Pulmonary arterial hypertension (PAH) is a rare but serious and potentially life-threatening condition that worsens over time. If you or a loved one was recently diagnosed with PAH, you might have a lot of questions on your mind such as: What causes it? Will it affect your day-to-day life? Is there a cure?
There isn’t a cure for PAH. But you can manage it with medications, regular care, and, if necessary, certain procedures like a lung transplant. For the best outcome, it’s important to work closely with your doctors and other members of your health care team.
In fact, research shows that if you maintain an open line of communication with your doctor, it can improve your odds overall. With routine care, your health care team can help customize a treatment plan that better serves your needs.
Communicating With Your Doctor
Good communication with your doctor is a two-way street. Clarity about what medications you need to take, how many times you need to take them, and possible side effects are all important things you should openly discuss with your doctor.
You’ll need to trust, share, and inform your doctor about your health care problems. In return, your doctor should listen to your needs and concerns, present options, and work with you and your family’s specific needs.
For your first appointment with a PAH specialist, try to come prepared. Soon after your diagnosis, the flood of PAH-related information might overwhelm or confuse you. It might be helpful to take a trusted friend or family member along for support.
Stay organized. Being on top of things can help reduce your anxious feelings or stress. Plus, it will help you maximize the short amount of facetime you get with your doctor and focus on the most pressing issues.
For example, before the appointment, write down any questions you may have. Your questions can look like:
- What is PAH?
- What’s the cause of my PAH?
- Can other conditions cause PAH-like symptoms?
- What tests or exams do I need?
- How advanced is my disease?
- What’s the standard treatment?
During the appointment, you should:
- Listen carefully to the recommendations.
- Take notes if it helps.
- Ask for clarifications, especially if your doctor uses medical terms you don’t understand.
You can also ask if you can record your appointment. This way, if you miss anything, you can come back to it.
Know your medical history. Your doctor will collect all relevant information to customize a treatment plan for you. This includes your medical history. List all medications and supplements you’re taking. Also, talk about all the health conditions you or your close family members have had in the past.
Advocate for yourself. Currently, there are over a dozen FDA-approved medications for PAH. Your treatment plan will depend on what caused your PAH and how advanced it is. If your doctor recommends certain medications, feel free to ask why they chose those particular ones.
You should ask questions like:
- What side effects can they have on you?
- Are there generic, cheaper options?
- Could they interact with any of your current medications?
- Are there new experimental drugs you qualify for?
Monitor your symptoms. Keep a close eye on your PAH symptoms. If you notice changes or new symptoms, let your doctor know as soon as possible. Don’t wait until your next appointment. If necessary, they’ll adjust your treatment plan accordingly.
Make a plan to stay in touch. Ask your doctor the best way to reach them between appointments for emergency and non-emergency concerns.
Follow Your Treatment Plan to Prevent Complications
Besides medications and routine tests, your doctor may recommend certain dos and don’ts such as exercise and lifestyle changes to manage your PAH symptoms.
Dos can include a heart-healthy diet and regular exercise, especially if PAH has caused you to have heart failure or high blood pressure. Before starting a new exercise routine, check with your doctor to see if it’s the right amount of physical activity for you.
Don’t plan a pregnancy without running it by your doctor. If you get pregnant, let your doctor know right away. That’s because PAH increases your odds of pregnancy complications. In some cases, it can be life-threatening.
If you’re planning to travel by air or to a mountainous area, check with your doctor. For some people with certain types of PAH, traveling higher than about 8,000 feet could make it harder to breathe and cause complications. At that height, oxygen levels are low and could make your symptoms worse.
Avoid saunas and hot tubs, as they could put a strain on your heart and lungs.
Follow-Up Appointments and Supportive Care
Make sure to go to your in-person follow-up appointments to keep your treatment plan up to date. Besides this, your doctor may recommend other forms of supportive care to improve your quality of life as you learn to live with PAH.
For additional care, you can:
- Join a support group.
- Get counseling or therapy from a mental health professional.
- Get your recommended vaccines to protect yourself from bacteria or viruses that could put your health at risk.
Know Your Health Care Team
Your health care team will include more than just your doctor. This can range from specialists to other health care workers, such as:
- Cardiologists (heart specialists)
- Pulmonologists (lung specialists)
- Physician assistants (PAs)
- Nurses and nurse practitioners (NPs)
- Respiratory therapists (RTs)
- Medical assistants (MAs)
- Registered dietitians (RDNs)
- Social workers
- Case managers
In some cases, your care team might include a palliative doctor. They are medical doctors who specialize in pain management. They can help control your PAH-related symptoms. Palliative doctors will also listen to your fears and concerns, whether they’re emotional, spiritual, or both.
If you’re seeing multiple specialists outside of your health care team, tell your doctor. This will help them coordinate your care and make sure your treatment plan doesn’t overlap or cause problems.
Photo Credit: John Fedele / Getty Images
American Lung Association: “Questions to Ask Your Doctor about Pulmonary Arterial Hypertension,” “How Pulmonary Arterial Hypertension Is Treated,” “Learn About Pulmonary Arterial Hypertension.”
National Heart, Lung, and Blood Institute: “Living With Pulmonary Hypertension.”
Pulmonary Circulation: “Improving communication between healthcare providers and pulmonary arterial hypertension patients: a survey of patient preferences.”
UCSF Health: “Communicating With Your Doctor.”
Pulmonary Hypertension Association: “Get to know your PH Healthcare team.”
The Open Cardiovascular Medicine Journal: “High-altitude Pulmonary Hypertension: an Update on Disease Pathogenesis and Management.”