Epoprostenol (Intravenous)

Overview

Epoprostenol is a vasodilator drug that helps to improve the health and physical stamina of patients with pulmonary hypertension. Pulmonary hypertension is the high blood pressure that forms in the major artery connecting the heart to the lungs. Smaller blood vessels in the lungs may become resistant to blood flow due to various reasons, which increase the workload of the heart as it then needs to pump harder to transport blood to the lungs. Epoprostenol helps to relax and dilate these blood vessels, which in turn allows for less work from the heart.

Patients starting with epoprostenol will usually receive treatment in a hospital or clinic, with the medicine administered by a healthcare professional. However they can request to receive the medicine at home, in which case the person giving the medicine (whether it is the patient themself or a caregiver) must be fully familiar with the procedure of preparing and infusing the medication.

Epoprostenol primarily comes in the form of a powder, which is to be reconstituted with a sterile diluent provided together with the medicine. Hygiene is of utmost importance while using this medicine.

Used correctly, epoprostenol that works as intended helps patients to maintain a good quality of life, by allowing them to exercise as normal and giving them the stamina they require to perform various daily activities.

Conditions Treated

  • Pulmonary arterial hypertension

Type Of Medicine

  • Vasodilator

Side Effects

When using epoprostenol, there is a chance that patients may encounter side effects, although not everyone who uses the medicine will do so. These side effects range from mild to severe. The following lists more common side effects that should go away after a while. However, if they persist or worsen, the patient should let their doctor know. They should also ask their doctor any questions that they have about any possible side effects

  • Nausea

More serious side effects may occur, although they are quite unlikely. If the patient experiences the following, they should seek immediate medical attention:

  • Changes in mental state or mood (such as anxiety, confusion or agitation)

Rare side effects on this medicine include:

  • Unusual sensitivity to touch and sensation

The above is not a complete list of side effects that a patient may encounter. If the patient notices any unusual sign or symptom, they should contact their doctor as soon as possible for further advice.

It is unlikely that a patient will have an allergic reaction to the medicine. However in the event that it happens, the patient should get emergency treatment. Patients should keep an eye out for:

  • Rashes

Dosage

Epoprostenol is infused into the patient via a catheter, which is placed into a vein in the chest which leads to the heart. Initial treatment with this medicine should take place at a hospital or clinic, with the medication administered to the patient. If the patient chooses to take the medicine at home, a qualified healthcare professional such as the doctor or nurse will show the patient or their caregiver how to use this medication. They will teach the patient or their caregiver how to prepare the medicine, and how to use the pump that is required for the infusion. This portable pump is operated by a computer, and will feed epoprostenol steadily and continously to the patient's heart, via the catheter.

During treatment, the doctor should perform regular checks on the patient to ensure that the medication is working as intended. If the patient notices any change or worsening of their condition, they should inform their doctor as quickly as possible.

As epoprostenol comes in a powder, it is mixed with a diluent to then be infused. It should be mixed only with the sterile diluent that is provided with the medication, and not with anything else. After reconstitution, the epoprostenol solution should not be mixed with other medicines or solutions.

The patient should follow the doctor's prescription exactly, and not use more, or less than the medicine they have been instructed to take.

The following describes the procedures used in handling epoprostenol for daily use. It should not be used as a replacement for a demonstration by a qualified healthcare professional such as a doctor or nurse. If the patient or caregiver has any questions regarding the procedures or medicine, they should clarify everything with their doctor or nurse prior to using epoprostenol.

For reconstituting epoprostenol daily:

  • Clear an area large enough to work in, and clean it thoroughly with alcohol. Ensure that all supplies are present.

Withdrawing the sterile diluent:

  • Attach a needle to the syringe if it has not already been attached. Without using force, pull the plunger out slightly, before pushing it back to break the syringe seal. Proceed to draw air into the syringe. The amount of air should be about the same as the amount of sterile diluent required.

Reconstituing the epoprostenol:

  • Take the vial of epoprostenol and push the needle from the same syringe past the rubber seal of the vial.

