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.

Oral inhalation treprostinil therapy is inhaled through the mouth to deliver the medication directly to the lungs. Treprostinil can be taken as a nebulized solution or by dry powder inhaler. The medication is inhaled three to five times per day with each dose at least four hours apart.

Tyvaso is a liquid version of oral inhalation treprostinil therapy. Patients inhale the medication by mouth using a handheld nebulizer supplied by the drug manufacturer. The medication can’t be used with types of nebulizer.

1. Set up and assemble the nebulizer each morning and add the medication ampule to the medication cup.
2. Take treprostinil four times a day, four hours apart: when you wake up, at lunch, dinner and bedtime. Each session takes two or three minutes.
3. Clean plastic pieces of the device each night. Plug in nebulizer each night to recharge battery.

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.

This medication comes in plastic ampules packaged in a foil pouch. Each ampule contains a one-day supply of treprostinil. Store in the pouch, away from light until you’re ready for use.

Use ampules with medication within seven days of opening the foil pouch.

You don’t need to refrigerate ampules.

Treprostinil 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. 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.