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Palynziq (pegvaliase-pqpz)
pegvaliase
Black Box Warnings .
Appropriate Use
available only through Palynziq REMS Program due to anaphylaxis risk; patients must have epinephrine available at all times while receiving tx; prescribers, patients, and pharmacies must enroll at 1-855-758-7367 or www.PALYNZIQREMS.com
Anaphylaxis
has been reported and may occur at any time during tx; administer initial dose under supervision of healthcare provider equipped to manage anaphylaxis; observe patient for anaphylaxis signs/symptoms for at least 60min after 1st dose; consider an adult observer for patients requiring assistance with recognizing and managing anaphylaxis; observer should stay during and for at least 60min after administration; confirm competency before self-administration and patient or observer's ability to recognize signs/symptoms of anaphylaxis; prescribe epinephrine and instruct patient or observer on administration and to seek immediate medical care upon use; instruct patient to carry epinephrine at all times while receiving tx; if anaphylaxis episode occurs, consider risk/benefit prior to tx restart; if decision is made to restart tx, readminister 1st dose under supervision of healthcare provider equipped to manage anaphylaxis and observe patient for at least 60min after dose
Adult Dosing .
Dosage forms: INJ (pre-filled syringe): 2.5 mg per 0.5 mL, 10 mg per 0.5 mL, 20 mg per mL
Restricted Distribution in US
- [1-855-758-7367 or www.PALYNZIQREMS.com for more info]
Special Note
- [drug name clarification]
- Info: nonproprietary name = pegvaliase-pqpz
PKU
- [20-60 mg SC qd]
- Start: 2.5 mg SC qwk x4wk, then 2.5 mg SC 2x/wk x1wk, then 10 mg SC qwk x1wk, then 10 mg SC 2x/wk x1wk, then 10 mg SC 4x/wk x1wk, then 10 mg SC qd x1wk, then 20 mg SC qd for at least 24wk, then may incr. to 40 mg SC qd; Max: 60 mg/day; Info: for patients with phenylalanine level >600 mcmol/L on existing tx; consider premed with antihistamine or antipyretic; may incr. dose to 60 mg SC qd after at least 16wk on 40 mg/day; D/C if inadequate response after 16wk tx on max dose
renal dosing
- [not defined]
- renal impairment: not defined
- HD/PD: not defined
hepatic dosing
- [not defined]
Peds Dosing .
- Dosage forms: INJ (pre-filled syringe): 2.5 mg per 0.5 mL, 10 mg per 0.5 mL, 20 mg per mL
Restricted Distribution in US
- [1-855-758-7367 or www.PALYNZIQREMS.com for more info]
Special Note
- [drug name clarification]
- Info: nonproprietary name = pegvaliase-pqpz
PKU
- [12 yo and older]
- Dose: 20-60 mg SC qd; Start: 2.5 mg SC qwk x4wk, then 2.5 mg SC 2x/wk x1wk, then 10 mg SC qwk x1wk, then 10 mg SC 2x/wk x1wk, then 10 mg SC 4x/wk x1wk, then 10 mg SC qd x1wk, then 20 mg SC qd for at least 24wk, then may incr. to 40 mg SC qd; Max: 60 mg/day; Info: for patients with phenylalanine level >600 mcmol/L on existing tx; consider premed with antihistamine or antipyretic; may incr. dose to 60 mg SC qd after at least 16wk on 40 mg/day; D/C if inadequate response after 16wk tx on max dose
renal dosing
- [not defined]
- renal impairment: not defined
- HD/PD: not defined
hepatic dosing
- [not defined]
Contraindications / Cautions .
- caution: hypersensitivity to drug or ingredient
Drug Interactions .
Overview
pegvaliase
metabolic enzyme
-
None
- monitor for hypersensitivity reaction (including anaphylaxis) in combo with products containing polyethylene glycol 3350 (PEG 3350) or other PEGylated products (as active or inactive ingredient); combo may incr. risk due to formation of anti PEG IgM and IgG antibodies
Monitor/Modify Tx
- certolizumab pegol
- elapegademase
- medroxyprogesterone (contraceptive)
- methoxy polyethylene glycol-epoetin beta
- pegaspargase
- pegfilgrastim (G-CSF)
- peginterferon alfa 2a
- peginterferon beta 1a
- pegloticase
- polyethylene glycol
- polyethylene glycol/electrolytes
Adverse Reactions .
Serious Reactions
- anaphylaxis
- hypersensitivity reaction
- injection site infection
Common Reactions
- injection site reaction
- arthralgia
- hypersensitivity reaction
- headache
- skin reaction, generalized
- fever (peds patients)
- nausea
- dizziness
- abdominal pain
- vomiting
- cough
- oropharyngeal pain
- pruritus
- diarrhea
- nasal congestion
- fatigue
- extremity pain
- anxiety
- alopecia
- myalgia
- erythema
- back pain
- anaphylaxis
- complement C3 levels low
- CRP elevated
- hypophenylalaninemia
- complement C4 levels low
- CPK elevated
- eosinophilia (peds patients)
Safety/Monitoring .
Monitoring Parameters
phenylalanine level at baseline, then q4wk during titration, then periodically or more frequently in peds pts during maintenance tx; signs/symptoms of anaphylaxis for at least 60min after initial dose or 1st dose following anaphylaxis episode
Pregnancy/Lactation .
Pregnancy
Clinical Summary
weigh risk/benefit during pregnancy, monitor phenylalanine levels closely during pregnancy; adjust dose or modify dietary protein and phenylalanine intake to avoid phenylalanine level <30 mcmol/L; inadequate human data available; possible risk of embryo-fetal toxicity and fetal malformations based on animal data at 5x MRHD
Pregnancy Reporting
report patients who become pregnant during tx or within 1mo after D/C to Palynziq surveillance program at 1-866-906-6100
Lactation
Clinical Summary
weigh risk/benefit while breastfeeding; no human data available to assess risk of infant harm or effects on milk production
Pharmacology .
Metabolism: for pegvaliase: unknown; CYP450: unknown
Excretion: for pegvaliase: unknown; Half-life: 47h
Subclass: Inherited Metabolic Disorders
Mechanism of Action
for pegvaliase: converts phenylalanine to ammonia and trans-cinnamic acid, resulting in decr. phenylalanine levels
Formulary .
No Formulary Selected
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