Understanding PAH*
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Living with PAH*

If you're like most newly diagnosed pulmonary arterial hypertension (PAH)* patients, you may be overwhelmed. Feelings of shock or uncertainty are common. At the same time, you probably have questions about your disease.

It is hard for physicians to predict how your disease will progress in the long run. Despite this, in the last decade a great deal has changed for the better in the treatment arena. There are medications that may improve some of the symptoms of PAH*.

To empower yourself, it is important that you understand your disease and how to live with it. You may need to adapt in a number of ways by making lifestyle changes and adjustments. The best way to get answers about managing PAH* is to talk with your healthcare team. Your healthcare team is most familiar with your medical case history, and they are most qualified to give you the best advice.

What lifestyle changes could I make?

Wondering how you can adapt and live with PAH* a little easier? Your healthcare team is most familiar with your case history and qualified to give you the best advice. You may also want to consider some of these small changes. They may add up to big differences in your day-to-day life.

1. Keep a journal. All you need is a notebook and a pen to help monitor your progress. The details you record about your disease may help your healthcare team give you the best possible support and advice. You may want to write down:

  • Lists of things to do, such as phone calls to make or household tasks
  • Symptoms you have each day and their severity
  • Questions for your healthcare team and then the answers you receive for future reference
  • Dates of medical appointments
  • Outcome of your appointments for future reference

2. Watch your diet. It's important that you eat a wholesome diet and get proper nutrition to keep your energy levels up. Eating fresh fruits and vegetables, whole-grain foods, and lean chicken and fish while limiting fatty foods and keeping sodium to less than 1,500 mg a day is part of a heart-healthy diet.7 Always check with your healthcare professional before changing any part of your routine.

3. Look into appropriate physical activity. While certain physical activities may put too much strain on the heart and lungs, many patients benefit from some level of physical activity. Talk to your healthcare professional about whether starting or continuing an exercise program is right for you.

4. Quit smoking. People with PAH* should not smoke because it may contribute to breathing difficulties caused by PAH*. Smoking also may cause the already narrowed blood vessels in your lungs to tighten even more—placing more stress on your heart.

5. Get support. There is a network of support available to you that includes medical professionals, family, caregivers, and patients like yourself. Communicating with and relating to others who are "in your shoes" may help you cope better with your disease. Take advantage of support networks.

6. Learn about working with PAH*. Having PAH* may not mean you have to quit your job. Many people continue their careers and are productive employees. Still, your disease may affect your ability to perform your job, so talk with your healthcare professional, monitor your on-the-job performance, and be sure to discuss with your employer any changes that need to be made.

7. Travel safely. Traveling can sometimes be challenging. Before traveling, check with your healthcare team and discuss any changes you might have to make to your routine. Consider these helpful suggestions:

  • Ask your healthcare team for a letter explaining your medical condition and treatment plan, and have the letter handy in case of an emergency.
  • Bring a cell phone and provide any travel companions with emergency instructions and phone numbers.
  • When flying, check with the airline to see if it requires advance notice if you wish to bring oxygen. Try to inform them of your condition well in advance. And ask your healthcare team if you should use supplemental oxygen while waiting in the airport.
  • Altitude affects air density and may result in a more difficult breathing situation. Find out ahead of time if your destination is at a much higher altitude than your home.

8. Talk to your doctor about your sex life. Ask your healthcare professional if there are any limitations. Always use contraceptives, since pregnancy can be very dangerous and some medications like Tracleer® (bosentan) can cause birth defects.

9. Enjoy each day. Deal with your reality as it unfolds, and look for the pleasure in every little thing.

10. Get involved. Be proactive and come to every doctor's visit prepared to discuss your questions, concerns, side effects, and progress. Share your feelings and frustrations. Make a list, and don't be embarrassed to ask anything and everything.

Also, be sure to tell your healthcare team what you are currently able to do. This can help them determine your functional class and better manage your treatment.

Because it can be a complicated disease, lifestyle changes may differ from one person to the next. Always check with your healthcare team before changing any part of your routine.

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.

Learn more about treatment options for PAH*.


*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.