by Rob Dunn
The idea of improving our health by planting trees is simple and primitive, particularly when compared to the sparkling innovation of artificial hearts or transplants. It is, at its root, a public-health measure, and public health is rarely sexy, even though it contributes far more to people’s health and well-being than medicine does. Public health brings us the power of sanitation, vaccination, and the monitoring of the spread of pathogens to save lives, billions of lives. Public health, as a field, also invites us to do something else, namely, to consider how we might improve the quality of life of the average person in a region, in a country, or even on Earth.
When treating an individual patient, a doctor (at least in the United States) is expected to do whatever can be done to prolong life. Not necessarily good life, or quality life, or one life over another. Just life. But from the perspective of society at large, from the perspective of public health, the goal is necessarily different. The goal is instead to increase the longevity or quality of the average life. Naturally, we tend to care most about our own health, the health of our family members, and the health of the people where we live. But let’s imagine for a second that instead of extending our own lives, the goal was to extend the average life. What would we do?
If we were considering the entire world, the first step would not be dealing with heart disease. It would be treating or preventing malaria, HIV, and other pathogen-borne diseases so that the average child could live long enough to worry about heart disease. It would be ensuring that everyone has clean water so that deaths due to diarrhea could be prevented. For much of the world’s people, heart disease remains a disease of the wealthy that affects people in relatively old age. May your children live long enough to worry about heart disease.
But that is globally. What about within the United States or within a particular city? Right now, part of America has experienced the benefits of the past fifty years of heart research, and life expectancy for them has increased. But many of the country’s citizens haven’t seen these benefits. For large segments of the American public, life expectancies are what they were in 1950, as are rates of heart-disease death. In the United States, genes can be predictive of heart disease, but better predictors, all other things being equal, are money and education. The poor and poorly educated have essentially missed out on the benefits of the past fifty years of medical science when it comes to cardiovascular problems. Individuals of lower socioeconomic status are at a 50 percent increased risk of developing heart disease than those of higher socioeconomic status. This is true even after risk factors such as diet, obesity, and smoking are taken into account, and it’s due to disparities in health care and access.
This discrepancy appears to be increasing rather than decreasing. If we wanted to make major advances in the life expectancies of individuals in the United States, we could invent new technologies that eke a few more years out of the human body. Or we could plant policies, trees, and equitably distributed health care. The key is not necessarily to plant it with greater innovation, just with greater equity, so that everyone might live a longer and greater life. If you are already among those fortunate enough to have good health care, this won’t improve your fate, but it will have a large effect on the average person’s fate, the average human condition, the condition of those many who are not so fortunate. Today, those who are less fortunate with regard to heart care are the poor, whether the poor in wealthy countries or the poor in the impoverished nations of the world. But until relatively recently, the category of those who were not so fortunate, who didn’t experience the benefits of the past decades of progress, included children, children born with congenital deformities and abandoned as beyond repair, children with broken hearts.
14
The Book of Broken Hearts
On November 29, 1944, Eileen Saxon, a fifteen-month-old girl, was on her back on a steel table at the Johns Hopkins Hospital. Inside her small body, her heart was doing something unusual, though just what, no one could say for sure. Her skin was blue. She was alive. Those who stood over her, including a chief surgeon and a cardiologist, thought she had blue-baby syndrome, in which the blood pushed through the body by the heart is low in oxygen. It is a common congenital disease, often caused by a suite of deformities collectively termed the tetralogy of Fallot, named for French physician Étienne-Louis Arthur Fallot. These four (“tetra”) deformities are ancient, predating the origins of humans or even primates. They have been found to occur in every mammal species that has been studied well enough for us to detect cardiac abnormalities. The malformation has occurred billions of times, but prior to 1944, not once among those instances had it been cured. Each time, the baby—be it gorilla, squirrel, or little girl—eventually died, literally suffocated. Now, here was another blue child, but her situation was different. Gathered around her in their ghostly gowns and masks were a dozen or so people, at least three of whom believed she might be saved.
