Pegvaliase-pqpz

(peg VAL i ase pqpz)

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Solution Prefilled Syringe, Subcutaneous [preservative free]:

Palynziq: 2.5 mg/0.5 mL (0.5 mL); 10 mg/0.5 mL (0.5 mL); 20 mg/mL (1 mL)

• Phenylalanine Ammonia Lyase Enzyme
• Phenylalanine Ammonia Lyase, Recombinant

There are no contraindications listed in the manufacturer's labeling.

Anaphylaxis has been reported after administration of pegvaliase-pqpz and may occur at any time during treatment. Administer the initial dose of pegvaliase-pqpz under the supervision of a health care provider equipped to manage anaphylaxis, and closely observe patients for at least 60 minutes following injection. Prior to self-injection, confirm patient competency with self-administration, and patient’s and observer’s (if applicable) ability to recognize signs and symptoms of anaphylaxis and administer auto-injectable epinephrine, if needed. Consider having an adult observer for patients who may need assistance in recognizing and managing anaphylaxis during pegvaliase-pqpz treatment. If an adult observer is needed, the observer should be present during and for at least 60 minutes after pegvaliase-pqpz administration, should be able to administer auto-injectable epinephrine, and call for emergency medical support upon its use. Prescribe auto-injectable epinephrine to all patients treated with pegvaliase-pqpz. Prior to the first dose, instruct the patient and observer (if applicable) how to recognize the signs and symptoms of anaphylaxis, how to properly administer auto-injectable epinephrine, and to seek immediate medical care upon its use. Instruct patients to carry auto-injectable epinephrine with them at all times during treatment with pegvaliase-pqpz. Consider the risks and benefits of readministering pegvaliase-pqpz following an episode of anaphylaxis. If the decision is made to readminister pegvaliase-pqpz, readminister the first dose under the supervision of a health care provider equipped to manage anaphylaxis and closely observe the patient for at least 60 minutes following the dose. Because of the risk of anaphylaxis, pegvaliase-pqpz is available only through a restricted program under a Risk Evaluation and Mitigation Strategy (REMS) called the pegvaliase-pqpz REMS

Uncontrolled maternal phenylalanine concentrations are associated with adverse pregnancy outcomes. Phenylalanine concentrations >600 micromol/L (10 mg/dL) may increase the risk of miscarriage, birth defects (including microcephaly and major cardiac malformations), intrauterine growth retardation, and future intellectual disability. Maternal phenylalanine concentrations should be controlled during pregnancy and for 3 months prior to conception to reduce the risk of adverse fetal events. Dietary control is currently recommended (ACOG 2015; Vockley 2014); information related to the use of pegvaliase-pqpz in pregnant women is insufficient.

Health care providers are encouraged to enroll women exposed to pegvaliase-pqpz during pregnancy or within 1 month following the last dose to the pregnancy surveillance program (866-906-6100).

Pegvaliase-pqpz is an enzyme.1

• If you have an allergy to pegvaliase-pqpz or any part of pegvaliase-pqpz.
• If you are allergic to any drugs like this one, any other drugs, foods, or other substances. Tell your doctor about the allergy and what signs you had, like rash; hives; itching; shortness of breath; wheezing; cough; swelling of face, lips, tongue, or throat; or any other signs.

This medicine may interact with other drugs or health problems.

Tell your doctor and pharmacist about all of your drugs (prescription or OTC, natural products, vitamins) and health problems. You must check to make sure that it is safe for you to take pegvaliase-pqpz with all of your drugs and health problems. Do not start, stop, or change the dose of any drug without checking with your doctor.

• Tell all of your health care providers that you take pegvaliase-pqpz. This includes your doctors, nurses, pharmacists, and dentists.
• Have blood work checked as you have been told by the doctor. Talk with the doctor.
• Follow the diet plan that your doctor told you about.
• Skin reactions that lasted at least 2 weeks have happened with pegvaliase-pqpz. Tell your doctor right away if you have a skin reaction while you are using pegvaliase-pqpz.
• This medicine may cause harm to the unborn baby if you take it while you are pregnant.
• If you get pregnant while taking pegvaliase-pqpz or within 1 month after your last dose, call your doctor right away.
• Tell your doctor if you are breast-feeding. You will need to talk about any risks to your baby.