47
HOW A TELLTALE HEART COULD CHANGE MEDICINE FOREVER
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I say, there came to my ears a low, dull, quick sound, such as a watch makes when enveloped in cotton. I knew that sound well too. It was the beating of the old man’s heart.
—EDGAR ALLAN POE, “THE TELL-TALE HEART”
“THE TELL-TALE HEART” is a chilling 1843 short story by Edgar Allan Poe about a man whose disturbed conscience is haunted by the sound of a heart that will not stop beating. The real-life telltale heart is the tragic story of seventeen-year-old Jahi McMath, who was diagnosed as “brain dead” over three years ago but whose heart still beats. The case generated a lawsuit that could overturn a half century of established belief and haunt the conscience of medicine.
McMath’s heart and blood pressure have been stable for more than three and a half years, an unanticipated finding extremely rare with the diagnosis of brain death. Perhaps in her case, the original diagnosis was wrong, a disturbing possibility, or more likely it simply means we must rethink the twentieth-century concept of brain death.
In December 2013, following a routine tonsillectomy for sleep apnea, McMath suffered a cardiac arrest and massive brain damage from lack of oxygen to the brain. Several neurologists found she demonstrated no sign of cerebral electrical activity, no blood flow to her brain, and could not breathe on her own. She was diagnosed as “brain dead”; a California coroner issued a death certificate, and the hospital prepared to discontinue her ventilator.
In a legal proceeding independent from the current lawsuit, McMath’s family went to court in 2014 to prevent her from being removed from the ventilator. The judge assigned an expert from Stanford to examine McMath, and he concurred with the diagnosis of brain death. Technically McMath, brain dead, was no longer a living person.
Despite this, her family subsequently received court approval and removed her from the hospital to a facility willing to care for her. She is currently on a ventilator, being fed through a feeding tube. Her heart has been beating far longer than that of nearly any other brain-dead patient, a troublesome fact most experts have failed to acknowledge since the first court hearing. When McMath’s family initially went to court, most bioethicists and doctors familiar with brain death felt the family’s interests, while understandable, should not supersede the law. The conventional thinking was that the judge’s decision to keep McMath on a ventilator was in error; once McMath was declared legally dead, the court should have ordered the ventilator disconnected. Without the ventilator to breathe for her, her heart certainly would have stopped in minutes.
The possibility remains, however small, that experts may have been wrong, and a medical malpractice lawsuit may force reconsideration of the issue of whether McMath is alive or dead. Here’s why: in cases of properly diagnosed brain death, no patient has ever recovered to come off a ventilator. The only things keeping the heart beating are the ventilator and occasionally drugs given to sustain the blood pressure, usually given when the patient is an organ donor. However, even in brain death, machines and drugs are usually not enough to sustain the heart. Even with the patient connected to the ventilator, the heart will usually stop fairly soon. Within a few hours to a few days, patients “die” in the widely understood sense. There have been isolated cases of pregnant women who were brain dead but kept on life support until their babies were delivered. Outside of those, there are only a handful of reports of brain-dead patients on ventilators whose hearts beat for more than three months, and in some of those cases, the correct diagnosis of brain death has been questioned.
Because McMath’s heart has continued to beat far longer, the new lawsuit against the hospital and doctors who originally performed the surgery reopens an area of life and death once thought settled. In California, damages are legally capped for the wrongful death of a child, but there is no cap, and damages could run into tens of millions of dollars, if the child is injured but still alive, as the suit claims. So the court might actually have to revisit the question, “Is Jahi McMath alive or dead?”
Unlike the cases of Terri Schiavo and Karen Ann Quinlan, who were not brain dead and could breathe without ventilator assistance, the issue in McMath’s case is not whether she will ever regain consciousness. That would be completely out of the realm of experience. The question is the meaning of brain death as death of a person and whether traditional diagnostic criteria require reexamination in view of McMath’s unusual situation. The answers are essential to neurology, organ transplantation, and public policy.
In 1968 the medical community outlined specific neurologic tests that, if fulfilled, indicated a person was brain dead. This created an alternative definition of death, which has been accepted legally in every state. Since then a number of new diagnostic tests, including PET scans and MRIs, have been developed that measure brain activity more precisely. With such an important medicolegal issue at stake and with the older criteria likely to be questioned in McMath’s case, these new tests, while not used routinely in the diagnosis of brain death, may have an important future role.
The question of the exact moment life ends is conceptual—it can never be conclusively settled. Albert Einstein once observed that a single experiment could prove his most elaborate theory wrong. Likewise, the sad case of young Jahi McMath and her “telltale heart” have the potential to change the medical community’s understanding of brain death.
48
THE NEW PARADIGM OF ASSISTIVE TECHNOLOGY
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Any sufficiently advanced technology is indistinguishable from magic.
—ARTHUR C. CLARKE
WHEN HE WAS IN HIS EARLY THIRTIES, former National Football League (NFL) player Steve Gleason contracted amyotrophic lateral sclerosis (ALS), a motor neuron disease that robs people of muscle control and currently affects about twenty thousand Americans. As the disease progresses, it eventually leaves patients paralyzed but spares their eye movements and leaves their minds intact.
