Preview Mode Links will not work in preview mode

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

A new podcast series devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. New Episodes every Monday & Thursday!

Use the search bar above to search episodes by topic. Search "phawareMD" to discover podcasts with medical professionals.

Get our custom smartphone app (for iPhone™, Android™, and Amazon™ devices). It's the most convenient way to access the phaware™ podcast.

The views and opinions expressed in the phaware® podcast do not necessarily reflect the official policy or position of phaware global association. Information on phaware.global and phaware social media sites is provided for general information only. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs.

To learn more about PH visit www.phaware.global. phaware® is a 501(c)3 organization. © Copyright 2015. All Rights Reserved.

Generously Supported in part by Actelion Pharmaceuticals US, Inc., Bayer, United Therapeutics Corporation, Acceleron, CVS Health & PhaseBio Pharmaceuticals.

Ryan D. Coleman, MD - phaware® interview 340

Sep 1, 2020

 

Ryan D. Coleman, MD specializes in pediatric critical care and pulmonary hypertension at Texas Children's Hospital. Dr. Coleman is also the medical director of the Right Ventricular Failure Program. In this episode, Dr. Coleman discusses how  #pulmonaryhypertension impacts both the parents and the patient, how hope drives science and why every child deserves to leave behind a legacy.

My name is Ryan Coleman, and I'm a pediatric intensivist at Texas Children's Hospital. I spend 50% of my time taking care of kids in the intensive care unit. Then the other part of my time, I spend as a member of the pulmonary hypertension service. I am one of the attending physicians and the medical director of the Right Ventricular Failure Program that we just started down here at Texas Children's Hospital.

We started the Right Ventricular Failure Program here, to really focus on those kids who have progressive pulmonary hypertension, despite being on maximum medical therapy, who are approaching the need for potential lung transplant, looking at ways to better optimize their management. Whether it's looking at changing their medical therapies to a different regimen, or considering some sort of intervention. If that intervention is a surgical one, such as placement of a Potts shunt or a unidirectional valve shunt. Or is it going to the cardiac catheterization lab for creation of a atrial septal defect, a ventricular septal defect, or trying to put a stent where their ductus arteriosus once was, to offload the stress that the right ventricle is under. [We] try to buy them some more time before they potentially, need to go to lung transplant.

One of the things that we've realized over time is that, while lung transplant is a great therapy, and sort of the only therapy available for some kids, there are other options. Sometimes, if we can delay transplant for whatever period of time, overall, it just adds to the total length of time the family has with their child. We know that the 50% survival mark for a pediatric lung transplant right now is around six years. So, anything we can do to delay the start of that clock, only goes to benefit patients and their families. One of the challenges that we realized a lot of these families face is that, when you are considering something as consequential as lung transplant, you really need to have a comprehensive multidisciplinary team that's there to help you, not only with transplant, but also to talk about any other options there may be. Transplant is a very intense, both medical procedure and then it involves a lot of lifestyle changes afterwards, including potentially where you even live.

So, what we aim to do with the Right Ventricular Failure Program is to work not only with our transplant colleagues, but we've got members from interventional cardiology, advanced cardiac imaging, palliative care, congenital heart surgery, representatives from pulmonary hypertension, from the intensive care units. This entire group of folks get together to talk about these patients, so that we can come up with a real comprehensive plan, including lung transplant, who's also a member of this team, so that we can try to create a roadmap for these patients to follow that may eventually get to lung transplant. But if we can do anything to optimize their transplant status before they get there, or even delay transplant, we try to do that. They may ultimately need transplant, but we're going to work with them to try to find the best solution for that family.

There are only a few centers in the US that really do the bulk of pediatric lung transplant. Texas Children's happens to be one of them. St. Louis is another big one. Same thing with Potts shunt, there's really only a few centers that do them. We are sort of new to the arena in terms of doing Potts shunts. St. Louis has the biggest experience in the United States. I work very closely with Mark Grady, who's the director of their PH program there. And then, Morgan Stanley up in New York, affiliated with Columbia, also has a big experience. Erika Rosenzweig is there, and she and I also worked together.

Undergoing these procedures is a very big deal, obviously. Performing a lung transplant following a Potts shunt is not a straightforward surgery, by any means. So, it can be a little bit more complicated. You have to go in eyes wide open, knowing that if you elect for a Potts shunt, it may make your transplant tougher in the future. Or depending on how your Potts shunt is performed and the surgeon's preference it, you may not be eligible for a lung transplant. So there needs to be a lot of planning that goes into considering what type of intervention you're going to look out for these patients when their right ventricle begins to fail.

Whenever we meet with families, first off, you know it's going to be a long day. So we try to make sure that we've got plenty of time allotted for these appointments. The last thing you want to do when you're talking about something this serious, is for anyone to feel rushed. Usually, I donate my entire afternoon to this conversation, whenever we have it. The goal is to figure out, number one, what are the parents and the patient's wishes? What matters most to them? What are their hopes? What are their fears? Where are they at in terms of their disease? I think one of the things, with something like pulmonary hypertension, that you need to be very cognizant of, is that there is no cure for this disease, and there's no right way to treat it.

There are certainly things you could do that may negatively impact a child's life or their quality of life. The patients that I meet with in particular, are patients whose disease is pretty severe. The last thing I want to do is present them with information that, ultimately, they're not going to be using or things that may sound scary. What we instead want to do is figure out exactly where the family and the patient is, and then take that knowledge and use it to help craft a care plan for them. We'll talk about all the different options, but when I start with tell me where you're at and tell me what your big goals are for the next stage of your life, I'll use that as the guide to then figure out, how much to get into with each of the different therapies and what are the options.

