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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!

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Emma Olson Jackson, MS, ARNP - phaware® interview 342

Sep 29, 2020


Emma Olson Jackson is a Pediatric Nurse Practitioner at Seattle Children's Hospital. In this episode, she discusses her history in helping develop pediatric pulmonary hypertension programs across three different specialty centers. Emma also discusses the challenges that pediatric programs face in treating this disease.

My name's Emma Olson Jackson, and I am a pediatric nurse practitioner in pulmonary hypertension here at Seattle Children's Hospital in the heart center. I've been a pediatric pulmonary hypertension nurse practitioner since 2012, where I started at UCSF Benioff Children's Hospital, developed that program there, and I was there almost four years and then I moved up to Portland, Oregon and was at Doernbecher Children's Hospital, which is an affiliate of OHSU Medical Center, and did pulmonary hypertension there, and now I'm at Seattle Children's.

I was a bedside nurse before I was a nurse practitioner in the cardiac ICU at UCSF, and I knew Jeff Fineman, MD really well. When I was finishing my graduate school education and getting my NP license, he was also at the same time trying to get funding to hire the first pulmonary hypertensive nurse practitioner at UCSF. He convinced me that this was the field that I should go into and work with him, and it was an awesome decision. I didn't really know what I was getting into, but he such a wonderful provider, and he's done so much research on pulmonary hypertension, and he's a wonderful teacher and just in general, a great team member. Starting in that environment was really wonderful, so I just learned so much. Then once I dove into the world of pulmonary hypertension, I knew I never wanted to leave it, and that's kind of where my passion has been. So the whole time I've been a nurse practitioner, I've specialized in pulmonary hypertension, which has been really valuable for me.

I am from the Pacific Northwest. I grew up in a town called Bellingham, that's north of Seattle, and the program in Portland, kind of cold called me and had heard my name, and had a position opening within cardiology in pulmonary hypertension. They were wanting to grow their pediatric pulmonary hypertension program. At that point in time, I really was not looking to leave UCSF. I was very happy at that program and I felt like we had accomplished a lot and we had a lot of momentum, and the program was growing and I felt really close to my patients there and the team, but at the same time, I knew eventually I wanted to be back in the Pacific Northwest to be close to my family.

I had a sister in Portland and when the opportunity presented itself and I started to be recruited there, I felt like there might not be another opportunity. There's very few pulmonary hypertension nurse practitioner positions in the nation, and to have one open up kind of where I thought eventually I wanted to end up (was a important). I wasn't really ready to leave San Francisco, but I was worried that if I didn't take the opportunity, I might not have another opportunity to move closer to my family. So that's why I decided to take that position.

Once I was in Portland, it was a big difference just from going from a really big pulmonary hypertension program to a much smaller pulmonary hypertension program. I pretty quickly realized that I wanted to be part of a bigger program, and lucky for me, Seattle Children's actually got funding for a nurse practitioner when I was in Portland. People already knew that I was there, and so Seattle reached out and then recruited me up to Seattle, which was a pretty easy recruit, because I wanted to be in a bigger program, so it was awesome.

I think it was a really good experience for me to be also in a smaller program to see the challenges. There's still patients in Oregon and everywhere else that do end up being seen in smaller programs, and they need to have strong pulmonary hypertension program, as well. I learned a lot now that I've been in three different pediatric pulmonary hypertension programs. Everybody does things a little bit differently, and every provider has a different style of care. I think it's been really amazing for me to learn from all those different providers and teams and take what I like and what I don't like from every place and just learn more about how to best take care of this pediatric population that's really challenging.

I think pulmonary hypertension is rare, as you know. In pediatrics, it's even more rare. I think it's a really niche specialty. One of the things more people are recognizing is that there kind of does need to be centralized care for these patients, because it's complex, and there needs to be experts caring for them. I think because the numbers in general are small, because it's a rare disease, that there's just less comprehensive centers that have enough volume to justify having advanced practice providers in that specialty.

Seattle Children's is both a comprehensive care center through the PHA for pediatric pulmonary hypertension, and they're also part of the PPHNet, which is the Pediatric Pulmonary Hypertension Network of North America. I think in general, the pediatric pulmonary hypertension world is small, and it's a really close knit community. That's something I love about this field of medicine, is that we're always communicating with each other and sharing ideas and protocols and guidelines and getting advice from each other. There's no secrecy from program to program about how people are doing things, and there really doesn't seem to be very much ego, which I think is sometimes rare in medicine between programs. We're not trying to compete with each other. We're really trying to be cohesive and collaborative to provide the best care for this population, which I love.

