Not long after the catheter exam, the hospital called the Spandaus with bad news. Dr. Himmelstein had taken sick. He had brain cancer. He would not be able to operate. The surgery would have to be postponed. The family had to wait another year, with their fainting blue baby at death’s door. Columbia was not able to find a replacement heart surgeon. Danny’s case was taken over by Himmelstein’s young chief resident, Dr. James Malm.
In 1960, six-year-old Danny Spandau was listless and weighed just twenty-seven pounds. When he rested, he tended to draw his legs up against his chest, a squat typical of children with tetralogy of Fallot; it relieves some of the pressure on their chest. The Spandaus met Dr. Malm up in Babies Hospital. He was tall and graceful, precise in his speech, elegant, and well-mannered. His hair was parted neatly. He had high cheekbones. His features were small, feminine, almost Asiatic, and his skin strangely flawless. In blue and red pens, Malm drew careful pictures of Danny’s heart and described to the Spandaus the surgery he would perform. He showed the four chambers of the heart, the big ventricles below and the small atria above.
In a normal heart, the blood flowed in through the vena cava, down into the small chamber on the right side, the right atrium. From the right atrium, it descended into the right ventricle, the big powerful pumping chamber that sent the blood up through the pulmonary artery into the lungs. From the lungs, the blood came back into the left atrium, then down into the big left ventricle, which shot the blood out through the aorta to fill the body with life. Danny’s heart, Malm explained, had four defects, but Malm focused on two of them. There was a big hole in the ventricular septum, the wall between the two big beating chambers of the heart, so the oxygenated and unoxygenated blood comingled and went out the aorta together. There was also a narrowing, a stenosis, in the valve between the left ventricle and the pulmonary artery—that stenosis limited the amount of blood that could get to Danny’s lungs. Malm was going to patch the hole in the middle of the heart, and he was going to widen the gap between the pulmonary artery and the left ventricle.
He called this operation a “complete repair.” What the Spandaus did not know in that first meeting was that Jim Malm had never in his life performed open-heart surgery.
3.
HEART DEFECTS ARE the most common of all life-threatening birth defects, affecting 1 in every 110 babies. Roughly one in every three kids born with a heart defect requires surgery, which means (more or less) that there’s now a kid with a surgically repaired heart at every school in the affluent world. Pediatric cardiologists and pediatric heart surgeons are good at what they do: these days, in the United States, over 85 percent of kids with deformed hearts survive surgery into adulthood. Still, congenital heart defects remain, of all birth defects, the number one cause of infant death.
Congenital heart disease is among the most common chronic diseases in the United States. People with repaired hearts are living long lives—healthy, give or take, but in need of constant monitoring and attention. You probably have a neighbor, a relative, a friend, or a coworker with a repaired heart defect. Most of us look entirely ordinary, even when we’re in deep trouble. We are invisible. Approximately nine in ten Americans with moderate to severe heart defects do not get the lifelong care and monitoring that they need.
Tetralogy of Fallot is the most common complex congenital heart defect. Fifteen years before Jim Malm, all the kids with tetralogy died—most died in infancy, but the healthiest lived crippled lives into their teens. Fifteen years after his breakthrough surgeries, almost all the tetralogy kids in the United States survived surgery, and many of us have thrived—snowboarder Shaun White, a three-time Olympic gold medalist, was born in 1986 with a tetralogy of Fallot.
In 1960, when Danny was rolled into surgery, a good portion of tetralogy patients didn’t make it through the operation. Mortality rates varied from hospital to hospital, but they were so bad that Helen Taussig—the most prominent pediatric cardiologist of all—argued that corrective surgery on “tet” patients wasn’t worth attempting. At Columbia under Himmelstein, more than half of the patients died.
In 1960, blood could not be stored safely long term. The donors had to be on hand for surgery, so Danny was accompanied by his older brother Stevie, one of the two sons from Ludwig’s first marriage—as a child during World War II, Stevie had hidden from Nazis. Now he was a US marine. He came to the hospital with twelve of his Marine Corps buddies, all of them ready to donate blood.
