Treatment for PAH: What You Need to Know

This article is for people who have pulmonary arterial hypertension (PAH), or anyone who wants to learn more about PAH. The goal of this patient education activity is to improve your understanding of treatment options for PAH.

You will learn about:

• What PAH is

• Treatment options for PAH

• Possible side effects of medicines used to treat PAH

• What to expect with your treatment for PAH

• Questions to ask your doctor

When you have PAH, it means that there is high blood pressure in your lungs. This happens because the blood vessels in the lungs constrict (tighten) and become narrow over time, resulting in less blood and oxygen being able to flow to the heart.

This pressure build-up causes your lungs and heart to work harder. It also leads to PAH symptoms like feeling short of breath, having chest pain, or low energy levels.

It's important to treat PAH so that it doesn't get worse over time. There are treatments that help manage PAH symptoms, and those that help to treat the condition itself.

General treatment for PAH symptoms can include:

• Diuretics (water pill) to remove extra fluid from the body

• Blood thinners to help prevent blood clots in the lungs

• Oxygen therapy to help you breathe more easily

• Treatment for other health conditions, such as high blood pressure or sleep apnea

• Healthy diet and exercise program

Treatment for PAH can include medicines, such as:

• Phosphodiesterase type 5 (PDE5) inhibitors/soluble guanylate cyclase (sGC) stimulators

• Endothelin receptor antagonists (ERAs)

• Prostacyclins

These medicines help treat PAH by opening up the blood vessels in the lungs.

For someone with more advanced PAH, a lung transplant may be recommended as a treatment option.

PDE5 inhibitors/sGC stimulators help the blood vessels in the lungs to relax and widen.

Possible side effects can include:

• Low blood pressure, headache, dizziness

• Stomach issues, such as heartburn, gastritis (inflammation of the stomach), nausea, vomiting, diarrhea, or constipation

• Nasal congestion, nosebleed, swelling in the legs, lung infection, numbness

ERAs stop the blood vessels in the lungs from constricting (tightening) or "clamping down."

Possible side effects can include:

• Swelling in the legs or abdomen

• Stomach issues, such as pain or constipation

• Sinus problems, such as nasal congestion or headache

• Lung or urinary tract infection

• Feeling like you have the flu

Prostacyclins help open up the blood vessels in the lungs and ease some symptoms of PAH.

Possible side effects can include:

• Headache

• Stomach issues, such as diarrhea, nausea, vomiting

• Muscle, jaw, or joint pain

• Flushing of the skin

Talk to your doctor openly about any difficulties you are having with your treatment for PAH, including any side effects. Your doctor may help you manage side effects by:

• Starting one medicine at a time

• Starting at a low dose and then slowly increasing the dose

• Spacing out the medicines during the day and night

• Recommending an over-the-counter medicine that you can get without a prescription for side effects like headache or diarrhea

If you cannot tolerate a side effect, talk to your doctor. Your doctor may lower your dose or recommend medicines to help with those side effects.

Three things you can expect with your treatment for PAH are:

• Most people will start with 2 medicines, an ERA and a PDE5 inhibitor. Some may need a third medicine to help them reach their PAH goals

• Side effects with PAH medicines may happen. Most of them get better over time and can be managed with the help of your doctor

• PAH is a lifelong condition, so it's important to keep taking your medicines every day

Along with taking your medicines for PAH as directed, some tips that can help you have an active, healthy lifestyle without over-exerting yourself include:

• Conserve your energy: Prioritize tasks, have your groceries delivered, and ask for help with errands

• Stay active: Ask your doctor about walking, biking, swimming, and light resistance training as acceptable exercises for short periods of time. Stop if you have a PAH symptom like feeling lightheaded, short of breath, chest pressure, or like your heart is beating very fast

• Eat healthy: Keep a nutritious diet with fresh fruits and vegetables and lean meats. Avoid fatty foods. Limit salt intake as that can cause swelling and may make your symptoms worse

It's important to have open communication with your doctor throughout your journey with PAH. Questions you can ask at your next visit include:

• What is my treatment goal for PAH?

• Which treatment options are right for me?

• What can I do to help manage side effects?

• When should I get emergency help?

Congratulations!

Congratulations! You have successfully completed the program Treatment for PAH: What You Need to Know

View Additional Materials on this topic that you may find useful:

Pulmonary Hypertension Association: Living With PH

Pulmonary Hypertension Association: Empowered Patient Online Toolkit

American Lung Association: Treating and Managing Pulmonary Arterial Hypertension