A Telltale Heart

The doctor. The heart transplant. The book. Features editor Claire seesbeyond the drama and finds urgent issues of health care, parenting, and personal responsibility. She asks impertinent questions and deliberates on ethics along the way.

A young person is going to die. That's why I am here, I am counting on it, staking my life on its occurrence....His death will occur whether I need a heart or not. It has been predetermined by an inscrutable god. Without this death, my life ends. I want this death to occur, I look forward to it happening. . .But ultimately these thoughts are flawed: I do not look forward to this death, and I do wish it were avoidable. What I look forward to is a new heart, a new life. That's all.

Just how much high praise can one man--and his book--take, I asked myself after reading yet another superlative-laden review of Raising Lazarus. It's an autobiography of a psychiatrist with a lethal heart disease. I found it on my desk with a yellow Post-It note hurriedly inscribed with my editor's scrawl: "Owen [the editorial director] says this is best-seller-bound." He was taken by what this man, Robert Pensack, endured--especially the drama of his heart transplant.

The accompanying press packet bulged with glowing reviews: The New York Times called it "an extraordinary coming-of-age story." The Kirkus Review lauded the "haunting story of a man's struggle to survive." It made the Library Journals list of best books of 1994, described as a tale of a man who "clung tenuously to the silver cord of life."

Book in hand, I drag my chair into the newly painted orangish conference room. Sealing myself from the office chaos, I inhale paint fumes, drink black coffee, and read.

We get a lot of medical hard-luck stories--read along as I lift myself heroically out of the depths of illness. But Bob Pensack's struggle is different. Not because it's better written nor because he's a physician. It's because the rational use of health care resources and even health care reform, though never mentioned outright, pull at the story like a lethal undertow.

I was bowled over on page one. Three-year-old Max stands stiff in wonderment above his dad, who pounds his fists into his own chest, hoping to shock his convulsing heart back to life. He struggles with the detached calm of a man who has been there before. As have his mother and brother before him, and nephew Benjamin after him.

The Pensacks carry the genetic curse of familial hypertrophic cardiomyopathy (HCM), a thickening of the heart walls. Most HCM patients's hearts become so muscle-bound that blood can't pulse through the organ's chambers, causing shortness of breath and ultimately heart failure. Many die from irregular heartbeats. Presumably, the disease took the life of Bob's mom at age 31, in 1955. Her doctor labeled it an unknown heart ailment. Pensack, then just one year older than Max, recalls his final memory of her as she was wheeled down a cold hospital corridor.

Back then, writes Bob, people went to hospitals "either to die or go home. . . . Little could be done for the chronically ill." By contrast, he says, he and his brother come to understand disease "as a way of life, mortality as something to be grinned at in the morning while shaving." What a difference 40 years make. In a society top-heavy with the elderly, health care has become disease management. Indeed, cancer is now thought of as chronic disease.

Richard, Bob's older brother, carries the first hint of his diagnosis home in his hip pocket during his freshman year of high school. A general practitioner detects a heart murmur after a routine medical cattle call in the gymnasium and sends a note home. The following fall the diagnosis is made after a week of tests at the National Institutes of Health. Bob made the trip too, but had no signs of the disease at that point.

The diagnosis changed his brother, who had lived for basketball and was now permanently benched from sport and, in a sense, childhood. Shades of Loyola Marymount University's Hank Gathers, who died of the same disease mid-game, flay my thoughts. "Richard now has, and always will have in my imagination, a face that doesn't match his years," writes Bob, "the face of a ruined child."

Bob managed to get away with childhood. His first traces of disease weren't felt until his second month of college, in the form of overwhelming vertigo, again on a basketball court. A week later he was on a plane to the NIH. The diagnosis is made, and Bob is put on a drug called Inderal to ease the dizzy spells. Its side effect, depression, casts a "desperate gloominess" over his return to school.

A depressive stupor doesn't stop him from graduating pre-med from University of Colorado or from medical school, for that matter. Yet during his second year at med school, he is forced to leave for open-heart surgery to pare the chambers of the heart, allowing for more blood to wash in and out. The procedure, a septal myotomy and myectomy, carries a one-in-five mortality rate. Bob beats the odds, but the surgeon carves too deep and cripples his heart's pacing system. He will now have to rely on a pacemaker.

Bob's illness starts to encroach on his sanity. Now 25 years old and anxious to gear back up for medical school, Bob can't concentrate. He can't even force himself out of his house. A friend prods him to an emergency room to see a psychiatrist. Some of his confusion and paranoia are temporary side effects of having spent time on a heart-lung machine during a traumatic surgical procedure. But Bob is convinced he is schizophrenic. His psychiatrist recommends he temporarily check into a facility that houses schizophrenics. Bob feels a great empathy for his fellow patients, but realizes immediately that he doesn't share their affliction.

