I'm Aware That I'm Rare: Steven Abman, MD
Dr. Steven Abman is Professor of Pediatrics and Director of the Pediatric Heart Lung Center (PHLC) at the University of Colorado Denver Anschutz School of Medicine and Children’s Hospital Colorado. Dr. Abman founded and continues to serve as Director of the Pediatric Pulmonary Hypertension Network (PPHNet), a multicenter clinical research and care group consisting of 10 leading PH centers from throughout North America, and initiated and led a joint American Heart Association/American Thoracic Society working group to establish the first joint guidelines for the care of children with pediatric pulmonary hypertension. Dr. Abman also serves on phaware’s Medical Advisory Board.
I’m Steve Abman. I’m a professor of pediatrics at University of Colorado, at Children’s Hospital Colorado.
My background is in pulmonary and critical care medicine but I’ve had a career-long interest in pulmonary hypertension both in terms of laboratory research, clinical research, clinical care and program development. It’s been my pleasure to work with Dunbar Ivy in Colorado as well. It’s been our passion is to try to improve the care and quality of outcomes of kids with PH. Years ago, we recognized that pulmonary hypertension is poorly understood in general, but especially in kids. The way we were approaching it was really by discipline.
Cardiologists may try one thing, neonatologists another, pulmonologists often not get involved. Really the sub-specialties weren’t communicating well together. There were huge gaps in our understanding of disease. What we thought we needed to do was to get multidisciplinary teams together so we could either talk to each other, give advice to each other, discover things together, really by taking care of kids but also doing intensive research. One thing we realized is that being a relatively rare disorder, that each site really didn’t have sufficient numbers of patients to get smart enough about the disease and how to manage it.
We developed what we call a network. Pediatric Pulmonary Hypertension Network, or PPHNet (www.PPHNet.org). We really just picked some of the sites that had the biggest programs and folks who were already invested deeply in terms of clinical care and clinical research. We got together. No money, no funding, but these are all leaders of programs that are really, again, passionate about trying to do better for families and kids with PH. As we started meetings one of the early ideas that came up was we really, really needed to have some sort of a starting point of how to think about taking care of these kids that we could agree on.
Where is the consensus? Where are the questions? In fact, the questions themselves are probably even more important than the consensus because it unmasked where the uncertainty was. Where do we need to go further with the research and things like that. We began this project. We first went to the American Thoracic Society. They were excited, gave us a little bit of help with organizing this. Then the American Heart Association, large part thanks to Stephen Archer in one of the committees of American Heart. They got really involved in this as well. Then over time it became a joint mission.
American Heart, American Thoracic societies getting together. We did a very extensive project that took actually a few years to complete. Peer reviewed, revised, peer reviewed again. Having to get both societies to sign off on it, and I came up with a document of what we call the guidelines for pediatric pulmonary hypertension. There are a number of drugs that are approved for adults, but when it comes to kids it’s really a different story. That’s where we still struggle. We really have relied on adult data, adult approvals and that sort of thing for the majority of what we do.
Just for ourselves, are we thinking the same way about this disease? What are you doing in Philadelphia or Boston that maybe I’m not thinking about in Denver, or vice versa? Where do we agree? Where don’t we understand it? We did actually try to present it so it would be useful for a wide variety of disciplines. Some of the elements we have in it are things like kids should be evaluated at experienced centers that have established clinics and programs in pulmonary hypertension, because it does take experience and different disciplines coming together, organized care that we think is important.
That’s a message for families, for insurance companies, as well as sub-specialist and finally the PH community, pulmonary hypertension community more broadly. There are elements of guidelines that are directed for everybody but there are some points that are more specific perhaps for the PH specialists, and some are more generalized. There are other examples too. If your child has pulmonary hypertension, even going under routine anesthesia we want to make sure we do it as safely as possible. Going to a center, even for having PE tubes or a tonsillectomy, if you’re going to have general anesthesia, let’s make it as safe as possible by having it somewhere where the anesthesiologist and the PH team can work together and anticipate the potential for adverse effects of the anesthesia and other kinds of things.
Those are things that families need to know about and hear about. General pediatricians need to know about and then finally within the realm of each sub-specialty we have things that are more neonatal, things that perhaps are more cardiac or surgical, but I think globally we try to serve a number of different missions. We began a registry with the hope that if we started with just the sites currently involved, establish the registry, we could then broaden it more nationally, include more centers and sites. There is a couple of things. A registry means we are collecting patient information, and we do it with consent of the families. We developed the questionnaires or we’ll call clinical research forms that could specify what kind of information we want to get.
We could then specifically do research projects and get smarter about the disease from that. The other side of it though is, these very questions we’re asking by implication tell doctors what they should test for. There is a positive impact on the clinical care simply as they fill out these forms. They go, “Well gosh, we didn’t do a brain natriuretic peptide level.” “We forgot to do a V/Q scan and yet the questionnaire’s asking for it.” In a way it’s also influencing care as we’re also concurrently collecting data for research and other kinds of things. Registry can be a very important tool. Then finally, we think down the road as we get more and more sites involved.
We could start looking at practices that differ from site to site. Are there more successes at one site than another? Why is that? If we identified what those care patterns are, can we improve outcomes that way? It sounds perhaps not that important but in cystic fibrosis for example, years ago a simple article brought out the fact that some centers had much lower mortality than other centers. They tried to track down why. One of the features was, centers with lower mortality were seeing their patients more frequently, making more active, proactive changes in therapy. Some were using lung function tests more actively in changing which kind of medications they’re on.
Certainly with pulmonary hypertension there are some things, some patients are maybe not followed as closely or as rigorously or as regularly as we could be or should be. I think those are the kind of things we could compare and contrast outcomes, and I think the whole community benefits from that. Sometimes it’s those little things between the gaps that we don’t think about. It could be that maybe some centers do a better job of transitioning patients into either adult care or into other kinds of clinics or getting more folks mobilized when it comes to routine surgeries, for example. All those little things that technically we may not think of… We may think, “Well, I’m going to use this drug first, second and third,” we may all agree on that but it’s all those little things may add up in terms of fine tuning care to improve outcomes.
Listen to “I’m Aware That I’m Rare: the phaware™ podcast” at www.phaware.global/podcast. Learn more about pulmonary hypertension at www.phaware.global. #phaware