Studies indicate that early diagnosis is associated with improved survival rates and will help with getting started on the right treatment path. Treatment options vary from person to person, so you will want to work closely with your healthcare provider to determine the best plan for you.
Your Treatment Plan
If you are newly diagnosed with PAH, you should be referred to an accredited pulmonary hypertension care center for thorough evaluation. Because PAH is such a rare disease, it is extremely valuable to see a specialist at an accredited center to ensure you are getting the most up-to-date treatment options.
Medications and Treatment Guide
PAH-specific medications come in multiple forms: oral, inhaled and subcutaneous (meaning delivered by an injection or IV). The medicines for PAH work in a few ways. Some allow blood to flow more easily through the arteries of your lungs. Others help your heart and lungs work better.
Treatment aims to restore balance among one or more of three substances that are produced by your lungs: nitric oxide, endothelin, and prostacyclin. Although a test does not currently exist to determine which of these substances is not balanced, PAH medications act on these three pathways to help slow how quickly your disease worsens.
Supportive Treatments
Your healthcare provider may recommend supportive treatment to manage your PAH.
Managing Pulmonary Arterial Hypertension
Being diagnosed with a chronic illness like PAH is life-changing. You will need to have a close relationship with a PAH expert and would benefit from a strong support system of family and friends.
Many pulmonary hypertension care centers require visits every few months and regular testing such as echocardiograms and 6-minute walk testing. Your doctor may also have you complete cardiopulmonary exercise testing (CPET), a specialized type of exercise test that measures your exercise ability. Some centers will do right heart catheterizations every year to see how well treatment is working on managing pulmonary pressures and heart function. Additionally, it is essential to take your medications exactly as directed, being careful not to run out or change your schedule unless directed by your healthcare provider. It is also important to stay up to date on all recommended vaccinations.
Lifestyle Changes
PAH can affect every aspect of your daily life. No matter where you are at in your PAH journey, making lifestyle changes can help you better manage your health and your quality of life.
Page last updated: September 3, 2024