In those days at Columbia Presbyterian, there were no operating rooms specifically designated for cardiac cases. “Every operating room was occupied every day,” Malm remembered. “Gall bladders, colon cancer, breast surgery, thyroids.” He had to fight for operating room space. “There was no room at the inn. If I increased my volume, then one of the other surgeons couldn’t do his thyroid, couldn’t do his breast.”
Danny was rolled in. The anesthesiologist put a mask over the boy’s face and told him to count down from ten. Something extraordinary happened as he passed out. “I’m a scientific guy,” Danny told me. “I’m an analytic chemist by training. So I’ve explained it not as an out-of-body experience, but maybe it was. Who knows? It may have been the reflection in an overhead lamp. All of a sudden I wake up and see the reflections of all the doctors around the table. I see the top of the doctors and me lying on the table. It was so clear. It was literally like being with my back on the ceiling of the OR looking down at the operation. I saw the doctors in all their scrubs. And I saw myself lying on the table, being worked on.” He felt completely removed from his body, a spirit looking down at the surgery. “And that’s a memory I’ve had essentially all my life.”
Carefully, Malm sectioned the little boy’s chest. The heart-lung machine was of the new disc oxygenator type, a device about the size of a big fat salami, stored in something the shape of an industrial-sized sewing machine. It sat on a rolling cabinet, and from it extended long clear tubes. The machine was primed with a mixture of blood and blood thinners. One tube’s canula was inserted into Danny’s aorta, the other into his vena cava above his right atrium. Danny’s blood came into the machine and coursed over a series of discs. Each bloody disc was perfused with oxygen, and the oxygen-rich blood was sent back into Danny’s circulatory system. The heart-lung machine regulated the speed of blood flow, and by slowing down the blood, Malm lowered Danny’s body temperature to eighty-two degrees. The induced hypothermia slowed Danny’s metabolism and gave him some protection from damage that might come from oxygen deprivation during surgery. The heart was drained entirely.
Malm cut right through the pericardium, the rough sack of protective tissue that contains the heart. Now he had to attack the heart muscle itself. Other surgeons had favored a “median,” or horizontal, slice across the right ventricle, but instead of going side to side, Malm made a “vertical right ventriculotomy incision.” He had devoted himself to study of the heart’s electrophysiology—the system of electrical currents that control the heartbeat—and this seemed less likely to interfere with the circuitry of the heartbeat. It also gave Malm a better view of the hole in the septum.
The interior of the right ventricle wall is not smooth but, in doctors’ terms, trabeculated, which means it’s made of a crisscross web of muscle fibers. Even with today’s powerful imaging technology, it can be difficult to locate the boundaries of each ventricular septal defect. Malm found Danny’s by feel. He took a Teflon patch and stopped the gap in the middle of the heart, between the muscular wall at the bottom of the ventricles and the soft tissue at the top. He used mattress sutures to attach the patch, the kind of big, railroad-track loops that are inelegant but extremely secure. Once Malm was sure there was no leak at all, he attacked the stenosis of the pulmonary valve, the blockage on the right side of Danny’s heart.
He made a generous hole between the right ventricle and the pulmonary artery, slicing any tissue that was in the way and putting in an outflow patch to smooth the passage of blood toward the lungs. He wanted to get as
much blood moving as he could, and he wanted the space wide open. Where a normal heart would have a pulmonary valve, impeding blood flow backward from the lungs, Danny would have an open passageway.
Malm shut the hole in the ventricular septum. He relieved the pulmonary stenosis. He sewed Danny’s heart shut, doing as little damage to the muscle as he could, and he stitched up the pericardium. The boy was taken off the heart-lung machine. Blood filled his heart and the chambers began to beat. The boy’s temperature returned to normal, and his blood pressure did too. The whole operation had taken about an hour and a half. The crucial thing, according to Malm, was to get the blood flow as near normal as possible. “No leaks!” he barked at me over the phone when I asked him his secret to keeping a child alive.
