Jimmy Ford, MD - phaware® interview 512

Jimmy Ford, MD discusses the 7th World Symposium on Pulmonary Hypertension and his work on the Exploring the Patient Perspective in PH Task Force.

My name's Jimmy Ford. I work at the University of North Carolina at Chapel Hill where I direct the pulmonary hypertension program. I got into pulmonary hypertension when I was a pulmonary fellow about 17 years ago. At our institution, there was not really a formally organized program or cohort of patients being followed by a pulmonary hypertension specialist. Also at that time, there were a number of new therapeutics coming online and being developed, so it was a very exciting time. I managed to cohort together an increasing number of patients throughout my fellowship to the point that once I was done, they didn't want me to leave. I managed to stay on and continue to grow the program, and here we are today.

I'd like to discuss really a new, important task force that was included in the World Symposium on Pulmonary Hypertension. At the previous event, in 2018 in Nice at the Sixth World Symposium, we had the first patient perspective task force. Then, back this past summer, in Barcelona, now the second patient perspective task force. It's really, I'd like to think evolved quite a bit since the first one, and appropriately was positioned as the first task force presented at the World Symposium fittingly, since everything should be centered around the patient. It really helped to set the stage for a lot of important discussions about patient care, and brought to the forefront a number of new issues with regards to patient's perceptions of care, how they access care, how they are or are not included in important shared decision-making approaches, and making sure that their individual treatment goals are being addressed and met.

I think it's a really important topic that we'll see increasing focus on as the years go by. I think as providers we tend to get tunnel vision sometimes about we have a disease and we must treat the disease, but all the while forgetting there's a patient who's dealing with that disease. I put a quote up by Sir William Osler when I presented our recommendations at the World Symposium, and he stated that a good physician treats the disease, a great physician treats the patient who has the disease. I think that's really the spirit of this.

Every patient's journey and experience of pulmonary hypertension is different. Every patient's goals for therapy and what they want to achieve in terms of treatment are different. I think if we just take a sledgehammer approach of we must treat this disease, must treat this disease, and don't consider the individual patient's journey and how they're dealing with it or struggling to deal with it and what they want to achieve with therapy, then we're really doing them a disservice. I think that's why we're seeing this percolate ever more to the top of the agenda on the World Symposium and other important pulmonary hypertension meetings, as well.

We'll really need to lean on patient associations and patient advocacy groups increasingly as time goes on to really get their input collectively, as well. They really serve as an important avenue for patients to collectively advocate for their voice to be heard, and also a mechanism for their voice to come through in cooperation with industry and provider professional societies.

One area that we talked about was patient reported outcome measures, or PROMs, as we call them for short, which are tools that have been systematically developed to essentially capture patient's quality of life. There are a few different tools out there, some of which are specifically developed for pulmonary hypertension, some of them are more generic. At the end of the day, the intention is to capture not only symptoms of the disease, but also how the symptoms affect the patient's life, and also how the effects of treatment affect the patient's life.

Trying to find ways to get clinicians to adopt a more systematic inclusion of the use of those tools in clinical practice is really something that we advocated for. Not really coming down to preferring any one particular instrument over the other, but simply just to try to encourage patients to do this systematically. There are a lot of potential benefits. It helps to facilitate the shared decision-making approach, in that you are collecting quality of life information from the patient in a structured manner that is repeated hopefully in serial encounters over time, such that you can compare and contrast how the patient may or may not be achieving a better quality of life with therapeutic interventions or having their treatment goals met. And really thinking about, well, how can we better facilitate their use in clinical practice? The one thing we always hear from providers is that there's just not enough time in the clinical encounter to do this, right? It's a tool you've got to give to the patient, they've got to fill it out, it takes some time.

Ideas like electronic versions of these tools, which are in development, and can be provided to the patient ahead of a visit. Patient can fill them out, it doesn't take time away from the clinical encounter. We can set reminders to have the patient fill them out or to remind us to have the patient fill them out at certain intervals. They also can be tools which help to determine whether quality of care that's being delivered at a particular center is at a certain desired level, and hopefully improving over time, as well. I think as these are developed further and developed into formats that are more readily digestible and can be completed more easily in the clinical encounter or even in advance of it, we will see better adoption of them.

Another issue is, thinking more globally, how many languages are some of these translated into? If you're looking at the pulmonary hypertension-specific PROMs, I think the most that any one of them is translated into is about 20 to 30 languages, whereas some of the more generic ones are available in well over 100 languages. We prefer to use a pulmonary hypertension-specific PROM, that was our recommendation. But we still have to do a lot more research about things like, how often should patients be filling these out? And do they potentially have some value in conjunction with the risk assessment tools to help actually inform the risk profile of the patient too, not just their quality of life? We do have a few studies out there that have shown that the PROM scores, particularly for EmPHasis-10 and the SF-36 tool ,at the time of diagnosis, have translated with outcomes over time. We still have a lot to learn and explore about how to best utilize these tools, but I think at minimum we need to find a way to start using them more routinely in clinical practice.

One thing we did is we polled a variety of patients, providers, and patient advocates throughout various areas of the world across almost every continent. They were distributed all throughout South America, Africa, Europe, Oceania, Asia, so a really varied sample of patients in terms of social situations and geopolitical situations. There were a lot of similar recurring themes that we saw from these survey responses, including generally an impaired lack of access to expert pulmonary hypertension care in centers. In conjunction with that, relatively poor access to diagnostic procedures, particularly the most important one in pulmonary hypertension, the right heart catheterization.

