Lyme

by Mary Beth Pfeiffer

  Joseph: Opportunity Missed

  Diane and Ben Elone were proud, doting parents. They had two boys who were honing talents and pursuing dreams in a hardscrabble city on the Hudson River north of New York, where being a young black male was a challenge all in itself. The boys, a year apart, attended a high school in which 81 percent of students were impoverished and 39 percent failed to graduate. No matter. Diane, who worked in medical billing, and Ben, a thirty-year civil servant, were determined that would not be their children’s fate. Emmanuel would go on to Georgetown and study in Europe. Joseph would sign up for a science program in which he caught, weighed, and released slithering eels at a local stream; he would attend a “summer scholars” program at Vassar College, a mile from his modest Cape Cod home on a tree-lined street in Poughkeepsie, New York. Usually reserved and quiet, Joe was jubilant after the program wrapped up, heading to a local hamburger joint where he and some friends spent several hours reliving the experience.

  By his junior year, Joseph, seventeen, had found a calling of sorts. A boy who was often photographed outdoors—knapsack and black-frame glasses on, the majestic Hudson Valley unfolding behind him—Joseph wanted to protect the environment. That led to two weeks at ecology camp just after school let out in June 2013. He came back on July 5th more committed than ever. Then the problems began. Joseph developed what was initially seen as a “viral syndrome”—a sore throat, cough, and fever. He was tired and had body aches. Twice he went to the pediatrician. “They checked him for strep throat. He didn’t have strep throat,” Ben Elone said later, sitting at his kitchen table, Emmanuel by his side. “They checked him for Lyme disease; he didn’t have that…The doctor figured that the coughing would stop. He just needed to drink a lot and take a lot of rest.”

  The clues, to be sure, were there. Just before he got sick, Joseph Elone had spent two weeks, largely outdoors, in Rhode Island, which then ranked seventh among states for Lyme disease in America, rising to fourth the following year. He lived in a county with a Lyme disease rate in 2013 that was four times that of sky-high Rhode Island’s. His symptoms had appeared in prime tick season, when summer flus should be viewed suspiciously. Some of his symptoms—the malaise, the fever, the achiness—suggested Lyme disease.

  But Joseph had no rash, at least not one that was seen against his dark skin, and his other symptoms suggested an upper respiratory tract infection. Moreover, his test had come back negative, falsely so as it does in more than half of early cases. The doctor merely followed the Lyme disease guidelines of the Infectious Diseases Society of America (IDSA).

  At three different points, as I reported in chapter 6, these influential directives advise physicians not to treat potential Lyme disease in people without a rash who do not test positive. Symptoms simply “are too nonspecific to warrant a purely clinical diagnosis,” the guidelines say, in cases involving neurologic, arthritic, and cardiac symptoms. When I asked if the CDC concurred, a spokesperson referred me to this sentence in the IDSA guidelines: “Erythema migrans is the only manifestation of Lyme disease in the United States that is sufficiently distinctive to allow clinical diagnosis in the absence of laboratory confirmation.” No rash, no positive test, no diagnosis. And so, for Joseph Elone, who lacked the tell-tale rash, no antibiotics were prescribed.

  Twelve days after his last doctor’s visit, Joseph needed cough drops—the doctor had said to try some—so his mother took him to a nearby pharmacy. His symptoms had persisted; his records state “he had developed diarrhea and lightheadedness and was reported to have photophobia,” or sensitivity to light. The Elones were planning a third doctor’s visit. Instead, just after returning home, Diane Elone looked out onto her small front lawn, with a large tree on each side of a walkway to the front door, and saw her youngest son lying face up. He wasn’t breathing. Emergency medical workers resuscitated him there. In the ambulance on the way to a hospital, Joseph’s heart stopped twice, and twice he was brought back. At the hospital, his pupils reacted to light, a good sign, but his other indicators were not good. His kidney and liver function were abnormal. His white blood cell count was high. A nurse suggested, not unusual for Poughkeepsie, that this young black man, unconscious and breathing shallowly, “must have taken something.” But his urine was clean. What Joseph had in his body were Lyme disease spirochetes. They were in his lungs, in his brain, and, most significantly, in his heart.

