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I'm Aware That I'm Rare: the phaware® podcast

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Angela Bates, MD - phaware® interview 445

Dec 5, 2023

In this episode, Angela Bates, MD from Stollery Children’s Hospital, discusses the importance of incorporating quality of life into the management strategies for pediatric pulmonary hypertension patients. 

Dr. Bates also talks about the importance of allowing patients to engage in activities they enjoy, even with limitations imposed by their condition, and the need for a multidisciplinary team to provide comprehensive care. 

My name is Dr. Angela Bates. I am a pediatric cardiac intensivist who also takes care of pediatric pulmonary hypertension patients up in Edmonton, Alberta, Canada. I just wanted to talk a little bit about what I've learned over the past several years as I've embarked on getting to know these patients and their families. I think one of the things that patients have taught me the most about is how important quality of life is in incorporating that in terms of our management strategies. It seems a bit counterintuitive for the physician to be talking about quality of life because, really, it's around what the patients feel is in the best interest of what they see as a management strategy that fits into their lives as well as the families. But I think they've taught me a lot about how we strategize around implementing different medication therapies, even in terms of constraints of different activities.

What I've learned, especially with some of my sickest patients, is how you really need to listen about what's important to them in terms of what fits into their family. And how do you manage different therapies, even different investigations, timing of those things? How does that fit into the strategy to take care of these patients to still optimize them to have the best outcome? I think those of us that take care of pulmonary hypertension patients, those of our patients that live with it, those of our families, their family members that have pulmonary hypertension understand that this is a really severe disease. It's really rare. There's a lot of people that don't understand, what pulmonary hypertension means. Every patient is so different and quality of life becomes the most important in the sickest of the sick. We know that the only cure for a true pulmonary arterial hypertension patient is lung transplant, which is its own disease ultimately. So you're sort of trading one disease for another disease.

So I think it's really important, especially in pediatrics, whether they're five-years-old, 10-years-old, 15-years-old, is to really listen to the patient and understand what's important to them. Because ultimately, if these patients go on to survive to transplant or don't, what’s going to be most important is how was their day? Did they get to do things that made them happy? Did they get to spend time with family? So we talk a lot about therapies. We're very fortunate, I'm very fortunate to get to practice in the era where there's a lot of oral therapies. So we really strive to push the envelope with oral therapies. We push our pharmacy counterparts to really try to advocate for getting drug studies in pediatric patients, but ultimately having access to oral medications. Then we really push our friends with different medications, such as United Therapeutics with Remodulin to how can we best support these families at home?

I work in an environment where we see patients from all across Western Canada. So some of these families live really remote. It's not, again, quality of life-wise, it's not always the best interest for these families to relocate to a big center. So we try to work with families to offer medications that seem a bit extreme to be in a remote area, but can we set them up with a really good family doctor, a really good pediatrician? How do we incorporate how often they have to come down for visits? That sort of thing. So all of these things become, I think, really important in terms of what is quality of life for these families and for these patients. Then the other thing I've learned about, especially when we talk about treating with prostacyclins and with things like Remodulin, is how do we allow our patients to still get to do all the fun things that they want to do?

So we all know that with Remodulin that there's limitations, even with subcutaneous sites or with central lines on things like water activities, for example. So a lot of our families, they really want to try to incorporate, how do we still incorporate swimming? Some of them are brave enough to try things like being on a boat, water skis. People think that's crazy that your patients are doing this. But us in the PH world know that, no, that's not crazy because these patients, if they're feeling well enough to do all these things, how can we safely support them to do these things? So sometimes it's as simple as hooking them up with other families that have been down this road and they have little tricks and tidbits and salient points of how can you still get your kids to enjoy these activities safely? Also, it's about listening and saying, "I'm not just going to plug my ears and say, 'No, you can't do that.'"

It's like, okay, well, I know you want to do these things. These are what make you happy in your life. How can we work with you to make those activities accessible and safe? Sometimes it's as simple as, okay, you know what? Let's time your new site start with a pool visit, for example, that sort of thing. So I think those are really important things. Then ultimately, we have a lot of patients that are quite sick, but they're at home. I think one of the other things that I've learned is, no matter what, if you can facilitate that patient to be at home, I think that is huge. It's in the best interest of the patient, of the family. I think that they eat better, they sleep better, they're happier. Then, just having an honest conversation with families about, okay, what happens if? What do we do if?

Sometimes it's being really honest and saying, "Look, the safest place would be closer to the hospital. But you know what? You guys are not naive to this disease. You're not naive to the things to watch for." Maybe we do weekly check-ins with the families, for example. So I think there's a lot of meet in the middle things that you can do to enhance quality of life. Then, sometimes what I've been learning a lot is we have some oral medications, at least in Canada, where, through different funding avenues, we can get access to oral selexipag. So for those of you that are familiar with it, it's a non-prostanoid analog of the prostacyclin group. We truthfully don't know how does that compare and stand up to things like epoprostenol or treprostinil, Remodulin. But can we advocate for certain patients that, for example, if they don't tolerate sites for Remodulin or they need a break from their pumps for a while, can we safely trial something like a selexipag agent?

So just having that conversation with families and just being honest that we're still learning about these drugs, I think, is also, again, a huge advocate for quality of life for these patients. There's a lot of times where we have patients that, for example, we know that it's safest to do things in hospital, but they're like, "Listen, I think we can manage this at home. This is not our first rodeo." So finding some sort of a middle road on what's safest for the patient, but also what works best for the patient and their family. So sometimes that meaning that's drug conversions at home, or maybe it means that you're doing some more, now that we've got lots of different venues to do telehealth, maybe we do a few telehealth visits.

