Tyvaso nebulized solution is an inhaled medication approved to treat two types of pulmonary hypertension:
- Pulmonary arterial hypertension, also known as Group 1 PH.
- Pulmonary hypertension associated with interstitial lung disease, also known as Group 3 PH.
This therapy helps improve the ability to exercise, reduce strain on the heart and prevent the disease from getting worse. The Food and Drug Administration approved Tyvaso in 2002. This medications also is available in a dry inhaled power, called Tyvaso DPI.
How treprostinil inhalation solution works
Treprostinil is a manufactured prostacyclin. Prostacyclin is hormone released by the lungs that affects how blood vessels dilate. Treprostinil increases the level of prostacyclin, which typically is low in people with PH.
Treprostinil inhalation solution is delivered directly to the lungs to widen narrowed blood vessels in the lungs. The medicine 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.
Dosing and administration
Treprostinil inhalation solution is administered with a handheld nebulizer. The portable nebulizer allows for dosing at home or on the go. Only treprostinil should be administered with the portable nebulizer.
- Your health care provider will work with you to determine the number of breaths per session you need each week until you reach the target maintenance dose of nine to 12 breaths per session. This process is called titration.
- Each breath equals a dose of six micrograms. Start with three breaths, four times a day.
- Three breaths equal 18 mcg.
- Increase the number of breaths per session weekly as directed by your health care provider.
- One ampule contains a one-day supply of treprostinil.
How to take treprostinil inhalation solution
- Set up the nebulizer each morning and add the medication ampule to the medication cup.
- Take treprostinil four times a day, four hours apart: when you awake and at lunch, dinner and bedtime. Each session takes two or three minutes.
- Clean plastic pieces of the device each night. Plug in nebulizer each night to recharge battery.
If you miss a scheduled treatment session, resume therapy 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.
Storage
Treprostinil comes in plastic ampules that are packaged in a foil pouch. Store in the pouch, away from light, until you’re ready to use. The ampules don’t need to be refrigerated. Use ampules with medication within seven days of opening the foil pouch.
Obtaining treprostinil inhalation solution
Treprostinil 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 have prior authorization from your insurance, a specialty pharmacy (Accredo Specialty Pharmacy or CVS Caremark Specialty Pharmacy) will send the inhalation system to your home. You will receive two nebulizer units. A specialty pharmacy home nurse will teach you how to administer the medication properly and care for the nebulizer.
Insurance coverage
Most health insurance plans will pay part of the cost of this medication. However, some plans leave you with high out-of-pocket costs. Depending on your insurance, your plan may cover treprostinil through your major medical or your prescription benefits. If your cost is high, talk with your specialty pharmacy or your prescribing physician about financial assistance through a charitable foundation or the drug manufacturer’s patient assistance program.
For questions, call 877-864-8437 to connect with a United Therapeutics Cares patient navigator. You may be eligible for the Tyvaso Co-Pay Assistance Program.
Visit PHA financial assistance resources or call us at 301-565-3004 if you have other questions.
Common side effects
Treprostinil inhalation solution is generally well tolerated. The most common side effects are:
- Cough.
- Throat irritation/pain.
Other possible side effects include:
- Headache.
- Nausea.
- Reddening of the face and neck (flushing).
- Dizziness/fainting or loss of consciousness.
- Shortness of breath.
Possible serious side effects include:
- Low blood pressure.
- Bleeding problems, especially for people who take blood thinners (anticoagulants).
Avoid getting treprostinil in your eyes or on the skin. If it gets in your eyes or on your skin, rinse with water immediately.
Monitoring side effects
Your doctor’s office and a nurse from the specialty pharmacy will monitor you for toleration/side effects at the initiation and titration phase of the medication. They will monitor your blood pressure frequently because of the medication’s potential to lower blood pressure. Your doctor will assess your ability to exercise while taking treprostinil.
Drug interactions
You should discuss medications (including over the counter) you take with your health care provider before starting a new treatment. Your doctor may need to adjust your med to treat systemic blood pressure or heart disease. Other potential interactions:
- 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.
Considerations for special populations
Pregnancy and breastfeeding: Treprostinil hasn’t been studied in pregnant or breastfeeding woman. It isn’t known 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.
Pediatric: Treprostinil hasn’t been studied in patients younger than 18.