John Kingrey, MD - phaware® interview 538

Dr. John Kingrey exposes the rising crisis of meth-associated pulmonary hypertension, a condition spreading fast across the U.S. He challenges misconceptions, highlights urgent research, and stresses the need for compassion in treating both PAH and addiction.

This Special Edition Episode Sponsored by: Johnson & Johnson

My name's John Kingrey. I live in Oklahoma City. I'm a pulmonologist. I've been doing pulmonary hypertension for 10 years now. I came to Oklahoma with my wife and three kids to try to put Oklahoma in the middle of the pulmonary hypertension community. Since our time there, we've been able to build a pulmonary hypertension center. We have done that with the strength of an amazing team. Now, fortunately, I also have a partner who's helping me with that, Dr. Nick Shelburne. We're growing and we're trying to do good things in Oklahoma.

I'm excited today to talk to you about a subtype of pulmonary arterial hypertension that is incredibly common and is getting more common by the day, which is pulmonary arterial hypertension due to methamphetamine use. I recently had an opportunity to present some new research at the Team PHenomenal Hope Research Day in Boston. I’m excited to be a part of research that is really kind of peeling back the covers of a problem that's been a known for a while, but is really now becoming mainstream and center court for a lot of us taking care of PH across the country.

Methamphetamine use is actually a global problem. Really since the turn of the century, it has just continued to grow in popularity among recreational drug users. We do see a heavy, heavy burden of methamphetamine use in the Western part of the United States historically, but part of the research that we have done collaborating with partners in industry have really demonstrated that it is not a Western phenomenon anymore.

I think it's important to understand that it is a global problem because some people think that it just doesn't affect them depending on where they live. Certainly there are areas of the country and areas of the world where it's not nearly as prevalent. I still have colleagues on the East Coast who do a lot of pulmonary hypertension who have never seen a case of methamphetamine use pulmonary hypertension.

When I moved to Oklahoma in 2014, I also had never seen a case of methamphetamine-induced pulmonary hypertension, because I trained in Ohio and we had other recreational drug problems, but meth was not the most prevalent and certainly we weren't seeing PH from that. But in my 10 years in being in Oklahoma, I have many colleagues across the country, those who are seeing meth pulmonary hypertension, though they predominantly are now currently in the Western part of the United States, it is something that's infiltrating everywhere.

When I first moved to Oklahoma, I would occasionally see someone that I suspected, and so we would drug screen, and occasionally we would identify a patient that was a meth user, but methamphetamine use has been exponentially on the rise, and our diagnoses of methamphetamine-induced pulmonary hypertension have increased dramatically to the point that now, by far, our most prevalent incident population of PAH diagnoses is methamphetamine-induced. When I say the predominance, that also means, in our case, greater than 50% of all of our new diagnoses of PAH are from methamphetamine use at our center. We are taking care of approximately 500 patients with PAH or chronic thromboembolic pulmonary hypertension, most of them PAH, so we're seeing a lot of people. Probably the biggest reason for the exponential rise in the volume that we're doing is undoubtedly from methamphetamine use.

Like most rare disease, we've long-struggled with the challenge of trying to identify patients sooner. We have a little bit of a different challenge in the meth population. There are some stereotypes out there, some of which are true, and some of which are not true. Typically, when people are thinking about a population that abuses recreational drugs, they think of people typically who are socioeconomically disadvantaged, and that is true. Not everybody, though. You cannot profile these people just by the way they look or walk in the room, because sometimes you'll be surprised.

Because of the socioeconomic disadvantage, larger populations have state-sponsored insurance. We actually have through our claims data, which is a lot of the information that I presented at the Team PHenomenal Hope Conference is based off of that because we don't have randomized control trials in these patients, but what we know is that a larger percentage of meth PAH patients present in hospital settings in the emergency room settings because they have not tended to healthcare. They're not seeing a primary care physician. They're not doctoring frequently, and so when they finally present, it's because they're so sick that someone around them said, "You got to go," or the patients literally can't breathe or can't stand or can't walk.

Many of these patients, and we do have data on this that substantiates that they're sicker when they present, they have bigger right ventricles, they have poorer hemodynamics, and are really hurting in a bad way. It's hard enough when you're trying to treat this disease that's bad and you feel like you're behind the eight-ball, but these people are presenting really, really late, large in part because they're just not seeking healthcare until they're really in dire straits.

