[COLD OPEN]

Drezden: Hey everyone, this is Drezden. Before we jump in, quick note, this is part one of a three-part series. Today you're going to hear an incredible story from 1952. Let's get into it.

[INTRO]

Drezden: This is The One Percent Heart, a podcast about the millions of people living with congenital heart defects. In this episode, we have the opportunity to speak to William "Bill" Causey, the longest living open heart patient. Before we begin, here is my normal quick disclaimer. Nothing said here constitutes medical advice. These are our own opinions, experiences, and stories. I also want to include a trigger warning as we will discuss medical related problems. Listener discretion is always advised. With that out of the way, Bill, thank you so much for joining me here today.

Bill: Thank you for having me.

---

[CONVERSATION]

Drezden: Now, I have struggled a lot with how to properly introduce you because you have done so much and seen so much for congenital heart patients in general. Do you mind giving a brief introduction?

Bill: Sure. I currently reside in Washington, DC. I've lived here for almost 60 years. Born and raised in Baltimore in 1949. I was fortunate to be born in Baltimore because of my congenital heart condition, I was living about three miles from Johns Hopkins Hospital. The other reason I was very fortunate was that my mother was a nurse. She was an Army nurse in World War II. In fact, she was a captain in the Army. My father was a sergeant. So my mother always used to say that she outranked my father, both in the military and in life generally.

When I was born, my mother could tell fairly quickly that something wasn't right. I wasn't like any other normal baby. I seemed to be a little slower than normal. I wasn't crawling around. She had a suspicion that maybe I had a congenital heart problem because I had the classic blue baby symptoms, the bluish fingernails, blue lips. She watched me closely and after about two years, she could tell that things were going downhill pretty quickly. I was not walking and moving around. I was lethargic. I wasn't responding quickly to stimuli around me.

So she decided to take me to Hopkins and I had an appointment with a pediatric cardiologist by the name of Helen Taussig. Dr. Taussig was probably at the time the number one pediatric cardiologist in the world. She had worked very closely with another Hopkins doctor named Alfred Blalock to develop the Blue Baby operation.

Dr. Taussig was really a remarkable person. She had her own incredible life story beyond just medicine. In fact, there's a biography that has just been written about her, a great biography by Patricia Meisel. Dr. Taussig was born basically deaf. I think she had scarlet fever when she was little. When she examined me, she didn't use a stethoscope because she couldn't hear. She used her fingertips. She used her fingertips to hear my heart murmur.

She diagnosed correctly that I had pulmonary stenosis. My pulmonary valve wasn't functioning properly. I wasn't getting the proper level of blood supply between the heart and the lung. That's why we were called blue babies. We weren't getting enough oxygen in our blood. Our blood wasn't bright red. It was sort of a bluish red.

Dr. Taussig followed me very closely for about a year. She could see that I was quickly going downhill as most babies two and three years old at that time were who had pulmonary stenosis. She went to Dr. Blalock. They were obviously very close already. She said, "You really need to operate on this kid. He's got pulmonary stenosis. It's clearly a bad pulmonary valve. I want you to see if you can go in and fix it."

Blalock, according to what Taussig later told me, looked at her and said, "Helen, I don't do three-year-olds. They don't survive the anesthesia."

She looked back at him and said, "Well, if you don't operate, he's going to die anyway."

And he said, "Yeah, I guess you got something there. Let's do it, Helen. For you, I'll do it."

So on April 5th, 1952, which happened to be Blalock's 54th birthday, Saturday morning, they took me into the operating room at Hopkins. Dr. Taussig was there as well. And of course, with Dr. Blalock was Vivien Thomas, his surgical assistant that had worked with him for over 10 years. There's a great story of how Blalock hired Vivien Thomas. If we have time, I'll get to that story as well.

But the three of them were there. This is before the heart-lung machine. The heart-lung machine wasn't developed until about '55, '56. They put me on a block of ice to get my heart rate down. Blalock knew he only had maybe 15 minutes to do what he had to do once they opened me up.

Once they got my heart rate down, he did his signature horizontal incision and got to my pulmonary valve. They could see that the valve wasn't working clearly. Blalock turned to Vivien Thomas, who was always over his right shoulder, and said, "Vivien, take a look. What do you think?"

Vivien looked at it and said, "Let me try something." He took his pinky finger, because he had surgical gloves on, and he manipulated the valve. Right in the heart. He manipulated the valve. They watched it for about a minute and he could see that he got the valve to function normally. It just needed a kickstart. That's what I always say.

After they looked at it for about a minute or so, Blalock said, "I think that looks pretty good." And they sewed me up. When they did my surgery, they had to break my sternum to get to my heart. They did that with a lot of kids back then.