His breakdown leads to a journey of self-discovery and to psychiatry as his ultimate professional destination. He later realizes that his sense of impending doom, panic, and out-of-body experiences are the symptoms of post-traumatic stress disorder (PTSD). It is an appropriate reaction to his deadly predicament. Not unlike a shell-shocked soldier, Bob bounds from one life-threatening episode to another, with his nose pressed up against his own mortality.

Two pacemaker operations later, he completes his M.D. at age 31, seven years after he started. And in 1988, at the ripe age of 38, he becomes a psychiatrist. Three years later he's married, with two kids, but can barely make it up the stairs to his house. It's dear that if he doesn't get a new heart within the next 12 or 18 months he will die. After two false alarms--one almost lethal--he has a not-so-smooth but successful transplant in his eighth month of waiting.

Recovery is rough. Bob is on the heart--lung machine for twice as long as he should have been as the surgeons try to make room for a heart too large for his swollen chest. When he awakens he is lost in ICU psychosis, imagining his doctors are secretly plotting his death. Bob experiences a brief bout of sanity when his kids visit him. Max curls in the space between his shoulder and neck and asks him when he will finally come home.

Once home, Bob is thrown into mania by the immunosuppressive drugs he's taking. His coauthor, Dwight Williams, leaves us with the image of Bob madly painting the inside of his garage red. But a switch of drugs brings him out of it. Today Bob is thinking of writing a new book and is fighting chronic tissue rejection. Since his transplant he has endured 40 heart biopsies.

I closed the book with no intention of excerpting it. To survive a transplant--to have your rib cage sawed open, a brain-dead Texan's size-D heart crammed into your chest cavity, and be wired shut like a barnyard gate--that's simply the reality of organ transplantation.

But Max's face kept popping into my mind. He and his sister Miriam boiled the book down to three questions for me: In this age of health care reform, will Max, Miriam, and nephew Benjamin be provided for? Is it ethical for one family to consume so many health care resources? And is it ethical to have kids, knowing the hardship they may face, the threat of early death, and the resources they too may consume?

Bob and Richard Pensack were playing genetic roulette. Each kid had a 50 percent chance of inheriting HCM. But it's not black and white. The disease has variable penetrance. That means it has a range of severity. An infant that inherits HCM can be born with heart failure, or he can go through his entire life not knowing he had the disease. The bulk of patients with HCM, however, lead lives limited in the ways that Bob's and Richard's were.

Richard's son Benjamin was unlucky. He has HCM, but a milder version. Max and Miriam have no signs of the disease as yet and may never.

Feeling guilty about my reaction to the book, I called some medical ethicists for guidance. Funny, they had no problems rattling off an opinion for me, based on my brief sketch over the phone of Pensack's life. (It's a little easier to think about in the abstract, without Max and Miriam staring at you,) I ask Bob Dougherty, M.D., director of the Center for Health Policy and Ethics at Creighton University, to clear up my first moral dilemma: At the rate we are going, will there will be enough health care resources for the littlest Pensacks?

Dougherty wants me to get one thing straight: There is no such thing as health care reform. Congress dropped the ball and we are left with a market-driven system that doesn't know ethics from bedpans. Whatever you thought about the Clinton plan, at least it provided a framework, one that allowed for consideration of ethics. "As it stands now," says Dougherty, "ethical issues are sort of fought out house to house." we're in a state of free-form health care.

Apparently, insurance plans in about a dozen states put caps on what any one insured person can spend on health care in their lifetime. So people with catastrophic illnesses, like the Pensacks, have a decent chance of blowing through their caps. Some states tax insurers to create a pool that these patients can draw from, and as you might imagine, the costs are very high. "These people would be uninsurable on their own," he tells me. "It's ugly out there."

So if, God forbid, Max and Miriam are saddled with severe HCM, they may or may not be covered by insurance, depending on what state they live in and whether they are insured by, say, Kaiser, Blue Cross, or Aetna.

There are limits to what people, states, and the federal government can pour into health care without depleting the coffers for other necessities--food and lodging for impoverished families, education, and job creation. Are these finite resources best spent on the Pensacks, or are they hoarding benefits; draining resources from others whose afflictions were not the result of a hopefully calculated game of chance?

Tom Raffin, M.D., codirector of Stanford's Center for Biomedical Ethics, offers an unmitigated no to the latter. The bulk of health care resources in this country, he contends, go to folks with undiagnosable--and constantly recurring--pains in their backs or heads that are more than likely psychosomatic. Big-ticket items like transplants are one-time-only costs.

But what about the weeks the Pensacks spent at the NIH, free of charge, getting diagnostic tests, new pacemakers, open-heart surgeries, better drugs? I'm not convinced.

