Understanding PAH

What is PAH?

Pulmonary arterial hypertension (PAH; WHO Group 1) is a narrowing of the arteries that carry blood from the heart to the lungs.

Narrowing of the arteries near the heart:

  • Puts extra strain on the heart, making it work harder to deliver blood to the lungs
  • Increases pressure (known as hypertension) in the blood vessel (called the pulmonary artery) that delivers blood to your lungs
  • Can lead to symptoms such as shortness of breath and fatigue when doing activities

Healthy vessel: blood can be pumped easily from the heart to the lungs

healthy blood vessel

Vessel with PAH: narrow vessels slow down the flow of blood and make the heart work harder to pump blood to the lungs

blood vessel with PAH

As PAH worsens, difficulty with breathing and fatigue may make it harder to carry out everyday tasks such as getting dressed, climbing stairs, or walking to the mailbox.

What are the symptoms of PAH?

Below are some common symptoms of PAH you might experience:

symptoms of PAH figure

Dizziness and/or fainting

Feeling tired or worn out

Shortness of breath

Chest pain

Swollen abdomen

Swollen ankles and legs

To help improve your symptoms of PAH, you and your doctor will create a treatment plan that may include medicines to help increase blood flow to the lungs.

What steps can I take to help my doctor provide the best possible care for me?

You have an important role to play and can partner with your healthcare team to determine your course of treatment.

  • Talk to your doctor about PAH and what treatment options would be best for you
  • Keep track of your symptoms and share with your doctor to help see how you are responding to treatment
  • Tell your doctor what your goals are for your future and what you want to accomplish (ie, be able to run daily errands, continue working, or take a family vacation)
woman speaking to her doctor

Why is it important to know your risk status?

Improving risk status is the main goal of PAH treatment, and having a lower risk status can improve your long-term outlook.

Your risk status tells your doctor how you’re doing today and whether your disease is getting better, staying the same, or getting worse. Your doctor uses risk status to help make decisions about your treatment and evaluate if more needs to be done to help reduce your symptoms and improve your function.

To determine your risk status, your doctor looks at many different measures, such as your 6-minute walk test and your heart function, to predict how your PAH may change over the next 5 years.

Risk status is divided into 3 categories—low, intermediate, or high. Low risk means that there’s a low chance of your PAH getting worse. If your risk level is intermediate or high, there is more of a chance that your disease will get worse. When PAH gets worse, it’s called disease progression.

Ask your doctor about your risk status and how you might improve it.

Go learn as much as you can. When you have your doctor appointments, bring a notepad and have someone go with you who can take notes and help you with all the information.

—A Remodulin patient

Get more advice from people living with PAH.

Learn More

Where can I find additional information on PAH?

Find information and support about PAH from the Pulmonary Hypertension Association (PHA) community. Visit phassociation.org

  • Connect with others
  • Join a support group
  • Find information for caregivers
  • Find a doctor or PAH specialist in your area who has pump experience
  • Discover additional support and resources
man and woman shaking hands

Learn about the PAH Initiative and their commitment to helping people living with PAH. Visit pahinitiative.com

Find out more about PAH, available treatment options by United Therapeutics, and support programs at Fighting PAH. Visit fightingpah.com

Learn more about the work being done to increase awareness and innovation in finding a cure for PAH. Visit PHAware.global

Seek out other patients. I highly recommend joining a local support group to meet others with PAH. They can share tips and knowledge or just be a shoulder to lean on.

—A Remodulin patient

Remodulin® (treprostinil) Injection

Important Safety Information for Remodulin

Before you take Remodulin, tell your healthcare provider if you:

  • Have other medical conditions or take other medicines that may affect your use of Remodulin by increasing the risk of side effects or decreasing the drug’s effectiveness.
  • Have liver or kidney problems. Your Remodulin dose may need to be adjusted if you have liver problems.
  • Have low blood pressure or bleeding problems.
  • Are taking gemfibrozil (for high cholesterol), rifampin (for infection) or other drugs that affect liver enzymes. Your doctor may need to adjust your Remodulin dosage.
  • Are pregnant, breastfeeding, or planning to become pregnant. It is not known if Remodulin will harm your unborn baby or if Remodulin passes into your breast milk.

What are the serious side effects of Remodulin?

  • Continuous intravenous (IV) infusions of Remodulin delivered using an external infusion pump, with a tube placed in a central vein within the chest, are associated with the risk of blood stream infections and sepsis, which may be fatal. Therefore, continuous subcutaneous (SC) infusion delivered just beneath the skin is the preferred type of delivery.
  • Worsening of PAH symptoms. Do not stop taking or greatly reduce your Remodulin dose without consulting your doctor.
  • Low blood pressure (symptomatic hypotension). If you have low blood pressure or are taking drugs that lower your blood pressure, the risk of low blood pressure is increased.
  • Bleeding problems. Remodulin may increase the risk of bleeding in people who take blood thinners (anticoagulants).