They finished the surgery, sewed me up, and Blalock went to his 54th birthday party after that.

I went to the recovery room. The experience in the recovery room is my very first recollection ever. It was Easter time. I was in a crib in the recovery room. To me now, thinking about it, it was like a boxing ring. But I'm sure it was just a baby crib. There was somebody running around dressed up like an Easter bunny, and it scared the hell out of me. That's my very first recollection ever, being in that baby crib with that Easter bunny.

Drezden: What a way to be introduced to the world, right? I think there's something poetic about your life beginning after the surgery. I've always regarded Easter as one of my favorite holidays because of that.

Bill: I recovered very quickly. I would see Dr. Taussig periodically after the surgery. She followed me very closely. I'm sure I was seen by Dr. Blalock a couple of times, although I don't have a recollection of that. I was followed by lots of cardiac surgeons and cardiologists at Hopkins over the years.

My mother realized that not only was I coming out of a medical surgery experience, but that there were mental health experiences that went along with that. She told me from the first days I can remember: "Look, you are very different. You've got a scar to show it. But you're not special and you're not better than other kids. Just grow up to be a normal kid. Take care of yourself. Make sure the doctors look at you every now and then. Listen very carefully to them. But just remember, you're just a normal kid."

That stayed with me. As I grew up through my early years and into elementary school, I tried to blend in. I knew I was different because I had a scar and every now and then kids would look at it. But it really didn't bother me. I was very active. I had lots of friends. I was very engaged in elementary school. I played a lot of sports. Went into high school. I played baseball in high school. The only physical limitation I had was swimming. I just didn't have the heart-lung capacity for swimming. But I would run, play ball, and do all the other things kids do.

Drezden: Before we go to the next part, for those who don't know, the Blalock-Taussig-Thomas procedure, the three doctors you mentioned, Thomas getting his honorary doctorate afterwards, is a very big deal for the congenital heart community. Before this procedure, patients like you and other blue babies would live very short and limited lives. Am I right in saying that?

Bill: Absolutely. Most of them died within the first year or two, if they survived surgery at all.

Drezden: And what blows my mind, my open heart surgery to repair my pulmonary valve was on April 15, 1996. That's 44 years after yours. They didn't put me on a block of ice. They didn't cut my chest horizontally. They didn't manipulate anything with their finger. They went straight down my chest. They cut through my sternum. They repaired the valve with synthetic material to fix the pulmonary valve stenosis. I was six months old when my mom said to my dad, "Does he look blue to you?" And by the next day it was done. There was no waiting, watching, following up. They found the issue. They airlifted me to a second hospital. And I have a funny story, my dad hopped in his car and beat the helicopter to the second hospital. That's probably something I shouldn't say.

The change in 40 years from deciding whether or not to do your surgery, saying you won't survive anesthesia, slowing your heart by putting you on a block of ice instead of a bypass machine, the comparison blows my mind. There's actually a great movie, Something the Lord Made with Alan Rickman, about this. Alan Rickman is Severus Snape, for those who don't know.

You talked about your mom's approach to mental health. A big part of what got me involved in the CHD community was how my parents were not told about mental health. We were always told I was one in a million. No one ever told us that one in a hundred kids are born with congenital heart defects. If it was not for my little brother, he was barely a year younger than me, he gave me someone to keep up with and compare myself to. My parents had their own trauma from the surgery and the lack of resources they got afterwards. I think if it was up to them, I would have been put in a plastic bubble a lot of the time. But my little brother just didn't let that happen.

That takes me to the mental health aspect. I'm wearing my Cardiac Neurodevelopmental Outcomes Collaborative shirt right now. They are a great research and provider organization for doctors, PhDs, and nurses in the CHD mental health space. The problems they're attacking now are remarkable, making subtle changes during surgeries and early life to keep patients' brains healthy and minds healthy, both physiologically and developmentally. But it sounds like your support consisted of your mom telling you you were normal and that you're not special. Is that right?

Bill: Absolutely. My mother did not put me in a bubble. She pushed me out into the street. She said, "Get out there and live your life. Be thankful that you're alive because a miracle happened. Just be like any other kid and you'll have a much better life." I followed that guidance. It was one of the most important things my mother and father taught me.

Drezden: You mentioned the movie. There are two movies about the Blalock-Taussig-Thomas shunt.

Bill: One is based on Vivien Thomas's memoir called Partners of the Heart. It's his autobiography. There's one page where he shows how he used his finger to get valves to operate. So I'm not making it up. PBS turned it into a movie, Partners of the Heart, which you can still get on YouTube. Let me tell you something about Dr. Taussig. She was truly a remarkable woman. I think she was the first woman to graduate from Hopkins Medical School. She tried to get into Harvard Medical School and they wouldn't let her in because she was a woman. She had a fabulous garden at her house. She was almost a professional gardener. She was always very interested in her patients, not only during their medical care, but after their surgeries and how they developed.