In 1784, William Hunter at St. George’s Medical School in London first described the causes of the blue-baby syndrome accurately and in detail: “(1) the passage from the right ventricle into the pulmonary artery, which should have admitted a finger, was not so wide as a goose quill; and (2) there was a hole in the partition of the two ventricles [a ventricular septal defect], large enough to pass the thumb from one to the other. (3) The greatest part of the blood in the right ventricle was driven with that of the left ventricle into the aorta, or great artery, and so lost all the advantage which it ought to have had from breathing.” Also, (4) the right ventricle becomes hypertrophied, enlarged, because it must work harder to get blood through the narrowed pulmonary artery and into the lungs. These were the syndrome’s four problems, which always seemed to occur together and which led to there being less oxygen in the body than was needed. Many cases of this disorder occurred in humans, about 1 in 3,600 births around the world, but to Eileen’s parents, there was only one case that mattered.
The chief surgeon was Dr. Alfred Blalock, a surgeon in the children’s cardiac clinic at Johns Hopkins. Blalock had gone to medical school at Vanderbilt (as the school’s first student); he would do the cutting and sewing. Beside him, standing on a stool and whispering in his ear, was Vivien T. Thomas. Thomas was the one with the surgical skills, a genius with his hands. Thomas had performed all but one of the practice surgeries necessary to prepare to work on Eileen. In dogs, he created and then repaired problems like those seen in the tetralogy of Fallot. He could predictably save dogs. Based on his skills alone, Thomas should have been the one to do the surgery. But Thomas had grown up poor and black in a time where both made access to good high schools, much less colleges, difficult. He started college but dropped out because of a lack of funds. His way into surgery would be through a back door. Blalock had hired him as a technician, but he proved to be a genius at surgery, even if not legally a surgeon.1 He would guide Blalock, his collaborator, much as if, some would later say, Blalock were a marionette and Thomas were the puppeteer.
These were the two people closest to the patient, but behind them was Dr. Helen B. Taussig. Taussig had invented the surgery that was about to be attempted, though she was not a surgeon either. Her gender had prevented her from access to surgical training. This was an unusual team. Even Blalock, its most orthodox member, was a bit marginal. He was shadowed by self-doubt and thought himself a failure; he had had trouble getting a surgical residency. Vanderbilt Hospital was the only place that would have him. He was among that hospital’s very first residents.
Blalock told Eileen’s parents about the dangers of the surgery the doctors were about to embark on. Her parents knew enough to realize this was the only choice. No child with blue-baby syndrome had ever survived past the age of four. Of course, there was a chance that she did not have blue-baby syndrome, but no one could know until they saw her heart. Blalock did not give the parents the complex biographies of his team. Nor did he mention his own hesitation—his persistent and gnawing fear. For the first time in the 130-million-year
history of tiny broken mammalian hearts, this baby, their baby, might recover from the tetralogy of Fallot. If the surgery worked, Eileen’s skin would go from blue to pink. Her fingers would flush with life, and, as they did, Eileen would get a second chance.
The buildup to this moment began with Dr. Maude Abbott, Maudie to her friends. Abbott was born in St. Andrews, Quebec, where adversity was her native substrate. Her father abandoned the family. Her mother died of tuberculosis. A grandmother raised her and her sister. Abbott was forged out of hardship.
Abbott entered McGill University in 1886 and graduated valedictorian of her class, but McGill did not yet accept women to its medical school, and so she had to find somewhere else to go. This led her to Bishop’s University in Lennoxville, Quebec, where she was the only female medical student in her class; although she was admitted, she was never quite accepted socially. Because of the persistent barriers to women in medicine, her drive, ferocious as it might be, could not push her straight ahead, and so she would go sideways when she needed to in order to keep moving. Once she obtained her medical degree (again with the highest honors), Abbott opened a clinic focused on women and children. But, in her own telling, she did not have the right kind of empathy to deal with children and suffering. She was better at autopsies; the dead demanded only her persistence. She gained some renown when she identified hemochromatosis (a disease due to too much iron in the blood) in one of the bodies she studied and wrote a paper on it. On the basis of this paper, George Adami, the chair of pathology at McGill, recommended her for a position as an assistant curator at the Medical Museum of McGill. She was offered the job in 1898, and she took it. There, she began the work that would lay the necessary foundation for Blalock, Taussig, and Thomas.
