WARNING

Palynziq (pegvaliase-pqpz) is an enzyme substitution therapy used to treat phenylketonuria (PKU), a genetic disorder that affects the body’s ability to break down an amino acid called phenylalanine. While Palynziq can be effective in managing PKU, like any medication, it comes with potential risks and side effects. Here is a brief overview of the risks associated with taking Palynziq:

• Anaphylaxis: There is a risk of severe allergic reactions, including anaphylaxis, which is a life-threatening condition. Patients may develop symptoms such as difficulty breathing, swelling, and a drop in blood pressure.
• Hypersensitivity Reactions: Some individuals may experience hypersensitivity reactions, which can include rash, itching, and fever. These reactions may require discontinuation of Palynziq.
• Injection Site Reactions: Common side effects include injection site reactions such as redness, pain, swelling, and itching. These reactions are generally mild, but they can be bothersome for some patients.
• Blood Phenylalanine Levels: Palynziq can lower blood phenylalanine levels, but excessively low levels can lead to adverse effects such as cognitive and psychiatric symptoms. Monitoring and adjusting the dosage are essential to maintain an appropriate balance.
• Liver Enzyme Elevations: Some patients may experience elevations in liver enzymes, which may indicate liver issues. Regular monitoring of liver function is necessary during Palynziq treatment.
• Pregnancy and Breastfeeding: The safety of Palynziq during pregnancy and breastfeeding is not well-established. Consultation with a healthcare professional is essential for women who are pregnant, planning to become pregnant, or breastfeeding.
• Neurologic Effects: Palynziq may cause neurologic adverse reactions, including seizures. Patients with a history of seizures should be closely monitored, and the benefits of treatment should be carefully weighed against the risks.

It is crucial for individuals considering or currently using Palynziq to discuss their medical history and any potential risks with their healthcare provider. Healthcare professionals can provide personalized guidance based on the patient’s specific health condition and monitor for adverse effects throughout the course of treatment.

Why is this medication prescribed?

Palynziq (pegvaliase-pqpz) is prescribed to treat phenylketonuria (PKU), a rare genetic disorder that affects the body’s ability to metabolize an amino acid called phenylalanine. PKU is characterized by the deficiency of an enzyme called phenylalanine hydroxylase, which is responsible for breaking down phenylalanine into other substances. Without this enzyme, phenylalanine can accumulate to harmful levels in the blood, leading to cognitive and neurological problems.

Palynziq is an enzyme substitution therapy that works by providing a modified form of the phenylalanine hydroxylase enzyme. This enzyme helps metabolize phenylalanine, reducing its levels in the blood and preventing the associated complications of PKU.

How should this medicine be used?

Here are general guidelines on how Palynziq should be used:

• Administration: Palynziq is administered by subcutaneous injection (under the skin). The injections are typically given daily.
• Dose Adjustment: The dosage of Palynziq is individualized based on the patient’s response and the goal of maintaining blood phenylalanine levels within the target range. Healthcare providers will monitor blood phenylalanine levels regularly and adjust the dose as needed.
• Initiation of Treatment: Palynziq treatment is usually initiated in a healthcare setting under the supervision of a healthcare professional. This allows for close monitoring of the patient’s response and any potential adverse effects.
• Titration Phase: The initiation phase involves a titration period during which the dose is gradually increased to the optimal therapeutic level. This is done to minimize the risk of adverse reactions.
• Monitoring: Throughout the course of treatment, regular monitoring of blood phenylalanine levels, liver function, and potential adverse effects is essential. Healthcare providers will assess the patient’s overall health and adjust the treatment plan accordingly.
• Patient Education: Patients receiving Palynziq should be educated about the importance of adherence to the prescribed treatment plan, including the dosing schedule and any dietary restrictions necessary for managing PKU.

It’s important for individuals prescribed Palynziq to work closely with their healthcare team, including physicians, dietitians, and other specialists. They should follow their healthcare provider’s instructions and attend regular check-ups to ensure the medication is effectively managing PKU while minimizing potential risks and side effects.

Other uses for this medicine

Palynziq (pegvaliase-pqpz) is specifically indicated for the treatment of phenylketonuria (PKU) and does not have other approved uses. Phenylketonuria is a rare genetic disorder, and Palynziq is designed to address the metabolic imbalance associated with this condition by helping to metabolize phenylalanine.

What special precautions should I follow?

Regarding special precautions for Palynziq, it’s crucial to note that medication guidelines and safety information may evolve, and it’s essential to consult with a healthcare professional for the most up-to-date information. That said, as of my last knowledge update, here are some general precautions associated with Palynziq:

• Allergies: Individuals with known allergies or hypersensitivity to pegvaliase-pqpz or any of its components should not use Palynziq.
• Anaphylaxis: Palynziq carries a risk of severe allergic reactions, including anaphylaxis. Patients and healthcare providers should be aware of the signs and symptoms of anaphylaxis, such as difficulty breathing, swelling, and a drop in blood pressure. Emergency measures and immediate medical attention are necessary if anaphylaxis occurs.
• Hypersensitivity Reactions: Patients should be monitored for hypersensitivity reactions, which can include rash, itching, and fever. If such reactions occur, healthcare providers may need to adjust the treatment plan or discontinue Palynziq.
• Liver Function Monitoring: Regular monitoring of liver function is important during Palynziq treatment due to the potential for elevations in liver enzymes. Any significant changes may require adjustments to the treatment plan.
• Neurologic Effects: Palynziq may increase the risk of neurologic adverse reactions, including seizures. Patients with a history of seizures should be closely monitored.
• Pregnancy and Breastfeeding: The safety of Palynziq during pregnancy and breastfeeding is not well-established. Pregnant or breastfeeding individuals should discuss the potential risks and benefits with their healthcare provider.
• Patient Education: Patients should receive thorough education on the proper administration of Palynziq, including the injection technique, dosage adjustments, and the importance of regular monitoring.

