Treprostinil Inhaled (Tyvaso)

Updated October 2023

Tyvaso DPI is an inhaled medication to treat pulmonary arterial hypertension, also known as Group 1 pulmonary hypertension, and interstitial lung disease, or Group 3 pulmonary hypertension. Tyvaso DPI may improve patients’ ability to exercise, reduce strain on the heart and lessen disease progression.

The Food and Drug Administration approved Tyvaso DPI to treat PAH and interstitial lung disease in May 2022.

How trepostrinil inhaled works

Trepostinil is a manufactured prostacyclin. Prostacyclins are hormones that affect blood vessel dilation and are low in people with pulmonary hypertension. Tyvaso DPI works by increasing the prostacyclin level. The medication is delivered directly to the lungs to widen narrowed blood vessels in the lungs. The medicine also can prevent platelets from clumping together to form blood clots. Relaxing and widening of the blood vessels in the lungs reduces pulmonary pressure. Lower pressure in the pulmonary arteries decreases the resistance or workload of the right ventricle to pump blood to the lungs.

Dosing and administration

Tyvaso is an inhaled medication formulated to use with the Tyvaso Inhalation System, a portable nebulizer that can be used at home or on the go.

Generally, therapy begins with three breaths four times a day for the first two week. After that, the dosage will gradually by one to three breaths four times a day, every week or two. The goal is to reach a target maintenance dose of nine to 12 breaths four times a day. You and your doctor will determine a maintenance dose that works for you. If you miss a treatment session, resume therapy as soon as possible at the usual dose.Initial dosage: One 16 microgram cartridge per treatment session. Dosage typically increases 16 mcg per treatment session at one- to two-week intervals, if tolerated. Your physician will determine a target maintenance dose. At higher doses, you might need two cartridge strengths per treatment session to achieve the dose.

Recommended daily routine

Set-up device every morning.

Take the medicine four times a day to get the full benefit of the medicine (morning, midday, dinner and bedtime). Space treatments at four to six hours apart. Each session takes two or three minutes.

Clean device every night.

Storage

Tyvaso comes in plastic ampules packaged in a foil pouch. When the ampules are stored in an unopened pouch at the recommended temperature, you can use them until the expiration date printed on the pouch. Don’t use the ampules after the expiration date. You don’t need to refrigerate the ampules, but you should store them away from light.

Use ampules within seven days after opening the foil pouch.

Don’t keep the medicine in the nebulizer cup for more than one day. Throw away medicine left in the medicine cup at the end of the day.

See the instruction manual for information on assembly, care and troubleshooting.

Obtaining treprostinil inhaled

Tyvaso DPI is a limited-distribution medication, which means you can’t buy it at a local pharmacy. It must be prescribed by a physician, and you’ll need insurance approval before starting therapy. Once you obtain prior authorization, a specialty pharmacy nurse will visit you at home to train you to set up and maintain the device. The nurse will show you how to administer and adjust treatments. Your specialty pharmacy will mail a four-week supply of disposable inhalers and cartridges directly to you. Specialty pharmacies include Accredo and CVS Specialty Pharmacy.

Insurance coverage

Most health insurance plans will pay part of the cost of this medication. However, some plans leave patients with high out-of-pocket costs. Depending on your plan, you might be eligible for financial assistance through a charitable foundation or the drug manufacturer’s patient assistance program. Ask your specialty pharmacy or prescribing physician about financial assistance. Find more information from PHA or call 301-565-3004.

Side effects

  • Cough.
  • Headache.
  • Throat irritation/pain.
  • Nausea.
  • Reddening of the face and neck (flushing).
  • Dizziness/fainting or loss of consciousness.
  • Shortness of breath.

Possible serious side effects

  • Low blood pressure. If you have low blood pressure, this medicine can further lower your blood pressure.
  • Bleeding problems, especially for people who take blood thinners.
  • Bronchospasm, a tightening of the muscles that line the airways in your lungs.

Drug interactions

Discuss the medicines you take with your health care provider before starting a new treatment.

  • Blood pressure or heart disease medications may need to be adjusted.
  • Medicines that decrease blood clotting.
  • Water pills (diuretics).
  • Gemfibrosil (Lopid) or Rifampin (Rimactane, Rifadin, Rifamate, Rifater).

Special considerations

Pregnancy and breastfeeding: Tyvaso DPI hasn’t been studied in pregnant or breastfeeding woman. It’s not known whether the medication will harm a fetus or can be passed into breast milk. Talk to your health care provider if you are pregnant, plan to become pregnant or are breastfeeding.

Children: Tyvaso hasn’t been studied in patients younger than 18 years old.

Geriatric patients: The treatment effects and safety profile observed in geriatric patients were similar to those for younger patients.

Liver impairment: People with mild to moderate liver disease may require lower doses and slow dose increase. Treprostinil hasn’t been studied in people with severe liver disease.

Kidney impairment: No dose adjustments needed for people with renal impairment.