You get used to it. The miracle of modern medicine is a little like the miracle of modern flight. Time in hospitals can seem a lot like time in airports. You sit in waiting rooms. You get antsy in your seat. There’s a lot of taking off shoes and going into scanning machines. You remove all the change in your pocket before you get weighed. People poke at you and ask the same questions every time: Swelling in your feet? Shortness of breath? Trouble climbing stairs? The ceremony becomes a recitation you half attend to, like the flight attendants’ ritual performed with the safety belts and oxygen masks and floatation devices under your seat, until all of a sudden—CATASTROPHE!!! The engines are dying! Cabin pressure fails! We’re going to plummet 39,000 feet! Then the plane rights itself. The crisis passes. You’re above the clouds again, cruising at five hundred miles per hour, with your legs uncomfortable, all the way from New York to Los Angeles.
6.
IN THE FALL of 1973, when I was seven years old, my dad and I took the subway from our apartment on 108th Street up to Columbia Presbyterian Hospital in Washington Heights, where just two years before I had had open-heart surgery.
This was before the trains were covered in spectacular graffiti. If my memory is right, there were still gum-vending machines on the platforms. Most of them were broken, the coin slots clogged, but I’d stick my finger in anyway and hope for one of those shiny little brightly colored Chiclets.
My dad and I were a mismatched pair. Brown Beatles bangs covered my forehead. His style was more Allen Ginsberg/Fidel Castro, with his dark hair combed back and a black beard covering his chin. The similarity in the shapes of our faces was obscured. I was tiny, all skin and bones, and he was six feet tall with a hefty gut, strong shoulders, and big hands. His anxiety filled his body with a watchful tension. Mine left me dreamy, not quite present in the physical world. My brother, Daniel, was there too—three years older than me, skinny, with a handsome, serious face and an enormous poof of curly Jewish hair. Daniel recalls the events of that day better than I do, but the truth is I have somehow excised him from my memories.
When the subway came above ground after 116th Street, I spun around on my knees and pressed my face against the window, fogging it with my breath and leaving the print of my nose. On one side were the buildings where I went to school; on the other was the Hudson River. I had been going to the hospital at least once a year, every year since I was an infant, but this time we didn’t go to the doctor’s office. We went to the auditorium. The place was packed with kids who’d had open-heart surgery. They all seemed very happy to be in the hospital—blond kids, black kids, boys and girls. Everyone seemed to be smiling and making friends. This was very confusing to me. Daniel remembers that lots of sick kids were there, kids not doing as well as me, and it freaked him out a little to see them.
I was shy. I wasn’t ashamed of my surgery. I didn’t try to hide the big scar on my chest, not at sleepovers or when I went swimming. But I thought the surgery was something private, mine alone, not to be talked about. I also thought the surgery was over, past and done, very unpleasant and nothing to be celebrated.
“Where are the grown-ups who had the operation?” I asked my dad.
“There aren’t any,” he said, looking around the lobby in undisguised wonder. “There’s no one here ten years older than you.”
The summer after the surgery was the summer I had begun to read C. S. Lewis. I had stumbled through the back of a wardrobe in an old castle in northern England, and the fur coats had become fir trees, and the mothballs had become snow, and I had entered the kingdom of Narnia, which was the place for me. My favorite of all the books was The Voyage of the Dawn Treader. Prince Caspian and the Pevensies sailed toward the end of the world. This idea fascinated me: the earth as a flat disc with a lip over which the oceans fell. When my dad said that there were no kids ten years older than me in the hospital lobby, no grown-ups who had had the same surgery I had, I felt the intimation of something similar, the end of the world, the oceans cascading into oblivion. In the hospital with all those happy, smiling, dying kids, I understood for the first time in my life that my little ship was sailing toward the edge of the world. I don’t know what everyone else does with their first childhood intimation of death, but I tried to swallow mine and push it way down deep inside me.
