Treprostinil is a manufactured prostacyclin, a hormone produced by the innermost layer of blood vessels. Prostacyclin helps those blood vessels relax and widen. Many PH patients don’t make enough prostacyclin.

Treprostinil inhalation powder is delivered directly to the lungs to widen narrowed blood vessels. It also can inhibit platelets from clumping together to form blood clots. Relaxing and widening of the blood vessels in the lungs decreases pulmonary pressures. Lower pressure in the pulmonary arteries decreases the resistance or workload of the right ventricle to pump blood to the lungs.

Treprostinil inhalation powder is taken with a small, dry powder inhaler device that fits in the palm of your hand. You must use only the inhaler supplied by the manufacturer. You will receive five inhalers each month.

How to use your device:

• Insert the medication cartridge into the Tyvaso DPI inhaler for each treatment.
• Take one breath (one inhalation) from a single-dose cartridge.
• Repeat every four hours.
• Discard the inhaler after seven days of use and replace with a new inhaler.

You will start with one 16 microgram cartridge per treatment session. Your dosage likely will increase 16 mcg per treatment session every week or two. Your doctor will determine a target maintenance dose. As you take higher doses, you can use two cartridges per session.

If you miss a scheduled treatment session, resume as soon as possible at the usual dose.

If you go to the emergency room or are admitted to the hospital, bring your nebulizer and medication ampules with you.

What the cartridges look like:

Treprostinil inhalation powder comes in single-dose, color-coded cartridges filled with treprostinil:

• 16 mcg (purple)
• 32 mcg (dark blue)
• 48 mcg (light blue)
• 64 mcg (light green)

Use unopened Tyvaso DPI cartridges within eight weeks if not refrigerated or stored at room temperature (68 F to 77 F).

Refrigerate cartridges at 36-46°F. You can store them in the refrigerator until the expiration date on cartridge.

If refrigerated, bring the cartridge and inhaler to room temperature for 10 minutes before using.

Use opened Tyvaso DPI cartridges or blister packs within three days. Don’t store them in the refrigerator.

Treprostinil dry inhalation powder is a limited-distribution medication, which means you can’t buy it at a local pharmacy. A physician must prescribe the medication, and you’ll need insurance approval before starting therapy. Once you receive prior authorization from your insurance company, a specialty pharmacy will ship the medication, two nebulizers and relevant supplies to your home. A specialty pharmacy nurse will teach you how to administer the medication properly. Specialty pharmacies include Accredo and CVS Specialty.

Inhaled treprostinil is generally well tolerated. Common side effects include:

• Cough.
• Throat irritation/pain.
• Headache.
• Nausea.
• Reddening of the face and neck (flushing).
• Diarrhea
• Dizziness

More serious side effects include:

• Low blood pressure. Your health care team will frequently monitor your blood pressure because of the medication’s potential to lower systemic blood pressure.
• Risk of bleeding, especially for people who take blood thinners (anticoagulants).
• Bronchospasm, a tightening of the muscles that line the airways in your lungs.

Avoid getting treprostinil in your eyes or on the skin. If the medication gets in your eyes or on your skin, rinse with water immediately.

Talk to your health care team if you experience side effects while using Tyvaso DPI. Your doctor’s office and specialty pharmacy nurse will monitor you for toleration/side effects when you start the medication and when your dosage increases.

Your doctor will assess your ability to exercise will taking treprostinil inhalation powder.

Let your health care provider know the medications you are taking (including over the counter) before starting a new treatment. Other possible interactions include:

• Medicines that decrease blood clotting (anticoagulants).
• Water pills (diuretics).
• Gemfibrozil (Lopid) or rifampin (Rimactane, Rifadin, Rifamate, Rifater)

Note: Treprostinil isn’t cleared by dialysis. Treprostinil doesn’t affect warfarin use.

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

Pregnant and breastfeeding patients
Treprostinil inhalation powder hasn’t been studied in pregnant or breastfeeding women. It’s unknown whether the medication will harm an unborn baby 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 and teens
Treprostinil inhalation powder hasn’t been studied in patients younger than 18 years of age.