Whether you’ve been on combination therapy or are just starting it for pulmonary arterial hypertension (PAH), you may have questions for your doctor. Changing medications or beginning a new treatment can be an overwhelming experience. Don’t be afraid to ask your doctor how your medications work and what to expect. It may help you feel more empowered in your PAH treatment journey.

Preparing your questions before a doctor’s visit can help you make the most of your time during an appointment and get the information you need. Research shows that effectively communicating with your health care team about your PAH can help improve outcomes.

The following questions can be used as a starting point to learn more about combination therapy for PAH.

1. What Is Combination Therapy for PAH?

You may have read about combination therapy for PAH, or maybe your doctor has brought it up during an appointment. Combination therapy involves taking two or three different medications to better manage PAH.

When you’re prescribed two medications it’s called dual or double therapy, and getting three medications is called triple therapy. Consider asking your doctor more about this type of treatment and why it may benefit you.

If you’re already taking one medication to treat your PAH, your doctor may suggest adding another to help better control your symptoms. If you’re newly diagnosed with PAH, they may prescribe you one or two medications to start — the choice typically depends on the severity of your PAH. Your doctor will use treatment guidelines to determine which strategy is right for you.

2. What Medications Will I Take for Combination Therapy?

If your doctor suggests combination therapy, ask about which medications you’re being prescribed and why. If you’ve been on one medication for a while, you may be given another to help further reduce your PAH symptoms and prevent the risk of complications, such as right heart failure.

PAH medications used as part of combination therapy include:

• Endothelin receptor antagonists (ERAs) — Ambrisentan (Letairis), bosentan (Tracleer), macitentan (Opsumit)
• Phosphodiesterase-5 (PDE-5) inhibitors — Sildenafil (Revatio), tadalafil (Adcirca)
• Soluble guanylate cyclase (sGC) stimulators — Riociguat (Adempas)
• Prostacyclin medications — Treprostinil (Tyvaso, Orenitram), iloprost (Ventavis), epoprostenol (Flolan, Veletri), selexipag (Uptravi)

“I take both ambrisentan and sildenafil and know quite a few people that are on double therapy. There are three pathways in treating PAH, and some people are on triple therapy. My understanding is they all target different pathways. I was just into my PAH clinic for my six-month checkup on Monday, and we discussed just that. My specialist did not feel the need to add a third, as he said the combination was working well even after 10 years,” shared one myPHteam member.

Others have asked about starting combination therapy: “Were you ever taking sildenafil alone? If so, how did that work for you? I’ve been on it for over three weeks, and everything has just gotten worse. Did they add the other drugs one at a time?”

Another member responded, “Yes, I was taking sildenafil alone. It didn’t really work, so they added Tyvaso. Then they added Opsumit, and I really started to improve.”

Ask your doctor questions about your medications, why you’re taking them, and whether a second or third treatment may be beneficial for you. Understanding your doctor’s approach to your treatment can help you feel more empowered on your PAH journey.

3. What PAH Symptoms Will Improve With Combination Therapy?

If your doctor recommends combination therapy, you may have some questions about the changes and improvements you can expect to see in your symptoms. If you already take medication for PAH, you may be curious about what other benefits an additional medication would offer.

At your next doctor’s visit, ask about the benefits of combination therapy for your specific case of PAH. Your doctor may recommend combination therapy as a way to improve your blood pressure or six-minute walk test results or to help you feel better overall. Studies show that combination therapies improve symptoms and PAH disease outcomes better than monotherapies.

You may want to see improvements in specific symptoms. For example, maybe you’ve been increasingly short of breath or you’ve noticed swelling in your legs. Ask your doctor which medications could best treat these specific problems.

4. What Side Effects Can I Expect From Combination Therapy?

When beginning new medications, be prepared for potential side effects. If you notice any new symptoms, check in with your doctor to see if it’s a common experience or if you might be having a bad reaction to a medication. Managing medication side effects can help you stay on track with your treatment — many people stop treatment when they begin having unpleasant side effects.

Combination therapy with two or more PAH medications has been shown to increase the risk of side effects. That’s because these medications work to widen your blood vessels and decrease blood pressure. If you’re getting more headaches or feeling faint from low blood pressure, tell your doctor.

Your doctor may also offer ways to help manage these side effects. If side effects begin to interfere with your day-to-day activities, your doctor may change your dose or switch you to a different medication. Side effects are an uncomfortable part of many treatments, but asking questions can help prepare you for what’s to come.

5. What Other Medications Can Help Treat My PAH?

In addition to PAH-specific medications, other treatments are available to help manage side effects and lower your risk of complications. If you’re interested in learning more about these, ask your doctor which medications may be best for you. Some include:

• Anticoagulants (blood thinners) — Prevent the formation of dangerous blood clots that may lead to heart attack or stroke
• Diuretics (also called water pills) — Help your body excrete extra fluid and reduce your blood volume to take pressure off your arteries
• Calcium channel blockers — Help widen your lungs’ arteries and decrease the amount of force your heart needs to pump blood

You may already take some of these medications to help manage your other health conditions. Ask your doctor how they help and if they may interact with medications you take for PAH. Some people see multiple specialists to treat their PAH, including cardiologists (heart doctors) and pulmonologists (lung doctors), so make sure to stay informed about your treatment plan.

Talk With Others Who Understand

On myPHteam, the social network for those who live with PH, more than 49,000 members come together to ask questions, give advice, and share their experiences of life with pulmonary hypertension.

Are you taking dual or triple combination therapy to manage your PAH? What questions have you found beneficial to ask your doctor? Share your experience in the comments below, or start a conversation by posting on your Activities page.