Diagnosing and Treating Kawasaki Disease: A Patient’s Journey

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Episode 14 Transcript

Diagnosing and Treating Kawasaki Disease: A Patient’s Journey

Hello and thank you for joining us today. My name is Abbie Cornett, and I am the patient advocate for IG Living magazine. This podcast is brought to you by IG Living magazine to give readers an opportunity to hear from healthcare experts on topics important to them.

In this episode, we will be discussing the critical importance of early diagnosis for patients with Kawasaki disease and how intravenous immune globulin plays a vital role in treatment. I'm excited to have the pleasure of speaking with Nick Burrus today. Nick is a Kawasaki patient who, due to a delayed diagnosis, had serious complications that led to him needing a heart transplant. He is here today to explain his journey with his disease.

Abbie: Nick, thank you for being here today.

Nick: Yeah, absolutely. Thank you for having me.

Abbie: First of all, could you tell us a little bit about yourself and your diagnosis, and how that came about?

Nick: I grew up here in California. I had somewhat of a weak heart from birth. And I got something called Kawasaki disease at a time it was a hard-to-diagnose disease because it only exists in a subset of the population, and medical training was not very good at the time for this disease. It happened to be discovered when a visiting doctor not a part of a hospital, as I was going to multiple hospitals, happened to recognize the disease when he was walking across my ER room. This is a very rare disease.

Abbie: That’s fascinating. Why do you think the hospital wasn’t looking for that disease?

Nick: So when I first got the disease, it was about 30 years ago, and back then, there was a lot less training on childhood diseases for ER doctors. And, because the disease was believed to only exist in Korean or Japanese descendant children at the time, they didn't think this disease would have been correlated with other populations. Medical schools also taught about skin conditions that are common with certain races, so it often gets skipped over other types of skin rashes, etc. So the only thing they could think of was a bug bite. This was not a disease that medical providers were trained on at the time.

Abbie: So what effects did that delayed diagnosis have on you and your health?

Nick: So Kawasaki disease basically inflames the arteries and your entire vascular system. The inflammation causes your skin to peel off like a red strawberry. You get a red tongue and your nails will start getting like bright red, and you just get like really sick with a high fever of 104 to 105 degrees. So with this, it causes a lot of damage to your cardiovascular system. It causes aneurism, and it causes calcium buildup. Calcium is not always the best thing to have for the heart. If you have too much calcium, it slows the heart down. And with this disease, there is a treatment for it, but they have to treat it within a certain amount of time or it causes permanent damage.

Abbie: So, I'm assuming from that you had permanent damage, and what was that permanent damage?

Nick: They believe it weakened my heart severely because of the possible aneurisms that damaged the cardiovascular system. And calcium deposits cause a lot of scar tissue to grow, which your body tries to heal. That, in effect, can make your vascular system or your heart even weaker or can cause blockages.

Abbie: So what did that mean for you? What did that lead to?

Nick: It led to an extremely weakened heart. So my heart had an ejection fraction of less than 30 percent because Kawasaki disease caused so much damage to the vascular system. I was always in some sort of chest pain. I was always short of breath. That was my entire life. A normal human could expect an ejection fraction about 60 to 70%. It’s normal when you're in your 20s and 30s for it to be a little above 70. When you get into your 50s, you expect it to be in the 50s. But anything above like high 40s is considered normal. If you’re below 40, it’s probably some sort of heart failure. Below 30 is usually like, OK, this is pretty bad; your heart's pretty weak; it only has a 30% ejection fraction and that’s a recording for how much blood your heart is pushing out. So, you want it to push out like 60 to 70%. My heart was pushing out less than 30%.

Abbie: So that means you're not oxygenating your blood and so you're having a really hard time just breathing.

Nick: Exactly. So, Kawasaki disease causes the scar tissue and all that, and your body's response to it is, OK, we need to push more blood out because we're not getting enough oxygen or blood, so we're going to make the heart grow bigger. And so when it grows bigger, the side of the left ventrical chamber gets weaker because the heart tissue is stretching out. So the heart is getting weaker so you pump less blood. And so to correct that, it grows bigger and it pumps less blood and so it starts the whole enlargement process.

