Uptravi lowers blood pressure in your lungs by dilating (widening) blood vessels that supply blood to the lungs.

Uptravi is used to treat pulmonary arterial hypertension (PAH).

Uptravi helps keep your condition from getting worse and helps lower your risk of needing to be hospitalized for PAH.

Tell your doctor about all your current medicines and any you start or stop using. Many drugs can interact, and some drugs should not be used with Uptravi.

Before taking this medicine

You should not use Uptravi if you are allergic to selexipag.

Many drugs can interact and cause dangerous effects. Some drugs should not be used together with selexipag. Your doctor may change your treatment plan if you also use:

• clopidogrel; or

• gemfibrozil.

To make sure Uptravi is safe for you, tell your doctor if you have ever had:

• a condition called pulmonary veno-occlusive disease (narrowing of the veins that carry blood from the lungs to the heart); or

• liver disease.

It is not known if selexipag will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

You should not breastfeed while using Uptravi.

Take Uptravi exactly as prescribed by your doctor. Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose.

Uptravi tablets are taken by mouth. You may take the tablets with or without food.

Swallow the tablet whole and do not crush, chew, or break it.

Uptravi injection is given in a vein by a healthcare provider if you are unable to take medicine by mouth.

You should not stop taking Uptravi without your doctor's advice. Stopping suddenly may make your condition worse.

Store tablets at room temperature away from moisture and heat.

Usual Adult Dose for Pulmonary Hypertension:

Initial dose: 200 mcg orally twice a day
Maintenance dose: Increase in increments of 200 mcg orally twice a day at weekly intervals to the highest tolerated dose
Maximum dose: 1600 mcg orally twice a day

Comment:
-If the patient reaches a dose that is not well tolerated, reduce to a previously tolerated dose.

Use: For the treatment of pulmonary arterial hypertension (PAH) to delay disease progression and reduce the risk of hospitalization for PAH

You should not use Uptravi if you are allergic to selexipag.

Many drugs can interact and cause dangerous effects. Some drugs should not be used together with selexipag. Your doctor may change your treatment plan if you also use:

• clopidogrel; or

• gemfibrozil.

To make sure Uptravi is safe for you, tell your doctor if you have ever had:

• a condition called pulmonary veno-occlusive disease (narrowing of the veins that carry blood from the lungs to the heart); or

• liver disease.

It is not known if selexipag will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

You should not breastfeed while using Uptravi.

Use the medicine as soon as you can, but skip the missed dose if your next dose is due in less than 6 hours. Do not use two doses at one time.

If you miss doses for 3 or more days, call your doctor before taking another dose. You may need to restart the medicine at a lower dose than you took before.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Get emergency medical help if you have signs of an allergic reaction to Uptravi: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Call your doctor at once if you have:

• low red blood cells (anemia) - pale skin, tiredness, feeling light-headed or short of breath, cold hands and feet; or

• new lung problems - anxiety, sweating, pale skin, severe shortness of breath, wheezing, gasping for breath, cough with foamy mucus, chest pain, fast or uneven heart rate.

Common Uptravi side effects may include:

• headache;

• jaw pain;

• muscle pain, pain in your arms or legs;

• joint pain;

• flushing (warmth, redness, or tingly feeling);

• rash;

• anemia;

• nausea, vomiting, loss of appetite, diarrhea; or

• pain, redness, or swelling where a Uptravi injection was given.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Sometimes it is not safe to use certain medicines at the same time. Some drugs can affect your blood levels of other drugs you use, which may increase side effects or make the medicines less effective.

Other drugs may interact with selexipag, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all other medicines you use.