Some families are very upfront and they say, "I'm really nervous about transplant. What else is there?" Then we'll spend time talking about shuts. Other families may say, we've read a lot about this, and we're really interested in going forward with lung transplant. Then, I shift my role to, how can I help make sure you get to transplant as healthy and as great of shape as you can be in before you get there.

It can be a very challenging situation when parents and the child are on a different page. One of the things that's most important, especially taking care of the teenage patient population, is that you give them a chance to have a voice in their care. Because even though they may be 12 or 13, 14 years old, they're often wise well beyond their numerical age. They've been living with this disease for a while, by the time I meet them. They know, kind of inherently, what they are comfortable with and what they aren't. We'll give them time alone with myself and with the other members of the team, so that they have the opportunity to express exactly what they're feeling without worrying about upsetting their parents. It's amazing to me and I think one of the great privileges we have as pediatricians as being able to interact with kids. These kids who have these amazing hearts and are so concerned about their parents. We always think of the parents as being the one to protect and care for the child. But it's amazing how many times it's actually the child, who's also trying to get care for and protect their parents from the bad things.

We try to give them first and foremost a forum to say what they really want to say. They get some time alone with us, and then, we do the same with the parents. Are there things the parents want to tell us, that they don't want their child to hear? Are there questions that the parents want to ask us that they don't want their child to hear? We give each member of the family their own space. Usually, when you think that they're on different pages, if you give them some space and talk to them separately, you can figure out where the disconnect is and get them realigned together. Thankfully, knock on wood, I haven't had a situation where both sides are just dug in on opposite sides of what they want. My approach has always the same, give each person a chance to say what they really want and what they're honestly really afraid of. You can usually use that information to craft a plan that everybody in the end is most comfortable with.

Sometimes, we meet these families at birth, and what goes from being one of the happiest moments of their lives, welcoming this new child to the world, suddenly becomes a very scary moment when something goes very wrong. Suddenly, the dream of that perfect child gets shattered. I think one of the great privileges of being a pediatrician is the kids are resilient. We may think we know what's going to happen, but it's not uncommon for the kid to prove us wrong. One of the things I try to do with all families is give them as much information as I can, and be as realistic as I can about what I've seen and not seen. But never to the point where you take away hope. Because the last thing on earth you ever want to do is take away the hope of a family.

Sometimes, we have to shift that hope. You go from the hope of having a perfectly healthy child to maybe the hope of having a child who stays very comfortable despite having to be in the hospital or a child who your hope shifts to, let's just try to get home. If we can get home, that's going to be a big win. So I never, ever wanted to take away anybody's hope. I just want to make sure that we keep their hope realistic, and that we set kind of reasonable expectations without ever making what's already a challenging time for a family, even more difficult.

I think the dream right is that in some ways the hope will drive the science. Anything and everything we do for any kid, whether it's treating pulmonary hypertension or a kid with a bad infection in ICU... everything we do is to stop the disease, stop the bad thing that's happening to this child and their family. As we learn more and more about pulmonary hypertension, for example, the genetics of pulmonary hypertension are just mind blowing. Even the genes that we test for when I first started learning about pulmonary hypertension as a fellow, the numbers are rapidly expanding in terms of the panel that we send today versus what we sent six, seven years ago. I think as we learn more about the causes, we learn more about kind of how this disease behaves. Because it's still a relatively young one in the grand scheme of things. We'll find new targets and new things that we can try to change. So, our hopes initially were to just get these kids to live.

When people first started treating pulmonary hypertension, years and years ago... It's a disease that has 100% fatality. Back then the hope was just buying a little bit more time. Then the first line of drugs came around, and then suddenly, we had a second line of drugs, and now a third line and a fourth line. So, our hopes are evolving and changing. So now, it's not just hoping for a year or two, now it's hoping to get to college, beyond college, or hopefully one day eradicating these diseases. So it would be fantastic if hope drives the science.

At the same time, the science can drive the hope a little bit too. As we get a better understanding of the right ventricle and how it responds to stress, as we get a better handle on how pulmonary hypertension develops, we can change what we hope for. We can have bigger hopes and bigger dreams for these patients. I think the two work hand in hand. As long as we as the physicians, who get the privilege of taking care of these kids are honest with families about what we can realistically do or not do, I think that's the best we can do in any of these situations.

Every kid deserves a chance to leave behind a legacy. One of the things that I make it a point to tell all of the fellows that I work with, whether it's on the intensive care side or the pulmonary /pulmonary hypertension side, is that you can't forget about the importance of a kiddo having a chance to say goodbye in the way they want to say goodbye. No matter how young they are, no matter what circumstances they come to you in, every kid deserves a chance to leave behind a legacy that's most important to them.

At the end of the day, we have to make sure that no matter how aggressive we're being with medical therapy, that that child doesn't lose a chance to impact this world in whatever way is most important to them. I've had a child recently, who decided to write a play, that he had different members of the medical staff act out. Because of COVID restrictions, he couldn't have just a ton of family and friends at the bedside. So he brought the medical staff and child life and the physical therapists into his play.

It's just remembering that behind every diagnosis is a human and behind that human is a family. If you do that, you do it right. That's what I'm really most proud of with the Right Ventricular Failure Program, is we're taking all the best advances in medical care and science, but we're coupling it with trying to really just provide 360 perspective on care for that child and their family.

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

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: www.phaware.global/donate #phaware #ClinicalTrials @TexasChildrens @TCHPulmonary