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Understanding PAH*
Use these links to navigate through your PAH* Journey. Visited pages will display with the following icon:

Understanding PAH*

If you've recently been diagnosed with pulmonary arterial hypertension (PAH)*, you may be feeling confused or overwhelmed. There's a lot to understand and the more you know, the better equipped you'll be to take a positive step toward participation in your own care. Your disease is a journey and we're here to help you get started off on the right foot. Let's begin by answering "What is PAH*?":

What is PAH*?

Pulmonary arterial hypertension, or PAH*, is a disease of the vessels that carry blood from the heart to the lungs. In PAH*, there is a constant state of high blood pressure in the vessels of the lungs. It is a progressive disease—meaning that it gets worse over time. It is not common but it affects people of all ages and ethnic backgrounds. PAH* can occur on its own, or it can be related to other causes.

The basics of PAH* Types of PAH* Diagnosing PAH*

Understanding the Basics of PAH*

How does the cardiovascular system work?

Knowing how the cardiovascular system works will help you better understand PAH*. The cardiovascular system includes your blood vessels and your heart. The vessels that carry blood from your heart to your lungs are called pulmonary arteries. When they are healthy, these vessels are large and flexible—this means they are very open and elastic, and blood can easily pass from the heart, through the pulmonary arteries, and into the lungs, ultimately moving oxygen out to the rest of the body.

In PAH*, the pulmonary arteries tighten up and become stiff. This causes more resistance to the blood flowing through the arteries.

Healthy pulmonary artery is open and elastic; blood flows through easily Artery with early signs of resistance to blood flow Artery with advanced narrowing and stiffening due to blood vessel wall thickening, scar tissue, and clotting4,5

† Statements are based on laboratory studies. The clinical significance in humans is not known.

PAH* causes changes in the heart

In PAH*, the heart gets bigger. The right ventricle has to work harder to pump blood against higher resistance in the arteries. As the right heart grows, it starts to compress its left side. This makes it harder for the left side of the heart to do its job of circulating blood to the rest of the body, in turn causing more and more symptoms to appear.

As this cycle continues, the strain on the right side of the heart may become too great, resulting in a condition known as "right-heart failure." This doesn't mean that the heart stops. But it does mean that the heart works less efficiently, because there is less blood flowing through the pulmonary artery.

Tracleer® (bosentan) has not been studied in all PAH* patient populations.
Studies showing Tracleer is effective included mainly people with mild to severe symptoms (called NYHA Functional Class II-IV). In these patients, PAH* was caused by: unknown causes (called idiopathic), hereditary causes (a family history), connective tissue diseases (like scleroderma or lupus), or being born with a hole in the heart between the left and the right sides.

Types of PAH*

Tracleer has not been studied in all PAH* patient populations.
Studies showing Tracleer is effective included mainly people with mild to severe symptoms (called NYHA Functional Class II-IV). In these patients, PAH* was caused by: unknown causes (called idiopathic), hereditary causes (a family history), connective tissue diseases (like scleroderma or lupus), or being born with a hole in the heart between the left and the right sides.

Pulmonary Hypertension (PH) vs Pulmonary Arterial Hypertension (PAH)*

Pulmonary hypertension (PH) is a broad term used for any condition in which the blood vessels of the lungs have high pressure. There are many types of PH with many different causes.

The World Health Organization (WHO) is considered an international authority on health definitions and disease information. The goal of the WHO classification system for pulmonary hypertension (PH) is to classify the disease into different groups according to their cause and treatment method. The most recent update to this classification system was completed during the 4th World Symposium on PH held in 2008 at Dana Point, California. PH is divided into five WHO groups depending on the cause.

The first group, or WHO Group 1, is otherwise known as pulmonary arterial hypertension (PAH)*. PAH* specifically affects the pulmonary arteries of the lungs, causing them to tighten and stiffen.

Types of PAH*, WHO Group 16

1.1 Idiopathic PAH (IPAH)*: This refers to PAH* that occurs without an apparent cause (no family history or identified risk factor). IPAH* used to be referred to as "primary pulmonary hypertension" or PPH, and some older information still uses this term.

1.2 Heritable PAH*: With this type of PAH*, a faulty gene is passed on through the family, causing PAH* to develop over time.

1.3 Drug-and-toxin induced PAH*: Certain drugs including aminorex, fenfluramine, dexfenfluramine, and toxins including rapeseed oil have been associated with the development of PAH*.

