Open Heart (38 page)

“You may not remember this now,” Rich says, “but I was pushing you very hard to get the angiogram a.s.a.p. You were sitting on a time bomb, and I knew that one hundred percent.”

“Yet you were the only person, in the two months before surgery, who believed this,” I say, and I remind him that Phil called a few times to suggest I have an x-ray, that he thought what I was describing—the discomfort between my shoulder blades—might be due to a dissection of the aorta (a condition in which the inner lining of the aorta is sheared off, so that the blood stream “dissects” its way through the lining of the blood vessel, forming a double opening that is life-threatening and, characteristically, causes pain between the shoulder blades).

“But Phil trying to diagnose your situation would be like me trying to diagnose a nuance of where in the hippocampus a stroke was taking place,” Rich says. “It's why I agree with him about the drive toward having more family practitioners and fewer specialists being the reverse of the way things should be.

“But that's another subject,” he says, and he backtracks to our discussion about what was happening in the weeks preceding surgery, and walks me through the experience again.

“There were two main reasons why I knew your condition was very severe,” he says. “First of all, not only did you have a clear-cut angina, even though it was in a somewhat unusual place—between your shoulder blades—but the symptoms were progressive. They were occurring more frequently, and they were occurring with less physical activity—and I learned this from your telling me about what was happening when you went swimming.

“The second critical factor was that along with the discomfort between your shoulder blades, you were also becoming short of breath, and this told me that when you were experiencing the anginal symptoms, a very large area of your heart was becoming dysfunctional.”

Rich explains: “What happens to the heart when you get angina is this—you are exercising, so your heart needs more oxygen, but because there's a blockage in an artery in that area of the heart which the artery is supplying, the heart is not receiving the oxygen it needs. So two things occur: One is that the heart says ‘Ouch'—which is
what you feel in the symptoms—but what also happens to that area of the heart muscle is that it quite literally
stops contracting
. And the reason it does this is because it's making an effort to stay alive—and the way to do that is to minimize the amount of oxygen it's going to use.

“But when a critical area of the heart muscle stops contracting in order to preserve its oxygen sources as well as it can, the blood backs up into your lungs, and you experience this as shortness of breath.

“Thus, a typical patient with angina will get chest discomfort, pressure, heaviness, or a squeezing—and it can be located in many places: the neck, the jaw, between the shoulder blades—but you will
not
get shortness of breath along with that unless there is a very large area of the heart involved.”

I ask why he thinks my family doctor diagnosed asthma, and Rich says that for a general doctor to diagnose my condition would be like having a neurologist look at a cardiology problem.

“A family doctor cannot have the depth of perception about the across-the-board panoply of diseases they are called upon to be insightful about,” he says. “So when you told me what your doctor said, I told you with exclamation points that this asthma diagnosis was absolute nonsense.” Rich adds that it
was
a good thing my doctor eventually realized this (when the inhaler he had prescribed had no effect on my shortness of breath), and that he had ordered a stress test.

But why, I ask, as the weather grew colder, did it get harder and harder for me to walk outdoors?

“Because cold tends to constrict your blood vessels,” Rich answers. “They constrict in order to shut down the blood supply to the skin so that you can maintain body heat. But by doing that, the work that the heart has to do is
increased—
so the harder the heart has to work, the more oxygen it needs. But a limitation has now developed on how much oxygen you can get through the blocked arteries—those coronary arteries that are the suppliers of oxygen to the heart muscle.”

Rich reminds me that the first time I called, he urged me to go to Boston and see two doctors he knew at Massachusetts General Hospital,
and I ask why he was so concerned at the outset, especially since I had few if any risk factors, and virtually no symptoms.

“I've always had an intuitive sense of when a patient is in danger—it's an instinct I've learned to trust,” he says. “In your case, even though you lived a healthy lifestyle and the only risk factor you really had was your father's history—his heart attack, but, then too, he was a chain smoker—all that stuff goes out the window. When tell-tale symptoms develop, factors like family history, cigarette smoking, and cholesterol levels no longer matter. The symptoms themselves are all that count, and they register as being significant and urgent or they don't.”

I ask what the results of the EKG and the echocardiogram—both of which I had faxed to him—told him.

“Well, the EKG showed an unequivocal abnormality, and an EKG is quite valuable, but only if it's abnormal. Then it can provide clues. But if it isn't, it can often be misleading and miss a lot of things. What the echocardiogram did was to confirm my concern about there being a lot of weakness in the way the heart muscle was contracting. This told me that there was a very extensive area of the heart involved in the process, which suggested that several of your coronary arteries had significant blockages. When added to the increasing frequency and severity of your symptoms, the picture that emerged was that you had widespread, severe coronary artery disease,
and
that the abnormalities had become unstable. One of your major arteries was about to close down and cause a massive, possibly fatal heart attack.”

I remind Rich that when I reported the results of the echocardiogram to him, and reported that the cardiologist had said, “I think it's viral,” Rich had exploded for the first time, telling me it wasn't viral—
“goddamnit!”—
and that he wanted me in the hospital as soon as possible.

“You know, my level of concern had been high from the outset,” Rich says. “Only I did not want you to know because I didn't want you to panic. What I wanted was to get you into a good hospital where they knew what they were doing, and to get this sorted out and fixed.”

But if what was happening was so obvious, why had two doctors missed it?

“Look,” Rich says. “The EKG and echo simply provided lab evidence that confirmed what your story was telling me loud and clear on the phone from three thousand miles away. And what happened was a microcosm of a central problem with technology—that a lab test can only be used correctly in the context of the patient's symptoms.

