Managing Your PAH

You’ve done everything you can to improve. Now, how can you keep improving?

Let’s look at where you are now.

You’ve made great strides in moving beyond your diagnosis and into a treatment plan. You’re probably feeling better than you were and are adapting to your “new normal.” You may have even hit an important milestone by getting to “stable.” They are all worth celebrating.

But what’s next? Are you still improving? If not, what could you do to see more improvement?

Are you still

  • Wishing for more good days?
  • Declining invitations or avoiding making plans because you don’t feel up to it?
  • Wanting to do more in your day?
Managing your PAH and life expectancy image

Today, there’s something more: Risk assessment

PAH Treatment Guidelines now recommend that healthcare providers conduct an evaluation called risk assessment every 3-6 months. Risk assessment is a powerful tool that helps determine whether your treatment plan is working. It can even help predict your chance of survival over the next 5 years.

PAH care isn’t the same as it used to be. New tools like risk assessment as well as earlier treatment can help better evaluate your PAH and treat it appropriately. There are also more treatment options available now than ever before. Today, more than a dozen PAH medicines are approved for PAH management and many can be taken in pill form.

Have you asked your healthcare provider about a risk assessment yet?

What is risk assessment?

Risk assessment is a different way of looking at your PAH. It combines your tests and other information to create a more comprehensive view of how you’re doing.

Your risk assessment can tell your healthcare provider how well your treatment plan is working and if adjustments are needed.

In this video, PAH specialist Dr. Lana Melendres-Groves provides a detailed explanation of what risk assessment is and why it’s so important for your health.

Click to expand transcript

So, what is risk assessment?

This concept is at times very difficult to understand, even for me, who’s been doing this for quite some time, but it’s a different way of looking at PAH. We now know that this risk assessment is so essential to how we treat our patients that it’s actually become part of our treatment guidelines. It’s recommended that patients receive a risk assessment from their provider on their risk status at least every 3 to 6 months. This measures how well your treatment plan is working for you and it informs your healthcare provider if potentially changes should be made.

What happens during a risk assessment?

Treatment guidelines recommend that your healthcare provider perform a risk assessment for you every 3 to 6 months. Your healthcare provider performs a risk assessment by combining many of your evaluations, including your 6-minute walk test, WHO Functional Class, and BNP or NT-proBNP.

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6-minute walk test

During a risk assessment, your healthcare provider will measure how far you’re able to walk in 6 minutes. This 6-minute walk test gives clues about how your heart, lungs, and blood vessels are doing. In fact, studies show that out of all the tests your healthcare provider gives you, the 6-minute walk test is the best predictor of how you will be doing over the next 3 to 5 years.

Are your walk distances changing?

What’s important about your 6-minute walk distance is improvement—try to improve your walk distance. Your results on this test may indicate whether your treatment is working effectively or if an adjustment is needed.

Able to walk farther

If you are able to walk farther than you did at your last appointment, your PAH may be getting better.

Distance hasn't changed

If your distance hasn’t changed, your PAH may not be improving.

Can't walk as far

If you can’t walk as far as you walked at your last appointment, your PAH may be getting worse.

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WHO Functional Class

The World Health Organization (WHO) developed a system to place people with PAH into 1 of 4 functional classes. Functional Class is based on the symptoms you experience when doing everyday activities.

Functional Class is a strong predictor of life expectancy, and it’s an important factor that your healthcare provider uses when choosing your PAH treatments.

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NT-proBNP/BNP blood test

When your heart works harder than usual, it releases high levels of molecules found naturally in your body. These include the hormone called B-type natriuretic peptide, or BNP, and its precursor, NT-proBNP.

If this test shows high levels of NT-proBNP/BNP, it means your heart is straining and working harder than normal. The strain may be caused by narrow blood vessels in the lungs because of PAH.

Risk assessment:
A closer look

In this PAH video, Dr Lana Melendres-Groves shows an example of how a risk assessment is completed.

Click to expand transcript

[On-screen text: Risk assessment: a closer look. Your PAH specialist reviews the results for many of the tests or evaluations you have during your visit. One way healthcare providers conduct a risk assessment is by assigning a score for each test or evaluation based on its result. Then they use these scores to calculate your risk status. on-screen visual: Example risk assessment form with example variables, low-risk, intermediate risk, and high risk columns.] This is just an example of what a risk assessment looks like. It’s a table that often we color code in green, yellow, and red, because I think it helps us, just visually, to understand if we’re getting somebody to where they need to be. There’s a lot of information in this table, but we use all of those tests that we ask you to get to be able to fill it out. And it often looks something like this, where maybe you’re feeling quite good and are in a low-risk category, in the green category for how you feel and what you can do. But maybe some of the blood tests that we’ve gotten or some of the other procedures that you’ve done would indicate that actually you’re in more the yellow zone.

