Russel Hirsch, MD - phaware® interview 551

Why Families Belong at the Table: PHriends of the PPHNet

Dr. Russel Hirsch breaks down the core pillars of the PPHNet, from shared research to consistent care standards, and explains why the next chapter in pediatric pulmonary hypertension care must be written with families, not just for them through the PHriends family-led committee.

My name is Russel Hirsch. I'm a physician, pediatrician, pediatric cardiologist, and I work out of Cincinnati Children's Hospital. I've been in the United States now going on about 33 years, actually. I grew up in South Africa and trained at the University of Cape Town. Then, knew that I was probably going to be leaving and trained for a further year and a half in the United Kingdom before moving to actually St. Louis Children's Hospital where I did my pediatric training and my pediatric cardiology training and actually specialized at that time in interventions. My initial appointment was actually doing interventional cardiology.

At St. Louis Children's Hospital, they had a very large lung transplant program. In fact, the biggest in the country at the time for pediatrics. They were doing a lot of pulmonary hypertension work, so I had a lot of background in pulmonary hypertension, cardiac catheterization, and also nascent interest in the therapies and the drugs for pulmonary hypertension.

After I'd been there for about five or six years, I was recruited to Cincinnati Children's Hospital where I've been ever since. That's going on about 22 years now, where initially, I work mainly in the cardiac catheterization laboratory, but from about 2003, 2004 onwards, it became apparent that nobody was really looking after pulmonary hypertension patients and they were all coming to the cath lab. I was cathing them and once they were cathed, they were all going to the cardiac intensive care unit or the general cardiology floor. By default, they simply started falling in my clinic. That was how the pulmonary hypertension service started. It was around about 2005 that we actually formed it into a formal pulmonary hypertension service. Now, I've got a training program and it's grown into something really quite amazing and quite big. I've been incredibly fortunate to be very well supported by those around me, because I work with incredible people and my hospital has been very supportive.

Our initial involvement in the Pediatric Pulmonary Hypertension Network came after I first met Steve Abman, actually. He came to give a lecture for a conference at Cincinnati Children's Hospital. It was a BPD chronic lung disease neonatology conference, and I met Steve there and he introduced us to the Pediatric Pulmonary Hypertension Network. He was the Director at the time. It was very interesting to me, but I didn't think we were ready and part of the readiness was that I didn't feel like we had our research and academic and scholarship credentials quite in line where they needed to be. So, we waited about two or three years before we put in for a formal membership. I think that was probably around about 2015, 2016, maybe a little bit later, 2017, 2018, but we became a member of the network.

Now, the Pediatric Pulmonary Hypertension Network is a consortium of currently 14 pediatric hospitals around the United States and Canada. It is not necessarily the largest of the sites, but it's the sites that have established themselves as creating lots of work in the academic sphere, in the clinical care sphere, and also in the advocacy sphere, and what it's allowed us to do is to combine all of those attributes into a network that is now very, very strong and serves for teaching advocacy, consistency of care, industry relationships, which are now getting larger and larger. My involvement in that organization, initially, was to sit in the back of the conference room and listen, because it was quite intimidating. These were the biggest names in pulmonary hypertension, but a wonderful group of people who all had one thing in common and that was the care of the pediatric patient. How could they do better? How could we as individual centers combine our efforts to actually advocate and do better for pulmonary hypertension?

It was remarkable, because the other thing which I recognized was that everybody at these meetings, at the PPHNet meetings, were voluntary. Nobody was being paid. They were all doing it on top of their academic and their clinical and their own institutional research activities. Nobody got any stipends. Nobody got any payment. They were doing it because of the love of children with pulmonary hypertension. It was remarkable to me because there was a need for some kind of financial background. Quite remarkably, at the second meeting that I was at, it just dawned me and I asked a question. I said, "Is there a finance committee for this organization?" Everybody looked at me and pointed at me and said, "Yes, there is now." That's how I became the treasurer of the PPHNet.

As I progressed through the organization and started understanding the chart and the bylaws of the organization, one thing became completely apparent to me. As much as that there were periodically involvements of families and parents, there wasn't a concerted family involvement. This was just before the pandemic. I had started developing some ideas as to how we could change that. In actual fact, I had started registering a completely separate organization as a nonprofit that was aimed at raising funds for pediatric pulmonary hypertension research, which I was going to use as a vehicle to involve families to actually contribute their time, their efforts, their own advocacy for their children into bake sales and golf tournaments and all other kinds of ways that we could start raising money to support families and children with pulmonary hypertension. Long and short of it is everything came to a crashing halt during the pandemic. As much as it was registered as a nonprofit in Ohio and I'd put through the application to the federal government, the IRS, I decided actually to stop there.

