by Bret Baier
Bret: Thank you. I mean, it was a family there at Children’s. It really was. We felt that way the entire time we were there. On another topic: have things changed since then in this whole area of early warning signs or diagnosis?
Dr. Jonas: Fetal echocardiography is really progressing. I mean it was only in the late seventies that echo machines were developed where you could put a probe on a baby’s chest and you could see anything at all in terms of figuring out the heart problem. Getting good images when the ultrasound has to pass through the mom’s abdominal wall through the uterus through the fluid. You have the baby moving around inside the amniotic fluid. It’s really amazing that those ultrasound machines can give you good images now. That’s where there has been a lot of advance in the last ten to fifteen years. We can even get fetal MRI scans that give you even more information than the echo and look at the baby’s brain as well as the heart. So, advances in fetal echocardiography are still moving along.
Bret: Isn’t there now an inexpensive simple oxygen test that can be done? A simple finger test of some sort?
Dr. Jonas: Yes. It’s called a Pulse Oximeter. Fetal problems are missed if a baby is born like Paul was. It’s even possible for a baby to go home from the hospital undiagnosed. Because while the baby still has the fetal circulation, it can take a few days before you get the transition to what we call a mature circulation.
Bret: So, in a sense, even though the heart is operating the wrong way—it’s still pumping.
Dr. Jonas: Yes. And it’s allowing a baby to stay alive. In some circumstances, the only change is a very slight change in the oxygen level. A baby doesn’t become really blue. Some babies will be really blue and get really sick. But others will stay relatively well and unless somebody is extremely well trained, has a really good eye for color change, they could miss the fact that there is a slight blueness. The child will leave the hospital and get really, really sick at home when they do have an abrupt change from the fetal circulation to the post-natal circulation. So, the Pulse Oximeter is a little machine that you put on a fingertip and can detect very fine changes in oxygen level. That’s been a big advance in screening and helps to pick up kids who would otherwise be missed.
Bret: But, they’re not yet mandated for hospitals to use right?
Dr. Jonas: Well, Dr. Gerard Martin’s been very heavily involved with this together with the American College of Cardiology and other cardiology groups. It is something that’s beginning to become the standard of care on a state-by-state, hospital-by-hospital level.
Bret: Along with being a surgeon and performing hundreds of complicated surgeries like Paul’s and others every year, you’re actively involved in various research projects at Children’s. I’m wondering if you could give folks a sense of what you’re up to?
Dr. Jonas: Well, the main focus of my research has been to try and optimize the development of the brain in kids who also have heart problems. In the early years of heart surgery, there were a lot of specific problems related to the heart and lung machine. A lot of pediatric devices we used on children in the early days were actually developed for adults. So, we would take a machine designed to support a 200-pound adult and connect it to a four or five pound baby. Needless to say, under those circumstances, the machines didn’t work as well as we would like. That’s really the way things worked in the ’50s, ’60s. It wasn’t until the 1970s that there were some early machines designed for bigger kids. It wasn’t really until the 1990s, early 2000s that we started to get the equipment we needed for newborn heart surgery.
The organ that’s most sensitive to the harmful effects of using a very big machine on a baby is the brain. You get tiny particles that can travel to the brain and block off blood vessels. This can cause strokes inside the baby’s brain. In the early years of heart surgery this was a very big problem. Now there are very good filters and surfaces designed to avoid platelets and white blood cells clumping together and blocking off blood vessels and allowing us to minimize the inflammatory response to the heart and lung machine. The heart and lung machine is perceived by the baby’s body as being a huge invader. So, when you send a baby’s blood through plastic tubes the baby’s body assumes this is a massive infection and you get a whole body inflammatory response.
The brain is developing very, very rapidly in the newborn and that inflammatory response affects particular cells within the brain. So, our particular focus in research right now is trying to figure out which cells are most sensitive to inflammatory effects and how can we potentially reduce those effects and even replace those cells that died because of the impact of the heart and lung machine.
Stem cell implants into the developing brain is also an area that we’re just beginning to explore.