Abbie: So, let's take a step back when you were diagnosed with Kawasaki disease. What is the standard treatment for Kawasaki?

Nick: Standard treatment is giving immune globulin.

Abbie: So did you get immune globin at that time?

Nick: I did get immune globulin after they identified what the disease was. My parents took me to multiple hospitals. They did not know what it was. They told my parents it was just a bug bite and sent me home. My dad felt like there was something deeper wrong. This is not a bug bite. He took me to another hospital and the doctor there was like, “OK, you're right, this is not a bug bite. But we don't know what this is.” And a traveling doctor just happened to be there. He did some training in Japan so he recognized the disease. He diagnosed it without even touching me.

Abbie: That's amazing. And because of the delayed diagnosis and the enlarged heart, in our previous discussion, you told me that you had to have heart transplant.

Nick: Yeah, absolutely. So because of the delayed diagnosis, I was delayed in getting immune globulin. The hospitals at the time didn't always have that in stock, because at that time, immune globulin was more common in trauma centers or surgical centers and not just the random country hospitals. So it did cause my heart to be weaker and caused so much damage because they weren't able to treat it on time. It eventually led up to so many complications where I had to have part of my heart replaced. And they found a replacement and an ultimate heart transplant.

Abbie: And then you had that heart transplant in 2020?

Nick: I did, yes, in 2020 just right before COVID happened.

Abbie: That's amazing because you had told me that originally they thought it might take up to two years for you to get a replacement heart, and you were able to get one within four or five months. That's astounding.

Nick: Yeah, absolutely. I believe it only took four to five months. They said I had about a year and a half left of life in about August of 2019. But the wait list might be two years, so I was running a risk there. But, it only took five months.

Abbie: That's amazing. And then after the heart transplant, you were back on immune globulin again?

Nick: Yeah. After the heart transplant, I took about, I want to say five days of immune globulin or IVIG. I was taking it morning and evening each day because I went into acute microcellular rejection.

Abbie: And the IVIG helps calm that down?

Nick: Yeah. It helps to calm that down. And you want your immune system to not respond to it — to the antibodies and things like that. It helps your immune system not be overreactive. IVIG, even though it helps fight off infections, also has that nice effect. It reduces the inflammation; it reduces your overresponse of some kind, which is how it also treats Kawasaki disease a little bit. It reduces the overreaction response.

Abbie: So, you know, your journey has been incredible. And it sounds like you had so many challenges in your life. What would you say is your biggest accomplishment? I heard you talking about getting your PhD and you've got two master's degrees. How did you do all of that? And do you consider that your biggest accomplishment?

Nick: Yeah, absolutely. So one of the big things to recognize, and it's kind of hard to explain sometimes, but before my heart transplant, I didn't really think I had a future. I try to be optimistic, I love the world, I love life and everything. But I grew up in a medical system where they're saying, like, “you only have a few years or you're always going to be weak; you can't do this; you're not allowed to play sports; you have to be careful; you can't go hiking” and things like that. So my my life was so very limited with extreme shortness of breath and extreme pain. So, all of that now, post heart transplant, all those issues went away. I'm not as short of breath anymore. I used to not be able to take five steps without needing to catch my breath. Now, I'm able to go on hikes, things like that. I could play a sport if I wanted to.

Abbie: That's amazing.

Nick: So after this, I really sat down and started thinking, like, OK, I need to think about what I want to do for my future. And because of the coronavirus world, every single university decided we're doing online programs. And because I was not allowed to interact because of the transplant, I went to school. So I went and got my master's degree from the University of Maine in organizational theory. After I finished it, I started working on an MBA on the side. It was just a class. I didn’t really intend on getting an MBA. and I enrolled at Virginia University of Lynchburg to get a doctor of business focused on healthcare administration because I've always been really interested in how organizations work. So, while I was doing that, I was also taking a few more classes from the MBA school. I was short three credits. So I'm like, OK, I'll just take a few more classes. In Virginia, they have a law that you have to have 90 credits in order to get a doctorate. I was only short three to qualify for the doctorate program. So, they go, as long as you're taking classes concurrently with the doctorate, you're good to go. So I just happened to get an MBA kind of by accident.

