Pulmonary arterial hypertension (PAH) is a form of pulmonary hypertension that causes the small arteries of your lungs to thicken and narrow. This can lead to high blood pressure in your lungs.
While thereтАЩs no cure for PAH, there are many treatment options that can help control your symptoms. ItтАЩs important to work closely with the doctor to ensure your PAH treatment continues to work.
тАЬTreatments range from medication all the way up to transplantation,тАЭ says Richard N. Channick, MD, a pulmonologist at UCLA. Your treatment plan might include:
Vasodilators. Blood vessel dilators, called vasodilators, help relax and open your narrowed blood vessels to help blood flow. Your doctor may give you treatment through an intravenous (IV) infusion, under the skin, as a pill, or through inhalation. With inhalation, youтАЩll breathe in the medication through a machine called a nebulizer.
Anticoagulant medications. These drugs can help prevent blood clots. The most common form is warfarin (Coumadin, Jantoven).
Diuretics. These are “water pills” that help get rid of extra fluid in your body.
Digoxin. This medication can help ease your symptoms, strengthen your heart muscle contractions, and slow down your heart rate.
Oxygen treatment. With this therapy, youтАЩll inhale air that has a higher concentration of oxygen than normal air.
Surgery. In some cases, you may need surgery. There are a few different types, including pulmonary endarterectomy, balloon pulmonary angioplasty, atrial septostomy, and transplant.
There are other treatments less commonly used for PAH as well.
тАЬWe have this big list of potential medications that we can choose from. Which medications we choose and how we use them is also a very important topic,тАЭ Channick says.
The main goal of treatment is to ease symptoms and slow the progression of your condition. If your PAH seems to be getting worse, you may need to explore new treatment options.
How Can You Tell if Your PAH Treatment Is Effective?
тАЬIt doesn’t matter as much how you are at day one; it’s really how you’re responding to therapies that will determine how you’ll do long term,тАЭ Channick says. There are a few different ways to measure the success of someoneтАЩs PAH treatment:
Functional class. Doctors may simply ask how youтАЩre feeling with the current form of treatment. TheyтАЩll have you rate your symptoms on a scale, which experts refer to as a functional class.
тАЬThe functional class ranges from one to four. One being the [person] has no limitations to activity, four means they get symptomatic even at rest or with minimal exertion, and two or three being in between,тАЭ Channick says. тАЬTheir functional class can help us determine how they’re going to do and whether they need additional therapy.тАЭ
Exercise capacity. тАЬWe can measure that using what we call the тАШ6-minute walkтАЩ test, or how far a patient can walk up and down a hallway in 6 minutes. It’s a pretty strong measure of how a patient is doing,тАЭ he says.
Other tests. тАЬThen we have things that we measure more directly, such as blood tests, an echocardiogram to look at how the right ventricle is functioning, or in some cases, even doing a repeat heart catheterization,тАЭ says Channick.
No matter which method your care team uses, itтАЩs important to check in with your doctor to let them know how youтАЩre doing. Every 3 to 4 months is ideal. DonтАЩt wait until you think your condition has gotten worse. ItтАЩs easier for them to determine your risk level with regular appointments and tests.
тАЬIt’s important that you come in regularly, regardless of the presence or absence of symptoms,тАЭ says Channick. тАЬWe have many examples of [people] who felt like they were doing pretty well, but maybe they weren’t doing as well as they thought.тАЭ
Symptoms donтАЩt always tell the whole story, but itтАЩs still important to pay attention to how youтАЩre feeling.
тАЬAre you noticing a decrease in you exercise tolerance? For example, things you could do a month ago, youтАЩre now no longer able to do,тАЭ Channick says.
Weight changes are another potential warning sign.
тАЬOne of the problems with PAH that isnтАЩt responding to treatment is fluid retention. It may not always be apparent. People hide fluid in places they can’t even see,тАЭ Channick says. тАЬGetting regular weight checks may help us prevent a real problem or even a need for hospitalization.тАЭ
Your treatment may also not be working well if you notice other symptoms, like:
- Shortness of breath with normal activities (like going up the stairs)
- Fatigue
- Dizziness
- Fainting
- Swelling in your ankles, belly, or legs
- Chest pain
- Bluish skin or lips
- A racing heartbeat
- An irregular heartbeat
- Trouble breathing even when youтАЩre not doing anything
What Happens if Your PAH Treatment DoesnтАЩt Work?
If one treatment doesnтАЩt control your symptoms, itтАЩs likely something else will.
тАЬMost [people] start on two different medications. Then, we do risk profiling, and if they’re not at a low risk, and they still have limitations, then we’ll often add a third drug to the regimen,тАЭ Channick says. тАЬSo they may end up on three different treatments for their pulmonary hypertension.тАЭ
How you respond helps determine whether youтАЩre a good fit for infusions.
тАЬItтАЩs generally thought that the infusions can help even when the pills or the other medicines aren’t working. Ultimately, if none of that is working, then we consider lung transplantation,тАЭ Channick says.
But that doesnтАЩt happen often.
тАЬThe majority of patients benefit from just the current therapies and don’t need an extreme approach,тАЭ Channick says. тАЬBefore these medical therapies, the average survival was less than 3 years with this condition. Now, we have long-term survivors. We still can do better, but we’ve certainly come a long way.тАЭ
