Kristin Highland, MD - phaware® interview 513

Mar 25, 2025

Kristin Highland, MD, from the Cleveland Clinic discusses the importance of shared decision-making and patient preferences when treating patients with pulmonary hypertension. Dr. Highland, emphasizes that the patient is the true expert on their own body and that it is crucial to understand their perspective when making treatment decisions. She highlights the need to consider factors beyond just clinical outcomes, such as side effects, costs, and the patient's ability to manage the therapy. She underscores the evolving role of patient-reported outcomes in medical decision-making and the need for open communication between providers and patients to ensure individualized and preference-aligned care.

This Special Edition episode Sponsored by: Johnson & Johnson

I'm Kristin Highland. I'm a pulmonary critical care physician at the Cleveland Clinic in Cleveland, Ohio. I'm also trained in rheumatology. My focus has been rheumatic lung disease. In that space, I see a lot of scleroderma patients, and pulmonary hypertension is a frequent complication seen in scleroderma. I started at the Medical University of South Carolina and saw that we had a need for helping patients with pulmonary hypertension. At that point, back in the early 2000s, there was really only one therapy and it was a new therapy at that. I feel like I almost got into pulmonary hypertension on the ground floor. I started the pulmonary hypertension program in Charleston and then moved to Cleveland and have just been touched by the pulmonary hypertension community. It's a small community. You get to really know your patients well and their family members and other providers who have similar passion for this disease.

Today, I'd like to talk to you about shared decision-making and patient preferences. When I think about taking care of a patient with pulmonary hypertension, I've come to realize that the patient is the true expert of their body. It is so important to meet the patient where they are, to understand their perspective when making decisions that are going to affect them. It's not uncommon that physicians and providers get caught up in the literature and which drug has the greatest survival benefit or is the gold standard treatment. We forget that there's always a cost to these therapies. There's side effects, expenses, there's pill burden, there may be self-image issues. There may be just the ability to execute the therapy, because you don't have good social support for some of these complicated therapies. I think there's a lot more that goes on in making these decisions. We have to make the right decision for the right patient.

Sometimes, the enemy of good is better and sometimes we shoot for better and we make things worse. Decisions are trade-offs. I find it easier to think about these trade-offs when I think about cancer. Everybody can kind of think about the old-fashioned way of treating cancer where you get this horrible chemotherapy and your hair falls out and you barf your guts out. Would you do that for an extra week of life, an extra month of life, an extra year of life or an extra 20 years of life? Your tolerance of that type of treatment is going to vary based on what you get out of it. Would you do it if you're 99 years old versus if you are 25 and you have a two-year-old kid? Those decisions might influence what you're willing to tolerate and what you're not willing to tolerate.

Patients are making these decisions all the time. They also are in the context of what else is going on. Often, pulmonary hypertension isn't their only problem. The average patient with pulmonary hypertension is on nine other medications. That's a lot to manage, a lot to deal with, a lot of side effects, potentially. I think it's important to really understand their perspectives, because I can prescribe medications all day long and if the patient doesn't want to take them for whatever reason, I'm not helping my patient. A medication cannot make you better if you don't take it. You have to find out what's going to work for that individual patient.

I've also learned the hard way that patients don't read the textbook. They don't fit into these neat perfect diagnosis with zero other comorbidities. What works for one patient might not work for another patient, so treatment needs to be very individualized. I also think when we talk about severe diseases like pulmonary hypertension, there's no cure. People will eventually die from pulmonary hypertension and we have great therapies that are extending life, but we don't have a cure. I think as providers, we get so caught up in offering hope and offering our best therapies that we forget to address symptom management, exercise capacity and overall goals of care.

Addressing the elephant in the room. Addressing that you're going to die from this disease, which is a really scary statement and is still an important one to have. We can have that conversation and say, I want you to have as many days on this earth as absolutely possible, and I want you to have the best quality of life with all of those days. But when you don't have a quality of life, what is your preference? And to meet the patient where they are, because that's a pretty individual decision, what the patient wants and what the patient doesn't want. It should not be my decision. It should not be my personal decision.

