Epoprostenol

WHY is this medicine prescribed?

Epoprostenol injection is used to treat certain kinds of pulmonary arterial hypertension (PAH; high blood pressure in the vessels carrying blood to the lungs, causing shortness of breath, dizziness, and tiredness). Epoprostenol may improve the ability to exercise and slow the worsening of symptoms in patients with PAH. Epoprostenol is in a class of medications called vasodilators and platelet-aggregation inhibitors. It works by relaxing the blood vessels, including those in the lungs, and improving blood flow.

Are there OTHER USES for this medicine?

Epoprostenol is also sometimes used to treat acute respiratory distress syndrome (ARDS; a condition in which fluid builds up in the lungs after a signficant injury or illness). Talk to your doctor about the risks of using this medication for your condition.

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

HOW should this medicine be used?

Epoprostenol comes as a powder in a vial to be mixed with a liquid to be given as an intravenous (into a vein) infusion. Epoprostenol is usually given as a continuous intravenous infusion (injected slowly). Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Use epoprostenol injection exactly as directed. Do not inject more or less of it or take it more often than prescribed by your doctor.

Epoprostenol injection is usually used under medical supervision when your treatment is started and when your dose is changed. Before you use epoprostenol injection at home for the first time, your doctor or nurse will show you how to use the infusion pump to receive your medication. Read the written instructions that come with the pump and the medication. Be sure that you understand these directions. Ask your healthcare provider if you have any questions about how to infuse epoprostenol injection.

Your doctor will probably start you on a low dose of epoprostenol and gradually increase your dose. Your doctor may need to slow down your infusion or interrupt your treatment if you experience certain side effects. It is important for you to tell your doctor how you are feeling during your treatment with epoprostenol injection.

Epoprostenol injection may cause low blood pressure. Your doctor will check your blood pressure when you initially receive the infusion and when your dosage changes. If you experience any of the following symptoms after you receive the medication tell your doctor immediately: flushing, dizziness, feeling faint, headache, or pounding heartbeat.

Epoprostenol may control the symptoms of PAH but does not cure it. Do not stop using epoprostenol injection without talking to your doctor. If you suddenly stop using epoprostenol, your symptoms may get worse. Your doctor will probably decrease your dose gradually.

Make sure you have another infusion pump to use right away if your device does not work for any reason. Be sure to ask your healthcare provider what to do if your infusion pump stops working properly.

Epoprostenol injection is not available in pharmacies. You can only get epoprostenol from a specialty pharmacy. Ask your doctor if you have any questions about receiving your medication.

Ask your pharmacist or doctor for a copy of the manufacturer's information for the patient.

What SPECIAL PRECAUTIONS should I follow?

Before using epoprostenol injection,

• tell your doctor and pharmacist if you are allergic to epoprostenol, any other medications, or any of the ingredients in epoprostenol injection. Ask your pharmacist 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. Be sure to mention any of the following: anticoagulants ('blood thinners') such as warfarin (Jantoven) and heparin; digoxin (Lanoxin); diuretics ('water pills'); medications for high blood pressure; and platelet inhibitors such as clopidogrel (Plavix), dipyridamole (Persantine, in Aggrenox), prasugrel (Effient), and ticlopidine (Ticlid). Your doctor may need to change the doses of your medications or monitor you carefully for side effects.

• tell your doctor if you have heart failure. Your doctor may tell you not to use epoprostenol injection.

• tell your doctor if you have or have ever had bleeding problems, pulmonary edema (excess fluid in the lungs), or liver problems.

• tell your doctor if you are pregnant, plan to become pregnant, or are breast-feeding. If you become pregnant while using epoprostenol injection, call your doctor.

• you should know that long-term, continuous use of epoprostenol injection may cause blood clots to form in your body. Your doctor may tell you to take an anticoagulant medication to help prevent blood clots during your therapy with epoprostenol injection. Call your doctor right away if you develop any of the following symptoms: chest pain; shortness of breath; coughing up blood; or lower leg pain, swelling, warmth, or redness.

What SPECIAL DIETARY instructions should I follow?

Unless your doctor tells you otherwise, continue your normal diet.

What should I do IF I FORGET to take a dose?

Your healthcare provider will tell you how to store your medication. Store your medication only as directed. Make sure you understand how to store your medication properly.

What SIDE EFFECTS can this medicine cause?

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

• dizziness
• jaw pain
• muscle and bone pain
• headache
• nausea
• vomiting
• diarrhea
• flushing
• anxiety, nervousness, or agitation
• rash

Some side effects can be serious. If you experience any of these symptoms, call your doctor immediately or get emergency medical treatment:

• shortness of breath, wheezing, difficulty breathing, or coughing up blood
• unusual bleeding or bruising
• fever, chills, or other signs of infection
• dizziness or fainting

Epoprostenol 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 (http://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

What should I know about STORAGE and DISPOSAL of this medication?

Your healthcare provider will tell you how to store your medication. Store your medication only as directed. Make sure you understand how to store your medication properly.

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. http://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 (http://goo.gl/c4Rm4p) for more information if you do not have access to a take-back program.

What should I do in case of OVERDOSE?

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.

Symptoms of overdose may include:

• flushing
• headache
• nausea
• vomiting
• diarrhea
• rapid heartbeat
• dizziness or fainting

What OTHER INFORMATION should I know?

Keep all appointments with your doctor.

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.