If you’ve been diagnosed with pulmonary arterial hypertension (PAH), you likely know that PAH is a type of high blood pressure that occurs when the lungs’ blood vessels are narrowed or obstructed. You also likely know that it causes the right side of the heart to work harder to keep up with blood flow and can lead to heart failure. What you may not know is when to see a doctor about PAH. This article discusses seven questions to help you decide if it’s time to make an appointment.

If you notice changes to your health or think it’s time to call your doctor, don’t wait for someone else to tell you it’s OK. You know your body best. But if you’re on the fence, asking yourself the following questions may help guide you.

1. Are Your Symptoms Progressing?

A form of pulmonary hypertension (PH), PAH is rare and progressive — it gets worse over time. Common symptoms include:

• Fatigue
• Shortness of breath
• Chest pain
• Palpitations (pounding or racing heart)
• Dizziness or fainting
• Coughing
• Discolored lips or fingers (blue, gray, or white, depending on skin tone)
• Swelling of feet, ankles, abdomen, or neck

Symptoms of PAH may be mild at first and often overlap with those of common lung diseases like asthma and chronic obstructive pulmonary disease (COPD).

Consider enlisting a physician who specializes in treating PAH, such as a pulmonologist or cardiologist, as it’s a rare disease. They can tell you which signs of disease progression to look out for and when to call for help. Progressing disease could be indicated by worsening of the common symptoms listed above, as well as the following:

• An increase in resting heart rate, typically higher than 100 beats per minute
• Unexplained weight gain
• Nausea or decreased appetite

If your PAH symptoms are getting worse, it might be time to start or change PAH treatments. Talk to your health care provider to ensure you get the care you need.

“I kept telling my cardiologist I couldn’t breathe, and it was getting worse,” said one member of myPHteam. “I had an echocardiogram and was sent to the pulmonologist, where I had breathing tests done. Within a few weeks, I had a right heart catheterization, which showed moderate to severe narrowing of my artery. That’s why I couldn’t breathe. They put me on a new treatment, and I feel great now. Tell your doctor how you feel, and be persistent!”

2. Do You Have New Symptoms?

Developing new symptoms can be another reason to see a doctor for PAH. Experiencing new symptoms after previously being asymptomatic (not having symptoms) could indicate that the disease is progressing. If you were experiencing chest pain or shortness of breath at the time of your diagnosis and now your ankles are swollen or your lips or fingers look blue, gray, or white, your condition may be advancing.

“I am on oxygen at night, but no PAH medication yet,” said one myPHteam member. “I am in between the mild and moderate stages. My doctor said he would prescribe medication when I reach the moderate stage.”

Another myPHteam member recommended, “Keep a journal of symptoms and what you were doing when you experienced them so you can discuss them with your doctor.”

3. Are Daily Activities Becoming Harder To Do?

Physical functioning refers to your ability to perform activities that are part of your daily life, such as:

• Bathing
• Getting dressed
• Eating
• Caring for family and pets

If you notice these activities are increasingly difficult, your doctor may be able to help. It’s also an important data point for your health care team as they monitor your disease progression and figure out how best to support you.

“I get tired easily and short of breath when I go up stairs mostly,” said one myPHteam member. “When I try to walk for exercise, I’m finding it harder to do because of shortness of breath, but if I can keep it from getting worse, I’ll be happy.”

4. Have Side Effects Become Unbearable?

Like all medications, treatments for PAH can lead to some unwanted results. Common side effects of PAH medications include:

• Headaches
• Nausea or bloating
• Vomiting
• Diarrhea
• Light-headedness
• Flushing (a feeling of warmth)

You may also experience a rash, upper respiratory congestion, or other side effects.

If you’re having trouble managing your side effects, tell your health care provider. Depending on how the medication works, your doctor may adjust your dosage, prescribe a different drug, or suggest waiting a bit before making a change. Ask your doctor what to expect anytime you start a new drug and when to call them if you have concerns.

“I just started on a third medication for my PAH,” said one myPHteam member. “Hopefully, all goes well this time, and I have no side effects.”

5. Is PAH Affecting Your Mental Health or Quality of Life?

Being diagnosed with a progressive disease can affect your mental health. If you’re struggling with your diagnosis, tell your health care provider. They can connect you with support groups and other mental health resources.

“This is a little out of my comfort zone, but I feel like it’s time,” a myPHteam member said about sharing their PAH experience with others. “I don’t think anyone in my family gets it at all. I get really anxious and very scared.”

PAH can also affect your quality of life, but your doctor can help. For example, they may prescribe a diuretic to reduce swelling and make you more comfortable, or they may create an exercise plan scaled to your current energy levels. Ask for what you need. If they can’t provide it, they should be able to refer you to someone who can. You and your doctor are a team.

“If you are in pain, ask your doctor for pain medication to help you sleep through the night,” recommended one myPHteam member.

6. Do You Want To Explore New or Different Treatments?

Treating PAH is not a one-size-fits-all approach. If you want to explore new or different options, talk to your doctor and tell them why. Did you read about a new PAH treatment or clinical trial (research study of a new treatment)? Have you been receiving IV treatments but want to try oral medication?

The general goal of PAH treatment is to improve quality of life and slow disease progression. You and your health care provider should work together to create a treatment plan based on your diagnosis, symptoms, overall health, personal treatment goals, and other factors.

“When I was first diagnosed, I was only seeing my primary care physician,” one member of myPHteam said. “He is certified in pulmonology and provided some helpful tips. However, it wasn’t until I started seeing a specialist for PAH that medications specifically for PAH were prescribed, and I began to see more progress.”

7. Do You Have Questions About Genetic Testing?

About 15 percent to 20 percent of people living with PAH have a hereditary form of the disease. In these cases, experts believe a genetic mutation causes changes to the cells that line the blood vessels, resulting in the narrowing or other pulmonary artery damage that leads to PAH.

Because parents can pass genetic disorders to their children, some people with PAH pursue genetic testing, which usually involves sending a blood sample to an approved laboratory. If you have questions about genetic testing, discuss them with your doctor. They can explain the risks and benefits and refer you to a genetic counselor for further support.

“I found out I have hereditary PAH, and my twin daughters are carriers of the genes,” one myPHteam member said. “They will have yearly echocardiograms to catch a change early if it ever occurs.”

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 49,000 members come together to ask questions, give advice, and share their stories about life with PH and PAH.

Do you have questions about when to see a doctor for PAH? Do you have advice for others who might be wondering if they should call their doctor? Share your thoughts in the comments below, or start a conversation with others on your Activities page.