As I recall it, they called us the Open Heart Club—I remember confusing it with the Beatles and Sgt. Pepper’s Lonely Hearts Club Band. We filed into the auditorium and took our seats. There on the stage were the most famous players from the New York Mets, Tom Seaver and Willie Mays. Willie Mays in 1973 was confusing to me. He had once been the greatest ballplayer ever, but now he was a forty-three-year-old man way past his prime, who couldn’t hit or run or throw or field, and it was incomprehensible that someone my dad’s age got called “the Say Hey Kid.” Tom Seaver, on the other hand, was dominant. My friends and I pretended to be him and Tug McGraw and Cleon Jones and Felix Millan. Seaver was on stage in a suit, smiling and waving at us.
He made a speech. He said something like, “You kids are the real heroes,” and I had no idea what he meant. I dreamed of becoming a hero, a baseball player like him (though, as I explained to my friends, I’d work as a veterinarian in the off-season). I didn’t want to grow up to be a heart patient.
When Tom Seaver’s Mets had won the World Series in 1969, they had been called the Miracle Mets, and this was a name Seaver had objected to. It was no miracle he said; they had worked like hell to win that championship. When he looked around the room, did Seaver call us miracles? I didn’t feel much like a miracle. I did not want to know how lucky I was to be sitting there in that auditorium with my dad and big brother and all those kids just like me, feeling so confused and alone and alienated.
Sitting up on stage with Tom Seaver and Willie Mays was Dr. Malm. This also confused me. He’d seen me as an infant and right before surgery, and probably he had come by my hospital bed to visit me at least one time after the operation, one of a hundred doctors and nurses poking at me, but I had no memory of Malm’s face. His name had been invoked over and over again in our home, and I had thought his name was spelled “Dr. Mom” and not “Dr. Malm,” and some of the magic of that misspelling had rubbed off on my conception of the man—the doctor named Mom who had fixed my heart. On stage, he seemed small and ordinary next to the ballplayers. There were speeches. There was clapping. The boy next to me seemed very excited. That was weird to me. Did he want to be part of the Open Heart Club?
We filed up on stage to get our programs and autograph books signed by the baseball players. When I interviewed him for this book, Malm told me a story: he saw a boy at the end of the line, an old patient of his waiting impatiently, and when Malm said, “Johnnie, I’ll sign your book for you,” the kid had refused. No. He wanted Tom Seaver’s autograph. Malm laughed when he told me the story, and I guess it’s a measure of his success. Kids like us didn’t think much about our hearts. We could dream about baseball. The more we took Jim Malm for granted, the greater his achievement.
My brother and I were the kind of kids who got everyone to sign. Daniel has kept his childhood autograph book, and he showed me Malm’s signature. Malm made a little heart under his name, with two x’s in it, like stitches or eyes. It’s hard to tell if the heart is dead or if it’s mended. A lot of the kids in the auditorium had their Mets hats on. A lot of them had brought baseball mitts. Danny Spandau wasn’t there.
In 1961, his family had moved out of Brooklyn to Plainview, Long Island. He had been followed by Malm in the years immediately after his procedure. Five years after surgery, Malm had pronounced Danny cured, and Danny came back to the hospital only once more, in 1971, for a ten-year checkup. He had a good relationship with his surgeon. “He exuded confidence. He exuded it,” Danny told me. “He had a great smile, and he had this clean-cut presence. He was a super professional, but also warm and he had a great bedside manner, and he was genuine. Very, very, very well groomed, very tight skinned, almost waxy face with perfe
ct hair. And he had this beautiful smile. He always smiled. And his hands were always warm and gentle. He was never the one who stuck me. He was never the one who gave me the shots or drew the blood or whatever. And he was always writing in his journal. I remember that because I was always inquisitive. I remember asking why are you writing that, now you’re drawing a picture. What are you doing?” Malm kept an enormous folder on Danny’s case, in which he kept all the detailed plans and pictures that he drew of Danny’s heart.
