Your pulmonary arterial hypertension (PAH) treatment plan is designed to help control blood pressure in the lungs and relieve your symptoms. PAH medications work well, but they can’t treat every possible symptom. Pulmonary hypertension supportive therapy is an add-on treatment to pick up where PAH treatments leave off.

In this article, we’ll discuss four supportive therapies used to manage PAH symptoms and how they work. We’ll also cover the benefits and risks of each therapy and how they can help you improve your quality of life while living with PAH.

What Is Supportive Therapy?

“Supportive therapy” is the term for therapies used in addition to medications that directly treat a condition, with the goal of improving your quality of life with a disease. It can include add-on treatments and lifestyle changes that address symptoms. Supportive therapy can also help lower your risk of complications and hospitalization.

PAH is most often treated with vascular (blood vessel) medications called vasodilators. These medications correct vascular dysfunction by relaxing and opening the blood vessels. The larger the blood vessels are, the more blood can easily flow through them. This helps lower your blood pressure and treat PAH.

There are four main pathways targeted by pulmonary vasodilators to relax blood vessels:

• Prostacyclin
• Endothelin
• Nitric oxide
• Activin

Drugs that target the prostacyclin pathway are available in various forms. Formulations of prostacyclin analogs include intravenous (into a vein) infusions, subcutaneous (under the skin) injections, inhaled medicines, and oral (by mouth) tablets.

Endothelin receptor antagonists block endothelin to relax blood vessels and reduce harmful inflammation.

For the nitric oxide pathway, medications called phosphodiesterase-5 inhibitors (PDE-5 inhibitors) and soluble guanylate cyclase stimulators help open blood vessels.
Activin-signaling inhibitors help prevent harmful changes in the lung’s blood vessels by encouraging smooth muscle cells to grow in a healthier way.

Many of the medications that target these pathways can be given together as combination therapy to better treat your PAH symptoms. Your doctor can prescribe supportive therapy along with vasodilators to better manage your disease. Here are four types of supportive therapy used in PAH, how they work, and when they’re used.

1. Diuretics

An important part of managing PAH is controlling your fluid levels. The more fluid there is in your body, the higher your blood pressure may rise. Your doctor will likely have you limit your salt intake and control the amount of liquids you drink every day.

Diuretics are a class of medications that help the kidneys remove extra fluid from the bloodstream. You may be prescribed a loop diuretic like bumetanide (Bumex) or furosemide (Lasix) to help treat PAH. Loop diuretics target the loop of Henle — a part of the kidney that absorbs water and salts, including potassium. These medications prevent this absorption, making you urinate more to help reduce swelling. Due to the potassium loss by urine, potassium supplements are usually prescribed with diuretics.

If you take an endothelin receptor antagonist or riociguat (Adempas), you may be more likely to develop edema (swelling). This condition causes uncomfortable swelling in the feet and legs. Doctors use diuretics to treat potential edema or fluid buildup from vasodilator therapy. Your treatment will depend on how severe your symptoms are.

One myPHteam member shared how they manage edema with a diuretic. “I take two PH medications. Sometimes, one of them makes me retain fluid. I check my weight three times every day. When I gain 2 pounds, I take a diuretic and potassium.”

“I take two PH medications. Sometimes, one of them makes me retain fluid. I check my weight three times every day. When I gain 2 pounds, I take a diuretic and potassium.”

— A myPHteam member

When taking pulmonary hypertension diuretics, you’ll likely need to urinate more often. This is how your body gets rid of extra fluid and salts. Other side effects of loop diuretics include diarrhea, upset stomach, and dizziness.

2. Digoxin

Digoxin is an oral medication used to treat heart disease and arrhythmia (abnormal heart rhythm). According to Cleveland Clinic, digoxin strengthens your heartbeat to help the heart muscle pump blood throughout the body. This drug also calms electrical signaling in the heart to regulate your heart rhythm.

People with PAH are prescribed digoxin if they have an arrhythmia or heart failure. Doctors might prescribe digoxin together with a diuretic. However, some specialists have suggested that more research needs to be done on the safety of long-term use of digoxin for people with PAH.

Side effects you may experience while taking digoxin include stomach pain, dizziness, and breast tissue growth.

People with PAH are prescribed digoxin if they have an arrhythmia or right heart failure.

3. Supplemental Oxygen Therapy

Supplemental oxygen therapy is additional oxygen given to people with breathing difficulties. People with PAH may need supplemental oxygen when they leave their house or perform daily activities due to reduced exercise capacity. This treatment isn’t used together with any specific PAH vasodilator — it mainly helps improve your quality of life.

One myPHteam member shared the positive impacts of oxygen therapy. They wrote, “I’ve had PAH since 2016. I’ve been on oxygen for two years. I invested in a portable oxygen concentrator. I still travel and watch my grandkids play sports.”

Generally, supplemental oxygen therapy is prescribed if your blood oxygen levels fall below a certain level. Doctors measure these levels in a few different ways. The most common way is by using a finger pulse oximeter that measures the oxygen saturation in your blood. The arterial blood gas test uses a small blood sample to determine your partial pressure of oxygen (PaO2) levels. This is the pressure of gas dissolved in your blood. Doctors use your PaO2 to see how well your lungs transfer oxygen into your bloodstream.

4. Blood Thinners

Blood clots are clumps of proteins that stick together and travel through your bloodstream. If a clot becomes stuck in a blood vessel, it blocks blood flow to an area of the body. Blood clots can be dangerous if they get stuck in the lungs, heart, or brain.

To help lower your clotting risk, your doctor will likely prescribe an anticoagulant (blood thinner). These medications interfere with how your body forms blood clots. For example, the blood thinner warfarin blocks enzymes from activating vitamin K. This vitamin plays a key role in the clotting process. Stanford Medicine notes that most people with PAH take warfarin.

Some studies show that warfarin may help reduce the risk of blood clots and improve life expectancy in people with PAH. While this treatment is effective, you need to be careful when taking it. Your doctor will regularly monitor how well your blood clots. A side effect of warfarin is an increased risk of bleeding.

Some studies show that warfarin may help reduce the risk of blood clots and improve life expectancy in people with PAH.

You’ll also need to avoid foods and beverages high in vitamin K when taking warfarin. Examples include:

• Leafy green vegetables like kale and spinach
• Brussels sprouts
• Green tea

These supportive therapies can assist the treatment of pulmonary arterial hypertension and improve your quality of life. Talk with your healthcare provider if you’re interested in learning more.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 56,000 members come together to ask questions, give advice, and share their stories with others who understand life with PAH.

Have you used any supportive therapies for treatment of PAH? Which helped the most? Share your experience in the comments below, or start a conversation by posting on your Activities page.