“This cardiologist was looking at the fact that the whole heart muscle was not contracting well—something that occurs in people with viral infections of the heart muscle—and he was making a misdiagnosis because he was forgetting that you had told him you were also having symptoms of angina. And people with viral heart disease do not get any symptoms of pressure, tightness, or pain—what you were having between your shoulder blades—they simply develop shortness of breath.

“So he misread the echocardiogram because he forgot the fundamentals—he forgot about you. You start with the patient's story, and the tests you run are only valuable if they add dimension to that story. But they do not let you be seduced away from the story.”

Rich talks for a while about what Phil and Jerry have been talking about: the ways technology has lured physicians toward quick-fix procedures, and away from the primary source of diagnostic information, and why, because this is happening, we are often kept from knowing what is really going on. He declares that I would not have been able to benefit from the incredible technological advances we
do
have at our disposal—bypasses, coronary care units, revolutionary medications—if people wedded to technology had had the final say.

“What has happened, it seems to me,” Rich says, “is that the diagnostic acumen of the physician at the bedside, on the phone, or in the office has been severely compromised because the mindset now—and this is also the main reason medical costs keep going up—has become, ‘Well, the tests will tell me anyway, so I don't have to spend a lot of time listening. I can just run a battery of tests, and the tests will tell me the diagnosis.'

“First the nurse said, ‘Why don't we just schedule you for a full
exam,' and then you had a diagnosis of asthma, and then you had a diagnosis of ‘Well we don't know,' and then you had a diagnosis of a heart attack, and then you had the doctor saying, ‘No, there's no heart attack, but we have a viral cardiomyopathy,' and all the while the symptoms are progressing, you're hanging on by a single artery which is itself hanging by a thread, and something catastrophic is about to occur. Let me say it again, Jay. There is no question in my mind that we wouldn't be sitting here today if you hadn't gone to high school with the right guys.”

In our talks in Palos Verdes, we return frequently to what we talked about in the days immediately following my surgery: what we do and don't know about heart disease.

“Theories have come and gone,” Rich says, “yet we are no closer to a true understanding of the causes of atherosclerosis—of what happened to you—than we were a generation ago. We do, however, know that smokers are several times more likely to develop heart disease than nonsmokers.

“The evidence is unequivocal there. We know that people with really high cholesterol levels are more likely to develop heart disease. The same is true for patients with hypertension, or with diabetes, or for patients who are obese—we have very strong
statistical
correlations in these instances. But if you compare what we know to what happened when medicine first became scientific—when Pasteur made his discovery of the germ theory: that germs cause infectious disease—and when Koch provided the scientific standard for proving cause and effect with respect to these diseases—we come up short. Statistics have almost nothing to do with cause and effect—they only show
associations—
and we cannot yet show cause and effect when it comes to atherosclerosis.

“If you give antibiotics to one hundred people who have pneumococcal pneumonia, all one hundred will be cured. Nothing like that exists regarding atherosclerosis—though we do know the causes of other heart diseases, such as rheumatic valve disease, which is caused by streptococcus.”

According to Koch's postulates, an organism must be present in every instance of a particular disease; it must be capable of being
isolated in pure form from the disease lesion and possess the capacity to reproduce the disease in a healthy animal through inoculation with a pure culture; and the same organism must then be capable of being retrieved from the inoculated animal—in the lesions of the artificially produced disease—and of being cultured anew.
^*

The assumption, and hope, still with us 120 years after Koch formalized his postulates in 1882—as we see in the immoderate rhetoric that accompanies much recent genetic and biomedical research and drug company advertising—is that each disease will be shown to have a specific causative agent, and that once this agent has been discovered and isolated, we will be able to control and cure the disease.
^*

But the world of biology and disease is rarely this simple. Even when we
do
find what appear to be single causative genetic abnormalities, as with Huntington's chorea, Friedreich's ataxia, sickle cell anemia, cystic fibrosis, and muscular dystrophy, developing means for controlling such agents (in these instances, defective genes) frequently eludes us.

“Phil was right when he said that except for the possible genetic link with your father, you had at very most only minor risk factors,” Rich continues. “Now, for people who have a strong family history of coronary disease, particularly early-age coronary disease—if, say, your father had had a heart attack at age thirty-eight or forty-two—then that would make the genetic link, and the danger, much more likely.”

I say that after hearing my stories of how the common early warning signs and symptoms for the illnesses Robert and I had (schizophrenia, coronary artery disease) were largely absent, people have usually responded by saying, “Oh, then it must be genetic.” Rich agrees that “It must be genetic” is simply another way of saying “We don't know why these things happen”—and that the use of a scientific term such as
genetic
does somehow reassure people.

It is much the same, I say, with diagnoses. For years, when people would ask what my brother's diagnosis was, and I said “manicdepressive,” or “schizophrenic,” or schizo-affective”—whatever the most recent diagnosis happened to be—people would nod knowingly, and then move on to another topic of conversation.

I had come to group such responses under the heading of “The Consolation of Diagnosis,” and had taken to following up with questions of my own: “So now that I've given you a word—a clinical term—what does it tell you about my brother?” I'd ask. “What do you know about him now that you didn't know before—when you met him, or heard me talk about him, or read about him—?” I would, that is, use the question as an occasion to talk about Robert as a man with an idiosyncratic personality, a complex history, an unenviable series of breakdowns and hospitalizations, and an identity at least as unique as anyone else's.

In point of fact, it turns out that the genetic basis for coronary artery disease, as for the major mental illnesses, is modest. Twin studies of coronary artery disease show a concordance rate between identical twins of 19 percent, and of 8 percent for nonidentical twins.
^*
(Compare this, for example, to a rate of 50 percent for identical twins in insulin-dependent diabetes, and of nearly 100 percent in non-insulin-dependent diabetes.)