There was no special recovery room for cardiac patients in 1960. Danny recalls a big room with lots of other kids. He woke up under a clear plastic oxygen tent. For the first two days after surgery, he lived in a high-humidity bubble. His parents hovered close by but could not touch the top of his body. IV tubes replaced his lost blood and body fluids and gave him the necessary sugars and electrolytes. Nurses came by with shots of penicillin and streptomycin and delivered the shots to the muscle of Danny’s legs.
“I pinked up right after surgery,” Danny told me.
For a time, after the oxygen tent was removed, Danny was confined to a wheelchair. “I remember my hands were so dirty from wheel-chairing, and it was so disgusting that I never sucked my thumb again.” After six days, he was allowed to walk. When Danny came home from the hospital, everything had changed. He could climb the stairs to his apartment. He began to put on weight and to grow. The darkness was lifted from the Spandaus’ lives. When Danny was strong enough, his brother Stevie took him to a marine ball to celebrate his resuscitation, and Danny was shown off in front of all the young men who’d given their blood to him.
In the late 1950s, a new era had begun, the era of open-heart surgery, and in the early 1960s a new kind of person was invented, a person like Danny Spandau, a person like me, who would live a whole lifetime with a surgically repaired heart.
4.
I WAS BORN WITH tetralogy of Fallot, the same defect as Danny Spandau, but I had the good fortune to be born a decade later, in 1966. My birth defect went unnoticed in the obstetrics ward. My parents thought I was perfectly healthy when they brought me home. My pediatrician, too, missed the squishy sound in my heart on my first visit. In 1966, there weren’t the same protocols for checking for heart defects that there are today—no sonograms, no fetal echocardiograms, just diagnosis with a stethoscope. The doctor caught it on my second visit, when I was already a month old, and he sent my parents to a pediatric cardiologist, Dr. Lucy Swift, at St. Luke’s Hospital on Manhattan’s Upper West Side.
Women doctors were uncommon in 1966, but not in pediatric cardiology. In the study of deformed babies’ hearts, women doctors were the rule. Helen Taussig, the Johns Hopkins doctor who had conceived of the Blalock shunt procedure in 1944, had founded the discipline of pediatric cardiology. She was still alive and at work in 1966, and her students, the so-called Knights of Taussig, dominated the field. Women, most of them Taussig’s heirs, ran most of the pediatric cardiology programs in the New York region: at Yale, Ruth Whittemore; at New York University, Janet Baldwin; at Columbia, Sylvia P. Griffiths; and at Cornell, Mary Allen Engle.
My mother remembers Dr. Swift as a small, strange, otherworldly, birdlike creature, but also as a woman of considerable will and professional devotion. Dr. Swift examined my fingers and toes. She listened to my heart with a stethoscope and then pressed her fingers to my abdomen and shut her eyes. Because she had been deaf, Helen Taussig had developed techniques for listening to infant hearts with her fingertips, and those techniques had been passed down to some of her heirs. After palpating my abdomen, Dr. Swift had me sent for X-rays.
My parents received the diagnosis, a heart defect, a tetralogy of Fallot, something neither of them had ever heard of. Untreated, I would probably not live past the age of fourteen. But yes, Dr. Swift explained, it could be cured. She gave my parents the name of Dr. James Malm at Babies Hospital up at Columbia Presbyterian, where Dr. Swift had done her residency. Jim Malm was the one man in the world most qualified to cure me.
I recently met a woman who was born in Chicago the same year I was, with the same condition I have, and when her parents first took her to see a doctor, the doctor advised them to give up. The baby would not live. But Lucy Swift knew exactly where to send us. My parents traveled up to 168th Street, just five subway stops from their apartment. This has been the story of my life: an expert clinician, right there every single time I have needed it.
My parents sat in a large, stuffy New York City waiting room, baby me fussing on my mother’s lap. Malm met them in his office. In my recent meetings with Malm, I’ve found him both reserved and very funny. Even in his nineties, he’s quick and intimidating and capable of dominating a room. I’ve interviewed his colleagues and his patients. Everyone I spoke to seemed to view Malm with affectionate awe. Everyone loved him—except my mother. Jim Malm scared her half to death.