Because patients with advanced ALS cannot talk, they need help communicating, and Gleason, confined to a wheelchair, has been working with Microsoft to develop “assistive technology”: new tablet computers that use eye-tracking technology and speech-generating software. Gleason has described his personal progress, “I can do anything an ordinary person can do on a tablet computer—talk, videoconference, text, stream music, buy Christmas presents online, pay bills, tweet.” He and Microsoft are currently working on a wheelchair he can drive with his eyes. What they are doing was unimaginable a generation ago.
As Microsoft demonstrates with Steve Gleason, this is a chance for the Silicon Valley companies like Apple and Google to develop research capabilities and customized products for the health care market. But it doesn’t even take a giant like Microsoft to create revolutionary assistive technology. Through crowdsourcing, a small Israeli company is currently developing an affordable prototype device for patients with locked-in syndrome, a type of brain damage that resembles ALS. The device, an infrared camera connected to a pair of glasses, records the user’s eye movements and communicates them to an attached microcomputer. The computer translates those eye movements into programmed commands, which can then be transmitted via headphones, speaker, or smartphone. Once, paralyzed patients with locked-in syndrome had no one to speak for them; now with the right training, they may soon be able to “speak” for themselves.
In rehabilitation medicine, the use of computers, tablets, and smartphones is only now emerging. As the American population ages, there are more people surviving with strokes, Parkinson’s disease, multiple sclerosis, and other similar disorders who could participate in everyday activities by using these assistive technologies. This situation is not limited to the elderly; there are currently thousands of young patients with spinal cord trauma, brain trauma, or neuromuscular diseases who would likewise benefit from the latest hardware and software. For patients with neurologic diseases, vision problems, or other disabilities, these tools are esse
ntial to communicate or get around. In some cases, these devices may also be the patient’s only means of interacting with others and combating loneliness.
These patients often need customized devices because standardized equipment may not be right—tablet screens are often too small, and patients may not have the dexterity or strength to use smartphone keys. However, it is not simply a matter of machinery. Just as important, these patients need a well-trained therapist to help them since they may not have the experience with new technology or may be unaware of what the technology can actually do. Hospitals currently employ specially trained therapists to help people move (physical therapy), to aid people with breathing problems (respiratory therapy), help those with difficulty communicating (speech therapy), and return people to daily activity (occupational therapy). But with the advent of new, customized devices, there is a need for specialized therapists whose primary focus is to evaluate the specific assistive technology needs of patients with disabilities and help them use personalized technology. With the exception of a limited number of specialty facilities, few hospitals have such a therapist.
Especially for tech-savvy young people, all this could present a genuine opportunity for a prospective start-up career—assistive technology therapy. The call should go out to hospitals and universities to devise curricula and work with other therapists, teaching prospective assistive technology therapists about different conditions and the specific limitations those conditions present for patients, as well as the solutions available through emerging technology. Assistive technology therapists could then become part of every hospital, large clinic, rehabilitation center, and nursing home. Imagine a cadre of trained professionals working with disabled patients, custom-fitting them for the right device, and helping them learn (or relearn) not just how to communicate and move about but how to search the Internet, use social media, and even play video games.
Medicine’s early adaptation to the computer age was to develop devices like the electronic medical record, the computerized scanner, and the robot that can perform surgery. Although these innovations have been undeniable advances in patient care, they have all had the perverse effect of causing less interaction between caregivers and patients. The resident typing into the electronic record does not make eye contact with his patient; the consultant looking at the scan no longer performs a physical exam on her patient; the surgeon, once intimately involved, now operates with a robot at a distance from the patient.
But medicine can also use computer technology and still reverse this trend. In the future we need more assistive technology therapists, a critical first step to creating a new, personalized “high-tech, high-touch” approach. Combined with new devices and software, you will see miracles.
49
GOOGLE, GENE MAPPING, AND
A CHRISTMAS CAROL
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Are these the shadows of the things that Will be, or are they shadows of things that May be, only?
—EBENEZER SCROOGE IN CHARLES DICKENS’S A CHRISTMAS CAROL
SERGEY BRIN, a forty-four-year-old Russian émigré, comes to work in T-shirts, jeans, and sneakers. He revealed a secret about himself on his personal blog that may be important to you. With slicked-back hair and a fair complexion that make him look ten years younger, he could easily be mistaken for your typical Starbucks trainee. But a quick Google search identifies Sergey Brin as the fourth-youngest billionaire in the world and fifth-richest man in the United States. That quick Google search increased Brin’s fortune since he happens to be a cofounder of Google Inc.
The secret Brin told the world was that he has a genetic mutation that increases his chances of contracting Parkinson’s disease later in life. Parkinson’s disease, a progressive degenerative central nervous system disorder, currently affects 1 percent of people over sixty-five. The mean age of onset is between fifty-five and sixty (some are stricken much earlier). As it progresses it causes tremors, and patients experience difficulty with everyday activities, including walking, speech, and swallowing. Mr. Brin’s mother suffers from Parkinson’s disease, as did his late aunt.