I told you that there are some stereotypes that are true and some that aren't true. Many people believe that once you identify these patients, let's say you do meet them in the emergency room or we meet a lot of them in clinic, it's not like they all present in the hospital, that you're going to see them once and you may never see them again, because maybe they finally came to you because they were so sick or someone in their family said, "You've got to go," or whatever. But it's not bearing out the prototypical way that a lot of people think, meaning that these patients do come back and see us.

Now, we have recidivism rates, we have dropouts just like any population of PAH, and is it a little bit higher in this population? Yes, but anybody who's socioeconomically disadvantaged, whether it be from substance abuse or just maybe the fact that they are otherwise disadvantaged, they have similar rates of dropout, no-shows, and those sorts of things, so we do see them. But this is why, okay, and this is the part that's really critical, is that these people are coming to us with two life-threatening problems. The first, which is why they're there, is because they have pulmonary hypertension. The second is their addiction. We have to address both of those things in some way, shape, or form.

Now, most people who are treating pulmonary hypertension are not addiction experts, but you have to be facile enough to understand where these people are coming from and what addiction in general means. What it means for them is that there's very few people in their lives that they can trust because they're used to being abused both emotionally, physically, sometimes sexually. They are patients largely who have very poor self-esteem and very poor confidence. The single most important thing that we have as our responsibility as a physician, particularly to this population, is to let them know that we care and that we love them, that we love them as a patient just like anybody else, that we're going to do whatever we need to do to help them irrespective of what they've done in the past.

Do many of these people get bounced around? Yeah, some of them do because some of them get treated, unfortunately, by the healthcare system as people who aren't worth our time or people who "have done it to themselves>" Somehow that means that they're supposed to pay a different price. In actuality, these are the people who need us the most, more than anybody else. They need us to be friendly to them. They need us to be a friend to them in certain times and to their family, and they have to know that we've got their back.

Most of the patients who present, and I think I'm speaking on behalf of all of my colleagues who deal with a large population of methamphetamine users, is that almost all of them are coming to us actively using. We do have some patients on occasion who are reformed and have gone through rehab and have put it in the rearview mirror. Even though they always have addiction, they're just not acting on it. But those patients who come to us, the majority of them are still using, we absolutely treat them. We treat them just like an idiopathic PAH patient showing up to us who needs treatment.

The reason is because they feel physically terrible. They're short of breath, they're swollen, they're passing out, they're exhausted. They can't do anything because they have really sick right hearts and they're in bad heart failure. If you consider how, any of the people listening to this, how you feel when you have the flu or if you have just undergone a surgery, are you at the top of your game? No, because when you feel physically terrible, it's really hard to feel mentally good, emotionally good, and because it weighs you down. It's why we're irritable when we're sick. That's exactly what these people have, except it never goes away. So, we have to start helping their heart, both their physical heart and their emotional heart, but we have to help them with therapies.

We start them on drug therapies, and are there certain considerations that you have to have with this population, things that you need to be aware of that can sometimes change the treatments that you use? Absolutely, but that's personalized medicine that we would do for anybody. But large in part, just like anybody else, we're going to treat them with a individualized treatment program that we feel is going to help them be most successful.

The fen-phen comparison, there are some nice parallels there, but there are also some differences, but the mechanism of action of how fen-phen led to pulmonary arterial hypertension is very similar to methamphetamines, because it's going down the same pathway, except one was approved by the FDA, the other one obviously isn't. There was a big epidemic after fen-phen came out and quickly was taken off the market, and obviously unfortunately, we can't take methamphetamines off the market.

I wasn't around in the late 1990s when the fen-phen epidemic occurred, but this much I know: they weren't being stereotyped as bad people. They were being stereotyped as people who were looking for a way to lose weight and got victimized, so to speak, by a drug that hit the market that didn't have enough data to know, at the time of its launch, that it was going to lead to both pulmonary hypertension, the mitral valve problems, and the other issues that came about. I can only imagine those people were embraced and treated accordingly, except the big difference there, as well ,is we didn't have any treatment in the late '90s, except for IV epoprostenol, which was the very late '90s. A lot of people didn't have access to that, so many of these fen-phen patients died.
We do have a problem with colleagues within pulmonary hypertension and outside of pulmonary hypertension. One of the things that we have to continue to stress to people is that these people are just as valuable as anybody else, and they deserve our attention just as much as anybody else. I have people, even within my own healthcare institution, and I know that other, my colleagues, have dealt with similar biases, is I've been approached and said, "Well, what do you do with these people? I mean, I know they got a big bad right heart, but you can't treat them. I mean, they're doing meth." It's like, "Well, hold on a second. Why can't we treat them? Because they have an addiction?"