Withdrawing epoprostenol that has been reconstituted:

  • Clean the top of the vial with the reconstituted epoprostenol by wiping with an alcohol swab. Allow to dry.

Injecting the epoprostenol solution into the cassette:

  • Take off the end cap of the cassette tubing.

Injecting remaining diluent into the partially filled cassette:

  • Affix a new needle to a 60 mililitre (ml) syringe. Follow the same instructions for breaking the rubber seal of the syringe, and wipe the tops of the sterile diluent vials as above.

Injecting the sterile diluent into the cassette:

  • Remove the cap from the cassette tubing (which is still clamped).

Removing air from the cassette:

  • Turn the cassette slowly, so that the air bubbles will combine to form an air pocket, and tilt the cassette so that the air pocket reaches the corner where the cassette is attached to the tubing. -Unclamp the tube. Pull the plunger of the syringe until the solution fills the tubing, before clamping the tube near the connector.
  • Put the cassette into the refrigerator. To prevent spilling food and drink onto it, it is advisable to keep the cassette on the top shelf of the refrigerator and in its own box.

Using the portable pump:

  • The portable pump that is required for using the epoprostenol solution comes in different makes and models. Therefore, instructions for the pump may vary. The doctor or nurse should be able to advise the patient or their caregiver on how to use and take care of their portable pump, as well as the accessories that will come with it.

If the entire vial has been mixed with the 5ml solution provided, the solution can be infused by the pump for up to 24 hours at room temperature. If a lower concentration is being used, it can only be infused for up to 12 hours. The solution should not be placed in direct sunlight.

In the case of any problems arising with the portable pump, the patient or their caretaker should contact their doctor. They may be given a second pump in the event that the original pump stops working. The patient should ensure that the back-up pump is within reach at all times.

Before using any of the vials, the patient or their caretaker should inspect the fluid in the vials. If they notice any solid pieces, specks, changes of color or any other signs of contamination or change, they should discard the vial and use another.

It is important to use only the supplies provided by the doctor or that are in the packaging, as supplies from other sources may not have the wanted effect. For instance, the provided infusion tubing has a filter to remove specks and particles in the liquid.

Epoprostenol may be required for several years by the patient. If the patient has any questions or concerns about the length of treatment, they should discuss it with their doctor prior to beginning treatment with epoprostenol.

The patient's dosage will depend on the concentration of their reconstituted medication, as well as the rate of the medicine's delivery by the infusion pump. They should take only the required amount of medication- no more, no less, and as their dosing schedule requires. The following lists an average dose, which may differ from the patient's:

For primary pulmonary hypertension, and pulmonary hypertension secondary to the scledroderma spectrum of disease:

  • For adults: The doctor will determine a dosage based on body weight. Initial dosage starts from about 2 nanograms (ng) per kilogram (kg) of the patient's body weight, infused per minute.

If the patient has missed a dose, they should contact their doctor right away to seek further medical advice.

Patients should not abruptly stop or decrease their intake of the medicine without prior approval from their doctor as it may lead to adverse and possibly serious reactions. On rare occasions these reactions may be fatal. If they wish to stop the medication, patients should consult their doctor and decrease their dosage gradually.

If dosage is interrupted at any point, patients should ask their doctor for further advice.

Interactions

Drugs may interact with each other, with various supplements or herbal products. These interactions may lead to an increased chance of side effects, or affect the efficiency of the medication. Patients should keep a list of any drugs, supplements or herbal products they are currently consuming so that they can share the list with their doctor or pharmacist. Sometimes, the doctor may prescribe a medicine that has been known to interact with epoprostenol if they deem it necessary. In these cases, patients should discuss with their doctor ways to reduce chances of side effects.

Patients should not start, stop or change the amount of any medicine or supplements they are currently taking without the consent or knowledge of their doctor.

