And how would Stephen take the disease? That mattered. What would he do? The show that 60 Minutes had run about Doctor Kevorkian, the show in which he killed a man with ALS on the air, was still in the news. It was one of the most controversial shows in the program’s history. Many terminally ill patients were outraged at the implication that life with advanced ALS or Parkinson’s was no longer worth living. The producers did a follow-up show about ALS patients with a passion for life.
Jamie was sure that Stephen would want to live. But he could not imagine how Stephen would feel when he could no longer do what he wanted to do. How would he react when the reality set in? It had not sunk in yet and it might not for a while. Stephen still felt fine, almost immortal. Jamie once took Stephen to meet a musician named Steven Fowler, who had an advanced case of ALS. Fowler was a skeleton in a wheelchair, rocking back and forth slightly to help his breathing machine force air in and out of his lungs. Stephen was full of pity, but he could not believe that he would be reduced to that himself. When Stephen was out of earshot, the musician whispered to Jamie, “He’s in denial.”
Jamie knew that Stephen might have only a year or two before he was in a wheelchair. To save him while he was still walking, Jamie had to start treating him within one year: He had to start early in 2000. To start then, he had to persuade ALS specialists to test a new approach by the end of 1999. To do that, he had to find an idea right away. He had almost no time at all.
“I really started having some hard sessions with Joe,” Jamie says. Gally would coach Jamie in all of the fields that radiated outward from Rothstein’s paper about EAAT2—genetics, neurobiology, neuropharmacology, neurogenetics—and he would help Jamie prepare lists of questions to ask specialists. “I would spend an hour on the phone with someone,” Jamie says, “and afterward we would spend an hour and a half debugging my notes, and we would do it again, and we would do it again. And there must have been twenty conversations like that.”
Jamie still has a sketch that they made after one of those talks, a rapid technical drawing of a section of human spine, drawn half in his own hand and half in Joe’s.
The information he was learning with Joe was coming together in his head in graphic form, an indescribable, three-dimensional pulsing matrix of elements that hit and pushed and brushed against each other day and night like nerves in a network or like molecules in a synapse. Not only was his dyslexia gone, but overnight he had acquired a photographic memory, just like Joe Gally’s. And the information he was learning kept coming together in his vision.
“I’ve never had anything like that in my life,” Jamie says. “I was daily listening to myself talk and not believing what I was saying. Between talking to the doctors and then talking to Joe, somehow I just learned what kind of data was important and what wasn’t. Like the needle of a compass.”
Sometimes now he remembered the hero of his favorite book, Ender’s Game by Orson Scott Card. He remembered how Ender felt when he had been flown up into a space station to be trained on an emergency basis in how to defeat the enemies of the human race, the Buggers. “So Ender withdrew into his studies and learned quickly and well,” Card writes. “Whenever he was given a problem that involved patterns in space and time, he found that his intuition was more reliable than his calculation—he often saw at once a solution that he could only prove after minutes or hours of manipulating numbers.”
But in the evenings Jamie and Melinda kept seeing Stephen in a wheelchair, Stephen with a plastic breathing tube in his trachea, images that brought on boiling nausea. To block out their visions of Stephen, Jamie and Melinda roller-skated themselves to exhaustion in Mission Bay, which sometimes helped. Then Melinda belly danced at a club—set after set, night after night. Jamie sat alone at a table for two and watched Melinda. At the institute, Jamie had talked a little about his crisis with a friendly Italian psychiatrist-turned-neurobiologist. So, Jamie, do you drink? the man asked kindly. A little. Good. Your parents? A little. Good. You’re going to need a few. Melinda danced in her costumes of dangling coins, and Jamie drank vodka and tonics, sometimes more than a few.
No matter how wildly they lived, they could not escape their dread. Their digestive systems were in revolt. “Biography becomes your biology,” Melinda wrote in her journal. “Every conscious thought generates a physiological response. It’s driving me mad. Everything is driving me mad. I’m mad!”
