Currently, there are six FDA-approved prostacyclin medications available. Of these available products,
none are orally administered, however there are some oral prostacyclin products under clinical
development.
1. Epoprostenol (Flolan®)
2. Epoprostenol (Veletri®)
3. Treprostinil, IV (IV Remodulin®)
4. Treprostinil, SQ (SQ Remodulin®)
5. Iloprost (Ventavis®)
6. Inhaled treprostinil (Tyvaso®)
Process to Initiate Prostacyclin Medications
These medications are provided through only a few specialty pharmacies.
- Accredo is the only pharmacy that may dispense epoprostenol.
- Treprostinil is provided by Accredo or Caremark specialty pharmacies.
Prescription forms are completed by the clinician and faxed to the specialty pharmacy, who then verifies the patient’s insurance benefits. Once this is completed, the patient (and their partner) undergoes extensive training by the specialty pharmacy nurse responsible for prostacyclin drug education.
Intravenous Prostacyclins (Epoprostenol IV, Treprostinil IV)
- All IV prostacyclin medications are infused continuously through a dedicated central line.
- Patients undergo extensive training on pump operation, mixing of their medication, and care of their central line catheters.
- Epoprostenol has been on the market the longest and is considered the gold standard for patients with advanced PAH.
- Flolan® and Veletri® are both epoprostenol. The difference is in the diluent and the buffer used with each product. Veletri® has a higher pH and is stable and room temperature. Flolan® requires ice packs if a single cassette is infused over 8 hours.
- Epoprostenol has a short half-life, 3 to 6 minutes.
- Treprostinil is stable at room temperature and has a 4 hour half-life.
- Epoprostenol can only be infused with a CADD Legacy pump.
- Treprostinil is generally started with use of a CADD Legacy pump. Some patients can be transitioned to smaller, miniaturized pumps.
- Epoprostenol is mixed daily whereas IV treprostinil cassettes are mixed every 2 days.
Subcutaneous Prostacyclins (Treprostinil SQ)
- Treprostinil was first approved for administration using a continuous SQ infusion, and later approved for use intravenously in patients who fail SQ therapy.
- In clinical trials, most patients experienced some degree of site pain, however only 7 percent discontinued therapy due to site discomfort.
- Many options are available to treat site pain, including topical or oral medications.
- Site pain is worse for the first 5 to 7 days after change of the site, thus patients typically leave sites in for extended periods of time.
- Site pain varies from patient to patient and from site to site.
Inhaled Prostacyclins
- Iloprost is an inhaled prostacyclin which is dosed 6 to 9 times per day. Treatments take 5 to 10 minutes.
- Inhaled treprostinil is dosed at 9 breaths QID.
- Inhaled prostacyclins do not deliver as much prostacyclin as IV or SQ therapies and should not be chosen over those prostacyclins in patients with advanced disease.
Prostacyclin Side Effects
- Prostacyclins have significant side effects, thus dosing begins in very low doses with small increases based on how well patients tolerate and manage the side effects.
- Side effects include jaw pain, headache, nausea, vomiting, diarrhea, extremity pain, flushing and thrombocytopenia.
- Most patients will experience side effects and will require medications for pain and nausea.
- Cough is the most common side effect with inhaled prostacyclins.
Continuous Prostacyclin Dosing
- Patients are generally started on IV therapy in the hospital setting after placement of an indwelling central line catheter.
- Dosing is calculated in nanograms per kilogram per minute and generally starts at 1 to 2 ng/kg/min. The dose is increased slowly by 1 or 2 ng/kg/min every 12 to 24 hours, then patients are typically discharged home with a dosing plan that continues dose increases 2 to 3 times per week. Dosing increments may need to be individualized based on side effects.
Safety Measures for Hospital Administration of Prostacyclins
- Most nurses will have limited exposure to home infusion pumps and will require on-going training if admitted patients are kept on their ambulatory infusion pumps.
- Prostacyclins have a very narrow therapeutic range and abrupt changes in dosing can be life threatening.
- Prostacyclin infusions should NEVER be abruptly stopped and dose changes over 3 ng/kg/min should be verified with the PH expert clinician.
- Policies should include a mandatory two nurse sign-off whenever a dose is changed or a new cassette is placed in the pump.
- No medications or other infusions can be infused in the same line as the IV prostacyclin.
- The patient’s back-up pump should be in the patient’s room and the patient should be involved in all aspects of the prostacyclin infusion, as they have been highly trained.
|