An introduction to VELETRI (epoprostenol) for Injection: I ... · between the heart and the lungs. VELETRI may improve your ability to exercise as measured by how far you can walk

An introduction to VELETRI® (epoprostenol) for Injection:I.V. therapy made a little easier for patients

It is important to use VELETRI as directed by your doctor. VELETRI should not be used by patients with heart failure due to severe left heart disease, patients who develop fluid in the lungs (pulmonary edema) during dose initiation, or patients who are allergic to epoprostenol.

• Flexible preparation, storage, and usage options

• No ice packs required

• Up to once-weekly preparation

What is VELETRI?

VELETRI is a prescription medicine that is given intravenously (in a vein). It is used to treat adults with certain kinds of severe pulmonary arterial hypertension (PAH) (WHO Group 1), a condition in which blood pressure is too high in the blood vessels between the heart and the lungs. VELETRI may improve your ability to exercise as measured by how far you can walk in 6 minutes (6-minute walk test).

Studies showing VELETRI is effective included mainly patients with NYHA Functional Class III-IV PAH. In these patients, PAH was caused by unidentified or hereditary factors or connective tissue disease.

Please see Important Safety Information on page 3 and back cover, and see enclosed full Prescribing Information.

Please see enclosed full Prescribing Information.

Table of contentsIntroduction

Pulmonary arterial hypertension: The disease process

About VELETRI® (epoprostenol) for Injection

Related symptoms

How to use VELETRI

Your first shipment

Emergency supply kit

Contacting Accredo

Additional resources

VELETRI inventory worksheet

Central venous line care log

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Important Safety Information

IntroductionLiving with pulmonary arterial hypertension (PAH) isn’t easy. Actelion Pharmaceuticals US, Inc., the pharmaceutical company that makes VELETRI® (epoprostenol) for Injection, and Accredo Health Group, Inc., the specialty pharmacy that cares for patients, understand the challenges you face every day. That’s why we are pleased to bring you VELETRI, an intravenous (I.V.) medication designed to help make therapy a little easier. It offers flexible preparation, storage, and usage options and can be used for 24 hours at room temperature without the need for ice packs.

Read on to learn more about VELETRI, how it may help improve your ability to exercise (as measured by how far you can walk in 6 minutes) and some of your PAH symptoms, and what you can do to get the most from your therapy.

What is VELETRI?VELETRI is a prescription medicine that is given intravenously (in a vein). It is used to treat adults with certain kinds of severe pulmonary arterial hypertension (PAH) (WHO Group 1), a condition in which blood pressure is too high in the blood vessels between the heart and the lungs. VELETRI may improve your ability to exercise as measured by how far you can walk in 6 minutes (6-minute walk test).


Please see Important Safety Information on page 3 and back cover, and see enclosed full Prescribing Information.

Who should not take VELETRI?

VELETRI should not be used if you have heart failure due to severe left heart disease, if you develop fluid in the lungs (pulmonary edema) when starting therapy, or if you are allergic to epoprostenol.

Some medications may interact with VELETRI. Please talk to your doctor about all of your medications.

What is the most important information I should know about VELETRI?

It is important to use VELETRI as directed by your doctor. VELETRI should be used only with Sterile Water for Injection, USP, or Sodium Chloride 0.9% Injection, USP. Do not mix VELETRI with other intravenous medications. Each vial is for single use only; discard any unused medication. Use at room temperature (77°F/25°C). Do not expose VELETRI to direct sunlight.

When you take epoprostenol for the first time, you must be in a setting (hospital or clinic) where you can be monitored for any serious side effects or in case of emergency. Your blood pressure and heart rate should also be monitored with any dose changes. If you are taking VELETRI, your doctor may prescribe another kind of medicine used to prevent blood clots. It is the use of these medications concomitantly (in combination) with VELETRI that may potentially cause an increase in the risk of bleeding.

Sudden and dramatic changes in dose may lead to unstable blood pressure, a return of pulmonary hypertension symptoms, or fatal low blood pressure (hypotension). Do not stop using VELETRI without first talking to your doctor.

To reduce the risk of infection in the bloodstream, it is important to know how to properly care for the catheter and infusion pump.

What are the possible side effects of VELETRI?

You may have side effects at the start of treatment or with dose increases. The most common side effects seen in at least 1% of patients were flushing, headache, nausea/vomiting, low blood pressure, anxiety/nervousness, chest pain, dizziness, slow heartbeat, abdominal pain, pain in the muscles and/or ligaments and bones, shortness of breath, back pain, sweating, upset stomach, numbness/increased sensitivity, and fast heartbeat.