Although Jamie was hardly sleeping now, on some days he still went racing in San Diego Bay. Water had always moved him spiritually: the surf at Duck, Boston Harbor, even the smoothness of the Charles River and the lagoon in the Boston Garden. Racing was awesome. That had not changed. It took only a little of his strength, but all of his attention. Racing was one of the few things that could make him stop thinking about ALS.
Once, at Duck, the Heywood boys and their friends and cousins were playing basketball while the elders sat and drank daiquiris and cheered them on. In the middle of the game, Jamie saw a young man drifting drifting too far out on a windsurfer. It was a Costa Rican exchange student. A cousin of theirs had brought him to Duck that summer. His windsurfer was broken, and he was drifting toward Costa Rica. It was Jamie who got on his surfboard, went out, and brought him back. The man was not in real danger yet and nobody else had been moved to do anything about it. Now sometimes when Jamie was looking out at the Pacific, he felt his brother drifting away.
Jamie and Melinda had spent that Christmas in Phoenix with Circus Flora. Back in Newtonville, John and Peggy had celebrated the holiday with Stephen, Wendy, and Ben. What a difference from the Christmas before, when the Heywoods were helping Stephen finish his house in Palo Alto. Stephen had not been diagnosed officially yet by Doctor Brown; the appointment was still a few weeks away. But they knew Stephen had ALS, and the thought of it, daily, nightly, hourly, hit them literally in their guts. They tried to bear the news and the suspense quietly in each other’s company. They were not people who let themselves wail out loud.
Peggy and John saw no point in keeping the news from their family and friends. They each write an annual Christmas letter. Peggy sat down at her computer with its desktop picture of her three boys digging in the sand at Duck. “I can’t even begin to describe what it has been like,” she wrote, “except to say that I now realize that all the metaphors of grief have their physical equivalents. I am thankful for the times when I don’t feel awful and for when I don’t wake up in the middle of the night. Stephen has been his usual low-key sweet self, and is able better than I to say, ‘We’ll see what the doctor has to say in January’…and I do pray for the best. The three boys are so close to each other, as is Melinda, my daughter-in-law, so it has hit us all this Christmas fiercely.”
John did not write his own Christmas letter until January. “A little later than usual, but you will see why below. As Peggy said in her end-of-year letter, it has been the ‘best and worst of years.’ Stephen is perhaps doing better than the rest of us—steady and low-keyed. As our friends and church community have learned about this they have been incredibly comforting and supportive. We will keep in touch as we learn more and things develop. I’m sure we will be spending as much time together as a family as we can.”
Stephen himself was working in slow motion on the house on Mill Street. He was laying tile and yellow limestone in the new master bathroom. He felt fine, except for his claw hand. He was also having some slight trouble now with his left foot.
People can react in several ways to a diagnosis of ALS. They can devote themselves to fighting the disease, they can close one eye to it and try to live as fully as they can, or they can give up—not an option with the Heywoods. Jamie was masterminding a war, and Stephen was calling friends from Alaska to Corfu to tell them his news and make plans to get together. They all heard him on the phone with his voice as dry, cheerful, and sardonic as ever: Yeah, I’m toast! They could not understand how he could keep his good nature. But good nature is strangely common in victims of ALS. Doctors often say that ALS
patients are some of the most painful patients to treat and lose. Surprisingly often, they are sturdy, athletic, sunny optimists, like Stephen. (Other diseases prey on couch-potato pessimists.) That year, ALS advocates would parade in a fund-raising demonstration on Capitol Hill—walking or in wheelchairs—wearing T-shirts that read, “ALS patients are such nice people, it’s a shame they’ll only be here two years.”