[on-screen text: Risk status. It’s more than a status—it can change your future.] We take all of those together and are able to come up with a score that indicates a risk status, and here, demonstrated in the bottom corner with 1.4. Knowing whether someone fits into a green zone, low-risk, an intermediate zone, the yellow, which is intermediate risk, or in the red zone, high risk, we’re able to know if somebody will do better and have a better chance of survival over the next 5 years or if maybe there’s a lower chance of survival over that time frame.

What happens after my risk assessment?

Once your risk assessment is complete, your healthcare provider determines your risk status.

The 3 different levels of risk status are:

Low risk

Low risk means there is a better chance of survival over the next 5 years

Intermediate risk

High risk

Intermediate and high risk mean there is a lower chance of survival over the next 5 years

Risk status helps your healthcare provider determine how your treatment is going and predict how you’ll be doing in the future.

How does your healthcare provider use risk status to help you?

In this video, PAH specialist Dr. Melisa Wilson explains why risk status is such an important part of creating the best possible treatment plan for you.

Click to expand transcript

Hi, I’m Melisa Wilson, nurse practitioner and program coordinator for the Pulmonary Hypertension Program at AdventHealth Orlando. I would like to talk to you today about doing risk assessment with my patients. In the past, when we discuss risk assessment, we used to talk really in terms of how a patient’s feeling, what we call your Functional Class, meaning you tell me if you feel short of breath with certain activities and then I take that and put that into a category. But as humans, we tend to be very smart. If there’s things that make us feel bad, we just don’t do them. So instead, what we have learned over time is that there’s multiple what we call variables or things that we look at, including like your labs and PFT findings and echocardiogram and of course your heart cath information that we can put all together and actually come up with a composite score, which puts you into a category of a high-, low-, or intermediate-risk patient.

This is more prognostic, meaning, telling me how you’re doing not just today but how you’re going to do long term. This number and this grouping is not permanent. It’s not that if you were categorized as high risk that that’s where you are forever. In fact, what that does is gives me, as your clinician, the opportunity to take all of this data that we used to get in pieces or parts. I get your cath data today, I get your lab some other time, I get your 6-minute walk distance, and then have to put that together to kinda say, “Hmm, am I doing enough for you as your provider?” This category or this scoring allows us to put that all together and take a look at everything at once. And then it tells me, if you’re high risk, for example, that I have a lot of opportunity as your clinician to partner with you to make you better. And I can do that through therapies, through rehabs, through diet. All of those things make a difference.

So, when I get you at high risk, my goal ultimately is to move you to low risk. Low risk is where you have the best outcomes. That’s going to happen over time. We’re going to go from high risk to intermediate risk to low risk. And it is our job together to get through that. I’m still going to consider, “How are you doing?” in all of that, but that’s just now going to be one part of the puzzle. So it gives me the opportunity to look at you holistically, as well it gives you the opportunity to have something to work towards in a notable way to notice your improvement in how you’re doing overall with pulmonary arterial hypertension. So it is an exceptionally helpful tool that we didn’t used to have in the past that I’ve become quite passionate about. In fact, with my whole clinic team, we all do this together, 100% involvement, and having these great conversations with all of our patients so that you understand how you’re doing, you can make modifications as well.

I find it changes things like your diet choices. Sometimes people, we make choices that we know aren’t always the best things for us. Maybe it has a lot of sodium. Well, now understanding that that sodium intake could increase what’s called a BNP or an enzyme your heart gives off when it’s under stress or failure, now will make many people, encourage many people, to not eat those high-sodium foods knowing that it can now impact how they’re doing long term. I also find that it helps with things like improvement in 6-minute walk test distance because it helps with the compliance with rehab because you have a goal, something to achieve.

Ultimately, our goal with treating you is to get you from high risk to intermediate risk to low risk, and low risk is where we want to keep you. Low risk we know is associated with better long-term outcomes, meaning that you’re really doing very well from a pulmonary arterial hypertension perspective. So we will partner together to get you through therapies, through rehab, to get you engaged in your healthcare so that we can ultimately get you to that low risk and help you live your best life with PAH. And that’s how I talk to my patients about risk assessment.

Deciding on the right goal for you

Did you know that according to today’s PAH Treatment Guidelines, improving your risk status is considered the key treatment goal for PAH? Improving your risk status is important for everyone. For many patients, the goal is to reach low-risk status because it is associated with better outcomes.

Your healthcare provider uses a risk assessment form similar to the example below to determine your risk status. Then, you and your healthcare provider will decide if low risk is the right goal for you—and whether changes to your treatment plan could help you get there.

Example of a PAH risk assessment form

Remember, the more scores you get in the low-risk column of your risk assessment, the better your overall risk status will be.

