The Heart Healers (12 page)
Authors: James Forrester
It was true. In October during his run of bad surgical outcomes, Lillehei’s anesthesiologist had allowed air to enter the pump tubing as the equipment was being set up. The curse that had killed Charles Bailey’s young lady when air entered her coronary arteries had resurfaced in Lillehei’s operating room. Again the simple error was devastating. In Lillehei’s case the air had gone to the brain of donor Mildred Jones, the mother of his eight-year-old VSD patient. Lillehei immediately canceled the surgery, but it was too late. Mildred Jones was permanently and severely brain damaged, unable to care for herself or her four small children.
In that sudden reversal of fortune Watson emerged the ethical victor. As world-famous pediatric cardiologist Dr. Helen Taussig sniped at Lillehei’s reported success with the tetralogy case, “Too bad, now he’ll continue.” The Mildred Jones story finalized the decision for most cardiologists and cardiac surgeons. The problem of adding oxygen to blood could not be circumvented by hypothermia or cross-circulation.
Walt Lillehei had proven that the heartbeat could be stopped, that the circulation could be maintained, and that successful surgery could be performed on the arrested heart. In principle no form of heart disease, including CAD, was any longer beyond the surgeon’s reach. But as Lillehei and the new breed of cardiac surgeons surmounted these obstacles, they had also proved that cross-circulation carried a terrifying, inescapable, unacceptable ethical risk. To save a life, a healthy volunteer was put at risk of permanent disability or even death. If monkey lungs did not work, hypothermia was inadequate, and cross-circulation was ethically prohibitive, what was left? Was human ingenuity exhausted? Or was there still another way?
There is no way that the heart-lung machine could have been devised and developed other than through studies on living creatures.
—PROFESSOR SIR NORMAN BROWSE, PRESIDENT OF THE ROYAL COLLEGE OF SURGEONS OF ENGLAND
AT PHILADELPHIA’S JEFFERSON
Medical College another war surgeon pursued a quite different dream. Dr. John H. “Jack” Gibbon set out to create a heart-lung machine. Gibbon was the polar opposite of his contemporary fellow Philadelphian Charles Bailey. Son of a prominent Philadelphia doctor, Gibbon was a fifth-generation physician, born to wealth and entitlement. He had an in-town home and a farm home. A Princeton-educated upper-class blue blood, he was both erudite and handsome. To round out his genteel Renaissance Man image, Gibbon also wrote poetry and painted. He and his wife entertained Philadelphia’s social elite at large parties with charades, ballet, and music, the privileged life of Gatsby’s East Egg. Yet as elegant as he was, John Gibbon was neither aloof nor pretentious. He was down-to-earth, sincerely interested in listening to others and in their ideas. A rather slow and deliberate surgeon, he was an exceptional teacher. His unique breadth of interests, achievements, warm personality, and modesty often led to leadership being thrust upon him.
Jack Gibbon’s philosophy of life was quite different from the other surgeons we have met. He enjoyed his surgical career but was not consumed by it. Personally secure, his research was more driven by its pure intellectual challenge than by the competitive desire for peer recognition. I believe this constellation of personal and professional attributes explains what was to be the strangest, most incomprehensible decision I have seen in my years in academic medicine.
In his graduate years, Gibbon was more inclined to creative pursuits than to science. He had to be dissuaded by his physician father from dropping out of medical school in his sophomore year to become a writer. Both smart and connected to the establishment, Gibbon eventually matriculated to surgical training at Massachusetts General Hospital. In those years, every doctor and nurse knew the letters MGH. Those three letters stood for one thing only: the nation’s premier hospital. If you worked there you were, by association alone, a superior physician. And if you contemplated leaving the fold, you were reminded to choose your new location well, because you only come from Harvard once.
