Prostanoids for Treatment of PH

Flolan, Remodulin, Veletri, Tyvaso, Ventavis, and Orenitram: Prostanoid therapies used to treat PH

Drugs targeting the prostacylin pathway are called prostacyclins and their goal is to increase the level of prostacylin in the body. Until recently prostacyclins were only available as continuous infusions or inhalations. The FDA approved the first oral prostacyclin Orenitram in December 2013.  It is important for each patient to discuss each therapy with their physician and to understand the risk, benefits, and alternatives of therapy before they choose which medication is right for them.

Flolan (Epoprostenol) a continuous infusion used to treat PH

Flolan was the first medication approved by the FDA for the treatment of pulmonary arterial hypertension. It is a prostacyclin only available as a continuous infusion requiring a central line to be placed and maintained for the duration of treatment. It is known as the gold standard of treatments for pulmonary hypertension because it has been used for an extended period of time, has been researched extensively, and physicians have a high comfort level of how to use it and what to expect from it. It has a short half-life, meaning if the medication is stopped the levels in the blood diminish rapidly. Because of the short half-life, any interruption in therapy from a pump or line malfunction or human error is considered a life threatening emergency. This medication is not stable at room temperature and ice packs must be used at all times to keep the medication cold. The patient should understand the risks, benefits, and alternatives of this medication before treatment is initiated.

Flolan is generally initiated while the patient is in a hospital. It is provided by a specialty pharmacy that is focused on providing therapies to pulmonary hypertension patients. The patient is educated either by a pulmonary hypertension nurse coordinator from the pulmonary hypertension clinic or by a nurse specializing in pulmonary hypertension with the specialty pharmacy. This medication requires mixing vials of the medicine with diluent, programming a small infusion pump, and maintaining a central line. The pump used for Flolan is called a CADD Legacy and weighs 3-5 pounds. The pump comes with a special carrying case that allows for ice packs to be kept around it at all times. Many patients carry the pump over their shoulder like a purse or around their waist like a belt. The patient will be managing the medication at home independently with assistance by the specialty pharmacy or clinic nurses as the need arises.

Flolan is initiated at a low dose and then slowly increased over time. A dosing sheet will be provided by the specialty pharmacy and outlines how to mix the medication and how to program the pump. A copy of the dosing sheet should be kept in the carrying case with the pump at all times as this is the only way to know what dose of Flolan you are currently on. It is important to stay in close communication with the physician’s office as the dose of Flolan is being titrated up. This medication, like all medications used to treat pulmonary hypertension, is known to have side effects. Not every patient will experience the same side effects while being treated with Flolan. Common side effects reported by patients using Flolan are headache, nausea, vomiting, diarrhea, jaw pain, leg pain, and flushing. Patients experiencing side effects should report their symptoms to their physician as there are several methods for helping to alleviate these effects.

It is very important that the infusion of Flolan is never interrupted as an unexpected stop in the medication can be life threatening. The patient is responsible for ordering supplies and medication through the specialty pharmacy monthly. It is important that the specialty pharmacy always have an accurate up-to-date address and phone number for the patient to avoid interruptions in therapy. Flolan is mixed daily using sterile technique. An emergency kit with extra medication, supplies, and a back up pump should be kept near the patient at all times.

Veletri (Epoprostenol) a continuous infusion used to treat PH

Veletri is the newest form of Epoprostenol. It is the same medication as Flolan but is stable at room temperature. It is infused continuously through a central line and is dispensed by a specialty pharmacy. Please see the above information under Flolan for more information. The patient should understand the risks, benefits, and alternatives of this medication before treatment is initiated.

Remodulin (Treprostinil) used to treat PH

Remodulin is a prostacyclin that is available as a continuous intravenous (IV) infusion or a continuous subcutaneous (SQ) infusion. The IV version is similar to Flolan in that it is infused continuously through a central line by a small pump, usually the CADD Legacy, that the patient carries on them at all times. It is generally initiated while the patient is in the hospital and is dispensed by a specialty pharmacy. The SQ version can be initiated in an outpatient clinic or in the hospital.  With either form the patient is educated either by a pulmonary hypertension nurse coordinator from the pulmonary hypertension clinic or by a nurse specializing in pulmonary hypertension with the specialty pharmacy. This medication is stable at room temperature alleviating the need for ice packs. It has a half-life of 3-4 hours making an interruption in therapy an urgency but not an emergency. The IV requires mixing vials of the medicine with diluent or saline, programming a small infusion pump, and maintaining a central line. The SQ form does not require mixing of the drug but the patient draws undiluted Remodulin up into a syringe and loads a small pump the size of a large cell phone. The medication is then infused through a small catheter that the patient places in their subcutaneous tissue usually on their belly.   The patient will be managing the medication at home independently with the assistance of the specialty pharmacy or clinic nurses as the need arises.

Remodulin is initiated at a low dose and then slowly increased over time. A dosing sheet will be provided by the specialty pharmacy and outlines how to mix the medication and how to program the pump. A copy of the dosing sheet should be kept with the patient at all times. It is important to stay in close communication with the physician’s office as the dose of Remodulin is being titrated up. This medication, like all medications used to treat pulmonary hypertension, is known to have side effects. Not every patient will experience the same side effects while being treated with Remodulin. Common side effects reported by patients using Remodulin are headache, nausea, vomiting, diarrhea, leg pain, and flushing. Patients experiencing side effects should report their symptoms to their physician as there are several methods for helping to alleviate these effects.