  In a helicopter on the way to a tertiary-care hospital several hours after his collapse, Joseph’s heart began beating wildly and attendants used electrical currents in futile attempts to reset it. He arrived at the hospital unresponsive, on a ventilator and with a pulse that went from intermittent to none at all. He died a little after midnight on August 5, 2013. A few days later, Joseph Elone’s second test for Lyme disease came back. It was positive.

  In 1997, an article in the Archives of Internal Medicine reported on fourteen cases of a Lyme-like rash in children at a summer camp in central North Carolina; eight of the campers had recalled tick bites. Near the end of the article the researchers reported, in a reference so passing that it appeared in parentheses, that one of the fourteen campers had died, from tick-borne ehrlichiosis. The victim was twelve years old. In 2017, a plethora of ticks were discovered at a day camp in Lake Forest, Illinois, located in a wooded ravine along Lake Michigan. Officials thought it wise to move the camp to a suburban park. Imagine how many other camps in tick-riddled natural areas chose to operate in their usual fashion, with a few perhaps, many hopefully, doing tick checks, educating campers on tick avoidance, and aggressively responding to reports of achiness, fevers, and odd rashes. A college senior who helped with my book contracted Lyme disease a decade ago at one such camp, where her symptoms were not quickly recognized. It changed her life.

  After Joseph’s death, I studied records of 1.2 million deaths in New York over a thirteen-year period; I found that just nine had been attributed to Lyme disease even though regions of the state have among the world’s highest rates of the illness. When I asked the IDSA to comment on my findings, a spokesperson pointed to a study that suggested that fewer, not more, people had died from Lyme disease than were being reported. The study, authored by researchers at the Centers for Disease Control, looked at 114 US deaths that had been officially attributed to Lyme disease from 2009 to 2013, of people aged nineteen to ninety-nine. Just one death, it concluded, was “consistent with clinical manifestations of Lyme disease.”

  In death as in life, late-stage Lyme disease is a controversial diagnosis, one that is disputed and discounted in ways that are not designed to ferret out truth. The CDC study looked merely at the coding of death certificates—some were incomplete, others deviated from procedure—and not at actual medical histories. These surely would have added insight. The study results were published as a “brief report” in the IDSA’s Clinical Infectious Diseases journal, indicative of the effort that went into it. Beyond this, pathologists have not been encouraged to look for evidence of the tick-borne toll in postmortem studies that could shed light on disease progression. No registry exists to investigate suspect tick-borne deaths like the young camper’s, or like three other North Carolina children and a two-year-old Kentucky boy, all who died from tick-borne Rocky Mountain spotted fever, their deaths only incidentally reported in the literature. Beyond sudden cardiac deaths like Joseph’s, there are Lyme deaths from entrenched, systemic infection and from suicide, among them a thirty-three-year-old woman who stepped in front of a train in Wisconsin in 2014 after every avenue was closed to her. We simply do not know how many die from Lyme and tick-borne disease because no systematic effort has been made to look.

  Kara: Search for Cure

  Kara Wilson missed eight years of school to Lyme disease, and instead was tutored, read to, and engaged in other ways at home. Each year her parents dutifully did her hair—long and curly some years, straight and short in others—and dressed her up to take school-style portraits, the kind that document childhood. Kara’s cheeks are f
ull and rosy in the second grade. Over the years, the teeth grow but the facial structure seems locked in place. Her neck is lined; her eyes hollow. “You see a healthy, vibrant six-year-old,” she told me, “and you see the decline into death.”

