Glossary of Terms

This information is provided for educational purposes only and is not meant to be a substitute for the advice of a physician or other healthcare professional. You should not use this information for diagnosing or treating a health problem or disease. You should always consult with your healthcare team for your personal medical needs.

(Quick links by letter)

A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

angina. A type of chest pain or discomfort caused by reduced blood flow to the heart muscle.

angiogram. An x-ray picture of blood vessels that's taken after a dye is injected into blood vessels to make them more visible.

anticoagulants. A class of medications used to prevent blood clotting in the body, particularly in the lungs of patients with PAH*.

breathlessness. Short of breath. Also called dyspnea. This is the most common symptom of PAH*.

cardiac output. The amount of blood pumped by the heart over a particular period of time, measured in liters per minute (l/min).

congenital heart disease. A problem with the heart's structure and function that a person is born with. One of the most common congenital heart defects is a hole in the wall between the right and left ventricles of the heart. Those with congenital heart disease have an increased risk of developing PAH*.

connective tissue disease. Disorders affecting joints (muscles, bones, tendons, cartilage). There is often an autoimmune component. Types associated with PAH* include SLE (lupus), scleroderma, and mixed connective tissue disease. Those with connective tissue disease have an increased risk of developing PAH*.

digoxin. A drug that helps the heart pump more effectively and is often prescribed along with PAH* medication (it's important to note that this drug has no effect on constricted blood vessels).

diuretics. A class of medications that can help reduce fluid in the body and reduce swelling in the feet and legs. Diuretics are often prescribed along with PAH* medication.

dyspnea. Shortness of breath or breathlessness; labored breathing. This is the most common symptom of PAH*.

Doppler echocardiogram. A test in which ultrasound (sound waves) is used to examine the heart. Doppler is a special part of the ultrasound examination that assesses the direction and velocity of blood flow as it makes its way through and out of the heart.

edema. Swelling caused by fluid retention. For PAH* patients, edema is often present in the ankles and lower legs.

electrocardiogram (ECG). A test in which electrodes are placed on the body to record the electrical activity of the heart. ECGs are used to learn the condition of the heart and to diagnose heart disease.

embolism. A blood clot or foreign substance that can block or obstruct a blood vessel, restricting vital blood flow.

endothelin. A chemical made by the body which may cause the smooth muscles in blood vessel walls to tighten up. Excess amounts of endothelin have been found in the blood of PAH* patients.

endothelin receptor antagonist (ERA). A medication that may help people with PAH* by blocking the effects of the extra endothelin their bodies produce. Tracleer is the first oral endothelin receptor antagonist approved for FC II, III, IV PAH*.

hypertension. Abnormally high blood pressure.

idiopathic PAH . This refers to pulmonary hypertension that cannot be attributed to any other cause.

lung scan. A diagnostic test that measures air and blood flow in the lungs and is used to rule out the presence of other lung diseases.

PAH Pathways^®. A patient service and support program that provides patients with access to Actelion's PAH* medications, insurance coverage information, financial assistance for eligible patients and Sure Steps™.

portal hypertension. An increase in the pressure within the portal vein (the vein that carries blood from the digestive organs to the liver). Those with portal hypertension have an increased risk of developing PAH*.

pulmonary arterial hypertension (PAH)*. A disease of the vessels that carry blood from the heart to the lungs. PAH* is caused by changes in the small blood vessels in the lungs that make it harder for blood to flow through these vessels.

pulmonary artery. The large blood vessel that carries blood from the heart to the lungs.

pulmonary function tests. A group of tests that measure how well your lungs work. Your healthcare provider might order this test to learn more about your condition or to rule out other lung diseases.

pulmonary hypertension (PH). Broad term used for any condition in which the blood vessels of the lungs have high pressure. PH comes in many types with many different causes. Pulmonary Arterial Hypertension (PAH)* is one such type.

right heart catheterization. A thin tube with a special tip is inserted into a vein in the neck or groin and threaded into the heart and the pulmonary artery, where it measures pressure. This is the only diagnostic test to confirm PAH*.

six-minute walk test. How far a patient can walk in 6 minutes is a measure of exercise ability that is usually a part of the diagnostic process for PAH*. It is also used during follow-up doctor visits to determine progress.

Sure Steps. An education and support program provided by PAH Pathways for Tracleer patients and caregivers. Patients will receive educational materials, one-on-one phone support from Nurse Counselors and online access.

Sure Steps Connect™. An online conference series where Tracleer Sure Steps members can watch PAH* experts discuss a variety of PAH*- and Tracleer-related topics.

syncope. Fainting. This can be a symptom of PAH*, and is often the symptom that gets people to see their healthcare team.

Tracleer Enrollment and Renewal form and HIPAA form. A form patients will complete with their healthcare team to enroll in the Tracleer Access Program (T.A.P.^®) in order to receive Tracleer. You can also enroll in voluntary patient education and support programs by completing the HIPAA form.

warfarin. A medication that makes the blood less likely to clot. Warfarin is more commonly known by the brand name Coumadin (see anticoagulants above).

WHO Functional Class. A measurement of a patient's ability to perform ordinary physical activities, such as walking, without feeling shortness of breath. PAH* patients are put into one of four possible classes depending on the severity of their symptoms (the lower the WHO Functional Class, the less severe the PAH*). WHO stands for World Health Organization.


*What is Tracleer?
Tracleer is a prescription medicine indicated for patients with certain types of pulmonary arterial hypertension (PAH), which is high blood pressure in the vessels of the lungs, (WHO Group I). Tracleer can improve your ability to exercise as measured by 6-minute walk test and can slow the worsening of your physical condition and symptoms.