What I ended up doing was I actually took the framework of that organization and turned it into a subcommittee of the PPHNet, which was accepted by the organization, the executive committee. That's called the PHriends of the Pediatric Pulmonary Hypertension Network. The whole point of the PHriends Network is to further involve families and parents and aunts and uncles and grandparents and anybody, neighbors, into the organization so that they could have an equal voice in the organization. They could actually contribute, they could come with their ideas. In reality, there are lots of needs that we could help those families with. Not everybody lives in a big urban area where they have access to medical centers. Not everybody has oxygen concentrators or the insurance companies pay for oxygen concentrators that allow the kids to be more mobile and get around rather than taking big oxygen tanks with them. Not everybody has access to understanding what research is out there for children with pulmonary hypertension. It's all of those and some other ideas that formed the basis for the PHriends Network.

So far, the PHriends committee has been in effect now for two years. It's been slow but steady, as we've grown. We started off just with an initial virtual meeting to get families together. We recognize that there is privacy and there's confidentiality that we want to be very, very sensitive of because some families don't want to know that they're out there, so we've taken that into account. I think that has been maybe a slight impediment to getting some families to become involved, but the Zoom meetings are open and they're open to the aunts and the uncles and the brothers and the cousins and everybody else. Having gone through surgery, pot shunts or lung transplant doesn't preclude families from contributing. I'm going to be very, very honest. We've got some families who've lost children to pulmonary hypertension who become members of the PHriends Network because they are incredible and they want to continue to contribute.

Having said that, we've extended the actual organization to now starting to do educational seminars. Eric Austin, my colleague from Vanderbilt spoke about the genetics of pediatric pulmonary hypertension. Rachel Harper spoke about medicines in pediatric pulmonary hypertension. We have a number of series that are planned coming forward. The dates are yet to be determined. We plan to have the next session, which will actually be myself talking about cardiac catheterization as it relates to pediatric pulmonary hypertension all the way from the newborn, all the way to the older adolescent that's transitioning to adult care and needs that last cardiac catheterization at a pediatric institution before they move on to the adults. I want to talk about the anesthesia. I want to talk about the risks, but in actual fact, what I plan to do is to go through a cath report so that everyone can understand what is the need for reliable, valid information and how that impacts care. The actual hemodynamics. The measurement of the pressures. What the tracings look like, what the environment looks like, show you a picture of a cath lab. It's really important for everyone to see that.

In that context as well, one of the things that I am, of course, proud of is we have started a program to try and work on decreasing the anesthesia exposure at the time of cardiac catheterization. We do know that particularly that first cardiac catheterization, there is a huge amount of anxiety both from the parents, but most importantly, for the child involved. We've invoked a program that is becoming more and more active to use our child life specialists to actually be not just in the room at the time, but to actually walk through the cath lab the day before with the child, the adolescent, to show them the environment, show them so they can ask questions so that it isn't a shock and surprise when they walk into that very clinical environment the next day with a lot of strange people. It gets them to say "hi" to everybody, "hi" to the nurses. We've done this now with a couple of patients and we've actually avoided any sedation or any anesthesia whatsoever. That doesn't work for every single child. These are ideas which I would like to be able to put out into the PHriends Network so that they can continue to advocate for their children, because not every program obviously has the means to do this, but I'd like to be able to do that in the future as we go forward.

The hope is that the actual office holders for the PHriends committee are filled by parents. The ideal is for families to be front and center in the PHriends Network. I serve as the Chief Facilitator and leader of the committee at the moment, but I have no plans to do that forever. As soon as I can move into the background and serve as a facilitator, a communicator, that would be better, because not only will that mean greater advocacy and personal advocacy for the families, but it gives them a seat front and center in the PPHNet on the steering committee. There is a chair waiting on the steering committee for the chair of the PHriends of the committee who can come to the meetings, can understand what we're doing as a network and can actually change us from just being the hardcore science, registry, advocacy, teaching [organization], to put the softer edges because it's the softer edges that really count.

At the end of the day, unfortunately, we're dealing with very sick children and we want to make sure that we are catering to every single need. Until such time as we have families deeply involved and embedded within the organization... and I'm not trying to imply that there aren't any families. There are some families involved in the organization, but just that greater advocacy, that greater visibility, ability to also just move the organization in slightly different directions other than what we see from big academic institutions, 14 big centers. Hopefully soon to have more network sites in that organization, because we will be expanding shortly.

I'm Russel Hirsch, and I'm completely aware that my patients are rare.

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