A telltale heart

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.