The most common side effects in patients with PAH due to unidentified or hereditary factors with at least 10% difference between the group that received epoprostenol and the group that received conventional therapy alone were flu-like symptoms, fast heartbeat, flushing, diarrhea, nausea/vomiting, jaw pain, pain in the muscles and/or ligaments and bones, anxiety/nervousness, dizziness, headache, and numbness/increased sensitivity/tingling.

The most common side effects in patients with PAH due to connective tissue disease with at least 10% difference between the group that received epoprostenol and the group that received conventional therapy alone were flushing, low blood pressure, lack of appetite, nausea/vomiting, diarrhea, jaw pain, neck/joint pain, headache, skin ulcer, and rash.

Talk to your doctor if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of VELETRI. For more information, ask your doctor or pharmacist.

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

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Once therapy with VELETRI® (epoprostenol) for Injection begins, it must continue uninterrupted, 24 hours a day, 7 days a week. Interruption of therapy administration may be life threatening because VELETRI stays in your body for only a short time.Because of this, it is necessary for VELETRI to be continuously infused.

VELETRI is administered intravenously (in a vein) and requires surgical placement of a long-term, specialized catheter (a thin, flexible tube) to provide an uninterrupted flow of the medication. VELETRI requires special handling, as well as a dedicated person—whether the patient or a caregiver—to perform the mixing procedure.

Your healthcare provider will choose the right dose of VELETRI for you based on your individual needs and will work with you to manage your PAH over time. Remember, never change your dose unless told to do so by your healthcare provider.

Pulmonary arterial hypertension: The disease process

Pulmonary arterial hypertension (PAH) is high blood pressure in the pulmonary arteries. Normally, the pulmonary arteries move blood from your heart to your lungs to obtain fresh oxygen. Once this oxygenated blood leaves the lungs, it goes back to your heart to be pumped out to all parts of your body, delivering oxygen and nutrients to your tissues and organs. When the pressure is high in the pulmonary arteries, as in PAH, the heart has to work much harder to get blood into the lungs, causing shortness of breath, fatigue, chest pain, heart palpitations, and/or fainting. These symptoms may occur with or without exertion.

About VELETRI® (epoprostenol) for Injection

The active ingredient in VELETRI is the same as a substance made by the body that helps regulate the flow of blood in the pulmonary arteries. VELETRI has been shown to be effective in improving activity tolerance and reducing some of the symptoms of PAH in some adult patients. Blood vessels respond to VELETRI by opening up and allowing more blood to pass from the heart to the lungs via the pulmonary arteries. The heart does not need to work as hard, and blood flows more easily back into the heart and out into the body.

There are possible side effects with VELETRI, including flushing, headache, and nausea/vomiting, that may require routine monitoring by your healthcare team.

Reference: 1. VELETRI (epoprostenol) for Injection full prescribing information. Actelion Pharmaceuticals US, Inc.

Please see Important Safety Information on page 3 and back cover.

Related symptoms1

Call your healthcare provider if you experience any of the symptoms below or have any questions about your condition.

Symptoms at the start of treatment or with dose increasesOnce your body gets used to VELETRI, your healthcare provider may determine that you need a slight change in dose. When you start VELETRI or have your dose increased, you may experience:

•Flushing

• Headache

• Nausea/vomiting

• Low blood pressure

• Anxiety/nervousness

• Chest pain

• Dizziness

• Slow heartbeat

What is VELETRI?



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• Abdominal pain

• Pain in the muscles and/or ligaments and bones

• Shortness of breath

• Back pain

• Sweating

• Upset stomach

• Numbness/increased sensitivity

• Fast heartbeat

How to use VELETRI® (epoprostenol) for Injection

Like all epoprostenol medications, VELETRI is supplied as a powder in small, sterile vials. Before VELETRI can be used, it must first be reconstituted (dissolved) and then further diluted with a liquid, either sterile water or a sodium chloride solution. Cassettes of the diluted drug can be prepared ahead of time, then stored in the refrigerator for up to 8 days. With VELETRI, you can wear a 24-hour cassette without ice packs.

When administering VELETRI at room temperature, short excursions at 104°F (40°C) are permitted for up to:

• 2 hours for concentrations below 15,000 ng/mL

• 4 hours for concentrations between 15,000 ng/mL and 60,000 ng/mL

• 8 hours for concentrations above 60,000 ng/mL

Please consult your healthcare provider for more information.