I can say as I have gone through different stages of my life from being a college kid to a mother of a teenager, my decisions about my life have changed. Some patients want to do everything no matter what forever, and they are true fighters. I support those patients 100%. That's not the right answer for everybody. I think it's so important to meet the patient where they are and to remember that it's not just treating this disease with medications, but the goal is treating the disease with these medications, but with an emphasis on the outcomes that are important to the patient, which generally means giving them the quality of life that they want. I try to think about decisions that way.

I think it's important to educate patients on what's out there. One of the things is that there has been a lot of great developments in pulmonary hypertension over the years. When I started, we had one therapy and now we're up to like 16 plus available therapies. Patients aren't dumb. They are on the internet. They are on this blog. They're members of PHA and other patient advocacy groups. They're wanting information. It is our job as providers to provide them that information and to provide it in a balanced way.

When a drug is studied, generally, it's a very carefully curated population of study patients that are put in a clinical trial, which doesn't necessarily translate to all patients. We need to say this drug was studied in this way, in this particular population of pulmonary hypertension patients, and this is what we found. These are also the side effects that patients who were in the population experienced. Based on what I know about you, I think this might be a good drug to try, but maybe patient X has a certain comorbidity where I can say, because you have this other thing going on, it's possible that you don't tolerate this medication as well as we would like. So, we need to keep in contact. I need to know what you're going through when you take this medication, so I can either help you with side effects or we can decide that this medication is not a perfect fit for you.

When we think about data that is on average the study of 1000 patients or the study of 200 patients or however many -- this drug improved hemodynamics or improved walk or improved time to clinical worsening. But that is a group of patients that this drug affected on average. There are patients in that one study that do great on that medication, and there's patients that do less great. There is a spectrum. I think it's important to say you're just one patient. You are an individual. These are things about you that I think might make this medication work or this recommendation of therapy, but here are things that we have to look out for.

When I approach a patient, I always say, let's do what I call an N-of-1 trial, your own unique trial of this therapy. It sounds like a good idea. We're all on board. We're going to try this therapy and we're going to follow the results together to see if it's worth it.

I learned this the hard way. My daughter has ADHD and I kept thinking her ADHD wasn't controlled, so I had just talked the pediatrician into increasing her dose and increasing it and increasing it. Well, she was hiding her ADHD medicine, because she didn't like the way they made her feel, which is why her ADHD wasn't controlled, which is why she needed escalating doses, because she never took the medication. I made that mistake with my own daughter. I could tell she was focused when she took her ADHD medicine, but she hated taking it, because it made her feel bad.

Now, in pulmonary hypertension, we have 16 plus available therapies, clinical trials, all sorts of different things that we can try. It's really important to find the right combination of things that makes the patient feel better as opposed to what I'm reading about in a journal article. I try to think about that.

In regards to clinical trials, it is so important for us to have clinical trials so that we can move the needle and the disease. This is how we're going to come up with a cure and we can't have clinical trials without patients. We do a lot of clinical trials at the Cleveland Clinic. I offer these trials as an option for patients. I make sure that they understand if you're participating in a clinical trial that is always voluntary. You can decide you want to do the trial today and tomorrow you can say, I don't want to do it anymore, and you can quit. Clinical trials are voluntary. Whether you participate or not should not affect your care with your healthcare provider. It certainly does not affect the care they get at the Cleveland Clinic, whether they participate or not. So that needs to be very clear.

I also say, the reason it's a clinical trial is it’s an experiment. We do not know if it works. It might not work. It might make you feel worse. We have no idea. That's why it's a clinical trial. It needs to be studied. But then I say, I am so grateful for the patients who do participate in clinical research so that we now have more and more options for our patients with whatever disease, but participating in a clinical trial, that's an individual decision. Sometimes, it's just a pain in the neck to come in as often as you have to for clinical assessments in a trial. Some patients work and they just can't take that amount of time off work or they live too far and they don't feel well enough to travel that far, that frequently. Some people don't want to feel like they're being experimented on. That is 100% understandable.