Maude Abbott pretending to read. (Courtesy of Harris & Ewing/McGill University Archives, PR023284)
The museum charged Abbott with organizing the collection of body parts—organs and other pieces—left in disarray by her predecessor, William Osler. Osler was a brilliant pathologist, so dedicated to understanding the body’s failings that he would drive a hundred miles to see a fresh cadaver.2 He didn’t care for medicine, for curing the living. His interest and his genius were in figuring out the cause of death, case by case, as in any modern autopsy. From each of the more than one thousand autopsies Osler performed in Montreal, there were parts arrayed in over a thousand jars. It was a liquid-y rogues’ gallery of how human bodies go wrong—valuable, but not yet valued. Abbott considered these parts and sought to organize them the way a taxonomist might group birds, according to their similarities and differences, into basic kinds. Based on these parts, Abbott compiled the most comprehensive list in the world of the congenital defects of the heart, including the one leading to blue-baby syndrome, the tetralogy of Fallot. It became a gruesome traveling exhibit of broken hearts. It was first shown at the Graduate Fortnight in Cardiology at the New York Medical Society in 1931 and then again and again. It ultimately became a standard feature of medical-school classes at McGill. Then, in 1936, the same year she officially retired, she published the book that helped to frame the understanding of what can go wrong in a heart, The Atlas of Congenital Heart Disease, which is still in print today, by the American Heart Association. The atlas showed the diversity of the human heart’s failings as recorded in one thousand congenital malformations, all of which, Abbott imagined, or at least hoped, might someday be remedied.3 Abbott wanted to build on the book’s success and write another book, this one a textbook on the heart. She received a grant from the Carnegie Foundation in 1940 for the project, but at the age of seventy-one, she died of a stroke before she could complete the work.
Many books have compiled the problems of the human body. Most have all the flair and seriousness of sideshow oddities. “Here is the woman with a hole in her heart. Come see the man with an extra ventricle!” Abbott’s book was different. It spoke authoritatively and comprehensively to what congenital deformities looked like in autopsies. Yet it still said very little of their signs and symptoms in the living, much less how to treat them. Few followed up on it other than to add new instances of problems of the heart, new jars on the shelf. Then came Dr. Helen Brooke Taussig.
Like Abbott, Taussig seemed to find an unusual level of adversity en route to her successes. Her mother, like Abbott’s, died when she was young. She struggled through high school with dyslexia. Then her hearing, the very sense she would ultimately use most to try to diagnose problems, started to mysteriously disappear when she was in her early thirties. Add to this her gender. Because she was a woman, she was not able to get an internship in surgery, so she accepted one in pediatrics. Then luck came her way. In 1928, one of her mentors, Edwards Park, opened a pediatric cardiology center at Johns Hopkins, the first of its kind and the only one for decades to come. Park thought children—chronically ill children in particular—needed better care; they were suffering and ignored.4 He sought out Taussig to lead the implementation of this vision, a radical vision that ill-fated youths might be saved. There, Taussig saw hundreds of desperate children suffering from disorders of the heart.