What are the possible side effects of Remodulin?

  • In clinical studies of SC infusion of Remodulin, most people experienced infusion site pain and infusion site reaction (redness, swelling, and rash). These symptoms were sometimes severe and sometimes required treatment with narcotics or discontinuation of Remodulin.
  • IV infusion of Remodulin delivered through an external pump has been associated with the risk of blood stream infections, arm swelling, tingling sensations, bruising, and pain.
  • The most common side effects seen with either SC or IV Remodulin were headache, diarrhea, nausea, rash, jaw pain, widening of the blood vessels (vasodilatation), and swelling from fluid retention (edema). These are not all the possible side effects of Remodulin. Call your doctor for medical advice about side effects.

You may report side effects to the FDA at www.fda.gov/MedWatch or call 1-800-FDA-1088.

The risk information provided here is not comprehensive.

What is Remodulin?

Remodulin is a prescription medication used to treat adults with pulmonary arterial hypertension (PAH; WHO Group 1), which is high blood pressure in the arteries of your lungs. Remodulin can reduce symptoms associated with exercise. Remodulin was studied mainly in patients with NYHA Functional Class II-IV symptoms. It is not known if Remodulin is safe and effective in children.

In people with PAH who need to switch from epoprostenol, Remodulin is approved to slow the worsening of symptoms.

REMISIconOct19

To learn more about Remodulin, talk with your healthcare provider. Please see Full Prescribing Information at www.remodulin.com or call Customer Service at 1-877-UNITHER (1-877-864-8437).

PAH=pulmonary arterial hypertension; WHO=World Health Organization.

Remodulin® (treprostinil) Injection

Important Safety Information for Remodulin

Before you take Remodulin, tell your healthcare provider if you:

  • Have other medical conditions or take other medicines that may affect your use of Remodulin by increasing the risk of side effects or decreasing the drug’s effectiveness.
  • Have liver or kidney problems. Your Remodulin dose may need to be adjusted if you have liver problems.
  • Have low blood pressure or bleeding problems.
  • Are taking gemfibrozil (for high cholesterol), rifampin (for infection) or other drugs that affect liver enzymes. Your doctor may need to adjust your Remodulin dosage.
  • Are pregnant, breastfeeding, or planning to become pregnant. It is not known if Remodulin will harm your unborn baby or if Remodulin passes into your breast milk.

What are the serious side effects of Remodulin?

  • Continuous intravenous (IV) infusions of Remodulin delivered using an external infusion pump, with a tube placed in a central vein within the chest, are associated with the risk of blood stream infections and sepsis, which may be fatal. Therefore, continuous subcutaneous (SC) infusion delivered just beneath the skin is the preferred type of delivery.
  • Worsening of PAH symptoms. Do not stop taking or greatly reduce your Remodulin dose without consulting your doctor.
  • Low blood pressure (symptomatic hypotension). If you have low blood pressure or are taking drugs that lower your blood pressure, the risk of low blood pressure is increased.
  • Bleeding problems. Remodulin may increase the risk of bleeding in people who take blood thinners (anticoagulants).

What are the possible side effects of Remodulin?

  • In clinical studies of SC infusion of Remodulin, most people experienced infusion site pain and infusion site reaction (redness, swelling, and rash). These symptoms were sometimes severe and sometimes required treatment with narcotics or discontinuation of Remodulin.
  • IV infusion of Remodulin delivered through an external pump has been associated with the risk of blood stream infections, arm swelling, tingling sensations, bruising, and pain.
  • The most common side effects seen with either SC or IV Remodulin were headache, diarrhea, nausea, rash, jaw pain, widening of the blood vessels (vasodilatation), and swelling from fluid retention (edema). These are not all the possible side effects of Remodulin. Call your doctor for medical advice about side effects.

You may report side effects to the FDA at www.fda.gov/MedWatch or call 1-800-FDA-1088.

The risk information provided here is not comprehensive.

What is Remodulin?

Remodulin is a prescription medication used to treat adults with pulmonary arterial hypertension (PAH; WHO Group 1), which is high blood pressure in the arteries of your lungs. Remodulin can reduce symptoms associated with exercise. Remodulin was studied mainly in patients with NYHA Functional Class II-IV symptoms. It is not known if Remodulin is safe and effective in children.

In people with PAH who need to switch from epoprostenol, Remodulin is approved to slow the worsening of symptoms.

REMISIconOct19

To learn more about Remodulin, talk with your healthcare provider. Please see Full Prescribing Information at www.remodulin.com or call Customer Service at 1-877-UNITHER (1-877-864-8437).

PAH=pulmonary arterial hypertension; WHO=World Health Organization.