“Something about his eyes,” she told me.
They were blue, extraordinarily, clearly, high-sky blue. My mom started seeing a therapist. She told the therapist how the surgeon spooked her.
The therapist nodded. “They give up something to be able to do that.”
Malm wanted to take a scalpel to the perfect skin of her baby’s chest. With a saw, he wanted to cut through her little boy’s breastbone. He’d take a stainless steel spreader and pry open her son’s ribcage. Wearing sterile rubber gloves, he’d press his fingers against the muscle of his heart, even as it was beating. He would slice the big vessels around the heart and shove plastic tubes into the holes. With those tubes, he would drain the heart of blood and then connect the child to a newfangled machine with a humming motor and a plug that went into the electrical outlet in the operating room wall, and while the lungs deflated, this weird invention of metal discs and rubber tubes and plastic casing would do the work of the heart, while Malm took his scalpel and cut open the heart’s chambers. He’d take a piece of Teflon and shove it in there. He would sew Teflon into the heart with a needle and thread. He would dig into the heart, into the pulmonary valve, and he’d cut a piece right out, her baby’s heart sectioned with a knife and a piece of it thrown into the garbage.
Malm was a superb clinician. Colleagues have noted that when things got catastrophic in the operating room, he got more focused and calm. He loved his work—that’s what he told me when I talked to him, loved every day in the operating room. There was no place he would rather be than masked and scrubbed, putting his clamp and scalpel into the hearts of young children. “I miss it still,” he told me. “The old dog wants to be in the operating room.” My mom must have recognized this in him—his uncanny cool, his fascination and eagerness—and these qualities unnerved her. She was required to do something out of folktale: to offer up her child to this handsome stranger, who was going to stick a knife into her son’s heart.
Everyone reading this book knows someone who’s had heart surgery, who’s gone under the knife and come out fine. But in 1966, heart surgery was new. When Jim Malm did his residency, there was no such thing as open-heart surgery. In the late 1950s heart bypass surgery, which is to say surgery where the patient’s heart is taken out of the circulatory system, was performed only at a few highly specialized hospitals, and all the patients were children. In 1966, my parents had never met anyone who’d had heart surgery or whose baby’s heart had been repaired. This was a thing outside the experience of everyone they knew.
My father was a doctor, a psychiatrist, but where he had been in medical school in Buffalo, New York, there had been no open-heart surgery. Someone tried to monkey with it, he told me, but the results had been terrible. He had grown up in a lower-middle-class Jewish enclave in Buffalo, the second child of an insurance salesman, the brightest boy in his
school. My mother was the daughter of two Jews from Mielecz, Poland, who spoke Yiddish in their one-bedroom apartment in Queens. She was a Yale PhD in nineteenth-century British literature and an English professor at City College. Her dissertation had been on Lord Byron.
My parents were brilliant, educated people who through diligence and education had broken out of the cloistered worlds of their beginnings. They were scientific, assimilated, secular Jews who saw their parents’ lives as rife with primitive mumbo jumbo. On an early date, my dad won my mom over by taking her out on Yom Kippur, the Jewish day of fasting and atonement, and ordering bacon and eggs. Their friends were Upper West Side artists and intellectuals. These were people who could talk about anything: Keats, Vietnam, orgasms, Robert Rauschenberg, Thelonious Monk, marijuana, or Freud. But this, tetralogy of Fallot, was beyond meaning. Even for an English professor, even for an MD, it verged on gibberish. “Tetralogy” is an English word, but the kind that no one ever speaks or writes, and if used at all, it’s for artworks and not medical conditions. (William Safire wrote the “On Language” column in the New York Times when I was growing up, and I remember when he discovered the word in 2002: “Now I know,” he wrote in a column when he was eighty-three years old, “three volumes is a trilogy, four is a tetralogy.”) But “Fallot”? “Who was Fallot?” my mother asked me last summer. This was like “Rumpelstiltskin,” a heap of syllables indicating nothing. And there was Malm sitting at his desk, with his neatly combed hair, his fine features, and his carefully clipped speech.
The Open Heart Club Page 2
