Uptravi

Uptravi is used to treat pulmonary arterial hypertension (PAH) in adults and children aged 2 years and older. PAH is high blood pressure in the arteries of the lungs. Uptravi is given orally (by mouth) or as an intravenous infusion.

Uptravi can help slow down the progression of your disease and lower your risk of being hospitalized for PAH.

Uptravi (selexipag) works by activating a target in the body called the prostacyclin (IP) receptor, though it isn't built the same way as natural prostacyclin. After you take it, your body converts selexipag into an active form that is much more potent, about 37 times stronger. Both selexipag and this active form act specifically on the IP receptor rather than other, related receptors, which helps focus their effects. Uptravi belongs to the drug class called prostacyclin receptor agonists.

Uptravi received FDA approval on December 21, 2015. Generic selexipag tablets are available.

It is not known if Uptravi is safe and effective in children younger than 2 years of age.

Do not take Uptravi if you or your child:

• are allergic to selexipag or any of the other ingredients in Uptravi (see the end of this guide for a complete list of ingredients).
• take gemfibrozil because this medicine may affect how Uptravi works and cause side effects.

Before you or your child take Uptravi, tell your healthcare provider about all of your medical conditions, including if you:

• have liver problems.
• have narrowing of the pulmonary veins, a condition called pulmonary veno-occlusive disease.
• are pregnant or plan to become pregnant. It is not known if Uptravi will harm your unborn baby.
• are breastfeeding or plan to breastfeed. It is not known if Uptravi passes into your breast milk. You and your healthcare provider should decide if you will take Uptravi or breastfeed. You should not do both.

Tell your healthcare provider about all the medicines you or your child take, including prescription and over-the-counter medicines, vitamins, and herbal supplements. Uptravi and other medicines may affect each other causing side effects. Do not start any new medicine until you check with your healthcare provider.

Uptravi tablets

• Take or give Uptravi exactly as your healthcare provider tells you to take it. Do not stop taking Uptravi unless your healthcare provider tells you to stop.
• Your child's healthcare provider will prescribe an Uptravi starting dose based on your child's body weight.
• Your healthcare provider will slowly increase your or your child's dose to find the dose of Uptravi that is right.
• If you or your child has side effects, your healthcare provider may tell you to change the dose of Uptravi.
• Uptravi can be taken with or without food. Taking Uptravi with food may help you or your child tolerate Uptravi better.
• Uptravi is usually taken 2 times each day.
• Swallow Uptravi tablets whole. Do not split or crush Uptravi tablets.
• If you or your child cannot swallow the tablets: Dissolve (disperse) the 100 mcg or 150 mcg tablet(s) in apple or orange juice. Do not disperse the tablet(s) in water or milk. Place the required number of tablet(s) for the prescribed dose in a cup. Do not crush or split the tablet(s). Add 1 or 2 teaspoons of apple or orange juice to the prescribed number of tablets. Wait for 5 minutes, then stir the juice and the tablet(s) until the tablets are dispersed. Take or give the mixture right away. Uptravi tablets can also be given with soft foods as follows: Cover the prescribed number of 100 mcg or 150 mcg tablets with a small amount of soft food, such as yogurt, applesauce, or mashed banana. Take or give the mixture right away. Make sure no remaining medicine is left in the container by adding more soft food, and take or give the mixture right away. Do not store Uptravi tablets that are already mixed with juice or soft food for later use.
• If you or your child misses a dose of Uptravi, take it as soon as you remember. If the next scheduled dose is due within 6 hours, skip the missed dose. Take the next dose at the regular time.
• If you or your child misses 3 or more days of Uptravi, call your healthcare provider to see if the dose needs to be changed.
• If you or your child takes too much Uptravi, call your healthcare provider or go to the nearest hospital emergency room right away.

Uptravi given by intravenous (IV) injection

• Your healthcare provider will give you Uptravi into your vein through an intravenous line.
• Your healthcare provider will decide how much Uptravi for injection you will receive each day, based on your current dose of Uptravi tablets.

The most common side effects of Uptravi in adults and children include:

• headache
• jaw pain
• muscle pain
• pain in arms or legs
• diarrhea
• nausea
• vomiting
• flushing

Additional common side effects of Uptravi in adults include:

• pain in joints
• low red blood cell count
• redness, pain, or swelling at the injection site
• rash
• decreased appetite

Additional common side effect in children includes stomach (abdominal) pain.

If you have a condition called pulmonary veno-occlusive disease (a narrowing of the veins in your lungs), Uptravi may cause fluid to build up in your lungs, known as pulmonary edema. If your doctor confirms this is happening, they will stop your treatment.

These are not all of the possible side effects of Uptravi. Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

• Store Uptravi tablets at room temperature between 68 °F and 77 °F (20 °C and 25 °C).
• Store Uptravi 100 mcg or 150 mcg tablets in the original package to protect from moisture.

Keep Uptravi and all medicines out of the reach of children.

Uptravi tablets

Active ingredient: selexipag

Inactive ingredients: corn starch, D-mannitol, hydroxypropyl cellulose, low substituted hydroxypropyl cellulose, and magnesium stearate. The tablets are film-coated with a coating material containing carnauba wax, hypromellose, propylene glycol, titanium dioxide, along with mixtures of iron oxide black, iron oxide red, and/or iron oxide yellow. The coating material of the 100 mcg and 150 mcg tablets also contains talc.

Uptravi for injection

Active ingredient: selexipag

Inactive ingredients: glycine, phosphoric acid, polysorbate 20, and sodium hydroxide.

Uptravi is manufactured for Actelion Pharmaceuticals US, Inc., Titusville, NJ 08560, USA.