  In their search for a cure, the Wilsons took Kara to eleven pediatricians, assorted other specialists, and a hospital in California. They fled to a natural therapy clinic in La Gloria, Mexico, one step ahead of child protective services. It was at an integrative practice in Las Vegas, Nevada, where she arrived barely cognizant and in diapers, that Kara was diagnosed with Lyme disease in 1989. She was thirteen, and had been sick for six years. From there, the couple exhausted the possibilities of antibiotics for their daughter. She received electro-magnetic therapy, acupuncture, and homeopathic treatments. Some of those treatments, on both sides of the US border seemed to help, at times startlingly so, but they did not cure.

  Then in 1992, the Wilsons signed Kara up for what became more than 150 sessions in a hyperbaric oxygen chamber, twice a day, six days a week for three months. The treatment, accepted today for control of serious infections and stubborn wounds but not for Lyme disease, delivers pure oxygen in a pressurized chamber, enriching the blood and prompting the killing of pathogens. Kara was part of a Texas A&M University study, designed by a researcher named William Fife to attack Borrelia burgdorferi. All ninety-one enrolled patients were positive for Lyme disease (though possibly not by CDC standards), and all had failed to get well with antibiotics. In 1998, after six years of trials, Fife reported that more than 80 percent of his patients improved significantly or were symptom-free. For Kara, it was the end of a long road. Then sixteen, her body may not have been restored, but her health was beginning to return. And so was her potential.

  Shortly after her treatments, Kara, still unable to walk, went with her family to a rodeo. She suddenly realized, her fused fingers, calcified ankle, and weakened bones notwithstanding, I can ride. She bought a saddle on the spot, was tied to a family horse and off she went. “I ripped the Band-Aid off,” she said, “and got back into it.” Two years later, at eighteen, she graduated as valedictorian of her high school class, having clawed her way back from infirmity. She still rides horseback almost daily, often taking visitors onto the high desert range. Kara Wilson is nothing if not a survivor.

  Bound to a wheelchair that does not much limit her, Kara told me her story in the midst of a roaring January snowstorm that had her worried for the ranch’s four hundred head of cows. Deep snow is treacherous to cattle, every one of which needs forty acres to survive. Kara lives on and loves the ranch as her ancestors did; she runs the Wilson bed and breakfast where, over the years, she has told her story to thousands of guests. In 1999, Kara graduated from Texas A&M, a popular and beloved student. In 2000, she went down the aisle, in a wedding under a tent, on a bay roan quarter horse named Dynamite, her father on another horse beside her, and married a student she had met when she needed help getting on and off her horse. In attendance were 650 guests, including five well-meaning people who had reported the Wilsons for child neglect. In 2002, Kara and her husband, Brian, adopted a boy and a girl from Russia, where Muscovites in Red Square took pity on Kara in her wheelchair and dropped rubles in her lap.

  Kara Wilson Anglin won her battle to survive, though in a changed and damaged body, in a way that Lyme disease patients occasionally do. Through the advantages of her birth, the fierce determination of a mother who rejected any hint of surrender, and a set of parents who fought off the sheriff and mainstream medicine to find what worked for their daughter.

  There are infectious disease physicians, those who wrote the prevailing Lyme disease guidelines and those who accept them, who will read her story and be doubtful. They will say it was not Lyme disease that laid her so low. Even if it did, they will say, there’s no proof that the treatment she received works. Perhaps. The problem is we have not studied what does work. There has not been a US clinical trial for Lyme disease care published since 2008, and the four done to that point were limited to three months of antibiotics. They did not consider the role of other pathogens carried by ticks. They did not try anything but two selected antibiotics, while Lyme specialists who deviate from the IDSA guidelines use a wide variety, sometimes in combination. The National Institutes of Health has spent an average $24 million a year since 2012 on Lyme disease funding, with nearly 400,000 cases in 2015, and, by contrast, $40 million on mosquito-borne West Nile Virus, with 2,000 cases. That’s a woeful picture of underfunding. Even the CDC and IDSA acknowledge that some people—from 10 to 20 percent, though figures vary—do not get well after treatment for Lyme disease. It is time to recognize a problem exists, and find out what does work.