During his years as an MGH surgical resident, Gibbon was called to assist on the removal of a large blood clot from the pulmonary artery, the vessel that delivers blood from the right ventricle to the lungs, in a desperate effort to save a healthy young woman who had entered the hospital for routine gall bladder surgery. The surgical team extracted the clot, but it was too late. She never regained consciousness. Gibbon was deeply affected by the premature passing of an otherwise healthy young lady of his age: “If only we could remove the blood from her body by bypassing her lungs, and oxygenate it, then return it to her heart, we could almost certainly save her life.” Jack Gibbon was describing the genesis of the heart-lung machine. The idea ricocheted within Gibbon’s psyche until it became an obsession. Despite universal discouragement from colleagues who bluntly told him that he was wasting a promising career, Gibbon blithely ignored them. Failure was postposed success; persistence was the habit of accomplishment. Like an artist finding joy in a single brushstroke, he was satisfied with the smallest advance. His research would be, as with his surgical technique, tranquil, slow, and deliberate.
Gibbon faced three daunting engineering obstacles that Lillehei had circumvented with cross-circulation. First he needed a method to oxygenate blood. Second, blood outside the body tends to clot, so every surface of his machine, including delivery tubing and the oxygenation device, had to be resistant to blood clot formation. Third, oxygen-carrying red blood cells are extraordinarily fragile. Broken red cells carry no oxygen, and when many cells are disrupted, their fragments obstruct the kidneys causing organ failure. So the entire oxygenation and pumping process had to be exceptionally gentle. In Boston, Jack Gibbon spent ten years tinkering and jiggering one prototype after another without success, but not without reward. One of his coworkers at Harvard, Mary Hopkinson, believed in the unruffled surgeon’s dream. As Hitler rampaged through Europe before the war, Mary and John married, then returned to join the staff at Philadelphia’s University of Pennsylvania.
As the United States entered World War II, Gibbon was already into his second decade of trying to create a functioning device when he and Mary finally achieved their first modest success. They developed a prototype heart-lung bypass machine, which kept a small laboratory cat alive for twenty-six minutes. But with their first success came terrible disappointment. Japanese bombs terminated their research. John was called to serve four long years as chief of surgical services at the 364th Station Hospital in New Caledonia, a South Pacific island east of Australia. The idea of a heart-lung machine became as remote as the narrow strip of land he now inhabited. Could his lifetime of research survive?
* * *
SOME YEARS LATER
on a June afternoon in Los Angeles, I sat sorting through a stack of phone messages while watching rush hour traffic from my fifth floor office window. One call had come from Sam Bachner, one of my favorite supporters of our cardiovascular research program. Sam charged enthusiastically though life with the rare and wonderful capacity to be genuinely interested in each person he met, and to draw them into his circle. Sam’s perspective on life was captured by his company name, La Mancha, and by his office cluttered with Don Quixote sculptures.
“Jim, I want to get your opinion about a heart problem in a child,” Sam said. “My wife and I have a housekeeper from El Salvador who has lived with us for a few years. She sends her money back home to help pay for her kids, who live with her mother in El Salvador. Last week she got a devastating letter about her twelve-year-old daughter, Maria. Here’s what I learned. As a little girl she tired easily, and had episodes where she would start gasping for breath and her lips would turn dark blue. Whenever she had an episode, Maria would squat to relieve her breathlessness. The doctor said Maria was a ‘blue baby’ and there was nothing he could do for her. Recently her grandmother took her to a doctor because she had begun passing out during these spells. He said that Maria has a heart murmur and that she would probably die in the next year, but that she could live if she had surgery. He said they don’t have that kind of surgery yet in El Salvador. So what I am calling to ask is what do you think is wrong with her? And would surgery really save her life?”
I winced, feeling a parent’s dismay at knowing her child is dying, a cure exists, but her child is denied. But the doctor in El Salvador was right. Although the heart-lung machine has made reconstructive, curative surgery for all types of congenital heart disease feasible in developed countries, in the developing world congenital heart disease still claims many children.
“I need a lot more information, Sam. If she is a blue baby, the odds are she has a congenital heart condition called tetralogy of Fallot,” I said. “But even if we knew her diagnosis we need two more critical pieces of information. Kids with tet often have other cardiac and noncardiac congenital abnormalities. So if you want my opinion, your first step is to get a copy of her doctor’s physical examination, and to find out if she has had an echocardiogram back in El Salvador.”