In the pulmonology world, we see people every day who have smoked their whole life and have developed, as a result of that, emphysema, lung cancer, vascular disease, all the things, right? Somehow it's different because cigarettes are legal? I don't know a pulmonologist on earth who says, "I'm not going to give you any inhalers until you stop smoking," because those pulmonologists are out of a job if that's their stance. No, it's just that somehow as a society, we valued being addicted to cigarettes is somehow being on a different level than being addicted to street drugs.

I maintain that there is no difference, especially if we're going to take the stance that people have done this to themselves. Well, oh my goodness, our largest burden of healthcare resources is devoted to people who have done things to themselves. Who hasn't? Whether it's smoking or obesity or having out-of-control cholesterol, because you like to eat hamburgers or whatever it is. We've done these things to ourselves except we have a street drug that is often associated with people who are dirty, who are somehow sub-societal, and then they have to pay the price for being addicted and then for getting a disease from their addiction.

They are a different phenotype in very many ways. We know that they have a different hemodynamic phenotype, they're sicker. They have a different echocardiographic phenotype. Just in that, it still looks like pulmonary arterial hypertension, but often, some of the biggest right ventricles and the worst-functioning ventricles that we see are from patients who are methamphetamine abusers, but there is also a very, very different emotional and societal phenotype that has to be acknowledged with these people, this very different population. Because again, you're dealing with not only their disease, but also an addiction. None of them are proud of it. Just like most people with addiction, they're not proud of it, and they would love to get out of it.

There are many, many places in Oklahoma, certainly many of my colleagues who deal with a high methamphetamine-induced PAH population see that these people come in and are disadvantaged. They want a way out, but the amount of times that I've heard a patient say, "Everywhere I go, meth is there." They live in a world where they can't get out of it. You have to be sympathetic towards that, and you have to recognize that that could have been me, right? It could be you, if you were raised in an environment like they have.

I talk a lot about the glass house in this population. We all live in glass houses, so before you pick up that rock and make a judgment call on somebody, you better look in the mirror, because we all have things that we've done to ourselves. We all have things that we wish that we could erase or that we could have a redo on. I'm pretty confident that a hundred percent of my methamphetamine-induced patients wish that they would've never picked it up.

We are learning a lot from what most would consider the poorest quality of data, which is claims data, but there are still things to be learned from that, because we can get general trends. In the process, we are raising awareness. What we know is that over a five-year period from 2018 to 2022, methamphetamine use is going up across the country. It's not just in the Western United States. Some of the highest percentage increases in the country are east of the Mississippi. So not surprisingly, the number of diagnoses of methamphetamine-induced pulmonary hypertension are also going up to some degree.

Now, it's not completely linear because you have to identify it and you have to attribute it to methamphetamine use. I know that there are probably thousands of patients in this country who are being treated as idiopathic pulmonary hypertension simply because they never had a urine drug screen to tip off their provider. Even though I'm in the thick of it, I get duped sometimes too. As a practice, we send a urine drug screen on everybody at the time of diagnosis, but sometimes it gets missed. Sometimes the patient didn't go and get their labs or whatever, and then you kind of get into the thick of it and you think you have an idiopath or you think you have some other form of pulmonary arterial hypertension, and then at some point, you'll find out, which I think is important to recognize too.

For the most part, patients are very honest. They feel horrible, and they realize that they have nothing to gain. If you set everything aside, look them in the eye, let them know that you care, they're going to tell you the truth almost always because they want to be on the same playing field as you. They want to help.

My general message from the research that we've done, and some of which we presented in the conference in Boston, is that methamphetamine use is a serious problem and it is on the rise. We have well over 5,000 patients that were identified in our claims data work that were diagnosed with methamphetamine-associated pulmonary hypertension. There were more than 2,000 of them who weren't being treated, so why are they not being treated? I think that's an important question that we have to ask. I've got my ideas. I think that some of them can be directly tied to our biases and our judgmental approaches. Other people, it may just be because they presented and then they don't come back, right? There's those reasons too, but we have a lot of work to do.

If you're going to be in the pulmonary hypertension space going forward, you better understand methamphetamines and you better understand drug addiction because it's here and it's not going to go anywhere for a really long time.

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

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