PAH Life Expectancy & Risk Status

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 is the life expectancy of PAH patients? That is a question that I would say is very individual to each patient, each provider in terms of that discussion, so I don’t want to try to put specific time frames because I think that this is something that has evolved over the past 30 years. We can look back to our trials and registries that looked at life expectancy for patients with PAH back in the 1980s and early 90s, and we know that prior to the advent of specific medications for PAH patients, the prognosis with the diagnosis of PAH was very poor. Often, my patients will google or find online that they may have 2 to 5 years to survive after their diagnosis. I would say to that is that, over the course of the last 25 to 30 years, we’ve now been able to develop a multitude of therapies and best practices to be able to care for our patients. We can look back to one of the United States registries that was performed back in 2006 to 2009 timeframe, and we saw that we were already improving our patients’ outcomes with therapies, and that was over 15 years ago. And since then, multiple other therapies have been approved as well as our understanding of what we should be doing for our patients. So I think that the way that we look at life expectancy now is more individual to how somebody is doing, so every time a patient comes into my clinic, the goal is to try to understand where they are in their journey. That means looking at multiple tests, including blood tests and walk test and echocardiograms, and maybe even a new heart catheterization. And then, through a lot of that data, being able to assess where their risk status is. So I want to always come back to risk status, because I would say that knowing what someone’s risk status is to, you know, in some ways, helps us to predict how they may do long-term. Getting individuals to that low-risk status we know improves prognosis tremendously—that out at 5 years upwards in the 90% rang, people are surviving, Now, in situations where we may not be able to get somebody to low risk, then there may be other conversations that need to be had. But that would be the question that I would advise you to ask your providers; it’s “what is my risk status?” What does that mean in terms of my prognosis?

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, you know, 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. 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.