A few weeks later, Sam sent over a few crude Polaroid snapshots of echocardiographic images, not the moving video images we typically evaluate. Maria’s physical exam apparently had got lost in translation. Even so, I saw she had all four components of the tetralogy. First she had severe stenosis of the pulmonary valve, which separates the right ventricle and the pulmonary artery. Normally, all blood returning from the body is pumped by the right ventricle across this valve on its way to reoxygenation in the lungs. The marked increase in force required to pump blood across a severely narrowed pulmonary valve leads to the second defect of tetralogy, an increase in the mass of the right ventricle (called hypertrophy). I saw that Maria also had the third defect, ventricular septal defect (VSD). Because of this open hole between the two ventricles, some of the deoxygenated blood returning to the right ventricle was being pumped into the left ventricle. The tetralogy was completed by a distorted connection of the aorta to the left ventricle. Maria turned blue when the deoxygenated blue blood from the right side of her heart was pumped across her VSD to mix with the left ventricle’s oxygenated red blood. When she squatted, resistance to blood flow into the aorta increased, so that less blue blood crossed the VSD. Remarkably, before they can talk and even without being told, virtually every child with tetralogy discovers squatting.
I called Sam to deliver the bad news. “Sam, I think it’s best that you are honest with Maria’s mother. We haven’t seen a child of this age with untreated tet in years. Here in the United States about half of tets are now detected before they are born, by fetal echocardiography. The remainder are detected in the first two years of life, and they all get surgery right away. So tet in a person of this age nowadays is really confined to children like this little girl, born to poor parents in a poor country.”
“So what happens in those countries? What’s the treatment?” Sam asked.
Musing how Lillehei’s breakthrough tetralogy case using cross-circulation forty years earlier was still irrelevant to this little Hispanic child, I replied, “For these kids the prognosis is dismal. It’s the same as it was here in the United States in the years before cardiac surgery. About a quarter of infants die in the first year. Most of the rest die before their teens. I looked up the numbers. About forty percent die by age three, and seventy percent by age ten. By all odds, Maria should already be dead.”
“But she isn’t.”
“That does not mean she’s escaped,” I said. “Untreated, only a tiny fraction, maybe five percent, of tets survive to middle age. Most die during an acute episode of lack of oxygen, gasping for air like a fish out of water. Or just as bad, their body recognizes that the blood isn’t carrying enough oxygen, and so their bone marrow goes into overdrive, producing massively more red cells to carry oxygen. But more cells make the blood more prone to clot. And so they have a stroke. Some even get a brain abscess.
“It is pretty grim,” I added unnecessarily, feeling the sterile insensitivity of my statistical recitation.
“So are you telling me it is too late for her, that the doctors in El Salvador are right that she is going to die soon?”
“I’m afraid so,” I said. “Fainting is an ominous sign.”
“But one doctor also said she has a surgically correctable condition, right? He seemed to be saying that if she did have an operation, she’d have a good chance at a normal life, and yet…” Sam said, his voice trailing off.
“That’s true too, Sam, it’s a personal tragedy played out every day in the third world,” I replied. “You are looking at the universal curse of being born poor in a poor country. Life just isn’t fair,” I said, adding a sop of conventional wisdom to deflect our discussion away from the tragedy of one hopeless little girl.
“So now we know our problem,” Sam replied as he said good-bye.
I could hardly miss that. Without any input from me, Sam’s maid’s daughter’s problem had become “our” problem, and in that fleeting instant, I had the sinking feeling that I was about to confront an entirely new problem. I was.
The next day, Sam called again.
“I don’t suppose there’s any possibility of doing this, but if you don’t ask you never know, right? If I pay to fly Maria up here, and put her up in my house before and after surgery, could you get her taken care of?” he asked.
I reflexively tapped my fist against my chin several times before I answered. When you are a chief, this is the kind of request you hate: the impossible problem over which you have no control. I tried diplomacy.
“Sam, I am sorry to tell you that she has no possibility of being done here, or anywhere else in the U.S. that I know of. This is very complex surgery that consumes tens of thousands of dollars in cost just during hospitalization. That’s if there are no complications. If the patient needs more prolonged hospitalization, the cost could go into six figures. Our administrators face these kinds of requests all the time. No matter how sad and compelling the story, the medical center just cannot become a charitable institution for needy kids from other countries, when there are a million heartbreaking stories out there.”