Abbie: I'm going to finish my MHA by accident the same kind of way.

Nick: It's just the love of learning, because in the hospitals, you just get so used to learning. When you're growing in the hospital, that's all you have.

Abbie: You have TV and books. So, what are your goals for the future now? You said that you didn't really ever think about the future before because they kept telling you that you didn't have one. And now you've been given this great gift and you have a future.

Nick: That's a very hard question because I know I definitely want to build a career. I'm trying out different paths to determine what kind of career I want to build because I like a lot of things. I am good at technology. I like healthcare. So trying to decide on a career path is something that I’m proud of. It's something that I really want to do. And travel much more. I finally left North America for the first time just a few months ago. I went to Asia for a few weeks and that was like life-changing. Before my heart transplant, I wasn't even allowed; I couldn't even consider that. I'm able to break out of my box a little bit — break out of my shell and not be as protected in my shell anymore.

Abbie: That's amazing. Who would you say has had the most influence on you growing up?

Nick: So, there are a lot of people who were part of that. Because I grew up in the hospital and I have always been interested in the pursuit of knowledge, like how things work or how things always worked. And my dad always said, “you should learn computers and things like that.” My mom and my dad always encouraged reading and things like that, and my aunt always bought me a bunch of books. And so, it's a little bit of everything I really think. That saying that “it takes a village” is really true. A lot of people really helped me with it.

Abbie: OK, one last question: If you could sit down and talk to other people who had similar circumstances — maybe not Kawasaki disease but another chronic illness and major challenges in their life — what would your advice be to them?

Nick: Be willing to fight for yourself because when you're in smaller hospitals, oftentimes you're only seeing like the base level providers and it's hard to get into a specialist — especially a specialist who knows what's going on. Don't be afraid to ask for a second opinion. When my heart first started getting really weak again and I was told I had about a year and a half left, the first cardiologist I went to told me it's time to let nature take its course. I refused to listen. I got a second opinion. The second opinion was a heart transplant.

Abbie: Yeah, that's the one thing as a patient advocate I tell everyone: The person who is going to advocate for you the most is you. I mean when you're a child, it'll be your parents. But as an adult, you have to be able to step up to the plate for yourself and demand the treatment, because a lot of times if you don't, you get pushed aside.

Nick: And don't be afraid to change doctors if your doctor's not listening to you.

Abbie: That's another thing I say. Even if they're listening to you and it's not a good fit. Sometimes you just don't mesh, and if you don't have that that connection, sometimes it's just detrimental to your health.

Nick: Yeah, absolutely. After my heart transplant, one cardiologist I had back in my own healthcare system, she reached out to me and asked if she could have access to my medical file just because she wanted to know what went wrong with me so she doesn't miss it on a future patient. Those are the kind of doctors I want.

Abbie: Yeah, that's the kind of doctor you want. Well, thank you so much Nick. I can't tell you what a pleasure it has been to meet you. It's been a really informative day and your journey has just been incredible. You're a real testament to the fact that early diagnosis really does make a huge difference in patients’ lives. Time to diagnosis for a lot of chronic diseases or rare diseases is years, and so much damage is done during that period.

Nick: Absolutely. I type in all my symptoms on those Internet symptom checkers and they actually come up with Kawasaki. Back then, those didn't exist.

Abbie: No they didn’t. A caveat to that: Don't believe everything you read on the internet.

Nick: That is true, but it still gives you an idea of what’s going on.

Abbie: Well, thank you so much, it's been a real pleasure.

Abbie: Listeners, thank you again for joining us today. Additional information regarding this podcast can be found on our website at www.igliving.com. If you have a question that was not answered, please contact me at acornett@igliving.com.

Look for the next IG Living Advocate podcast announcement on our website for the opportunity to submit your questions.

IG Living Advocate is a copyright production of IG Living magazine published by FFF Enterprises. It is the only magazine for the immune globulin community comprised of patients who suffer from chronic illness and their caregivers.