Your healthcare provider will closely monitor your therapy and make any necessary changes. For more information, please talk to your healthcare team. For information about specific preparation and storage instructions, please talk to your healthcare provider or your Accredo home healthcare nurse.

For more information about preparing and using VELETRI, please refer to the Step-by-Step Preparation Guide flip chart included with the VELETRI Patient Starter Kit or call Accredo at 1-866-344-4874.



Emergency supply kit

Take your emergency supply kit with you whenever you leave home. Put all of your supplies and medication in a bag you can carry with you so it cannot get lost. Many hospitals do not normally stock the medication or special supplies needed to administer your therapy. Being prepared can help you avoid many problems.

Ask your Accredo nurse about talking with your local emergency medical services (EMS).

Your list may vary. Consult your nurse to determine exactly what you need.

* You can prepare and store fully diluted cassettes in the refrigerator (36°F to 46°F/2°C to 8°C) for up to 8 days and then administer at room temperature (77°F/25°C) for up to 24 hours, without ice packs. Do not expose VELETRI to direct sunlight. Talk to your doctor about the storage conditions for the VELETRI backup cassette.

Your first shipment

Your first shipment of VELETRI® (epoprostenol) for Injection from Accredo will contain:

• VELETRI medication

• Either Sterile Water for Injection, USP, or Sodium Chloride 0.9% Injection, USP

• CADD-Legacy® 1 pump

• Pump carrying case

• Catheter flushing supplies, when applicable

• Dressing change kits

• Batteries

• All necessary mixing supplies

Suggested items:

• Backup CADD-Legacy® 1 pump

• Extra AA batteries (new)

• Prepared/mixed VELETRI*

• Alcohol pads or skin prep pads

• Tape

• Syringes

• 18-gauge needle

• Extra CADD-Legacy® 1 cassette

• Vials of VELETRI (48-hour supply) and either Sterile Water for Injection, USP, or Sodium Chloride 0.9% Injection, USP, unmixed

• Extra extension tubing with attached filters

• Injection cap (if needed)

• Central line dressing change supplies

• Sodium chloride (if flushing catheter)

• Plastic clamps (hemostats)

Contacting Accredo

Phone: 1-866-344-4874

Please see Important Safety Information on page 3 and back cover.

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Accredo Health Group, Inc.

Phone: 1-866-344-4874

Actelion Pharmaceuticals US, Inc.Manufacturer of VELETRI

Phone: 1-866-ACTELION (1-866-228-3546)www.VELETRI.com

National Institutes of Health

Phone: 1-301-496-4000www.nih.gov

PHCentral

www.phcentral.org

Pulmonary Hypertension Association (PHA)

Phone: 1-800-748-7274www.phassociation.org

Scleroderma Foundation

Phone: 1-800-722-HOPE (4673)www.scleroderma.org

Additional resources

Other resources for PAH patients and families:

If you have any questions regarding your therapy, contact your healthcare provider.

VELETRI® (epoprostenol) for Injection inventory worksheet

This worksheet will help you manage your inventory so you can provide Accredo with an accurate account of your supply needs. Please have this worksheet available for your monthly call with your patient care coordinator.

Worksheet

Accredo will need to know the following information:

1. The rate of your pump is __________________ mL/day.

2. The number of vials you are using is _____________ per day.

3. The total amount of medication and supplies you have on hand: _______________.

Item UsageQuantity on hand

Quantity to order

Vial(s) of VELETRI 0.5 mg or 1.5 mg powder

50-mL vials of either Sterile Water for Injection, USP, or Sodium Chloride 0.9% Injection, USP

100-mL cassette

60-mL syringe

Dressing change kits

Extension tubing

Batteries

Alcohol prep pads

18-gauge needles

5-mL or 10-mL syringes

Tape

Sharps container

Cassette labels

Miscellaneous items

Please call 1-866-344-4874 if you have less than a 7-day supply of medication on hand or if you have a change in the number of vials you use per day.

