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PALYNZIQ
Inborn errors of metabolism
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Drug Name:

PALYNZIQ Rx

Generic Name and Formulations:
Pegvaliase-pqpz 2.5mg/0.5mL, 10mg/0.5mL, 20mg/mL; soln for SC inj; preservative-free.

Company:
BioMarin Pharmaceuticals

Therapeutic Use:

Indications for PALYNZIQ:

To reduce blood phenylalanine levels in adults with phenylketonuria (PKU) who have uncontrolled concentrations >600μmol/L on current management.

Adult:

See full labeling. Consider premedication with H1- or H2-receptor antagonist, and/or antipyretic prior to administration. Give by SC inj into the front middle of thighs, abdomen, top of buttocks, back of upper arms; rotate inj sites. Inject ≥2 inches apart if >1 inj required. Induction: initially 2.5mg once weekly for 4 weeks. Titration: in a step-wise manner, titrate over ≥5 weeks to achieve 20mg once daily, as tolerated; see full labeling. Maintenance: 20mg once daily for ≥24 weeks. Consider increasing to max 40mg once daily if maintained continuously on 20mg once daily for ≥24 weeks and have not achieved ≥20% reduction from pre-treatment baseline or a concentration ≤600μmol/L; discontinue if no response after 16 weeks of treatment at 40mg dose.

Children:

Not established.

Boxed Warning:

Risk of anaphylaxis.

Warnings/Precautions:

Anaphylaxis may occur during treatment. Have epinephrine readily available. Perform 1st initial dose and/or readministration after anaphylaxis under supervision of a healthcare provider (consider having an adult observer as needed during therapy); monitor closely for ≥60mins post injection. Dose adjustment, interruption, or treatment with antihistamines, antipyretics, and/or corticosteroids to manage hypersensitivity reactions. Obtain baseline blood phenylalanine concentration prior to therapy, every 4 weeks until maintenance dose established, then periodically thereafter. Assess dietary protein and phenylalanine intake throughout treatment. Do not inj into moles, scars, birthmarks, bruises, rashes, or areas where skin is hard, tender, red, damaged, burned, inflamed, tattooed. Use lowest effective dose. Pregnancy: monitor and maintain phenylalanine levels 120–360μmol/L for 3 months prior to and during pregnancy; adjust dose or dietary intake to avoid <30μmol/L. Nursing mothers: monitor.

Pharmacological Class:

Phenylalanine-metabolizing enzyme.

Interactions:

Concomitant other PEGylated products; monitor for hypersensitivity reactions.

Adverse Reactions:

Inj site reactions, arthralgia, hypersensitivity reactions, headache, generalized skin reactions (lasting ≥14 days), pruritus, nausea, abdominal pain, oropharyngeal pain, vomiting, cough, diarrhea, fatigue; anaphylaxis.

Note:

Report Palynziq exposure in pregnant patients by calling (866) 906-6100.

REMS:

YES

Generic Availability:

NO

How Supplied:

Prefilled syringes (2.5mg/0.5mL, 10mg/0.5mL)—1; (20mg/mL)—1, 10

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