In their last meeting, teenaged Danny asked a favor of Malm. Could he see an open-heart surgery in process? Malm consented. He didn’t let Danny see a child being operated on, and he didn’t let him see the actual sectioning apart of the chest, but in the middle of an operation on a grown man, Danny was allowed to walk onto the balcony of the operating theater and look down on Malm in his scrubs, and at the open cavity of the patient’s chest, and at the humming heart-lung machine. Danny did not visit a cardiologist again until he was fifty years old, and by that time his heart was in trouble, and Danny was told that he was highly susceptible to sudden cardiac death.
In the auditorium with me that day in 1973 were children with many different kinds of heart defects. We are all lumped together, sometimes, as patients with congenital heart disease, but that is an oversimplification. There is not one single congenital heart disease; rather there are many different kinds of defects, some arising from chromosomal disorders, some from accidents of development in the womb, and some of unknown etiology. There are correlations between certain congenital heart defects and certain genetic conditions: for example, there is a relatively high incidence of tetralogy of Fallot among children with Down syndrome. Because a fetus doesn’t breathe in the womb, because the mother supplies the blood with oxygen, an infant can develop and be born with even the most extreme heart defect.
The most common defects are the simplest: a single hole in the central wall between the chambers of the heart. A tetralogy like mine is the most common complex congenital malformation of the heart, and it is usually described as a collection of four distinct defects. In fact it’s the result of a single problem in fetal development. The ventricular septum, the wall between the two large chambers at the bottom of the heart, emerges in the developing fetus in two parts, strong and muscular at the bottom and thin and membranous at the top. In a normally developing heart, these two pieces connect and merge, but in my mother’s womb, the two parts failed to do so. The top part of my septum, the membranous part called the conal septum, drifted off to the side, blocking my pulmonary valve.
“Tetralogy,” as one cardiologist explained to me, “is the anterior displacement of the conal septum.”
So I had the same defect as Danny Spandau. If, after failing to knit with the muscular bottom part, the membranous part had moved in the other direction, then a different kind of heart defect would have ensued. My aorta and not my pulmonary valve would have been blocked. The doctors would have called this “subaortic stenosis,” and the defect might have caused a narrowing, or coarctation, of my aorta. Jim Malm repaired those too, and kids with subaortic stenosis and coarctation of the aorta were sitting in the stands with me.
So many things can go wrong in a developing heart. Heart valves are made of tiny leaflets, little flaps of flesh that come together and move apart as the heart contracts and expands. The pulmonary valve has two leaflets, and the tricuspid valve has three, and the leaflets open and shut with each heartbeat, allowing the blood to circulate when they open and preventing it from flowing backward when they close. If the leaflets of the pulmonary valves don’t develop separately in the fetus, if they fuse together and form a blockage, this is known as pulmonary atresia. If the tricuspid’s leaflets fuse, it’s tricuspid atresia. A fused valve can affect the fetus like a heart attack. The blood stops running through one chamber of the heart. That chamber fails to develop.
Some authorities claim there are at least eighteen different kinds of congenital heart defects; others will say at least thirty-five. The distinctions between anomalies can be subtle. “Pulmonary atresia with VSD” refers to a defect a lot like mine, a hole in the ventricle (a ventricular septal defect), but with a total blockage in the pulmonary valve. In some cases, the whole right side of the heart can fail to develop, and then the baby comes out with something called hypoplastic right heart syndrome, or HRHS. The kid only has one ventricle. There is no chamber to push the blood to the lungs. In 1973, cardiac surgeons couldn’t save kids with just one ventricle.
There are genetic and environmental factors in the etiology of congenital heart disease. People who have heart defects represent about 1 percent of the population, but in their children the incidence of congenital heart defects rises to 3 percent. Exposure to certain kinds of environmental factors may correspond to higher rates of congenital heart defects. If you look around a movie theater or subway car and try to approximate the number of people there with congenital heart defects (something you’ve probably never done, but something I do frequently), 1 percent can seem a large number, but if you think in terms of the complexity of the human heart and the number of things that can possibly go wrong in the womb, 1 percent seems tiny. It’s amazing that babies’ hearts develop perfectly more than 99 percent of the time.