It’s crucial for individuals considering or using Palynziq to have open communication with their healthcare providers, disclose their complete medical history, and follow their healthcare team’s guidance. The information provided here is a general overview, and specific precautions may vary based on the individual’s health status and any updates in the product’s prescribing information.

What special dietary instructions should I follow?

• Phenylalanine-Restricted Diet: Palynziq is intended to be used in conjunction with a phenylalanine-restricted diet. Follow your healthcare provider’s dietary instructions to manage phenylalanine levels effectively.
• Nutritional Monitoring: Regularly monitor nutritional status, including phenylalanine levels and protein intake.

What should I do if I forget a dose?

• Contact Your Healthcare Provider: If you miss a dose of Palynziq, contact your healthcare provider as soon as possible. Your healthcare provider will provide guidance on whether to administer the missed dose or adjust your treatment plan.
• Do Not Double Dose: Do not administer a double dose to make up for a missed one unless specifically instructed by your healthcare provider.
• Follow Instructions: Follow your healthcare provider’s instructions for managing missed doses to maintain the appropriate treatment schedule.

What side effects can this medication cause?

Palynziq (pegvaliase-pqpz) is a medication used to treat phenylketonuria (PKU). Like all medications, Palynziq can cause side effects. It’s important to note that not everyone will experience these side effects, and some individuals may experience side effects that are not listed. If you are prescribed Palynziq, it’s crucial to discuss any concerns or symptoms with your healthcare provider. Common side effects of Palynziq include:

• Injection Site Reactions: Redness, pain, swelling, or itching at the injection site are common side effects. These reactions are typically mild.
• Hypersensitivity Reactions: Hypersensitivity reactions, including rash, itching, and fever, can occur. If you experience these symptoms, it’s important to notify your healthcare provider promptly.
• Anaphylaxis: Anaphylaxis is a severe allergic reaction that can be life-threatening. While rare, it is listed as a potential side effect. Symptoms include difficulty breathing, swelling, and a drop in blood pressure. Seek immediate medical attention if you suspect anaphylaxis.
• Neurologic Adverse Reactions: Some individuals may experience neurologic adverse reactions, including seizures. Patients with a history of seizures should be closely monitored.
• Elevations in Liver Enzymes: Palynziq may lead to elevations in liver enzymes. Regular monitoring of liver function is important.
• Nausea and Vomiting: Gastrointestinal symptoms such as nausea and vomiting may occur.
• Joint Pain: Joint pain has been reported as a side effect.
• Headache: Some individuals may experience headaches.

It’s essential to report any side effects or concerns to your healthcare provider promptly. Your healthcare provider will assess the risk-benefit ratio of continuing Palynziq based on your individual response and overall health. The information provided here is a general overview, and the side effect profile may be updated, so it’s important to consult with your healthcare team for the most current information.

What should I know about storage and disposal of this medication?

Storage of Palynziq:

• Refrigeration: Palynziq should be stored in the refrigerator at a temperature of 36°F to 46°F (2°C to 8°C).
• Protection from Light: The medication should be protected from light. Keep it in its original carton until the time of use.
• Do Not Freeze: Do not freeze Palynziq.
• Avoid Shaking: Avoid shaking the medication.

Disposal of Palynziq:

• Dispose of Needles and Syringes Properly: Dispose of used needles and syringes in a sharps disposal container or follow your healthcare provider’s instructions for proper disposal.
• Unused Medication: Consult with your healthcare provider or pharmacist on the proper disposal of any unused Palynziq.

In case of emergency/overdose

• Seek Medical Attention: In case of an emergency or suspected overdose, seek immediate medical attention.
• Symptomatic Treatment: There is no specific antidote for Palynziq overdose. Treatment is generally supportive and symptomatic.

What other information should I know?

• Regular Monitoring: Regular monitoring of blood phenylalanine levels, liver function, and overall health is crucial during Palynziq treatment.
• Adherence to Treatment Plan: Adhere to your healthcare provider’s prescribed treatment plan, including dosing instructions, dietary recommendations, and follow-up appointments.
• Communication with Healthcare Provider: Keep open communication with your healthcare provider. Report any new symptoms, concerns, or changes in your health promptly.
• Pregnancy and Breastfeeding: If you are pregnant, planning to become pregnant, or breastfeeding, discuss the potential risks and benefits of Palynziq with your healthcare provider.
• Allergies and Hypersensitivity: Be aware of potential allergic reactions, including anaphylaxis, and seek immediate medical attention if symptoms occur.

Always follow the specific instructions provided by your healthcare provider and the medication’s prescribing information. If you have any questions or concerns about Palynziq, do not hesitate to contact your healthcare team for guidance.