Until recently, Parkinson’s was not thought to be heritable since there was no obvious pattern running in families. That thinking has been changed by genetic mapping, a technology that will soon be commonly available and inexpensive. Brin acknowledges that the implications of his results aren’t yet clear because it’s still uncertain which patients with the mutation will ultimately develop Parkinson’s. Studies suggest having the mutation does confer a much-higher chance of developing Parkinson’s for him than for the average person, though the exact probability is not known. In addition it is unknown whether the risk can be minimized or, alternatively, increased by cofactors such as diet, lifestyle, or environmental exposures.
The mutation was discovered when he took a mapping test of his DNA offered by a company called 23andME. (Coincidentally, 23andME was originally a partner to Google and was cofounded by Brin’s wife. Silicon Valley legend is that Brin and his partner started Google in her sister’s garage after leaving Stanford in the mid-1990s.) Brin said he didn’t intend to have his DNA mapped to check for a Parkinson’s risk but was glad to have the information.
Here’s why his secret is important. Brin has taken a proactive approach to the news.
I know early in my life something I am substantially predisposed to. I now have the opportunity to adjust my life to reduce those odds. I also have the opportunity to perform and support research into this disease long before it may affect me. And, regardless of my own health it can help my family members as well as others. I feel fortunate to be in this position. Until the fountain of youth is discovered, all of us will have some conditions in our old age only we don’t know what they will be. I have a better guess than almost anyone else for what ills may be mine—and I have decades to prepare for it.
Many people would, quite understandably, elect not to know they carry the genes of a serious disease that may manifest itself in the future. Yet Brin’s revelation about his personal DNA is a watershed moment in twenty-first-century medicine. With a personal fortune of billions of dollars, Google’s immense resources, and the support of the scientific community, he can explore the frontiers of medical genetics, including how diseases are expressed through genes, which patients will respond to specific medications or experience serious side effects, and whether there are ways to prevent the diseases patients are predisposed to—in effect, how to change their destiny.
Part of Sergey Brin’s quest is to change his own destiny. It is a timeless theme of literary and cinematic fiction. In classic Greek literature, Oedipus Rex was told his future but still couldn’t prevent his tragic fate. In contemporary fiction, heroes with extraordinary powers like Superman valiantly attempt to alter the future to rescue people. Now in real life, scientists will observe, and the rest of the world may benefit from Sergey Brin’s case history to see whether genetic destiny can be modified.
The plight of the young billionaire with slicked-back hair recalls that of a fictional wealthy man who was also shown a glimpse of his future, Charles Dickens’s elderly Ebenezer Scrooge. After Scrooge had his own ignominious end revealed in A Christmas Carol, he vowed to change his ways—and in doing so, he changed his future and that of those around him. Scrooge presaged Brin’s current efforts by musing, “Men’s courses will foreshadow certain ends, to which, if they persevered in, they must lead. But if the courses be departed from, the ends will change.”
50
I HAVE LOST MY MENTAL FACULTIES BUT AM QUITE WELL
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Young men be not proud in the presence of a decaying old man; he was once that which you are, he is now that which you will be.
—POPE CLEMENT III
WHEN ROBERT BYRD was ninety-one years old, he had been a senator for fifty years and was fourth in line of presidential succession. At ninety-two, Sumner Redstone was the executive chairman of CBS and Viacom, two of the world’s most powerful media companies. Fide
l Castro, supreme leader of Cuba since the 1959 revolution, was a relative youngster when he died at ninety. No one in the inner circles of these three powerful men publicly questioned their mental capacity to do their jobs until near the end of their lives. Their aides would be understandably reluctant to openly utter the R word, perhaps fearing involuntary retirement themselves.
But when he was seventy-five, another US senator, Pete Domenici (R-NM), announced he would not seek a sixth Senate term, in part because of a debilitating central nervous system disease that commonly causes behavior disorders and dementia. By doing so, Domenici avoided the experience of the late Strom Thurmond (R-SC), the longest-serving senator in history when he retired in 2002 at age one hundred. During his last years, Thurmond chaired the Armed Services Committee amid rumors he wasn’t always cognizant of the goings on around him. His final appearances consisted primarily of reading opening statements prepared by staff members, who nervously hovered close by.
This portends an impending societal dilemma: When people in positions of authority and responsibility can no longer function effectively, who will decide whether they should continue, and how? In the next three decades, several million Americans are projected to develop Alzheimer’s disease and related degenerative neurologic conditions similar to that of Senator Domenici.
These neurologic diseases and the demographics of aging baby boomers present a huge problem for both the private sector and the government. Everyone knows someone in the office who lingered in a prominent job as their faculties declined—the one who became the subject of lunchtime gossip and averted eyes. But it’s no longer just that person in the corner office, overly ego-invested in his job. There won’t be enough generous retirement packages for the huge demographic swath of those people on the horizon. Anticipate a spate of disgruntled workers, management struggles, lawsuits, as well as the sporadic incident of workplace violence when things really go haywire.
The Doctor Will See You Now Page 16