Some of the challenges in this field is because it's rare, and the numbers are relatively low when you compare it to other types of heart disease, that it makes it hard to really figure out best practices or best medical systems. We might think what we're doing is best, but actually we don't know what someone else is doing, or when we're comparing all the numbers together, it's really difficult to do randomized controlled trials to figure out what combination of medicines are the best. Working with a rare disease, it's challenging to have enough numbers that bring valuable research. So it's imperative that we form these different networks of multiple pediatric pulmonary hypertension centers, because that's the only way we're going to really answer big questions is by collaborating, putting together all of our data to figure out what's the best practices for this group of patients. But it's also a challenge, because that also takes a lot of time and effort in enrolling patients and putting in their data into these big registries, and then at the same time, it takes years of putting in that data and then multiple more years of interpreting that data before there is a change in practice.

We're doing such a better job of treating pulmonary hypertension than we were even 10 or 15, 20 years ago, and these patients are doing better and they're surviving longer and they're having higher quality of life, and we have more medications available. I think we're treating earlier and more aggressive and giving kids a really fulfilling, healthy, longer life. It's great that we do see these patients transitioning, and that's something we think about, and it's really imperative that we are working with adult centers that we trust and that we have our values and the way we care for patients aligned. We really work really closely with our adult colleagues, and really do the transition to adulthood in a thoughtful manner and in a way that prepares the child for the differences in an adult center, and at the same time, still offering our support. We tend to do a staggered transition where they meet the adult provider, sometimes we try to have them meet the adult provider before they actually go and transfer care. We're hoping to work through a system where then maybe we actually attend maybe the first appointment at the adult center so that we can act as a buffer and fill in the gaps of the clinical history that maybe didn't make it in the record change.

Another big piece of transitioning to an adult program is making sure that the child or adolescent really understands their disease and their medications, and getting them independent as possible, because once you transitioned to that adult program, there's much, much less ... I don't want to say handholding, but they assume that you take charge of your own care and that they're not double checking that you understand every piece of it.

It's our job as pediatric providers to make sure, in the years before transition, and we start talking about this sometimes when a patient's 11 or 12, even though they're not going to be transitioning until they're 18 or 19, sometimes 21, is that every time we see them in clinic, we try to think of a new topic to teach them of part of their disease, and are working with them understanding their disease and understanding their medications and being able to tell us what their medications are instead of us telling them and having them agree. When we go over medication lists when patients are younger, we just say, "Are you taking this, this and this?", and as they get older we say, "You tell us what you're taking, and what's the dose, and how frequently?", to empower that child to really take charge of their own health and understand their disease and the medications and why they're taking them.

The diagnosis of pulmonary hypertension is a challenging diagnosis to receive, and I think that when you're a newly diagnosed patient it’s really to try to think about one thing at a time and to remember, especially in pediatric, that every patient is really different, and the etiologies for pulmonary hypertension can be very different from kid to kid. I find sometimes that patients, especially newly diagnosed, kind of compare themselves to other patients with pulmonary hypertension, and that's a really challenging thing to do, because I think every case is so different to really hone in on your own child and their issues and level of disease and medical management and try to get to know that and really learn what's best for your child before you try to compare your child to other cases, especially cases that have had pulmonary hypertension for a long time, or that have really different reasoning for having pulmonary hypertension.

I think one of the challenges I see over and over again with newly diagnosed pulmonary hypertension is that because it's rare, parents want to reach out and find similarities with other pulmonary hypertension patients almost too soon before they really get to know their own child and their own child's disease, and that's not to discourage connecting with other parents, but that's just to learn your own child and their own disease and their own medications before you start to compare another child to your own.

Every parent wants to do their best for their child, and every care provider wants to do the best for their patients, and sometimes it takes time to figure out what that best practice is, and what I like to tell patients, especially new patients, is that we need to have patients and us getting to know the child and the parents getting to know their child with this new diagnosis. It takes time to figure out which medications or combination of medications work. Sometimes there's not really quick, fast answers, and it's a process that we get to do together, and it takes time. So sometimes there's not answers right away.

My name is Emma Jackson, and I'm aware that I'm rare.

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