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PAH* Overview
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- What is pulmonary arterial hypertension*?
- Symptoms of PAH*
- Symptoms of PAH* may include
- Treatments for PAH*
- Why WHO Functional Class is important to you
- WHO Functional Class
- How can you help?
- Living With PAH*
What is pulmonary arterial hypertension*?
Pulmonary arterial hypertension (PAH)* is a disease that affects your pulmonary arteries—blood vessels that carry blood from your heart to your lungs. PAH* starts when these small vessels tighten and become stiff. This makes it more difficult for blood to get to your lungs. Your heart has to work harder to push blood through the narrowed blood vessels, creating a constant state of high blood pressure in the vessels.1
Take a look at how PAH* affects and changes the pulmonary artery2,3:
Healthy pulmonary artery — open and elastic; blood flows through easily
Artery with signs of PAH* — resistance to blood flow
Artery with advanced PAH* — vessel narrows and stiffens from blood vessel wall thickening, scar tissue, and clotting1,4
Over time, scarring (fibrosis) of the blood vessels makes them stiffer and thicker, and some may become completely blocked. The extra stress causes the heart to enlarge and become less flexible. As this cycle continues, less and less blood is able to flow out of the heart, through the lungs, and into the body, and more and more symptoms can begin to appear.2
Symptoms of PAH*2
Symptoms of PAH* are caused by a lack of oxygen due to narrowed blood vessels in the lungs and increased stress on the heart. Symptoms may not be obvious at first, but over time they can become more limiting.
Symptoms of PAH* may include2†:
- Breathlessness or shortness of breath (dyspnea), the most common symptom
- Feeling tired all the time (chronic fatigue)
- Dizziness, especially when climbing stairs or when standing up
- Fainting (syncope)
- Swollen ankles and legs (edema)
- Chest pain, especially during physical activity (angina)
Without treatment, patients may experience increased breathlessness, so that even walking short distances may become difficult.2
†Tracleer® (bosentan) and Ventavis® (iloprost) do not improve all of the symptoms of PAH*.
Treatments for PAH*
Several medications have been approved by the FDA to treat PAH*. Talk to your healthcare provider to determine which medication is appropriate for you.
Why WHO Functional Class2 is important to you
Once a diagnosis of pulmonary arterial hypertension (PAH)* has been established, your healthcare provider will determine your WHO Functional Class. The World Health Organization (WHO) has established the WHO Functional Class System to determine, according to your symptoms, the severity of your disease. This is a measurement of your ability to perform ordinary physical activities, such as walking, without shortness of breath. Placing patients into WHO Functional Classes helps the healthcare team make decisions about your treatment.
The WHO Functional Class scale is divided into 4 categories and considers how symptoms such as tiredness, shortness of breath, chest pain or light-headedness limit physical activity.2 The lower the WHO Functional Class, the less severe the symptoms of PAH* are. One of the goals of treatment is to improve your Functional Class.4 Here are the 4 categories:
WHO Functional Class2 | ||
| Functional Class I | No limitation in usual physical activity (ordinary activity does not increase symptoms) | Activity example: Walking |
| Functional Class II | Some limitation in physical activity (no discomfort at rest, but normal activity increases symptoms) | Activity example: Walking |
| Functional Class III | Significant limitation in physical activity (no discomfort at rest, but less than normal activity increases symptoms) | Activity example: Putting away dishes or folding laundry |
| Functional Class IV | Symptoms are present with any activity or even at rest. | Activity example: Resting or sitting in a chair |
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.5
- 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.6,7
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.
How can you help?
You can help your healthcare provider determine your Functional Class. Tell them what activities you were able to do before you had symptoms of PAH*. For example, "A month ago I could walk to the post office. Now I get out of breath before I get there." This information can help your healthcare provider make treatment decisions with the goal of helping you improve your Functional Class.
Living With PAH*
Diet
Good nutrition can benefit everyone, including people with PAH*. Eating healthy is important. Choosing foods wisely may reduce your risk for other illnesses, help you feel better, and boost your energy levels. Plus, a healthy diet can be satisfying—and tasty too!