I can remember one story. In 1973, 20 years after my surgery, I was up in Baltimore at my parents' home. They had a TV on in the background. Dr. Taussig had just retired from Hopkins and was being interviewed on a local television show. Of course I stopped and sat down and watched with awe. That night I wrote her a letter, before email and all that. Within a week, she wrote me a letter back with her eight-cent Eisenhower stamp on it.

I still have the letter right here. Let me read a part of it. It's dated October 11th, 1973. "My dear William", she always called me William, "Your letter to me after watching my profile on television was one of the nicest letters I have ever received. You are quite right that learning from my former patients that they are fine and doing significant work", and she underlined the words "doing significant work", "means the world to me. Thank you for taking the time to write. You truly warmed my heart. Most sincerely, Helen Taussig."

So not only did she fix my heart, I was very happy to be able to warm her heart. Needless to say, I have this letter today. I keep it stuck between the two big volumes of a box set called The Alfred Blalock Papers. I've always been a book collector. About 40 years ago, I was at a book auction. I noticed a big two-volume box set on the floor. Lo and behold, it was the papers of Alfred Blalock. Needless to say, I grabbed those up in a heartbeat, so to speak. I still have those books today. I put this letter right between the two volumes. That's where I keep it.

Drezden: My wife says I should get it digitized because it's seen a lot of sunlight. You should scan it, laminate it, do everything to preserve it.

Bill: The other story I want to tell, and then I want to come back to say something about my follow-up surgery at Hopkins. My wife and I, she's also had some medical issues, so we're very conscious of staying healthy, keeping fit. We usually do a combination of a short nap and a long walk every day. We would always go to a resort in Tucson called Canyon Ranch for a week every August or September. About 20 years ago, I was in the men's locker room changing to go do a workout. There was a guy who kept looking at me. I thought, well, this is a little odd. That night we were in the dining room having dinner and the same guy walks over to our table. He says, "I want to apologize for staring at you in the locker room today." Then he said, "Did you have surgery by Alfred Blalock?" I said, "Yes, in fact I did." He says, "Well, I'm a cardiologist, and all cardiologists recognize the Blalock incision." Because it's horizontal instead of vertical.

Now, not to feel left out, I did eventually get a vertical incision. Because 10 years after my surgery, Hopkins said, "We think we can fix your sternum." It had caused a depression in my chest. In 1963, I had my second surgery at Hopkins. This time it was performed by a doctor named Alex Haller. Dr. Haller was an incredible person. He was about 45 years old, completely bald, from Southern Virginia with a thick accent. He had a great sense of humor. He was wonderful with kids. I was 14 at the time. He said, "I think we can repair that sternum." They operated on me. Unfortunately, they weren't able to repair it as much as they had hoped. But Dr. Haller became a lifelong friend. Sadly, he passed away in 1991. So I have a vertical incision and my Blalock horizontal incision. Best of both worlds.

Drezden: I've had three surgeries. The first was open heart, but the other two, the stents, they went through my leg. They went through an artery in the groin. Maybe one or two stitches at most afterwards. Metal in my heart, but the open heart scar is the only scar I can find.

Bill: Well, Drezden, not to let you take the better of me, but I've had that too. About 20 years ago, when I was going to Mayo regularly, I'll get to how that developed, apparently I've always had a small hole between the chambers in my heart, as a lot of CHD patients do. My doctor at Mayo, Dr. Carol Warnes, was the top cardiologist at Mayo. She said, "Bill, you're here for your annual checkup. Want to stay a couple days extra? I think we ought to fix that." So they did. They went in through the groin and arthroscopically fixed that hole in my heart. So I've had all three, and I hope I don't add a fourth one to it.

Drezden: I'm really happy you mentioned that because your story means a lot to me. You are the longest living open heart patient. You're 76, I'll be 77 in January. You don't sound like you're 77. I want everyone listening to realize how impactful this is. I had open heart for the pulmonary valve. I had two stents for the pulmonary artery. Also the hole in the heart, the ventricular septal defect. I had one between the two lower chambers that they stitched up during open heart. This episode is important because we can talk about how care has progressed over time. And just how lucky patients like me are today. But the parallels are dramatic for me. I'm thinking your job is to go from 77 to 100, 150, and my job is to beat you. Sound like a deal?

Bill: If I go to 100, we're going to do volume two of this podcast.

Drezden: It's a deal.

---

[OUTRO]

Drezden: And that's it for part one. Next time we'll hear what Bill did with the rest of his life after surgery. Thank you guys for listening and I'll talk to you soon.