• Treatment of phenylketonuria (PKU) in adult patients who have uncontrolled blood phenylalanine concentrations >600 micromol/L on existing therapy.

Contraindicated in:

• OB: Pregnancy.

Use Cautiously in:

• Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant
• Pedi: Safety and effectiveness not established in children.

Derm: alopecia, pruritus, rash.

EENT: nasal congestion, oropharyngeal pain.

GI: abdominal pain, diarrhea, nausea, vomiting.

Local: injection site reactions.

MS: ↑ creatine kinase, arthralgia.

Neuro: anxiety, dizziness, headache.

Resp: cough.

Misc: fatigue, hypersensitivity reactions (including anaphylaxis and angioedema), serum sickness.

Drug-Drug:

• Use with other PEGylated products may ↑ risk of anaphylaxis.

• Solution for injection (prefilled syringes): 2.5 mg/0.5 mL; 10 mg/0.5 mL; 20 mg/mL;

• SC (Adults): Induction phase: 2.5 mg once weekly for 4 wk, then proceed to titration phase; Titration phase: 2.5 mg twice weekly for 1 wk, then 10 mg once weekly for 1 wk, then 10 mg twice weekly for 1 wk, then 10 mg 4 times weekly for 1 wk, then 10 mg once daily for 1 wk, then proceed to maintenance phase; Maintenance phase: 20 mg once daily for ≥24 wk; may consider ↑ to 40 mg once daily if adequate response (blood phenylalanine concentration ≤600 micromol/L) has not been achieved after patient receives 20 mg once daily for ≥24 wk. May consider ↑ to 60 mg once daily if adequate response (blood phenylalanine concentration ≤600 micromol/L) has not been achieved after patient receives 40 mg once daily for ≥16 wk. Discontinue therapy if adequate response (blood phenylalanine concentration ≤600 micromol/L) has not been achieved after patient receives 60 mg once daily for ≥16 wk.

Palynziq

• As a phenylalanine ammonia lyase enzyme, it acts as a replacement for phenylalanine hydroxylase (as enzyme that is deficient in patients with PKU), and converts phenylalanine to ammonia and trans-cinnamic acid.

• Reduction in blood phenylalanine concentrations.

Therapeutic Classification: n/a

Pharmacologic Classification: enzymes

Absorption: Unknown.

Distribution: Well distributed to tissues.

Metabolism/Excretion: Metabolized via catabolic pathways into small peptides and amino acids; excretion pathway(s) unknown.

Half-life: 14–132 hr.

(plasma concentrations)

• REMS: Instruct patient in correct technique for injection, storage, and disposal of equipment. Omit missed doses and administer next dose a scheduled; do not double doses. Explain purpose and requirements of REMS program to patient and caregiver. Advise patient to read Medication Guide before starting therapy and with each Rx refill in case of changes.
• Advise patient to monitor dietary protein and phenylalanine intake and counsel patient on how to adjust dietary intake of protein and phenylalanine based on phenylalanine blood levels.
• Inform patient and caregiver on risk of anaphylaxis. Instruct patient to carry auto-injectable epinephrine with them at all times. Instruct patient and caregiver on how to recognize signs and symptoms of anaphylaxis (fainting, dizziness or lightheadedness, sudden confusion, trouble breathing or wheezing, chest discomfort or chest tightness, fast heart rate, swelling of your face, lips, eyes, or tongue; throat tightness, flushed skin, rash, itching, raised bumps on skin, nausea, vomiting, or diarrhea, losing control of urine or stools), how to properly administer auto-injectable epinephrine, and to seek immediate medical care upon its use.
• Instruct patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other Rx, OTC, herbal products.
• Rep: Advise patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding. If pegvaliase is administered during pregnancy, or if patient becomes pregnant while receiving pegvaliase or within one mo after last dose, report pegvaliase exposure to pregnancy surveillance program by calling 1-866-906-6100.
• Emphasize importance of carrying Palynziq Patient Wallet Card and auto-injectable epinephrine with you at all times.

peg-VAL-i-ase