There were kids in the Open Heart Club who had been born with startlingly dangerous defects—for instance, transposition of the great arteries, a condition in which the pulmonary artery and the aorta are switched, so that the heart is essentially set backward in the circulatory system, with blood from the veins routed out to the body, and blood from the arteries routed to the lungs. No baby born with transposition of the great arteries can survive long without surgery. Without intervention, these babies will die within the first months, weeks, days, or hours after birth.
Through the 1950s, the great Canadian surgeon Dr. William Mustard devoted himself to the cure of children with transposition of the great arteries. His colleagues called him “Wild Bill” Mustard, and I heard one old doctor say of Mustard that his surgical technique was so powerful that he could sew together two farts. In the 1950s, Mustard experimented with new techniques in experimental heart bypass surgery. He invented an organic heart-lung machine (it didn’t work): he cleaned the lungs of freshly killed monkeys so they were translucent and put them in jars, then directed his patients’ blood through the cleaned and suspended monkey lungs so as to perfuse it with oxygen. In 1957, he published the results of twenty-one such surgeries; three of his patients were alive at the time of publication, but they all died soon after.
Beginning in 1952, Mustard attempted surgical repairs of babies with transposition of the great arteries. For roughly a decade, he had a 100 percent mortality rate in these operations. Baby after baby died on the table as he struggled to find a way to reroute their blood. On May 16, 1963, after eleven consecutive years of failure, Mustard performed an operation called an “atrial switch” on an eighteen-month-old girl who had been born with transposition. On this girl, Mustard finally succeeded in redirecting the blood flow at the top of the heart, putting a baffle between her left and right atria so that the venous blood would go to her lungs and the arterial blood would go to her body. This procedure, called the Mustard procedure, was the standard treatment for transposition of the great arteries for the next decade.
Malm performed Mustard surgeries in the mid- and late 1960s, so there must have been kids in the audience with baffles in their hearts, and they must have clapped for the ballplayers, too, and lined up for autographs, but no one knew how long the baffles in the tops of their hearts would last. No one knew how long any of our hearts would last, really.
We were the first large cohort of heart-repair patients. After 1973, the technology advanced, and Malm was able to perform surgery on infants. No tetralogy patient younger than me really remembers being operated on as a small child: their defects were repaired when they were babies.
Patients like me and Danny Spandau seemed to do great after surgery, but our he
arts were jerry-rigged. None of us had valves between our right ventricles and our pulmonary arteries. In a normal heart, a pulmonary valve opens and closes with each heartbeat. We had an open passageway in that place. Our blood flowed up from the heart to the lungs, and then leaked back into our big, overdeveloped right ventricles. In 1963, Malm had used the term “complete repair” for his tetralogy surgeries, but that terminology was soon dropped and altered to “total correction.” We didn’t have the right number of heart valves.
Hang a big bookshelf from your wall using just three anchors when you ought to use four, and it’s likely that in the first decade the bookshelf will hold up just fine. But after a while it might begin to wobble. And it’s a good idea to check on the bookshelf to make sure it’s secure. The installation might require some revisions.
We all looked great when we got to Shea Stadium to continue our celebration, but no one knew when our baffles might deteriorate, or how well our hypertrophied right ventricles would do in the long term, or who would go into a sudden fatal cardiac arrhythmia. Danny Spandau lived for decades without worrying. He told me (smiling) that as a teenager he had lived an “experimental life.” When he finally saw a heart doctor, thirty-five years after his last visit with Malm, Danny was aware of no symptoms—he’d gone to the cardiologist as part of an insurance application—but the backward leak in his heart had enlarged his right ventricle severely. Not too long after that visit, he was told that he was in need of heart surgery. This scared the crap out of him. The great trauma of his life, long repressed, had returned. My heart repair didn’t last as long as Danny’s. By my mid-twenties, my heart was pretty wobbly, and no one was quite sure what to do about me.
The Open Heart Club Page 4
