Managing Your PAH^*

What lifestyle changes could I make?

Wondering how you can make living with pulmonary arterial hypertension (PAH)^* a little easier? Consider some of these small changes. They may add up to big differences in your day-to-day life.

• Keeping a journal
• Watching your diet
• Activity
• Quitting smoking
• Be consistent with your medications
• Getting support
• Working with PAH^*
• Traveling with PAH^*
• Your sex life and PAH^*
• The team approach

Remember: the best way to get advice about making lifestyle changes is to talk with your healthcare team. Your healthcare provider and the other members of your healthcare team are most familiar with your case history, and they are most qualified to give you the best advice.

Keeping 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

Watching your diet

It's important that you eat a wholesome diet and get proper nutrition to keep your energy levels up. A healthy diet includes fresh fruits and vegetables, whole-grain foods, and lean chicken and fish while limiting fatty foods, including fatty meats. Always check with your healthcare provider before changing any part of your routine.

Activity

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

Quitting smoking

People with PAH^* should not smoke because it contributes to breathing difficulties caused by PAH^*. Smoking also causes the already narrowed blood vessels in your lungs to tighten even more—placing more stress on your heart.

Be consistent with your medications

One of the biggest lifestyle adjustments for people with PAH^* is to make taking medications a part of your routine.

• Ask your healthcare team the proper way to take your medicine and then be consistent.
• Be sure to tell your healthcare team about all other medications, vitamins and supplements, prescribed and over-the-counter, that you take or are thinking of taking.

Getting support

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

Working with PAH^*

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

Traveling with PAH^*

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.

Your sex life and PAH^*

Check with your healthcare provider if there are any limitations. Also, be aware of the importance of using contraceptives because the strain of pregnancy can be very dangerous, and some medications like Tracleer can cause birth defects to a fetus.

The team approach

Your medical team is called your medical team for a reason—you have to make the most of your treatment and manage your PAH^* successfully. Be proactive and come to every appointment prepared to discuss your questions, concerns, side effects and progress. Be willing to share your feelings and even 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 PAH^* 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.

*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.

Learn more about how to work with your healthcare team

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.