After the shock of the news, one of the first things Stephen discovered was that he wanted to have a child. “I don’t want this to get me in trouble, because my family loves Grace Church—but I’m an atheist,” Stephen explained to me a little later on. He said this with a small smile, because his family’s faith was complicated. John and Peggy were both unbelievers, or so they claimed, even though they went religiously each Sunday. “I’m so atheist,” Stephen said, “and have been since I was twelve or eleven. So if you’re atheist and you know you’re gonna die before you get so exhausted from life that you don’t really care, you’re kind of fucked, because there’s nothing to look forward to. And one of the first things I thought about was kids. Well, maybe I would feel differently if I had left behind a whole bunch of buildings, too. But kids—that’s what you can leave behind.”
Even though he and Wendy were not even talking about marriage, Stephen found himself thinking more and more about having a child with her. She was thirty-four. He knew she wanted a family. One of her older sisters had chosen artificial insemination at about her age.
Those who loved Stephen cried when they listened to him, and thought how short a time he had left. “I mean, my mom was bawling,” he told me. “But Wendy and I started talking about babies. Wendy would make a great mom. I mean, shit, I could go out and plant my seed if I wanted to, but you know, how do I know what’s gonna happen after I’m done? How can I trust to that? And I felt I could trust Wendy. So it was very intense and very strange.
“It’s atrociously bad timing, all of this, I guess. It’s a very hard decision. I knew I was in love back in December, before any of this shit happened, which is great. But normally you’d just be like, ‘Wooh-hooh, we’re in love! And now we can just hang out and go to the movies and shit like that, and maybe we’ll live together and maybe we won’t.’ ” Now almost nothing was normal. On the one hand, Stephen and Wendy were talking about a baby. On the other hand, they did not even want to move in together without being engaged. Stephen had done that before, with Stephanie, and it had been a mistake. “Everybody knows that if you live together before you get married, it’s not gonna work.”
So there he was, in love and dying. Stephen knew that Wendy loved him, too, and what was the right or fair thing to do? “I mean basically Wendy’s fucked, you know. No matter how this works out, it’s not good.”
Thirteen
Prisoners in Outer Space
On January 13, 1999, Stephen went back to Massachusetts General to receive the official diagnosis from his neurologist, Robert Brown. Normally he preferred to go to doctors’ appointments alone. But his mother wanted to come to this one. Jamie asked Stephen if he could meet Doctor Brown, and Stephen decided to let him come, too. So Jamie flew home again, with his multilayered vision of molecules still forming in his head.
John Heywood did not ask to come, and Stephen understood. John bicycled to the Sloan Automotive Laboratory that morning, just as usual.
Bob Brown has been working on ALS for more than a quarter of a century, and he is one of the most distinguished scientists in the field. In 1993, Brown and his colleagues discovered that a small fraction of ALS patients inherit a mutation in a gene called SOD. Brown’s discovery enabled ALS researchers to genetically engineer a mouse with a bad SOD gene. For the first time, scientists could study the course of the disease in a laboratory animal. Normal mice live two years. ALS mice die at about five months, paralyzed and choking.
The creation of an animal that suffered and died like that was a miserable thing for the mice, but it brought hope to any human being concerned about ALS and the other nerve-death diseases. It allowed Brown and many others to experiment and try to figure out what is going wrong and how to fix it, although no one had found a cure. In medicine the easy diseases to cure were already curable. As a colleague of Brown’s, another Harvard neurologist, once put it, “The low-hanging fruit has been picked.”
It is one of the many coincidences in Stephen’s story that he found his way to Brown. Stephen had not known that he had ALS when he made his first appointment. Bob Brown is white-haired, patrician, and, for a Harvard man, extremely self-effacing. Delivering a diagnosis of motor-neuron disease is equivalent to handing down a death sentence. As a world-renowned authority on the disease, he has to deliver it at least once a day. He always hates it. The mice in his laboratory never know what is happening to them. But most of Brown’s patients, people in their fifties and sixties, feel as if they have a long time to live. Some of them are young and vigorous, like Stephen. Bob Brown is a kind man and he has never gotten used to any of it.
