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

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Steven Abman, MD - phaware® interview 411

Apr 18, 2023

Dr. Steven Abman is Professor of Pediatrics-Pulmonary Medicine and Director of the Pediatric Heart Lung Center (PHLC) at the University of Colorado Denver Anschutz School of Medicine and Children’s Hospital Colorado. 

In this episode, Dr. Abman gives an overview of The Pediatric Pulmonary Hypertension Network (PPHNet), a network of clinical specialists, researchers, and centers bringing a collaborative and multidisciplinary approach to improving care for children with pulmonary vascular disease. He also discusses takeaways from the 15th International Neonatal & Childhood Pulmonary Vascular Disease Conference.

My name is Steven Abman and I'm a pediatrician trained in Pulmonary and Critical Care Medicine from the University of Colorado. I've been working locally with our team here in Colorado at the Children's Hospital to really develop our own pulmonary hypertension program.

What we recognized very early on, of course, is that it's a disease that knows no boundaries, that occurs in various settings and affects neonates or newborns, infants, children, older children, and then can continue into adulthood. We also realized like most, that this is a disease without knowing boundaries. That means it crosses over into different specialties. So we include members of our cardiology team, our pulmonary team, our critical care team. It takes a village of really talented advanced practice nurses and therapists of all sorts. So in other words, it's an interdisciplinary program that manages children of all ages with various forms of pulmonary hypertension.

The program links inpatient with outpatient care. But what we realized quite a while ago is that there aren't many places that do this kind of thing. Also, we had very limited data, very limited information on what treatments really work, what kind of diagnostic studies are really necessary, and in what sequence. We relied very heavily on our adult colleagues and friends and collaborators of what works in adults and how do we apply them to children? If we apply them to children, how do we apply them to infants and newborns? We know that infants and newborns, of course, have different physiologies, meaning how the heart and the lungs work together and how pulmonary hypertension develops. We also know that their bodies metabolize drugs differently and the effects of growth and what that means with early interventions that perhaps one cannot do in the adult. So many, many things were just unknown at the time.

We went ahead and we were in touch with some of the sites around the country, in North America more broadly. This was a little bit over 15 years ago. We met together, first of all 8 sites, and now we have 13 sites. These are sites in major institutions that include Boston, Cincinnati, Philadelphia and Columbia in New York. In California, we have Stanford and the University of California in San Francisco, Texas Children's Hospital, Vanderbilt University. We have Alberta in Canada. So we have many different teams of folks from all over who are engaged here. So as these programs began to develop, we formed this network, the Pediatric Pulmonary Hypertension Network, or PPHNet (
www.pphnet.org). Over the years we've had some grant dollars from the government, the NIH. We have had many, many meetings, have done research together, written papers together. What we've done is really formed the infrastructure that we can now move forward to advance our studies and understanding of pediatric pulmonary hypertension.

Now we have a number of programs that address several missions. Number one is how to overall improve the outcomes of our children and how to work closely with families to achieve that goal. Number two, how could we better inform ourselves so we do the best thing? Educate the community, meaning other medical centers that are seeing children with pulmonary hypertension to develop optimal programs in their region or through centers. Also to then advocate for our families and our kids through the FDA to the National Institute of Health for research through government. Then finally, how to educate the next generation to make sure that our younger subspecialists in the field that I mentioned can be actively engaged in solving the problems related to pulmonary hypertension.

Over the 15 years then, the PPHNet has really developed a nice patient registry, which provides a lot of information from well over 1600 patients already. That provides us with a sense of the natural history of the disease, how we're treating them, thinking about their disease, how we can improve outcomes in the different diseases associated with pulmonary hypertension. These include the idiopathic forms, that is, we don't really know their cause, the genetic or heritable causes, causes related to lung diseases, heart diseases or systemic, meaning diseases from anywhere in the body that could be liver, blood cancer, many other things where we see pulmonary hypertension.