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10











Worksheet




Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items


Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items

11


12 13

VELETRI® (epoprostenol) for Injection inventory worksheetVELETRI® (epoprostenol) for Injection inventory worksheet

Worksheet












Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items


Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items


14


Worksheet








Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items


Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items

15






16


Worksheet












Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items


Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items

17


18 19


Worksheet












Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items


Quantity to order



100-mL cassette

60-mL syringe


Extension tubing

Batteries

Alcohol prep pads

18-gauge needles


Tape

Sharps container

Cassette labels

Miscellaneous items


The central venous line (CVL) care log is designed to help you document your routine for changing your CVL dressing and any related health problems, and to facilitate a discussion of any issues with your healthcare provider at your next appointment. Additionally, an Accredo representative will contact you on a monthly basis. Your CVL care log will be a key tool in providing additional information regarding the status of your therapy. Please follow the instructions below for completing the enclosed treatment logs.

Central venous line care log Central venous line care log

• Each time you change your CVL dressing, enter the date and time of day.

• Please answer the questions on the log that relate to your dressing change.



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DateDid you use a dressing kit?

Which antiseptic did you use?

Any redness, soreness, or broken

skin around the site?If yes, please describe

Any fever since last change?

Have you seen your

doctor?Date

Did you use a dressing kit?





Have you seen your

doctor?

Care log







22 23






Have you seen your

doctor?Date






Have you seen your

doctor?

Care log






24 25






Have you seen your

doctor?Date






Have you seen your

doctor?

Care log







26 27






Have you seen your

doctor?Date






Have you seen your

doctor?

Care log







28 29






Have you seen your

doctor?Date






Have you seen your

doctor?

Care log







30 31






Have you seen your

doctor?Date






Have you seen your

doctor?

Care log


Please see enclosed full Prescribing Information.

This educational resource was created and funded by Actelion Pharmaceuticals US, Inc.

VELETRI is a registered trademark of Actelion Pharmaceuticals Ltd Accredo is a registered trademark of Accredo Health Group, Inc. All other trademarks are the property of their respective owners.© 2017 Actelion Pharmaceuticals US, Inc. All rights reserved. VEL-00279 1117

This guide is provided for informational purposes only. Please contact your healthcare professional or Accredo if you have any questions or need specific instructions on how to administer your medication.

1-866-344-4874

What is VELETRI® (epoprostenol) for Injection?



Who should not take VELETRI?

VELETRI should not be used if you have heart failure due to severe left heart disease, if you develop fluid in the lungs (pulmonary edema) when starting therapy, or if you are allergic to epoprostenol.

Some medications may interact with VELETRI. Please talk to your doctor about all of your medications.



When you take epoprostenol for the first time, you must be in a setting (hospital or clinic) where you can be monitored for any serious side effects or in case of emergency. Your blood pressure and heart rate should also be monitored with any dose changes. If you are taking VELETRI, your doctor may prescribe another kind of medicine used to prevent blood clots. It is the use of these medications concomitantly (in combination) with VELETRI that may potentially cause an increase in the risk of bleeding.

Sudden and dramatic changes in dose may lead to unstable blood pressure, a return of pulmonary hypertension symptoms, or fatal low blood pressure (hypotension). Do not stop using VELETRI without first talking to your doctor.

To reduce the risk of infection in the bloodstream, it is important to know how to properly care for the catheter and infusion pump.

What are the possible side effects of VELETRI?

You may have side effects at the start of treatment or with dose increases. The most common side effects seen in at least 1% of patients were flushing, headache, nausea/vomiting, low blood pressure, anxiety/nervousness, chest pain, dizziness, slow heartbeat, abdominal pain, pain in the muscles and/or ligaments and bones, shortness of breath, back pain, sweating, upset stomach, numbness/increased sensitivity, and fast heartbeat.

The most common side effects in patients with PAH due to unidentified or hereditary factors with at least 10% difference between the group that received epoprostenol and the group that received conventional therapy alone were flu-like symptoms, fast heartbeat, flushing, diarrhea, nausea/vomiting, jaw pain, pain in the muscles and/or ligaments and bones, anxiety/nervousness, dizziness, headache, and numbness/increased sensitivity/tingling.

The most common side effects in patients with PAH due to connective tissue disease with at least 10% difference between the group that received epoprostenol and the group that received conventional therapy alone were flushing, low blood pressure, lack of appetite, nausea/vomiting, diarrhea, jaw pain, neck/joint pain, headache, skin ulcer, and rash.

Talk to your doctor if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of VELETRI. For more information, ask your doctor or pharmacist.

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Documents

An introduction to VELETRI (epoprostenol) for Injection: I ... · between the heart and the lungs. VELETRI may improve your ability to exercise as measured by how far you can walk