If we're looking at this globally and we can't get a significant number of patients to right heart catheterization, the discussion about the disease is moot because we don't even have a diagnostic foundation to begin with. We talked about some initiatives that have been employed in the past and that might be expanded upon to improve access to the right heart catheterization, whereby medical centers throughout Europe, South Africa, United States, have partnered with underserved areas, particularly two in Africa, Uganda, and the Morning Star Clinic in Zambia, where cardiology teams have actually traveled to these places and provided expertise, equipment and ability to get those sites set up to do right heart catheterizations. But again, that's just a few places, and there's still large swaths of the world where access to that remains poor.

Similarly, distance that patients are traveling to pulmonary hypertension centers can be quite far either in time and or distance. Clearly, we need more expert centers, more expert providers throughout the world in underserved areas. It's interesting, even in the relatively underserved or global south areas of the world, even beyond those in fairly well-resourced nations within them, we see significant disparities in healthcare access and delivery. It's really a complex problem, which certainly is not limited to just pulmonary hypertension, but I think when you've got a serious chronic, relatively rare disease where the therapeutic regimens can be quite complex, these obstacles become all that much greater to overcome and, more importantly, to address.

Another few areas that we addressed in our task force included, for example, climate change. In general, the idea of adverse humanitarian situations, which can include climate change, armed conflicts, pandemics, all of which we are seeing unfortunately throughout our world with increasing intensity and frequency. So recognizing what are some of the challenges that each of these adverse situations brings, particularly to a pulmonary hypertension patient.

For example, with the topic of climate change, we know that poor air quality, increased air pollution, extreme heat and weather events leading to storms, flooding, et cetera, can really cause a lot of issues for patients with cardiopulmonary diseases, can increase symptoms of dyspnea, cause acute exacerbations. There's the potential for waterborne and vector borne illnesses with flooding, all of which on top of a complex serious disease like pulmonary hypertension can really spell a lot of trouble.

Then, with regards to armed conflict, like any individual, and especially those dealing with chronic rare complex diseases like pulmonary hypertension, life is disrupted significantly, right? People are displaced potentially, their access to medications and care is interrupted substantially due to their likely geographic displacement or to the destruction of that healthcare infrastructure or medications. Really the best way that we could think of to try to address or mitigate some of these things is with advanced contingency planning of multiple different types, forming cross-border partnerships, really educating the patient about what to do in these situations, making sure that they are maintaining a supply of their medications, that they know where to go in the event that they cannot reach their usual care team, that they have access to telecommunication devices if telemedicine or e-health is necessary to be employed.

Every situation is different, of course, with these adverse humanitarian situations, but we thought it was important to call them out as they're becoming increasingly problematic, and to try to come up with strategies to preemptively deal with them.

Unfortunately, methamphetamine use is becoming an increasingly noted cause of pulmonary hypertension that we see in a lot of pulmonary hypertension clinics across the US and even the world depending on where you are specifically. We didn't address that specifically from the context of the patient perspective, but it certainly is an emerging problem, which does have some geographical predilection. In my part of the US, it's not so much of an issue. I know in many parts of California and Western US up to and including regions around the Mississippi River it is a huge problem, and probably other urban centers throughout the eastern US too. I think it really depends on where your practice is located.

Meth use really plays into the idea of social determinants of health. The reasons that people get into the use of methamphetamine are likely related to a lot of what we call allostatic load metrics that affect one's health. Being poorly, financially resourced, having low general and or health literacy, and having a lot of life or psychosocial stressors which lead one down the path of substance abuse. It is relevant in that regard in terms of addressing the social determinants of health. So probably should be something that is addressed in the next patient perspective task force, particularly if it continues to be an ever-increasing problem in pulmonary hypertension clinics.

One other important aspect is increasing the diversity of patients that we include in our registries and our clinical trials. For the longest time, those cohorts have consisted largely of non-Hispanic white patients, if you look at the percentage. It's not getting any better as time goes on from when we really started doing pulmonary hypertension trials a few decades ago, to even our most recent trials with Sotatercept, for example, we're still seeing overwhelmingly non-Hispanic whites being enrolled.

That's problematic in the sense that we sort of take the findings and results of these trials as generalizable to our entire patient population that we treat in the real world outside of clinical research. That may not be true. As it is, consider that we already see significant variability in treatment responses to pulmonary hypertension therapeutics between individuals even within the same race. You can imagine that genetic pharmacogenomic differences certainly exist on a racial basis that we're not capturing in our clinical trials. I think we need to be much more intentional about enrolling certain percentages of races and ethnicities in clinical trials so that we can stand on a better and firmer ground when we say that we can truly generalize these findings across our clinic patients.

This is really just a top-line look, a 30,000 foot view, if you will, of the world of the patient perspective and pulmonary hypertension. I think it's a really challenging area because so many factors inform it, right? We'll continue to uncover more, and we should continue to uncover more as time goes on. But I think it's important to really tackle the ones we've identified now head-on, and be ever mindful of emerging ones like methamphetamine, as I mentioned.

My name is Dr. Jimmy Ford, and I'm aware that my patients are rare.

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