Be sure to talk to your healthcare team before making any changes. They know your situation best and can design a diet appropriate for you.
How can I work with my healthcare team?
Successful treatment for PAH* takes a team effort. Working closely with your healthcare team members may help you manage your PAH* in the best way possible.
Here's how you can help your team:
- Tell them what you are currently able to do, which can help them determine your Functional Class and better manage your treatment.
- Tell them about all other medications, vitamins, and supplements, prescribed and over-the-counter, that you are taking or are thinking of taking.
- Use a journal to keep track of your progress and to write down your questions. Share this information with your healthcare team.
- Ask questions to make sure you understand information and recommendations.
- Talk about what you can expect from your treatment.
- Learn how to identify side effects and immediately alert your healthcare team if you experience any while taking your medication.
- Tell them how you feel, including how your symptoms have affected your ability to do ordinary physical activities.
- Discuss changes to your lifestyle, diet, or activity level that you or your healthcare team feel you need to make.
Other resources are also available to patients with pulmonary arterial hypertension (PAH)* who may be looking for information and support.
Patients should always talk with their healthcare team first if there are any questions about their individual treatment.
*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.5
- 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.6,7
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 for Tracleer.
*What is Ventavis?
Ventavis is a prescription medicine used to treat adults with certain kinds of severe pulmonary arterial hypertension (PAH), a condition in which blood pressure is too high in the blood vessels between the heart and the lungs. Ventavis may improve your ability to exercise and your symptoms for a short time by lowering your blood pressure and opening up the blood vessels in your lungs.
- In the key clinical study, Ventavis showed clinical improvement as defined by 3 specific measurements: ability to exercise as measured by the 6-minute walk test, symptoms (NYHA Functional Class), and decrease in the worsening of PAH symptoms.
- Ventavis is a medication you breathe in through a special device called the I-neb Adaptive Aerosol Delivery (AAD) System.
The study showing Ventavis is effective included mainly patients with NYHA Functional Class III-IV PAH. In these patients, PAH was caused by unidentified or hereditary factors (65%) or connective tissue diseases (23%).
Ventavis has not been studied in children younger than 18 years old.
What is the most important information I should know about Ventavis?
Ventavis may not be right for you. Before taking Ventavis, tell your doctor about all of your medical conditions, including if you have liver or kidney problems; are pregnant, or plan to become pregnant; or are breast-feeding. Tell your doctor about all the medicines you take, including prescription and nonprescription medicines, vitamins, and herbal supplements. Especially tell your doctor if you take medicines used to treat high blood pressure or heart problems or medicines that lessen blood clotting (warfarin, Coumadin, Jantoven). Ventavis and other medicines may affect each other, causing side effects.
What are the possible side effects of Ventavis?
- Ventavis may cause side effects, including feeling dizzy, lightheaded, and faint. If you have any of these side effects, you should stand up slowly when you get out of chairs or bed. Tell your doctor if your fainting gets worse during treatment with Ventavis. Your doctor may need to change your dose or treatment.
- Do not drive a car or operate any tools or machines if dizziness or fainting from low blood pressure is a problem for you.
- You may have trouble breathing after taking Ventavis, because it may cause the muscles around your airway to tighten (bronchospasm). Get emergency help right away if you have trouble breathing.
- The most common side effects of Ventavis include red face (flushing), increased cough, low blood pressure, headaches, nausea, spasm of your jaw muscles that makes it hard to open your mouth, and fainting.
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 Ventavis. For more information, ask your doctor or pharmacist.
Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.
Please see full Prescribing Information for Ventavis.
References: 1. Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet. 1998;352:719-725. 2. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(Suppl S):40S-47S. 3. McLaughlin V, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006;114:1417-1431. 4. Barst RJ, Gibbs SR, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S78-S84. 5. TRACLEER® (bosentan) full Prescribing Information. Actelion Pharmaceuticals US, Inc., February 2011. 6. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346(12):896-903. 7. 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.
