Questions to ask when starting a new medication for PAH

In simple terms, blood pressure is the force that blood exerts on the walls of the blood vessels as it moves through the circulatory system.

Pulmonary arterial hypertension (PAH) is a rare disorder that causes high blood pressure in the lungs. PAH causes the blood vessels in the lungs to become narrow and inflexible. This causes the blood vessel walls to push back harder against moving blood, and blood pressure goes up.

PAH also affects the right side of the heart, which can become enlarged and weakened as it is forced to work harder to pump blood through the lungs. Right-sided heart failure and other life-threatening complications can occur.

PAH is a progressive condition

Early signs and symptoms of PAH can be subtle and “nonspecific.” Nonspecific means that signs and symptoms overlap with many other conditions. Delays in diagnosis are common.

As PAH progresses, symptoms become more noticeable and severe.

Symptoms of PAH can include:

• Shortness of breath, especially during physical activity
• Fatigue
• Muscle weakness
• Chest pain or discomfort
• Dizziness or fainting spells
• Edema, a type of swelling caused by fluid buildup, which can affect the feet, lower legs, and abdomen
• A bluish color to the lips or skin
• Arrhythmias (fast or irregular heartbeat)
• Dry cough

There is no known cure for PAH, but there are treatments that can help reduce symptoms, slow disease progression, and improve a person’s quality of life when living with the condition.

What to ask when starting a new treatment for PAH

Medications are the main treatment for PAH. Treatment often begins with one or more vasodilating medications, drugs that help relax and open up the blood vessels to help blood move easily and reduce blood pressure. Other medications slow or reduce remodeling. Many new drug therapies are under investigation, and clinical trials may be an option for some people with PAH.

As the disease progresses, a treatment plan will need to evolve to keep up with treatment needs. For example, it’s common to begin treatment with one or two oral medications, but a person will often switch to a combination of oral and infused medications if symptoms progress.

Additional therapies like diuretics to manage edema and oxygen therapy may be needed. In severe cases, a healthcare team may discuss a lung or heart-lung transplant.

Because progression can be subtle, without obvious signs or symptoms, regular checkups with a healthcare provider are essential to monitoring PAH.

Questions when starting a new medication

• What is the name and dosage of the medication?
• How does this medication work to treat PAH?
• What is the goal of treating PAH with this medication?
• How is this medication administered? PAH treatment can include medications taken as pills, inhalers/nebulizers, infusions administered every few weeks, and medications delivered continuously through an infusion pump.
• How do I store this medication?
• How will we know this medication is working?
• How can I expect to feel while taking this medication?
• What side effects can this medication cause? Is there a risk of serious side effects?
• What kind of monitoring do I need while on this medication? Medications called endothelin receptor antagonists (ERAs) require monthly pregnancy tests due to the risk of severe birth defects. Some require monitoring for liver function.
• Is there anything I need to avoid? For example, certain foods, alcohol, or activities.
• Can this therapy interact with other medications I am currently taking? Keep a list of all medications you take, including prescriptions, over-the-counter medications, and supplements (including herbal supplements).
• Who can I talk to if I have concerns about the cost of this medication
• What do I do if I miss a dose?

Questions when you are prescribed an infusion pump

Mentioned above, medications delivered with an infusion pump are commonly used in the treatment of PAH. In addition to the questions above, it’s important to review these questions when starting a medication delivered with an infusion pump:

• What medication does this infusion pump administer?
• How do I operate this device? Your healthcare provider will provide training on how to safely use the infusion pump, including cleaning hands and surfaces, changing tubing and batteries, and how to dispose of needles.
• How often do I need to change or add medication to this device?
• What are some common problems/issues and how do I troubleshoot these?
• Is there a support line I can call if I have a question or technical issue?
• How do I clean and maintain the device?
• What do I do if the device stops working? What do I do if the device becomes damaged?
• What backup supplies should I always have on hand?
• Do I need special documentation to travel with this device?

You and at least one caregiver should have training on how to use any medical device.