Living With PAH: How to Manage Oral Treatment

This article is for people that have pulmonary arterial hypertension (PAH), or anyone who wants to learn more about treating PAH. The goal of this patient education activity is to give you tips for taking pills for PAH so that you can achieve your treatment goals. 

You will learn about:

• Medicines used to treat PAH and their side effects

• How side effects for oral treatment are managed

• Tips for staying healthy

• Questions to ask your doctor

Before you start, please answer this question. At the end of the program, you'll have a chance to answer the question again and then see the correct answer.

PAH happens when there is high blood pressure in your lungs. Over time, the blood vessels in your lungs constrict and become narrow, resulting in less blood and oxygen flowing from your heart to your lungs. This also increases pressure in your lungs and heart, making them work harder.

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There are 3 main types of medicines used to treat PAH:

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

• Endothelin receptor antagonists (ERAs)

• Prostanoids

These medicines work in different ways to open up the blood vessels in the lungs. Most people with PAH will start on 2 medicines, a PDE5 inhibitor and an ERA. Some people may need to add a third medicine like a prostanoid. 

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Taking 2, sometimes 3, medicines for PAH can help you achieve your treatment goals, like improving your symptoms or having more energy to do the things that you love. Most importantly, it will help slow down the progression of PAH.

Remember, adding another medicine does not necessarily mean that your PAH is getting worse, but it may help you to stay ahead of this serious disease.

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PDE5 inhibitors and sGC stimulators help the blood vessels in the lungs to relax and widen. This helps decrease pressure in the lungs and on the heart so that they can work better.

Pills available include: sildenafil (Revatio®), tadalafil (Adcirca®), riociguat (Adempas®).

Common side effects of PDE5 inhibitors and sGC stimulators include:

• Low blood pressure, headache, dizziness

• Stomach issues: heartburn, gastritis, nausea, vomiting, diarrhea, or constipation

• Others: nasal congestion, nosebleed, swelling in the legs

Some more serious side effects include: lung infection and numbness.

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ERAs stop the blood vessels in the lungs from constricting or "clamping down." This way, the blood vessels become less narrow and relax, allowing more blood and oxygen to flow from the heart to the lungs.

Pills available include: ambrisentan (Letairis®), bosentan (Tracleer®), macitentan (Opsumit)®.

Common side effects for ERAs include:

• Swelling in the legs or abdomen

• Stomach issues: pain or constipation

• Sinus problems: nasal congestion or headache

• Urinary tract infection

Some more serious side effects include lung infection or feeling like you have the flu.

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Prostanoids help open up the blood vessels in the lungs and help ease symptoms of PAH, including chest pain and shortness of breath.

Pills available include: selexipag (Uptravi®) and treprostinil (Orenitram®).

Common side effects of prostanoids include:

• Headache

• Stomach issues: diarrhea, nausea, vomiting

• Muscle or joint pain

• Flushing of the skin

A more serious side effect is jaw pain.

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Pills for PAH are generally well-tolerated. Most side effects can be managed at home and many improve over time.

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

• Having you take an over-the-counter medicine for mild side effects like headache or diarrhea

If side effects are intolerable, talk to your doctor. Your doctor can lower your dose or prescribe medicines to help with those side effects.

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Along with taking your medicines for PAH, here are some tips so that you can have an active, healthy lifestyle without over-exerting yourself:

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

• Stay active: Walking, biking, swimming, and light resistance training are acceptable for shorter periods of time. Stop the activity if you have a PAH symptom like feeling lightheaded, short of breath, chest pressure, or like your heart is beating very fast

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

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• How can I reach my treatment goals for PAH?

• Which medicines are right for me?

• What are the side effects I should know about?

• How can I prepare for the side effects?

• When should I get emergency help? 

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Congratulations!

You have successfully completed the program Living With PAH: How to Manage Oral Therapy

View Additional Materials 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