe intake1
In clinical trials, consistent dietary Phe intake was recommended until therapeutic dosage was achieved2
- Obtain baseline blood Phe concentration prior to treatment initiation and assess blood Phe levels every 4 weeks until therapeutic dosage is achieved1
- After therapeutic dosage is achieved, periodic blood Phe monitoring is recommended1
- Monitor patients’ dietary Phe intake throughout treatment with PALYNZIQ and counsel them on how to adjust their intake, as needed, based on blood Phe concentrations1
- Most patients were not on a Phe-restricted diet prior to and during the trials2
- Patients’ total protein intake remained relatively stable throughout treatment, while dietary Phe intake increased2
- If patients experience blood Phe levels
≤30 µmol/L (0.5 mg/dL) , reduce dosage of PALYNZIQ and/or modify dietary Phe intake to keep levels within a clinically acceptable range and above30 µmol/L (0.5 mg/dL)1
A 20% reduction in blood Phe levels or blood Phe concentration was defined as the first sign of response in clinical trials1
- Treatment may be continued to reach individualized Phe targets once this threshold is met, based on patient tolerability
References: 1. PALYNZIQ [package insert]. Novato, CA: BioMarin Pharmaceutical Inc; 2018. 2. Thomas J, Levy H, Amato S, et al, for the PRISM investigators. Pegvaliase for the treatment of phenylketonuria: results of a long-term phase 3 clinical trial program (PRISM). Mol Genet Metab. 2108;124(1):27-38.