Again, that always makes us feel a bit more uncomfortable because we're used to seeing our patients in person. But I think as you get to know patients and families and you know how honest families can be with you and you've built that relationship, I think then building a telehealth into the practice of seeing your patients can be very useful. Then, I think the other quality of life piece that I've learned about is what is the best in terms of investigating our patients? So I think most of our patients will say, "ECGs, six-minute walk tests, echoes. Those are bread and butter. We can do all of that." You start to talk about things that might need sedation like cardiac MRIs or the big, scary cardiac catheterization. Now you're talking, well, higher risk if you're needing sedation. Of course, our cardiac caths, I mean, at least at our center, we do general anesthetics for all our pediatric patients. That comes with a bit of more anxiety for our families.

So I think pushing the envelope as to how frequently do we need to do a cardiac cath? Can we get away with the least invasive test for a while if we've got enough data based on the other imaging and what we see with our patient clinically? Then, with cardiac MRIs, can you get away with using child life to help you instead of using sedation? I think those are huge benefits. So I think that also speaks to the need for a multidisciplinary team that really knows this patient. So it's not just the physician that's so important in treating these patients, it's the whole team. So that includes your nurse practitioner, your clinic nurses. A really important part of our team is the social worker, the child life specialist, right down to the person doing the six-minute walk test, whether it's a physiotherapist or an exercise therapist.

We even have really invested members of the team on our research side of things that get to know the families. I think all of those people are so important in listening to the families, picking up different components that help you to, again, mold that treatment plan around what focuses on quality of life for that family. So I've really learned a lot from just taking in what my team also learns from meeting the families and the patients. It's really interesting in our center, even our echo sonographers get to know these patients really well and are invested. So I think that can be really important feedback. And how are our patients doing with this strategy plan? How are they doing in general? Does this fit with what their quality of life goal and vision is?

Alongside that is when we have a really difficult decision with a family in terms of an intervention or a treatment strategy. So for example, we've had some of our patients that were listing for transplant, they're really sick, idiopathic pulmonary arterial hypertension patients, they're on triple therapy and oxygen. Some of them have had different interventions like Potts' shunts and we're waiting for lung transplant and they've got really bad scoliosis and they've got back pain, but also in terms of making them a better candidate for a lung transplant. So we have a really difficult decision about do we go for scoliosis surgery? So including in that discussion, members of your team that know the patient very well and your physician and your nurse practitioner, the parents, the child, but also your surgeons, your anesthetists. Those are also really important things, I think, in terms of, again, addressing what makes this a best quality of life for the patient and the family, but talking really honestly and openly about what are the risks to this patient?

I think the one thing that I try to always impress upon with patients and families is that I'll always be honest with them. I think that's a really important strategy moving forward, even though some discussions are really difficult. But I think honesty is always the best policy. But that's saying, "I can't promise you what's going to happen in the future, but I can promise you I'm going to walk this road with you." I think that's the best we can do. We're human as practitioners, but our patients and our families are human. So I think, to me, that's provided the best way to implement some of these treatment strategies. Then, I've got a couple patient examples where I've dealt with where there's been scenarios where we've had to really work together to focus on quality of life. I think the common one, and I won't just say a specific patient, but just going back to activity.

So I have patients that want to water ski, they want to play baseball, they want to play badminton, they want to play volleyball, they want to go on hikes, they want to go camping. Sometimes that's one patient wanting to do those all. I say, "Great. Okay. I think that's excellent that you want to do that. How do we do that safely?" So you're on a Remodulin pump, you're on Remodulin through a central line. Okay, so how can we do that safely? Even though, yes, ideally you don't ever want to think about putting a PICC line or anything close to water in terms of infection risk, that sort of thing. However, this is really important to this family. This is really important to this patient. How can we facilitate that? So the biggest thing I've learned is hooking them up with families that have been through this. They can give them different examples on how do you access different tools that can make the line safe or the site safe, for example?

Then, just working with the patients to say, "Okay, look, you just need to know your body and listen to your body. So you need to promise me that when your body says, 'This is too much,' you take a break." Even some things as simple as oxygen. We all know that oxygen can benefit our patients, but sometimes it's as simple as, well, okay, I understand that you're a teenager and you don't want to wear your oxygen the whole time. But can you promise me that when you're walking up the stairs or you're walking between class, that you try to put it on as much as possible? Or if you feel like you need oxygen, you take a bit of a time-out to put the oxygen on. Those are simple things that, even though it's sort of meeting your patients in the middle, which I think is important, especially in your teenagers where they need to have some autonomy over their care.

Then, even trips. So camping is one of them. So how do we facilitate making sure they have enough oxygen on their trips going to somewhere like Jamaica? I've had family members that want to go to Jamaica. Okay, well, what's a safety plan? Where do they go if they get in trouble? Do you have enough supplies? Providing them with a medical letter. I think those are all important things where instead of saying, "No, you cannot go to Jamaica." 

To finalize things, I think what I've learned most from my patients is quality of life has to be at the forefront of all of our decisions that we make with families. Again, I stress making decisions with families. Our job is to provide the best care, but that also includes keeping in mind, what is the best strategy that works within the life of that child and that family. I hope that we continue to get better management strategies to make this less onerous on families. But until then, we'll work with what we have. And again, focusing on quality of life to really guide those therapeutic strategies.

My name is Dr. Angela Bates and I'm aware that my patients are rare.

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