Epoprostenol may increase the chance of unusual bleeding, and certain medicines may make it worse. Patients are highly advised to consult with their doctors if they are using or are planning to use:

  • Antiplatelet drugs (such as clopidogrel)

In certain cases, the doctor may prescribe a low dosage of aspirin for the patient in order to help prevent strokes or heart attacks. Digoxin may also be prescribed in conjunction with epoprostenol. If the patient has any questions about the interactions between aspirin or digoxin with epoprostenol, they should speak with their doctor for further advice.

The following is an incomplete list of medications that may interact with epoprostenol:

  • Acetaminophen

Alcohol and tobacco are common products that may interact negatively with epoprostenol. Patients should check with their doctor on whether they should use either alcohol or tobacco during treatment with this medicine.

Certain diseases may also affect the use of epoprostenol. Patients with the following conditions should let their doctor know prior to starting treatment:

  • Congestive heart failure

Warnings

Prior to starting epoprostenol, patients should tell their doctor if they have any allergies, whether it is to the medication itself, any other drugs, or food, dyes or ingredients. This is to prevent an allergic reaction, either to the drug, any inactive ingredients it contains, or materials from the accessories required for the infusion of the drug.

Patients should also tell their doctor about their medical history, especially if they have had the following conditions:

  • Heart failure (on the left side of the heart)

Epoprostenol may cause dizziness. Patients on this medicine should not perform any activities that require mental alertness until they are certain they can do them safely, such as driving or operating machinery. It is also advisable for patients to limit or even refrain from alcoholic drinks. Patients can help lower the chances of dizziness by rising slowly, if they have been in a sitting or lying down position.

For pregnant patients, this medication should only be used if necessary. Patients who are planning to become pregnant should inform their doctors prior to starting epoprostenol. Currently there is insufficient information on whether epoprostenol passes into breast milk. Breastfeeding patients should check with their doctors for further medical advice.

Epoprostenol is not expected to work differently with elderly patients. However they may be at increased risk for age-related heart, kidney and liver problems, which may require an adjustment in their dosage, which will be determined by their doctor.

If the patient is scheduled to go into surgery, they should let their doctor or healthcare givers know if they are on any medication.

Storage

The medicine vial should be stored in its original carton in a cool and dry place, at about room temperature. It should not be exposed to freezing temperatures at any point. Epoprostenol should also not be stored in the bathroom as it may be too humid.

Any mixed solution of epoprostenol may be kept in the refrigerator for up to 5 days, away from direct light. It may also be stored at room temperature, up to 77 degrees Fahrenheit, for up to 48 hours. After 5 days in the refrigerator or 2 days in room temperature, the solution should be thrown away. Medicine that has been frozen should also be discarded.

The medicine should be kept out of the reach of children or animals.

Once the medication is expired or no longer needed, the patient should dispose of it through the proper channels. If they are unsure on how to deal properly with medical waste, the patient should ask their doctor or local waste management.

Needles and syringes should not be reused. Once they have been used, they should be put in a puncture-resistant container to be disposed of. They are also considered to be part of medical waste.

Summary

Epoprostenol is a medicine used to treat pulmonary arterial hypertension, or high blood pressure in the main artery that connects the ventricle, or right side of the heart, to the lungs. Due to several factors, smaller blood vessels in the lungs may become more resistant to blood flow, which forces the ventricle to work harder in order to pump blood to the lungs. Epoprostenol works to decrease the workload on the heart, by relaxing these blood vessels and by increasing blood flow to the lungs.

By helping blood flow more easily from the heart to the lungs, epoprostenol helps patients to have an active and healthy life by allowing them to exercise and perform various daily activities. It also helps patients if they have trouble with breathing, and fatigue, thereby improving their quality of life.

This medicine primarily comes in the form of a powder, and is mixed with a sterile diluent that comes with it, before it is slowly and steadily infused into the patient via a portable infusion pump. Epoprostenol should not be mixed with any other solutions or medicine.

Before starting treatment, a doctor or nurse will show the patient or their caregiver how to properly prepare the medicine for use, and how to infuse it into the patient. Patients should clarify the procedure thoroughly before attempting it on their own. It is advised that patients make two cassettes to begin with, and then thereafter use the cassette of medicine that they prepared the previous day, so that they will have a back-up if it is required. It is also advised that they have a back-up infusion pump, as it may be detrimental to the patient's health if they miss a dose.