I seek out yet another opinion. Bruce Jennings, M.D., director of the Hastings Center, frames my moral dilemma as an issue of individual versus statistical lives. Huh? On one hand you have Bob, or some kid appealing for funds on TV for a heart transplant that, according to Corporate Health Strategies Inc. of Connecticut, would run him around $145,000, not to mention a lifetime of biopsies and anti-rejection drugs. On the other, you have an inner-city prenatal health care program in the heart of Chicago. Let's say that $145,000 touches the lives of 300 mothers and saves 25 fetuses from a lethally early birth. Of those 25, 10 survive neonatal intensive care. What should our priority be, saving Bob or 10 inner-city kids yet to be born?

But Bob has a decent shot at a good quality of life and many productive years ahead of him. "If we try to ration in that way, taking health care that is clearly successful away from identifiable individuals to improve general health statistics like pre-maturity," says Jennings, "it's bound to cause a lot of political and moral anguish."

Phew, I wanted Bob to live. But his transplant is a done deal. It's Benjamin and, God forbid, Max and Miriam we have to worry about.

Next question: What were Bob and Richard thinking when they had kids? I thought it only fair to ask Bob. "I struggled with that decision every day," says Bob. "At one point I condemned my brother for it because he had a good chance of passing on the disease." His NIH physicians had always told him "even though the disease could be devastating, there was no reason not to go ahead and have kids." And that was before transplantation.

For him, as you might imagine, the decision came down to whether or not his own life was worth living. Given the option of living his life with all its suffering versus never living at all, he would live. He doesn't say this lightly. And he is mindful of the amount of precious health care resources he has already consumed to date--there are only so many beds at the NIH and transplantable hearts in the United States. But it's one thing to say, in retrospect, that your life was worth living, and quite another, says Dougherty, to risk injuring others merely by propagating your genes. What of the pain the kids may endure?

In deciding to have kids Bob was heartened by the prospect of being there to help them through it. Though he cannot know that for sure, he might just be right. After all, he has already outlived his mom by 14 years and has a brand new heart to boot. (In case he doesn't live long enough to shepherd his kids through their illness, he wrote his book as a survival kit.)

That leads us into his next argument: medical progress. Transplantation didn't even exist during his mother's short life. And its current incarnation may be crude and obsolete in a decade's time. Who knows, we may be transplanting monkey hearts by the time a little Pensack is in need (if ever). Or we may have a prenatal test for HCM so they won't be plagued with the same child-bearing issues.

Bob also feels his illness propelled science forward. As a guinea pig of "heroic" medicine, he taught us about how the heart and immune system work, he contends. If health care is reduced to primary care and prevention, medical science will come to a standstill, he insists. It's heroic medicine--transplantation, genetic engineering, and the like--that drives scientific progress.

I had to ask him fiat out: What if we are forced into a system of health care rationing and your children consume an enormous share of resources? Bob's passage about organ scarcity tugged at my question. Only 4,000 people a year donate their organs, while every 30 minutes another patient lines up for one. Thousands die each year awaiting an organ of the right size and blood type. Should the Pensack kids have equal footing in the line?

And aren't insurance plans built on the notion of spreading risk within large pools of people? Isn't Bob's decision to have kids with a 50 percent likelihood of being ill skewing the odds and, in effect, undermining the foundation of the insurance system? What if every person with a potentially lethal genetic malady decided to have kids?

"If it comes down to rationing, are you going to sterilize me?" he asks. "If you don't and I have children anyway, will you deny them care?" That's morally untenable. Economics cannot outweigh our sense of decency and humanity.

He's right. The right to bear children is fundamental. A health care system that encroaches upon it would be not only untenable, but perhaps unconstitutional. The decision will likely always be our own, as individuals. And Bob chose to have kids. But he wanted to parent his own genes, not adopted ones. Couldn't he have been a superb father to the 45,000 to 100,000 foster kids awaiting permanent homes in this country? If it were just parenting he wanted out of fatherhood, why did he have to raise kids in his own genetic likeness?

Though he had the right to make the choice, did he make the right decision? What could be more disturbing than second-guessing the lives of Bob and three children who are already here, clinging to the planet? I have renewed respect for the cold and tidy hypotheticals the ethicists spoke in. Believe me, I've seen It's a Wonderful Life as many times as the next person. I have no doubt Bob's soulful insights have been invaluable to his patients, and his readers. And his own research, early in his career, testing anti-rejection drugs, as well as his experience as a transplant patient, have added to an ever-expanding medical knowledge base.

Even so, I'm not sure I could watch my children rise to the top of a transplant list knowing they may receive an organ that would otherwise go to someone whose parents had no conscious control over their child's fate. It's easy to have individual rights and exert them when society shoulders your moral and financial burden.

As you might imagine, Bob is an advocate of a nationalized health care system (so am I). Come to think of it, he's been in one his whole life--the NIH and the University of Colorado, a state-funded medical school. And that makes perfect sense. If we didn't have health insurance, much less state and federally subsidized health care, could anyone afford to make the choice Bob made? I mean, anyone besides doctors?

ILLUSTRATION