William Osler standing beside a live patient in front of a captive audience. This surgical theater, like modern ones, is a direct descendant of both the coliseums in which gladiators fought and the stages on which Galen performed. (Courtesy of the Osler Library of the History of Medicine, McGill University)
It was becoming clear at the time that rheumatic fever, the result of an overreactive immune response to an infection by Streptococcus pathogens (the bearers of strep throat), was the leading cause of heart failure in children. The reaction of the immune system to a particular group of Streptococcus pathogens (group A) leads to a thickening of the heart valves, particularly the mitral valve, and a restriction in the valve’s movement. When the restriction is complete, or nearly so, heart failure results. Rheumatic fever and, with it, rheumatic heart disease seemed like an obvious target for research, as early medicines had already emerged to treat the pathogens (sulfonamide drugs, long the most effective treatment against bacteria), if not necessarily the immune response. Also, the sense that new antibiotics were on the horizon seemed to be in the air. Taussig found joy and fulfillment in working with patients with rheumatic fever (which was a leading killer of children in the United States then, as it is today in much of the rest of the world), but others in the hospital objected when she treated these children. She was “stealing their cases,” and so she focused on the other heart problems, the harder ones, the congenital ones, the ones with absolutely no hope; this was her allotment in life, an allotment of death.
In response to such challenges, Taussig was “aggressive, defensive, combative, sometimes triumphant, and often defeated. She suffered.”5 Her friends told her she had chosen (though that hardly seems the right word) the wrong field. As historian Laura Malloy put it, “The prevailing viewpoint was that even if congenital malformations could be accurately diagnosed, nothing could be done about them.”6 But Taussig believed in what might someday be possible. Unlike Abbott, her empathy knew no bounds. She felt she had to work on what she had been given, these children who were beyond cure and whom no one else would even try to help—children like Eileen.7
A piece of rheumatic heart from the Osler collection curated by Maude Abbott, one of her many “jars” in which the variety of our dysfunction could be made obvious. (Courtesy of the Maude Abbott Medical Museum)
As Taussig began to work on the hearts of children, she carefully read Abbott’s book. She even saw her display and met her. Later, the two developed a relationship that would have been described as mentor and mentee if the two women were not both so strong-willed. Their relationship, like much about each of their lives, defied simple characterization. In looking at what Abbott had compiled and in talking with her, Taussig was struck by the diversity and frequency of human maladies. Roughly one in 125 children is born with a congenital heart deformity, and this is not even accounting for those stillborn babies whose hearts nev
er fully developed; heart deformities are the most common congenital problems. She was also struck by something else. Taussig noted that although there did not seem to be that many kinds of congenital heart problems, they repeated. One could imagine that, with all its genes and stages of development, the heart might develop an infinite variety of problems, one for each of the combinations of mutant cells, but Taussig saw something different, something hinted at by Abbott’s work. To her, the same problems seemed to recur, as if the problems themselves had some predictable genetic origin, not in randomness but in evolution. At the time, congenital defects were thought to be due exclusively to exposures to dangerous environments and substances that affected the genes, broke them. To Abbott, this, from the very beginning, seemed wrong, or at least only partially right. The stories were not an infinite number of unique kinds of damage, but rather a smaller number of repeated forms. Abbott had hoped for cures for the congenital disorders she found, but Taussig was determined to do more than hope. She would try to find cures, ideally for the most common forms.
To do so, she had to figure out how to diagnose more of these problems in living children. MRIs, catheterization, and angiograms had not yet been invented. Forssmann had not yet probed his heart. The insides of the body were invisible unless one cut in. When Taussig was given her job in 1930, her equipment for studying the heart consisted exclusively of an EKG, the device used to track the electrical rhythms of the heart. If she was to learn anything more than what the EKG could tell her, she would have to rely on her own senses. So, she looked and listened. She described the endeavor as being like a crossword puzzle in which the answer was a disease and the clues were those garnered from listening, from blood pressure cuffs, and, later in her career, from the hazy images on fluoroscopes. Fluoroscopes are simply x-rays observed in real time so that the body’s internal movements can be captured. Each puzzle’s tragic conclusion, though, was that Taussig would know if she had answered correctly only after her patient died. Then she could look inside the tiny body and see if she was right. This was awful, soul-crushing work. There were almost never happy endings—only tiny tragedies from which she could learn incrementally more.