Hygiene during the procedure of infusion and preparation of the medicine is highly important when taking this medicine.

Certain other medications and conditions may affect the effectiveness of epoprostenol, or even increase the chances of the patient developing unwanted side effects. Medications of note include drugs that may increase chances of bleeding, such as antiplatelet drugs or NSAIDs. Patients who have congestive heart failure or pulmonary edema may be asked to refrain from using epoprostenol altogether as the negative effects of the medication on their health may outweigh any benefits it brings.

Pregnant or breastfeeding patients may want to discuss how epoprostenol may affect them as there are currently insufficient information and studies on how the drug affects babies and breastfeeding infants. For elderly patients, there is an elevated risk of age-related problems with the kidney, liver and heart.

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Epoprostenol belongs to a class of drugs known as vasodilators and work by relaxing blood vessels in the lungs so as to prevent work overload on the heart.

    • Heartburn
    • Vomiting
    • Diarrhea
    • Difficulty with bowel movements
    • Excessive gas
    • Dizziness
    • Unusual fatigue or sleepiness
    • Headaches
    • Lack of appetite
    • Lack of strength
    • Redness of the skin or flushing
    • Sweating
    • Pain in abdominal area
    • Pain in jaw
    • Pain or stiffness in muscles or joints
    • Hives or welts
    • Skin rashes and irritation
    • Swelling, redness or pain at the site of injection
    • Shaking or trembling in the limbs and extremities
    • Loss in interest or pleasure
    • Signs of infection (such as fever or chills)
    • Cold hands and feet
    • Skin that becomes pale, numb or tingles
    • Changes in skin color
    • Heartbeat that is irregular, or becomes faster or slower
    • Extreme fatigue
    • Slurred or incoherent speech
    • Changes in vision
    • Pain or discomfort in chest
    • Pain in the arm, back or jaw
    • Pain in bladder
    • Bloody or black, tarry stools
    • Unusual increase or decrease in urination
    • Bloody or cloudy urine
    • Difficult, burning or painful urination
    • Unusual bleeding or bruising, such as bleeding gums
    • Increase in menstrual flow or unusual vaginal bleeding
    • Coughing up blood
    • Metallic taste in mouth
    • Hoarseness or coughing
    • Dry mouth
    • Increase in thirst
    • Constipation
    • Convulsions
    • Difficulties in breathing or swallowing
    • Dilated or swollen neck veins
    • Unusual or rapid gain or loss in weight
    • Lips or fingernails turning blue
    • Sudden trouble with breathing
    • Noisy breathing
    • Seizures
    • Sudden or severe headache
    • Severe pain in chest
    • Temporary blindness
    • Weakness on one side of the body
    • Weakness in limbs
    • Pinpricks of red on the skin
    • Feeling of fullness or bloating
    • Sores, ulcers or white spots in the mouth or on lips
    • Insomnia
    • Swelling and itching, especially of the face, tongue and throat
    • Severe dizziness
    • Trouble with breathing
    • Wash hands with soap and water carefully.
    • Open all packages, and remove the vial cap from the container of sterile diluent. Clean the tops of the vials with alcohol swabs and ensure that the tops dry before continuing with the rest of the procedure.
    • Insert the needle through the vial's rubber seal at an angle, before turning both vial and syringe upside down into a vertical position. Press the plunger carefully to inject the air into the vial.
    • Place the tip of the needle into the sterile diluent and slowly pull the plunger back to retrieve the diluent, and allow the pressure to fill the syringe with it. If there is remaining air, push it into the vial so that the correct amount of sterile diluent is taken up into the syringe.
    • Tap the syringe lightly to allow any trapped air bubbles to rise to the top of the syringe. Do not withdraw the needle yet. Push the plunger gently if there are air bubbles, so they can be pushed into the vial. If needed, withdraw more sterile diluent so that the syringe maintains the required volume.
    • Allow the pressure in the syringe and vial to equalize once there is enough diluent, before withdrawing the needle slowly.
    • To help minimize the appearance of air bubbles, keep the syringe and vial in a vertical position. Ensure also that the needle tip does not leave the fluid while withdrawing the sterile diluent.
