Living with Pulmonary Arterial Hypertension
What to Expect
Patients with PAH may have very different lives after they are diagnosed with the disease. The therapies that exist for this disease range from pills that can be taken between one to three times a day to medications that have to be given continuously through an IV line using a pump that is similar to an insulin pump used by diabetic patients. How well a patient responds to medicine is somewhat unpredictable and depends on how advanced the disease is at diagnosis as well as the underlying cause. Patients without treatment usually quickly progress to death over the course of months to years. However, with treatment, survival has improved significantly.
Typically patients will be seen in the doctor's office at least every 3-4 months at first. In many centers, there will be regular testing such as echocardiograms and 6 minute walk testing. Some centers will obtain yearly right heart catheterizations to assess the impact of therapies on pulmonary pressures and heart function.
Special considerations for patients with PAH need to be made. For example, pregnancy should be avoided at all costs as it represents significant risk to the female patient with PAH and to the fetus. Also, many of the medications used to treat PAH may harm the fetus as it develops. Because PAH is a rare and complex disease, good care for a patient with PAH involves a close relationship with an expert in PAH and support from family and friends.
Managing Pulmonary Arterial Hypertension
To best manage your PAH you should:
- Take your medication exactly as directed.
- Do not stop a medication without consulting with your doctor.
- Take care to never run out of medication.
- Avoid things that can put strain on the lungs and heart. This would include lifestyle choices such as smoking and the use of recreational drugs such as cocaine and amphetamines. There are also potential risks of worsening PAH during the course of normal pregnancy. These issues should be discussed between you and your doctor.
- Since PAH can lead to a form of heart failure, adhere to a low salt diet and limit fluid intake.
- Monitor your weight to recognize fluid retention. If your weight goes up, it is often helpful to let your physician or healthcare provider know, as an adjustment of medications may be needed.
- Develop strategies to help cope with fatigue and shortness of breath.
- Develop a careful exercise program with your healthcare providers.
- Get immunized against flu and pneumonia.
- Talk to your doctor about anxiety and depression, which are common in patients with PAH.
The Lung Association recommends patients and caregivers join our Living with Lung Disease Support Community to connect with others facing this disease. You can also call the Lung Association's Lung Helpline at 1-800-LUNGUSA to talk to a trained respiratory professional who can help answer your questions and connect you with support.
Learn more at the Pulmonary Hypertension Association.
Approved by Scientific and Medical Editorial Review Panel. Last reviewed August 4, 2016.