Bret: So, paint a picture, five, ten, twenty years down the road if you would. Look into your crystal ball and tell us what your field will look like.
Dr. Jonas: Well, I think in the very long term. I really don’t know if we’re talking twenty years or fifty years or 100 years. The whole genetic area is going to have the biggest impact. There’s no question that genetic factors have a lot to do with the development of heart problems. It’s not hereditary. It’s not as though you have heart conditions that are passed from one generation to the next. Usually, it’s random. Congenital heart defects just suddenly pop up in families with no history of heart problems. We need to understand what it is about that genetic code that causes that to happen and what we can do to neutralize that change so it doesn’t result in heart development problems. So, I think that’s going to be the biggest area where we are going to see the greatest impact.
Ventricle assistic devices for kids are also going to be important. Right now there are no implantable assist devices for kids even though there are several for adults. Those will probably benefit mainly kids who are born with a single pumping chamber. Most people have two pumping chambers, one for the lungs and one for the body.
A lot of our most complex kids have only a single pumping chamber and they end up with an operation called the Fontan Operation, which doesn’t give them a pump for the lungs. Blood gets through the lungs, but at higher pressure than it should. It backs up into the body a little bit in some kids and that can have bad affects on the liver, kidneys, intestines, and so on.
If you could implant a little tiny pump—just a little booster—then that would give all of those kids a much better quality of life. And there are already implantable battery systems, so you can charge the battery of an implantable pump-up just by laying a charger over it. You don’t actually have to have wires coming out through the wall of the abdomen or the chest anymore.
Bret: Circling back to Paul’s case. He’s had three surgeries now. And it looks like he’s going to have a fourth sometime in his teens.
Dr. Jonas: Yep.
Bret: We obviously try to pin you down all the time about exactly when you think that fourth operation might be needed. But, beyond that—is there anything on the horizon that could negate the need for that surgery or might change Paul’s scenario going forward.
Dr. Jonas: Well, the entire area of growing replacement parts.
Bret: Stem cells?
Dr. Jonas: Right. That would definitely benefit kids like Paul because he had a coronary artery running right across the front of his heart in an unusual location. That was one of Paul’s big challenges. We had to bridge over that coronary artery with a non-growing tube and we used a human artery with a valve in it from a child organ donor. Paul’s outgrown them, of course. He’s a lot bigger now than he was then. He was a newborn when he had his first operation. So, if we could use tissue engineering and stem cells to develop a growing tube so a newborn baby would take some of his own cells or maybe even stem cells from a family member.
Bret: Like cord blood cells?
Dr. Jonas: Yes, like stored cord blood that would potentially give you stem cells that could grow in a tube that his body wouldn’t reject and would be able to grow with him. That would be anothe
r very big advance.
Bret: Couple more things. I know some folks would be interested in how you get ready for surgery. How you map it out. As you described to me before, you plan it out the night before. You see it. Describe that. And then you kind of sleep on it?
Dr. Jonas: I do. I really think that it’s important the night before complex operations to allow your brain to think about it all through the night. It’s actually quite helpful because the sequence of these operations is really important. The exact order. I mean it’s a little like potentially painting yourself into the corner of a room. If you don’t do things in exactly the right sequence—A: it takes a lot longer and—B: you might actually have to take part of the operation down and get back to an area that requires additional suturing or additional implantation of a patch. So, we do have to think about time. There’s a limited amount of time. We’re stopping the heart. We’re starving it of blood during the time we’re operating inside of it.
Bret: It actually stops. You’re stopping the heart. Putting the body on this machine. It’s actually coming to a full stop?
Dr. Jonas: Right. The body is being supported with the heart and lung machine. So, the blood is pumping to the brain and to the kidneys but the heart itself has to stop because you’re working inside it. So, the way we do that is to put a clamp across the main artery to the coronary arteries and the heart is now effectively having a massive heart attack, except we’ve cooled it down to a very low temperature and we’ve injected a chemical solution that paralyzes that muscle. Stops it consuming energy. And that gives you around about two to three hours when you can work safely inside the heart. So, you’ve got the stopwatch ticking. That’s where you really have to plan it out. I’ve watched plastic surgeons—terrific plastic surgeons—re-do a flap, something complex, four or five times until they’re completely happy with it. We don’t have that luxury. We have to get it right the first time.