Every year we've had a meeting, our organizational meeting in San Francisco. It's also paired with a scientific conference, which is called the Neonatal and Pediatric Pulmonary Vascular Disease Conference, organized by Dr. Jeff Fineman as our leader. He's been fantastic pulling members from the PPHNet and others together to help organize the best of experts in various aspects of childhood diseases.

We're also expanding our engagement of families, getting them more involved so we can make sure we advocate for them, meet their needs, do the educational part that's so vital, and get families talking to each other. So most recently we formed in the network, largely thanks to Dr. Russell Hirsch at Cincinnati Children's, this idea of PHriends of PPHNet. The PHriends really means non-medical people and people who could really help us make sure that we're meeting our goals of being advocates and colleagues and meeting the needs of the community and their families. So we're launching that as a major activity and that's quite exciting.

Along these lines, we've developed a website and the website will include a number of resources and information about our centers, but also different projects and goals and activities that PPHNet will be achieving. We also opened it up now from our 13 centers to individual members. So we'll have a number of folks from all over the country and from all over Canada as well, North America right now, who could join us. Then they could work towards developing their sites into successful programs. Then their sites can subsequently join us as we get the word out on how to better approach children from both our clinical research as well as our laboratory research, how to then communicate advances into effective therapies by working closely with pharmaceuticals, with regulatory agents, especially the Food and Drug Administration, the FDA and others, and to really finally try to just change the course.

Things are already getting better. Very exciting data was presented at this year's meeting. We're already seeing nice responses with very complex diseases to some of the therapies that we're using when applied to the right populations who are evaluated in the right fashion and followed closely. The meeting also highlighted certain causes of pulmonary hypertension or associated diseases. Some of them are what we call developmental lung diseases where there is a genetic basis for the lungs not growing properly, which then contributes to the pulmonary hypertension.

So how can we get smarter about that? One of our big workshops included experts in genetics, in pathology, to look at the lung tissue, the structure of the vessels and the airspaces that they travel with. We had members from neonatology and cardiology speak out. We had family and parents' perspective talking about all the issues of the care of their child and what it was like being in the intensive care unit going through such a difficult course when we've had very little to offer in that time window to improving the outcomes of their children. So we've had all these folks coming together, and that's a good marker for the kinds of missions that we have.

One of the major goals of the PPHNet is to really work closely with physicians and care providers from all over the country. We provide a resource for families as well. That's what PPHNet does. We talk with all sorts of very, very talented care providers, physicians and nurses and others. We advise and we'll discuss details on cases and we're available for those kinds of consultations. There is a lot of talent around the country absolutely, that are not necessarily PPHNet sites, but what we try to do is encourage people then to develop programs that really sort of provide the kind of depth and breadth of support from having multiple disciplines involved. Again, lung specialists, pulmonologists, neonatologists, specialists for the newborn, hematologist, oncologist. We work as a team to make sure that we could provide a team approach by talking about the kids together, caring for them together. We're trying to optimize care at as many sites as possible, but sometimes because we have these as sites that have this experience and expertise, and also we have striking interest in research with the registry and collecting patient data and things like that. That's where the PPHNet provides additional service.

So if you're the parent of a child who has pulmonary hypertension in a place where the medical center just doesn't have all these specialties, or they haven't yet formed a pulmonary hypertension program, that don't have talented individuals who really could provide great care for your kiddo, we're a resource to help advise and counsel. At those centers, they could then work with us to develop the kind of care program more broadly. Short of that, of course, we do get children who will travel to some of the sites that are close to their home for further in-depth evaluation. We're really not trying to take over the care, but simply to really help highlight and review and offer other opportunities or recommendations for care. So it really is still a part of the team with your local doctor and your local center. Communication's essential. We're certainly available to consult and get engaged with local care providers and to work together to help the families.

Overall, the scientific conference that we've just had, our organizational meeting for PPHNet and its growth, all of these things are very, very exciting times. We're really looking forward to really changing the course of all forms of pulmonary hypertension, improving not just survival, but the quality of life. I think exciting advances are happening. So it's wonderful to be part of the PPHNet.

My name is Steven Abman, and I'm aware that our patients are rare.

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