Less than a month had passed since Stephen’s neurological workup on December 16. In the old days, doctors did not even give patients a diagnosis of ALS at this early stage. Since there was nothing they could do for them anyway, they kept the bad news to themselves for a while. But that was before the ALS drug riluzole. Now that doctors could offer even a few months’ extension of the death sentence, they told their patients the diagnosis promptly.
Brown’s main goal in that first appointment was to acclimate his patient to the diagnosis. He knew where he was going and so did the Heywoods, but they all found it hard to take the steps one by one that would lead them there.
Brown’s office is a cubicle with just enough room for a desk and a small curtained alcove. Stephen and his mother sat down in chairs facing the desk. Jamie pulled in a chair from outside and sat by the door. The room was so small that both brothers could hold Peggy’s hands while they waited for Brown to begin.
Jamie had brought a tape recorder so that he could listen to Brown’s diagnosis again later. But when he pushed the button he discovered that he had forgotten to bring a tape. Before they started, he borrowed one from Brown.
“Any changes since December?” Brown began in a doctor’s studied, friendly, professional voice.
Stephen told Brown that he thought he noticed a little weakness in his left foot.
“Mmm-hmmm.”
“And in my right foot it’s much more pronounced.”
“Ah-huh.”
“Other than that, no, not really any changes.”
“OK.”
“The last couple of weeks have sort of been strange because we had Christmas, and, uh, I’ve had these stomach problems that have been very painful,” Stephen said. He meant the hemorrhoids, and also a case of stomach flu. “So I have felt a little under the weather in general. But, you know, you start looking at everything and wondering if that’s related, and you get a little paranoid.”
“Sure.”
“So I think I’ve just had a bad couple of weeks,” Stephen said, with a chuckle. His tone was eagerly manly, as if to say, I can talk about this straight, no big deal, I can handle all this.
“OK,” said Doctor Brown. “So you’re essentially functioning.” The phone rang. He dealt with the call, and turned back to Stephen. “Your voice sounds normal to me, it’s been fine?”
“Yeah.”
“Great. And chewing and swallowing are fine?”
“Fine.”
“OK, good.” He asked Stephen about the stomach problems, and gave him a little advice about the hemorrhoids.
“Alrighty. Well, let me examine you. And then we can sit down and talk a little.”
So I can try to summarize what we have at this point,” said Doctor Brown, after the exam was over and he and Stephen had settled themselves again on opposite sides of his desk. “And then I’ll of course try to answer your questions.”
“OK.”
“You have a history of motor weakness, which started very s
lowly and focally and has kind of slowly increased a little bit in its distribution and certainly in its severity.
“The EMG testing that you’ve had showed focal motor-nerve abnormalities at first, and now shows more widespread abnormalities, which involve the arms and the legs, as well as the initial site of onset. Not the face or the tongue.
“Um, and so that’s the picture of a progressive motor-neuron problem. And, um, in addition, ah, your examination shows, ah, some suggestion that what we call upper motor neurons that reside in the brain and affect the motor neurons that reside in the spinal cord, also may not be functioning quite normally. And so—”
Just as Doctor Brown steeled himself to deliver the death sentence, Jamie interrupted from the doorway.
“In the cortex?” he asked.
“Yeah,” said Doctor Brown. “I’ll draw you a picture of that.” He got out a pen and a legal pad and they all gathered to look. “So there’s a suggestion then that you have involvement of both upper and lower motor neurons,” Doctor Brown said. “So, for example, if this is, let’s say, your brain, and this is the base of the brain, and this is, let’s say, the spinal cord down here, this is—apologies to those of you who are real artists—a stick figure of you.” The doctor chuckled apologetically. “What we’re saying is that there are motor neurons that live in the motor cortex and descend to different levels of the base of the brain and the spinal cord. These are so-called upper motor neurons. And then there are others that reside down at the base of the brain and go up to muscles—for example, of chewing and swallowing and of the tongue. Other nerves reside in the neck and go down to muscles that have to do with arm and hand movement. And then still others that live elsewhere and go to other muscles down further in the body.
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