Bret: And then there’s a point when you’re undoing that clamp and you’re ready to take him off the machine and that heart needs to start again.
Dr. Jonas: Parents often express to me that they’re worried the heart won’t start at all but it will always develop some activity. The question is that we want to see that it’s going to take the load—whether or not it’s strong enough to take over from the heart and lung machine. So, the real critical time is when we transition from the heart and lung machine to the heart itself. How well will it take that load? That’s really the key time. That’s when we will hold our breath.
Bret: You have an amazing ability to explain things and be calm about it. You have an amazing bedside manner. These are kids that you’ve opened up. You deal with the kids and with the parents in the waiting room. How do you process all that? I mean, it’s not the carburetor of a ’57 Chevy that you’re working on. You have to detach a little bit right?
Dr. Jonas: Yep. It’s the paradox of surgery. You do have to be caring and empathetic but you also have to be able to switch off and detach and become a bit of a robot in a sense. I’ve been called “Iceman.” I’m not sure how kindly that’s been said by some people but it takes a certain personality type. We have many people interested in the field of pediatric heart surgery, but it does require a certain personality type to be able to master that detachment. Many, many very outstanding adult heart surgeons say to me that they would never try to do what we do. They just feel it’s not in their make-up to do it. So, even within the field of heart surgery, pediatric heart surgeons are considered a little bit different.
Bret: But later when you see a kid who’s been on that operating table graduate from college and write you a letter, or writes his college entrance essay on his or her experience or write you a thank-you note—or—you hear about a little boy running faster on the basketball court—that’s gotta be pretty special.
Dr. Jonas: It really is. It’s truly been remarkable that my career started almost the day that this field started because I started out just as Prostaglandin was being released and that really allowed me to be part of the neonatal revolution. That totally changed pediatric heart surgery. So, (those kids) feel like my own kids really. They’re graduating from college and getting married and writing letters to me and it’s really an incredibly warm and rich experience to be able to feel like I’ve got this whole family out there—just like my own kids. You know, you go through the ups and downs and all the challenges. You remember some of those difficult times when you faced very serious challenges with the kids themselves and with their parents. To finally get them to that college graduation or their wedding day is really terrific.
Bret: Dr. Richard Jonas—Thank You.
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Contents
Cover
Title Page
Welcome
Dedication
Prologue
Chapter One: Young Man on the Move
Chapter Two: Together as One
Chapter Three: Complicated Heart
Chapter Four: Through the Storm
Chapter Five: One Day Closer
Chapter Six: Things Unseen
Chapter Seven: Special Hands
Chapter Eight: Miracles Do Happen
Chapter Nine: To Whom Much Is Given
Chapter Ten: Special Heart
Epilogue
Photos
Acknowledgments
An Interview with Paul’s Heart Surgeon—Dr. Richard Jonas
Newsletters
Copyright
Copyright © 2014 by Bret Baier
Cover design by Roger Gorman
Cover photograph by Joyce Boghosian
Photo of Bret Baier interviewing President Obama is an official White House photo taken by Chuck Kennedy and is used with permission. Photo of President Bush with Paul by Chris Greenberg, courtesy of the George W. Bush Presidential Library. All other photos are from the author’s personal collection.
Cover copyright © 2014 by Hachette Book Group, Inc.
All rights reserved. In accordance with the U.S. Copyright Act of 1976, the scanning, uploading, and electronic sharing of any part of this book without the permission of the publisher constitute unlawful piracy and theft of the author’s intellectual property. If you would like to use material from the book (other than for review purposes), prior written permission must be obtained by contacting the publisher at [email protected]. Thank you for your support of the author’s rights.
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ISBN 978-1-4555-8364-5
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