Other patients say, I don't know if this'll benefit me, but if I can help contribute to the knowledge about pulmonary hypertension, that's my way of giving back. A lot of patients want to participate in clinical trials for altruistic purposes. They just hope that someone else can benefit from the science. I'm certainly grateful and amazed by the number of patients that want to do that. Whether a patient participates in a clinical trial or not, again, I think is 100% their decision. I just put it out there as this is also an option. Just the same way I say, and so is participating in pulmonary rehabilitation.

Shared decision making, I think has evolved over the last 20 years. The FDA, for instance, seems to have put more emphasis on what we call patient reported outcomes, how a patient functions and feels. Not just do they live longer, do they walk farther? Does their PVR drop? The FDA recognizes the importance of therapies. Not only do they want to have a biological improvement, but they need to have an improvement that the patient cares about. We're getting more and more data. Although there's some alignment between the providers and the patients, it's not complete.

For instance, I've recently been on a project where we've looked at the provider recorded functional class of a patient. So WHO functional class is something that is recorded in pulmonary hypertension. Functional Class I patients don't have symptoms. Functional Class IV, they're more or less symptomatic at rest. Function Class II is they have some symptoms. Function Class III, they have more. Function Class IV, they're at rest. I was involved in a project where providers, which is the usual way of assessing functional class. But then, we had patients report their own functional class, assess it themselves, say, “Well, I think I'm a Functional Class II or I'm a Functional Class I.

What we found was about 50% of the time the provider and the patient matched, but 50% of the time they didn't. The providers both over and underestimate functional class. The cool thing was that the patient reported functional class had better alignment with quality of life measures like emPHasis10, which is a pulmonary hypertension quality of life tool, and the SF-36, which is like a general health quality of life tool. What the patient says about their functional class aligns more strongly with these other measures than what the provider thinks about the patient's functional class. Although there is some alignment, it's not perfect. I think we're getting more and more data that patient perspectives matter.

I also think patients have more ready access to information at their fingertips, because of the internet and blogs and websites. They are coming in educated, asking questions about various therapies. They want to know about them. They want to know if they're a candidate. They're hearing about other patients' experiences, because patients are blogging and saying, “Well, I tried this and this happened and it was great,” or “I tried this and this happened and it was horrible.” They're hearing all these stories. Patients certainly come in with a lot of questions. I think that's the challenge to make sure that we are spending enough time with our patients to answers those questions, to discuss the options, to provide them fair, balanced information. This is what the drug can do, but here's also the side effect profile of the drug. And to think about, you have to take this drug three times a day or four times a day or once a day, or it's a continuous infusion, what does that look like? Or you can take this drug and oh, by the way, your copay is humongous, because these drugs are expensive. It's going to be challenging to get prior authorization. There is a very, very unfortunate financial piece to these decisions.

Also, I find as a provider, sometimes it's really hard to discuss. You're talking about therapies. You want to help your patient. You want them to live as long as possible and have the best quality of life as possible. It's hard to have a conversation around hope and then also have a conversation around goals of care and what are your thoughts about death and dying, or do you have a living will or palliative care, which I think are really important topics.

Sometimes, these are topics that scare patients that they don't want to talk about, they're not ready to talk about, but sometimes they're topics that patients do want to talk about, but they don't know how to bring the question up. There's this elephant in the room that nobody's talking about. We're doing a disservice to our patients if we don't bring that part up of care. Often, patients hear the word palliative care, and it's scary. Our palliative care group prefers to be called quality of life doctors. Just because you have this disease doesn't mean we can't really work on addressing your symptoms the best way possible.

Treating someone with pulmonary hypertension medications and also better managing their symptoms are not mutually exclusive. You can do both at the same time. I think providers have a hard time balancing those conversations, because you certainly want to leave the patient with hope. Always want to leave the patient with hope. But honesty and addressing the scary topics is super important too. I try to figure out where the patient is and how receptive they might be. Sometimes, these harder conversations have to build with time and patients have to be ready to have them, but they have to be given permission that the conversation can be had when they are ready. It's hard. It's hard to get through all of this in a short follow-up clinic visit. That's the challenge of taking care of the patient, but probably the most important part of taking care of the patient, in my opinion.

I'm Kristin Highland, and I am aware that my patients are rare.

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