In PAH, arteries in the lungs become narrow, thickened and rigid. Narrowing of the blood vessels increases lung pressures, which causes stress and strain on the right side of the heart. The right heart must work harder to push blood to the lungs through those arteries. Over time, the right heart function declines as lung pressures continue to increase, leading to right heart failure.

Selexipag is a prostacyclin-receptor agonist. Prostacyclin receptors are located in the arteries of the lungs. Selexipag blocks abnormal growth of the muscular layer of the blood vessels, which widens the blood vessels and lowers lung pressures.

Low lung pressures reduce stress and strain on the right heart, which improves heart function. Improved heart function generally slows disease progression and increases survival for people with PAH.

Selexipag comes in 60-tablet bottles in the following doses: 200 mcg, 400 mcg, 600 mcg, 800 mcg, 1,000 mcg, 1,200 mcg, 1,400 mcg or 1,600 mcg. Selexipag also comes in a dose titration pack that includes a 140-count bottle of 200 mcg tablets and a 60-count bottle of 800 mcg tablets.

How to take this medication:

• Take the table twice a day, once in the morning and once in the evening.
• Take the tablet with food for better toleration.
• Swallow tablets whole. Do not split, crush or chew.
• Your doctor will start you at the lowest dose — one 200 mcg tablet — and slowly increase the dosage to find what works best for you. Dosage generally increases 200 mcg weekly.
• Your long-term (maintenance) dosage is determined by how well you tolerate side effects. Your doctor will increase the dose until side effects are intolerable.

Note: If you can’t eat or drink for any reason, your doctor should have an alternative treatment plan for periods when you can’t ingest anything by mouth.

Uptravi is a limited-distribution medication that you can’t buy at a local pharmacy. You’ll need a prescription from your pulmonary hypertension specialist and prior authorization from your insurance. After your insurance is approved, one of two specialty pharmacies — Accredo or CVS specialty – will ship the medication to you.

These side effects are more common while your dose is increasing the dose and less common on your maintenance dose.

• Headache
• Diarrhea
• Jaw pain
• Nausea
• Muscle pain
• Vomiting
• Pain in arms and legs
• Flushing
• Joint pain
• Anemia
• Decreased appetite
• Rash
• Hyperthyroidism (overactive thyroid)
• Allergic reaction is possible but unlikely

Certain patient populations must take precautions while taking selexipag, while others should avoid it. Learn about who can and who shouldn’t take this medication:

Pregnant and breastfeeding patients: There are no adequate, well-controlled studies of pregnant women. However, in most circumstances, women shouldn’t become pregnant while being treated for PAH. In animal studies, maternal and fetal weights fell slightly when pregnant rats received 47 times more than the maximum human dose during the stage of pregnancy when tissues develop into organs. However, there was no evidence of other abnormalities in the rats and none when pregnant rabbits received selexipag at 50 times higher than the maximum human dose.

Selexipag has been detected in milk from lactating rats. Although it’s unknown whether it shows up in human milk, many drugs pass to milk. Nursing mothers should discontinue breastfeeding or selexipag because of possible adverse effects.

Children and teens: Safety and effectiveness for pediatric patients is unknown.

Older adults: No significant differences have been seen or reported in older patients compared to younger patients, but a greater sensitivity to the medication can’t be ruled out.

People with liver disease: No dose adjustment is required for people with mild liver impairment (Child-Pugh Class A). Selexipag should be taken once daily for people with moderate liver impairment (Child-Pugh Class B). People with severe liver impairment (Child-Pugh Class C) shouldn’t take selexipag.

People with kidney disease: People with moderate or severe kidney disease (Stage 3-4 kidney disease) don’t have to adjust their dose. There is no clinical experience for people with end-stage kidney disease (Stage 5) or who are on dialysis.

Avoid taking medications such as gemfibrozil, which can increase selexipag levels in the blood. Tell your PH provider or pharmacist about other medications you take (including over-the-counter drugs and herbal supplements) before starting any new drug.

Dose adjustments aren’t necessary for other PAH medications, warfarin or lopinivir/ritonavir.