    • In order to prevent foaming of the medication, the diluent should be injected in a slow stream towards the side of the vial and not the powder itself. Allow the pressure in the vial and syringe to equalise before removing the needle from the vial.
    • To mix the medicine, swirl the vial gently, and turn it upside down as well to dissolve any bits of untouched powder which may be stuck at the top of the vial.
    • It is important not to shake the vials.
    • If more vials of epoprostenol are required, repeat the process as described.
    • Change the needle on the syringe. Pull back the plunger on the syringe until there is an equal amount of air in the syringe to the amount of reconstituted epoprostenol required.
    • Push the needle through the vial's rubber seal and inject the air into the vial.
    • Ensure that the tip of the needle is kept in the epoprostenol solution while withdrawing the fluid. Gently pull the plunger up so as to withdraw the needed amount of epoprostenol.
    • Follow the same instructions as when withdrawing the sterile diluent to remove or prevent any air bubbles that may have appeared.
    • Remove the needle from the vial and place the needle cap onto the syringe.
    • Remove the needle from the syringe and dispose of it properly. Be careful while removing the needle.
    • Connect the cassette tubing to the syringe. Hold the cassette in one hand while pushing the plunger so that the epoprostenol solution can be injected into the cassette. Use a table or similar structure to help steady the plunger if it is necessary.
    • Clamp the cassette tubing near the syringe once the syringe has been emptied of the solution. Remove the syringe and place the end cap onto the cassette tubing.
    • Pull the plunger of the syringe so that it is filled with the same amount of air as the amount of sterile diluent required from the first vial.
    • Push the needle past the rubber seal and inject some of the air into the vial. Allow some of the diluent to flow back into the syringe. Inject the remaining air into the vial slowly until all the diluent has been withdrawn into the syringe.
    • Remember to keep the tip of the needle in the fluid while removing the sterile diluent.
    • Wait for the pressure to equalize between the vial and syringe before removing the needle. If the pressure has not been equalized before the needle is removed, some of the sterile diluent may flow back into the vial. In this case, the procedure will need to be repeated from the very beginning.
    • Replace the needle cap after the needle has been removed.
    • It may be easier to withdraw the diluent while holding the syringe in a vertical, upside-down position.
    • Remove the needle from the syringe carefully, and dispose of it properly.
    • Connect the syringe to the cassette tubing before unclamping the tubing. Inject the solution into the cassette carefully.
    • Once the syringe is empty, clamp the tubing near the syringe again before removing the syringe. Replace the end cap of the cassette tubing.
    • If more sterile diluent is required to fill the cassette, repeat the above procedure with a new vial of diluent.
    • Once the required amount of sterile diluent has been added to the cassette, clamp the tubing. This time, do not remove the syringe while mixing the solution.
    • Turn the cassette upside down for ten times, if not more, so that the reconstituted epoprostenol can be mixed completely with the additional sterile diluent. Do not shake the cassette.

-Remove the syringe and replace the end cap of the cassette tubing. Label the cassette with the time and date of the cassette preparation.

  • It is also advisable to make a new cassette a day, and to use the stored cassette from the previous day, so that there will be a back-up if needed.
  • Instructions for the pump should include how to change battery for the pump, as well as for the cassette and the tubing.
  • Gel packs should be changed every 8 to 12 hours, especially if temperatures around it reach or are close to 86 degrees Fahrenheit.
  • It is important to make sure everything is clean and sterile. In the case of contamination, even if it is only suspected, all accessories should be discarded, and the process should be repeated from the beginning.
  • For children: The doctor will determine both usage and dose.
  • Pain or fever relieving medication (such as aspirin, ibuprofen or naproxen)
  • Medicines that increase blood pressure or heart rate
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