It is very important that Remodulin is not stopped abruptly.  A sudden stop in the medication can be life threatening. The patient is responsible for ordering supplies and medication through the specialty pharmacy monthly. It is important that the patient always provide an accurate up-to-date address and phone number to their physician and specialty pharmacy to avoid interruptions in therapy. IV Remodulin is mixed every other day, SQ Remodulin is changed every 3 days using sterile technique. An emergency kit with extra medication, supplies, and a back up pump should be kept near patient’s on Remodulin at all times.

Tyvaso (Treprsotinil) an inhaled therapy for PH

Tyvaso is the inhaled version of Remodulin. It can be started in the patient’s home, pulmonary hypertension clinic, or hospital. It is dispensed by a specialty pharmacy and requires a special piece of equipment that allows the patient to inhale the medication. The patient is educated either by a pulmonary hypertension nurse coordinator from the pulmonary hypertension clinic or by a nurse specializing in pulmonary hypertension with the specialty pharmacy. The patient will fill the inhalation device once at the beginning of the day and clean the device once at the end of the day. The patient will take several breaths of the medication four times per day.

Tyvaso is initiated at a low dose and then slowly increased over time. The dose is increased by adding breaths per treatment. Treatment is initiated at 3 breaths four times a day and eventually increased to 9 breaths four times a day. It is important to stay in close communication with the physician’s office as the dose of Tyvaso is being titrated up. This medication, like all medications used to treat pulmonary hypertension, is known to have side effects. Not every patient will experience the same side effects while being treated with Tyvaso. Common side effects reported by patients using Tyvaso are headache, cough, sore throat, and flushing. Patients experiencing side effects should report their symptoms to their physician as there are several methods for helping to alleviate these effects. The patient should understand the risks, benefits, and alternatives of this medication before treatment is initiated.

The patient is responsible for ordering Tyvaso supplies and medication through the specialty pharmacy monthly. It is important that the patient always provide an accurate up-to-date address and phone number to their physician and specialty pharmacy to avoid interruptions in therapy. The patient should notify all parties involved in their care if they will be undergoing any type of procedure that would make it difficult for them to perform their inhalations, any procedure that will require anesthesia, as the patient may have to switch to a different continuously infused prostanoid therapy to avoid a gap in therapy. Hospitals do not have the special inhalation device used to administer Tyavso so the patient should bring their own equipment whenever they are admitted.

Orenitram (Treprostinil) an oral therapy for PH

Orenitram is the oral version of Trepostinil.  It can be initiated as an outpatient or inpatient.  The drug was approved by the FDA in December of 2013 and became commercially available in June 2014.  Orenitram is dispensed by a specialty pharmacy and is a very complicated oral therapy requiring titration, several pill strengths, a dosing sheet, and visits from a specialty pharmacy nurse.  It can be taken twice a day or three times a day.

Orenitram is started at a low dose and slowly titrated over time.  It is important to stay in close contact with your PH specialist and specialty pharmacy nurse during the titration process.  Common side effects from Orenitram include headache, nausea, vomiting, diarrhea, leg pain, and flushing.  Notify the prescriber’s office if you should experience these side effects.

It is very important to take every dose of Orenitram, an abrupt stop can lead to worsening PAH.  Notify your physician right away if you have missed a dose or if you will be undergoing any kind of procedure that would require you to be NPO (nothing by mouth).  It may be necessary to transition patients to Remodulin in order to continue to safely administer Trepostinil around and during a procedure. The patient should understand the risks, benefits, and alternatives of this medication before treatment is initiated.

Ventavis (Iloprost) an inhaled therapy for PH

Ventavis was the original inhaled prostacyclin. It can be started in the patient’s home, pulmonary hypertension clinic, or hospital. It is dispensed by a specialty pharmacy and requires a special piece of equipment that allows the patient to inhale the medication. The patient is educated either by a pulmonary hypertension nurse coordinator from the pulmonary hypertension clinic or by a nurse specializing in pulmonary hypertension with the specialty pharmacy. The Ventavis is added to the inhalation equipment before each dose of medication and is administered as a breathing treatment rather than separate breaths (more like a nebulizer than an inhaler). The drug is administered six to nine times a day and the equipment is cleaned once a day.

Ventavis is not titrated up like other forms of prostacyclins. The patient is on a steady dose from the initiation of therapy. It is still important to stay in close communication with the physician’s office after initiated Ventavis as the drug does have side effects such as headache, cough, sore throat, and flushing. These side effects should be communicated with the physician as there are several methods for helping to alleviate these symptoms. The patient should understand the risks, benefits, and alternatives of this medication before treatment is initiated.

The patient is responsible for ordering Ventavis supplies and medication through the specialty pharmacy monthly. It is important that the patient always provide an accurate up-to-date address and phone number to their physician and specialty pharmacy to avoid interruptions in therapy. The patient should notify all parties involved in their care if they will be undergoing any type of procedure that would make it difficult for them to perform their inhalations, any procedure that will require anesthesia, as the patient may have to switch to a different continuously infused prostanoid therapy to avoid a gap in therapy. Hospitals do not have the special inhalation device used to administer Ventavis so the patient should bring their own equipment whenever they are admitted.