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 dilates the narrowed blood vessels in the lungs, which decreases lung pressures and reduces strain on the right heart. Lower lung pressures can improve heart function and alleviate shortness of breath, which helps people be more active.

Subcutaneous treprostinil is delivered continuously through a tiny tube (cannula) connected to a small battery-powered pump. Infusion catheters are placed under the skin, often in the fatty tissue in abdominal area, and must be changed periodically.

Available subcutaneous pumps:

• Remunity pump
• CADD-MS 3 infusion pump. The manufacturer no longer makes this pump, but a limited supply is available.

Intravenous treprostinil must be administered through a surgically placed central venous catheter. IV treprostinil is delivered via the CADD-SOLIS VIP pump (the same pump used for IV epoprostenol administration).

Available IV pumps:

• CADD SOLIS VIP
• CADD Legacy pump. There is a limited supply of this older model.

Don’t stop taking treprostinil or reduce your dosage without consulting your doctor. Abruptly stopping treprostinil can be fatal.

Your doctor generally will start you on 1.25-2.5 ng/kg/min of Remodulin and slowly increase your dose until your PAH symptoms improve with minimal side effects.

If you need IV treprostinil, a surgeon will implant a central venous catheter into your chest for IV infusion therapy. You will need to stay in the hospital for a few days. During your stay, your health care will teach you step-by-step to prepare, administer and store your medication. You will learn central line care, infection precautions, and how to operate your pump, troubleshoot pump problems and reach the 24-hour support hotline for the CADD pump.

If you need subcutaneous treprostinil, you can start in the hospital or at home. The specialty pharmacy nurse will come to your house when the drug is delivered and teach you how to use the medication.

Treprostinil comes in 20 ml vials. Vials come in four concentrations:

• 1 mg/ml
• 2.5 mg/ml
• 5 mg/ml
• 10 mg/ml

Don’t use a single vial more than 14 days after you open it. Make sure you follow proper storage procedures. The half-life of treprostinil is about four and a half hours.

Treprostinil is stable at room temperature for at least 48 hours for IV therapy or up to 72 hours with subcutaneous infusion. It doesn’t have to be refrigerated or kept cold with ice while being infused, and you can pre-mix and store cassettes.

Treprostinil has limited distribution, which means you can’t buy it a local pharmacy. You usually need approval from your insurance company before starting this therapy. Your insurance company will identify a specialty pharmacy, such as Accredo or CVS specialty, to fill your prescription every month. The specialty pharmacy will ship the medication, pumps and supplies to your home.

The side effects of treprostinil are similar to those of all prostacyclin agents. They include:

• Jaw pain
• Flushing of the skin
• Headache
• Low systemic blood pressure (hypotension, dizziness)
• Nausea/vomiting
• Diarrhea
• Muscle or joint pain
• Leg pain
• Subcutaneous or intravenous infusion-related reactions
• Infusion site pain/reaction, such as redness, swelling and rash (more often seen with subcutaneous infusion)
• Infection and bleeding at infusion site
• Blood stream infection (more often seen with IV infusion)

No other solution or medications should be infused with IV treprostinil. If you are admitted to a hospital that isn’t a Pulmonary Hypertension Care Center, inform the care team that your central line for IV treprostinil must not be flushed and shouldn’t be used for other medication infusions or blood draws. The infusion shouldn’t be stopped in any circumstance, including an emergency, unless the PH doctor orders it.

Allergic reactions to this medication are possible but not likely.

Certain patient populations must take precautions while taking ambrisentan, while others should avoid it. This section addresses special populations and considerations.

Pregnant and breastfeeding women
It’s not known whether Remodulin will harm your unborn baby or if treprostinil can pass through breastmilk. However, there are risks to the mother and the fetus associated with pulmonary arterial hypertension. If you are pregnant or plan to become pregnant, please talk to your doctor.

Children and teens
Treprostinil has been used in children. However, safety and effectiveness haven’t been established.

People with kidney disease
No dose adjustments are required in patients with renal impairment. Treprostinil is not cleared by dialysis.

People with liver disease
Treprostinil is cleared slowly in patients with liver disease. In patients with mild or moderate liver disease, treprostinil can be started at one-third less than the usual dose, such as 0.625 ng/kg/min of ideal body weight, and must be monitored closely. Remodulin hasn’t been studied in patients with severe liver disease.