1.4 PAH* associated with other diseases and conditions. This category includes PAH* associated with:

  • Congenital heart disease
  • Connective tissue disease
  • HIV infection
  • Portal hypertension

Diagnosing PAH*

The importance of an accurate diagnosis

Because of the challenges involved in making a diagnosis, the diagnosis may be delayed for months or even years. Treatment can improve your ability to exercise and can slow the worsening of your physical symptoms.1

PAH* is a rare disease that starts with symptoms that are easily confused with those of other conditions, such as asthma or other lung diseases. Thus, people may be misdiagnosed and receive inappropriate treatment for their symptoms. That's why a series of tests may be performed to rule out other conditions and confirm PAH*.

Making a diagnosis

Once symptoms are detected, there is an array of diagnostic testing tools that your healthcare team may want to use. For an accurate diagnosis to be made, certain tests may be performed either to rule out other conditions and/or to confirm PAH*. If PAH* is suspected, your doctor may perform the following tests:

  • Electrocardiogram (ECG)
  • Chest X-ray
  • Doppler Echocardiogram; lung scan
  • Pulmonary function test; formal sleep study; laboratory tests.

The definitive test to make a diagnosis is right-heart catheterization. This test may also evaluate the severity of PAH* by measuring pressure and blood flow changes in the pulmonary arteries and right side of the heart.4 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.4

Your healthcare professional might order other kinds of tests to learn more about your condition or to rule out other diseases.

Classifying your symptoms

Once a diagnosis has been made, your healthcare professional may determine your functional class. The functional class categorizes the severity of your disease according to your symptoms. Functional class describes how symptoms, such as shortness of breath, chest pain, or lightheadedness, limit your physical activity.

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 Functional Class II symptoms, Tracleer slowed the worsening of PAH*, however, it did not show meaningful improvement in walk distance. If you are a patient with Functional Class II symptoms, your healthcare professional 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.

The 6-Minute Walk Test

The 6-Minute Walk Test (6MWT) is a tool to evaluate exercise capacity. For the test, you walk as far as possible in 6 minutes. Before, during, and after the test you will be asked to report how short of breath you are. Your healthcare professional may ask you to take the 6MWT before you begin and several times during your treatment.

Tracleer® (bosentan) has not been studied in all PAH* patient populations.
Studies showing Tracleer is effective included mainly people with mild to severe symptoms (called NYHA Functional Class II-IV). In these patients, PAH* was caused by: unknown causes (called idiopathic), hereditary causes (a family history), connective tissue diseases (like scleroderma or lupus), or being born with a hole in the heart between the left and the right sides.

Learn more about the symptoms of PAH*.

*PATIENT INDICATION AND IMPORTANT SAFETY INFORMATION EXPAND COLLAPSE

*What is Tracleer® (bosentan)?

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 1). 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.1-3

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 Functional Class II symptoms, Tracleer slowed the worsening of PAH, however, it did not show meaningful improvement in walk distance. If you are a patient with Functional Class II symptoms, your healthcare professional 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 professionals who are enrolled in the Tracleer Access Program (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 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 as small as possible, Tracleer may be prescribed only through 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 your blood tested to check your liver function before you start Tracleer and each month after that. Your healthcare professional will order these tests. Regular blood tests are important because they will help your healthcare professional adjust or stop your treatment before there is permanent damage.
  • Tracleer can cause serious liver damage, including in rare cases liver failure. 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 professional if you have had liver problems, including liver problems while taking other medicines. Call your healthcare professional 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 professional 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 professional right away if you miss a menstrual period or think you may be pregnant.
  • Females who are able to get pregnant must use two forms of birth control (contraception) during Tracleer treatment because there is a possibility of birth defects.
  • Birth control pills, shots, patches, and implants should not be used alone because they are not reliable when you are taking Tracleer. You must choose and use two reliable forms of birth control at the same time.
  • If you have had a tubal sterilization or have an IUD, these methods can be used alone. Otherwise, you should use two forms of birth control.

Talk with your healthcare professional about which 2 methods of reliable birth control you should use. Your healthcare professional 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?"
  • 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 professional.

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 professional 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 professional 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 professional if fertility is a concern for you.
  • Low red blood cell levels (anemia). Your healthcare professional 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, including BOXED WARNING about liver injury and birth defects, and Medication Guide.