Download Risk Assessment (PDF)

Achieving more low-risk criteria improves your chance of survival

One study of the risk assessment criteria that are easiest to measure showed a clear trend: The more low-risk criteria you achieve quickly, the higher chance of survival over 5 years.

Watch this quick video to take a closer look at the study’s findings with Dr. Lana Melendres-Groves.

Click to expand transcript

Overall, we know that achieving more low-risk criteria improves your chance of survival. This is a graph from one of our European registries that shows your prognosis can be determined in terms of the number of low-risk criteria that were looked at at their first follow-up visit. You can see that those patients who were able to achieve 3 low-risk criteria had a 97% survival out to 5 years, while those who were unable to achieve that didn’t do quite as well. [on-screen visual: Chart showing achieving more low-risk criteria improves your chance of survival. Patients who achieved 2 low-risk criteria had about 76% chance of survival, patients achieving 1 low-risk criterion had about 67% chance of survival, patients achieving 0 low-risk criteria had about 34% chance of survival. Note: Prognosis was determined by the number of low-risk criteria at first follow-up visit. Follow-up visit had to occur within the first year of diagnosis.]

Improving your risk status

If your risk status is low today, chances are you’ll be doing better over the next 5 years.

The key to achieving low-risk status is reaching the low-risk goal on several assessments. Achieving low risk on 1 or 2 assessments might make you stable, but you still may not have achieved low-risk status.

6-minute walk test goals

Goals will vary for each person, and your risk status will affect your goal. The average 6-minute walk distance for a person with PAH is about 350 meters (about 380 yards) and the low-risk goal is walking more than 440 meters. Your healthcare provider will work with you to set your appropriate goal and value.

6 minute walk test norms for PAH patients and the low-risk goal

Functional Class goals

Functional Class 1 and Functional Class 2 are associated with lower risk status, whereas Functional Class 3 and Functional Class 4 are associated with higher risk status.

Functional Class 1

Low Risk

Functional Class 2

Low Risk

Functional Class 3

Intermediate Risk

Functional Class 4

High Risk

BNP or NT-proBNP goals

Based on guidelines, your NT-proBNP/BNP results can be categorized as follows:

Low Risk

NT-proBNP <300 ng/L

BNP <50 ng/L

Intermediate Risk

NT-proBNP 300-1400 ng/L

BNP 50-300 ng/L

High Risk

NT-proBNP >1400 ng/L

BNP >300 ng/L

It’s your move. With modern advancements, you and your doctor now have the tools to have a more complete view of your PAH. Now is the time for you to invest in yourself and your future. Ask your healthcare provider about your risk status today, so you may live better tomorrow.

Risk status: What can you do?

Are you asking your healthcare provider about all of your treatment options?

There may be more you can do to improve your symptoms. Talk to your healthcare provider often and work together to explore your PAH treatment options.

Have you found a PAH expert?

It’s important to find a PAH expert who understands the complicated nature of PAH. Find a PAH expert who has the knowledge and experience to treat this complex disease.

Have you started keeping a symptom journal?

Tracking your symptoms can help you and your healthcare provider know more about how your treatment is working. Write down your symptoms and the activities that cause them. Then, share this with your healthcare provider at every visit to help determine which treatments are right for you. Write down symptoms in a place that’s most natural for you so you’ll stick with it.

Track your symptoms

PAH symptom tracker journal

Remember, lifestyle changes may also help you feel better

In addition to taking steps to improve your risk status, you can also try making some lifestyle changes, such as

Things to think about

In this short video, Dr. Lana Melendres-Groves summarizes what you learned on this page and offers some questions you can ask your doctor at your next visit.

Want to learn more from Dr. Lana Melendres-Groves? Our site now features full-length event videos covering the latest topics in PAH care. You can also download Frequently Asked Questions to learn information that was important to others like you. Visit our events page to watch them all.

Click to expand transcript

Some other things to think about, maybe, how do you prepare for your next visit? I really ask that my patients think of questions that they can write down such as, what is my risk status? Or, is low-risk status the right goal for me? I think that also understanding your walk distance and is it improving. I ask my patients to get echocardiograms frequently. So understanding what those tests results mean may be important to you. Or, how can you improve your Functional Class? When you do improve your risk status and experience fewer symptoms, it may help you to do more in your day and build confidence about your future.

In summary, today, PAH specialists perform a risk assessment and then calculate your risk status to show how you’re doing today and whether you’re on the right track, whether more could be done to improve your symptoms and increase your chances of survival. Risk status helps the healthcare provider determine whether changes in your medication or treatment plan could help you reach more low-risk goals, lower your risk status, or improve your symptoms. [on-screen text: Achieving more low-risk goals improves your chances of staying healthier longer]

How is PAH treated today?

PAH is going to slow you down, but you can adapt and move forward. And the right treatment can help you manage your symptoms so you can pursue your goals.

How Is PAH Treated?