Pegvaliase-pqpz Injection

Pegvaliase-pqpz injection may cause serious or life-threatening allergic reactions. These reactions may occur soon after your injection or at any time during your treatment. The first dose should be given by a doctor or nurse in a healthcare setting where these reactions can be treated and where you can be closely observed for at least 1 hour after the injection. Your doctor may give you certain medications before you receive pegvaliase-pqpz injection to help to prevent a reaction. Your doctor will give you a prefilled automatic epinephrine injection device (Adrenaclick, Auvi-Q, EpiPen, others) to treat a life-threatening allergic reaction. Your doctor will teach you and your caregiver how to use this medication and how to recognize the signs of an allergic reaction. Carry the epinephrine injection with you at all times. If you experience any of the following symptoms at any time during your treatment, use the epinephrine injection and get emergency medical care immediately: difficulty swallowing or breathing; shortness of breath; wheezing; hoarseness; swelling of the face, throat, tongue or lips; hives; flushing or sudden redness of the face, neck or upper chest; rash; itching; redness of the skin; fainting; dizziness; chest pain or discomfort; tightness of the throat or chest; vomiting; nausea; diarrhea; or loss of bladder control.

Because of the risks with this medication, pegvaliase-pqpz injection is available only through a special restricted distribution program called Palynziq^® Risk Evaluation and Mitigation Strategies (REMS) Program. You, your doctor, and your pharmacist must be enrolled in this program before you can receive pegvaliase-pqpz injection. Ask your doctor if you have any questions about how you will receive your medication.

Your healthcare provider will give you a Palynziq^® patient safety card that describes the allergic symptoms that you may have with this medication. Carry this card with you at all times during your treatment. It is important to show your Palynziq^® patient safety card to any other healthcare provider who treats you.

Keep all appointments with your doctor and the laboratory. Your doctor will order certain lab tests before and during your treatment to check your body's response to pegvaliase-pqpz injection.

Your doctor or pharmacist will give you the manufacturer's patient information sheet (Medication Guide) when you begin treatment with pegvaliase-pqpz injection and each time you receive the medication. Read the information carefully and ask your doctor or pharmacist if you have any questions. You can also visit the Food and Drug Administration (FDA) website (http://www.fda.gov/Drugs/DrugSafety/ucm085729.htm) or the manufacturer's website to obtain the Medication Guide.

Pegvaliase-pqpz injection is used along with a specific diet to reduce blood phenylalanine levels in people who have phenylketonuria (PKU; an inborn condition in which phenylalanine may build up in the blood and causes decreased intelligence and a decreased ability to focus, remember, and organize information) and who have uncontrolled blood phenylalanine levels. Pegvaliase-pqpz injection is in a class of medications called enzymes. It works by helping to reduce the amount of phenylalanine in the body.

Pegvaliase-pqpz injection comes as a solution (liquid) to inject subcutaneously (just under the skin). It is usually injected once weekly for 4 weeks, and then increased gradually over the next 5 weeks to once daily. Your doctor will change your dose based on your body's response to the medication. Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Use pegvaliase-pqpz injection exactly as directed. Do not use more or less of it or use it more often than prescribed by your doctor.

Before you use pegvaliase-pqpz injection, look at the solution closely. The medication should be clear to pale yellow and free of floating particles. If the medication is cloudy, discolored, or contains particles, do not use it. Do not shake the prefilled syringe.

You may inject pegvaliase-pqpz injection on the front of your thighs or anywhere on your stomach except your navel (belly button) and the area 2 inches around it. If another person is injecting your medication, the top of buttocks and the outer area of the upper arms also may be used. Do not inject the medication into skin that is tender, bruised, red, hard, or not intact, or that has scars, moles, tattoos, or bruises. Choose a different spot each time you inject the medication, at least 2 inches away from a spot that you have used before. If more than one injection is needed for a single dose, the injection sites must be at least 2 inches away from each other but can be on the same part of the body or a different part of the body.

This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

Before using pegvaliase-pqpz injection,

• tell your doctor and pharmacist if you are allergic to pegvaliase, any other medications, or any of the ingredients in pegvaliase-pqpz injection. Ask your pharmacist or check the Medication Guide for a list of the ingredients.
• tell your doctor and pharmacist what other prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take while using pegvaliase-pqpz injection. Your doctor may need to change the doses of your medications or monitor you carefully for side effects.
• tell your doctor if you are pregnant, plan to become pregnant, or are breastfeeding. If you become pregnant while using pegvaliase-pqpz injection, call your doctor.

Follow your diet plan carefully. Your doctor will monitor the amount of protein and phenylalanine that you eat and drink during your treatment.

If a dose is missed, inject your next dose as scheduled. Do not inject a double dose to make up for a missed one.

Pegvaliase-pqpz injection may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:

• redness, itching, pain, bruising, rash, swelling, tenderness at the injection site
• joint pain
• headache
• stomach pain
• mouth and throat pain
• feeling tired
• anxiety
• hair loss

Some side effects can be serious. If you experience any of these symptoms or those listed in the IMPORTANT WARNING section, stop using pegvaliase-pqpz injection and call your doctor immediately or get emergency medical treatment:

• rash, itching, hives, or skin redness that lasts at least 14 days

Pegvaliase-pqpz injection may cause other side effects. Call your doctor if you have any unusual problems while using this medication.

If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online (https://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

Keep this medication in the container it came in to protect from light, tightly closed, and out of reach of children. Store it in the refrigerator; do not freeze. It also may be stored at room temperature for up to 30 days. Once the medication is stored at room temperature, do not return it to the refrigerator.

It is important to keep all medication out of sight and reach of children as many containers (such as weekly pill minders and those for eye drops, creams, patches, and inhalers) are not child-resistant and young children can open them easily. To protect young children from poisoning, always lock safety caps and immediately place the medication in a safe location — one that is up and away and out of their sight and reach. https://www.upandaway.org

Unneeded medications should be disposed of in special ways to ensure that pets, children, and other people cannot consume them. However, you should not flush this medication down the toilet. Instead, the best way to dispose of your medication is through a medicine take-back program. Talk to your pharmacist or contact your local garbage/recycling department to learn about take-back programs in your community. See the FDA's Safe Disposal of Medicines website (https://goo.gl/c4Rm4p) for more information if you do not have access to a take-back program.

In case of overdose, call the poison control helpline at 1-800-222-1222. Information is also available online at https://www.poisonhelp.org/help. If the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services at 911.

Do not let anyone else use your medication. Ask your pharmacist any questions you have about refilling your prescription.

It is important for you to keep a written list of all of the prescription and nonprescription (over-the-counter) medicines you are taking, as well as any products such as vitamins, minerals, or other dietary supplements. You should bring this list with you each time you visit a doctor or if you are admitted to a hospital. It is also important information to carry with you in case of emergencies.

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