• In two different studies, Tracleer improved 6-minute walk distance by 35 and 76 meters. These studies focused on patients with WHO Functional Class III-IV.¹
• Clinical worsening was defined as any one of the following: a need to go to the hospital for PAH, stopping treatment because of worsening of PAH, a need for intravenous (IV) treatment (epoprostenol), or death.^2,3

Studies showing Tracleer is effective included mainly patients with NYHA Functional Class II-IV PAH. In these patients, PAH was caused by: unidentified or hereditary factors (60%); connective tissue disease (21%); being born with a hole in the heart between the left and right sides (18%).

In patients with WHO Functional Class II symptoms, Tracleer slowed the worsening of PAH, however, did not show meaningful improvement in walk distance. If you are a patient with WHO Functional Class II symptoms, your healthcare provider will consider whether the potential benefits of Tracleer outweigh the risk of liver damage, which may prevent future use of Tracleer as your disease progresses.

Tracleer is only:

• prescribed by healthcare providers who are enrolled in T.A.P.
• available to people who understand and agree to enroll in T.A.P.

It is not known if Tracleer is safe and works in children below 12 years of age.

What is the most important information I should know about Tracleer?
Tracleer is only available through the Tracleer Access Program (T.A.P.). Before you begin taking Tracleer, you must read and agree to all of the instructions in T.A.P. Because of potential liver damage and in an effort to make the chance of fetal exposure to Tracleer^® (bosentan) as small as possible, Tracleer may be prescribed only through the Tracleer Access Program (T.A.P.), by calling 1-866-228-3546. Adverse events can also be reported directly via this number.

Liver damage:

• Liver damage may not cause symptoms at first. Only a blood test can show if you have early liver damage. So you must have a blood test to check your liver function before you start Tracleer and each month after that. Your healthcare provider will order these tests. Regular blood tests are important because they will help your healthcare provider adjust or stop your treatment before there is permanent damage.
• Tracleer can cause serious liver damage, including in rare cases liver failure. The contribution of Tracleer in these cases could not be excluded. Even in situations with regular liver testing, rare cases of unexplained liver cirrhosis (scar tissue in the liver) have developed in patients with other medical conditions and taking other medications in addition to Tracleer for over 12 months. Tell your healthcare provider if you have had liver problems, including liver problems while taking other medicines. Call your healthcare provider right away if you have any of these symptoms of liver problems while taking Tracleer: nausea, vomiting, fever, unusual tiredness, stomach area (abdominal) pain, or yellowing of the skin or the whites of your eyes (jaundice).

Serious birth defects:

• Tracleer can cause serious birth defects if taken during pregnancy. You must not be pregnant when you start taking Tracleer or during Tracleer treatment. Serious birth defects from Tracleer can happen early in pregnancy. Females who are able to get pregnant must have a negative pregnancy test before starting treatment and each month during Tracleer treatment.
• Talk with your healthcare provider or gynecologist (a doctor who specializes in female reproduction) to find out about how to prevent pregnancy. Do not have unprotected sex. Tell your healthcare provider right away if you miss a menstrual period or think you may be pregnant.
• Females who are able to get pregnant must use birth control (contraception) during Tracleer treatment. You must choose and use two reliable forms of birth control at the same time, unless you have had a tubal sterilization, or have a Copper T 380A IUD or LNg-20 IUS. These methods can be used alone.
• Talk with your healthcare provider about which 2 methods of reliable birth control you should use. Your healthcare provider may recommend that you use a different method of birth control to help lower your risk of problems with your pulmonary arterial hypertension.

Who should not take Tracleer?
Do not take Tracleer if you:

• Are pregnant, plan to become pregnant, or become pregnant during Tracleer treatment. Tracleer can cause serious birth defects. All females should read the birth defects section of "What is the most important information I should know about Tracleer?"
• Have a blood test that shows possible liver injury.
• Take one of these medicines:

  —	Do not take Tracleer with cyclosporine-A, a medication used for psoriasis and rheumatoid arthritis, and to prevent rejection of heart or kidney transplants. Using cyclosporine-A with Tracleer may increase your chance of side effects.
  —	Do not take Tracleer with glyburide. Glyburide is used to treat diabetes and when used with Tracleer it may increase your chance of liver injury.

• Are allergic to any of the ingredients in Tracleer. If you have a rash, hives or your lips swell after taking Tracleer, it may be a sign of an allergy. You should stop taking your Tracleer and talk to your healthcare provider.

What are the possible side effects of Tracleer?
Tracleer can cause serious side effects, including:

• See "What is the most important information I should know about Tracleer?"
• Fluid retention and swelling of ankles and legs. Tracleer can cause your body to hold too much water, and you may get swelling of your ankles and legs. Tell your healthcare provider if you have swelling of your ankles and legs that happens either with or without weight gain, or if you have more trouble with your breathing than normal. Your healthcare provider will look for the cause of this.
• Lower sperm count. Some men who take Tracleer may have lower sperm counts. This may affect your ability to father a child. Tell your healthcare provider if fertility is a concern for you.
• Low red blood cell levels (anemia). Your healthcare provider will do blood tests to check your red blood cells during treatment with Tracleer.

The most common side effects of Tracleer are: respiratory tract infection, headache, fainting, flushing, low blood pressure, inflamed nose passages (sinusitis), joint pain and irregular heartbeats.

Tell 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 Tracleer. 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.

Please see full Prescribing Information and Medication Guide.

REFERENCES:

1. TRACLEER^® (bosentan) full Prescribing Information. Actelion Pharmaceuticals US, Inc., February 2011.
2. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346(12):896-903.
3. Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet. 2001;358:1119-1123.