  Troy: A Disease of the Brain

  “He’s not walking. He can’t sit up. He can’t hold his head up. He acts like he’s autistic or something.”

  —Alison Murphy, on her son Troy, 11.

  That Lyme disease sometimes infects the central nervous system and brains of children, and adults, is not in doubt. Starting in the early 1990s, Brian Fallon and researchers at Columbia University had shown that children with long-standing Lyme disease suffered “significantly more cognitive and psychiatric disturbances,” including behavioral, anxiety, and mood disorders. Tourette’s syndrome, depression, bipolar disorder, attention deficit disorder, and dementia have all been associated with Lyme disease—and sometimes have improved with treatment. The physical manifestations of neurologic Lyme disease are equally varied: headaches, numbness and tingling, facial palsy, dizziness, unsteady gait, hypersensitivity to stimulation, and fatigue. This is a disease that affects both physical and mental health.

  Within months of his twin bouts of flu at age ten, Troy Murphy had regressed in every way possible. He could no longer walk. He wasn’t speaking. He could not eat. Neurologist Elena Frid looks at children like Troy, who was her patient, and sees brains on fire from tick-borne disease. The mechanics of this dynamic aren’t fully clear, and cases like Troy’s are not common. But the effects of late-stage Lyme disease have been documented in living color brain imaging called Single Photon Emission Computerized Tomography, or SPECT, scans. In a study of 183 patients with long-standing Lyme disease, published in the journal Clinical Nuclear Medicine in 2012, 75 percent had “discernible abnormalities” in the frontal, temporal, and parietal lobes of the brain. Here was something rare: what three physicians led by Sam Donta of Boston University Medical Center called “objective evidence” of damage to the brain in late Lyme patients.

  Columbia researchers, similarly, used SPECT scans in 2009 to show how white matter in the brains of eleven Lyme patients had been starved of blood and therefore oxygen, fogging memory and sapping the ability to do everyday tasks. Ben Luft is a physician-researcher who was an author of the IDSA’s Lyme guidelines in 2000, but who long ago broke ranks with IDSA over its dogmatic view of Lyme care. Fallon’s Columbia team, he told me, unprompted, showed “a whole host of different abnormalities” that, nonetheless, traditionalists have ignored. “It’s current, it’s contemporary, it’s cutting-edge,” he said. “It hasn’t been embraced.” In a world in which IDSA tenets rule, doctors do not acknowledge symptoms like Troy’s as physiological. Instead, he had schoolitis. Donta, similarly, comes from the other side of the Lyme divide. Also on the 2000 IDSA panel, he refused to go along with the finished guidelines in the belief they did not accurately reflect late-stage Lyme disease. As a consequence, his subsequent research, while peer reviewed, is dismissed by the IDSA side.

  In 2016, a German researcher named Rick Dersch looked at all the studies—six of them—on treatment of children whose central nervous systems and brains had been infected with Lyme disease. The findings of the various publications were not informative. Studies were old, few, hampered by research biases, and confined to short treatment regimens. “Data is scarce and with limited quality,” Dersch and his team reported in the journal BMC Neurology. Of great concern, the existing research was limited to treatment
in the early stages of neurologic Lyme, when it is easiest to resolve. Tell us something we don’t already know. As for cases like Troy’s, there are no treatment studies to guide and inform physicians, and moreover no incentive to perform them. Both funding and urgency are in short supply. Yet the potential effect and toll of tick-borne disease on the brain is only growing.

  In early November of 2016, a five-month-old boy ran a fever and began to vomit. The right side of his face took to twitching, and seizures set in. The boy, like Troy from Connecticut, had been bitten by a tick, this one armed with Powassan virus, a deadly pathogen transmitted in as little as fifteen minutes of attachment. Five of fourteen victims hospitalized in New York died within months, according to 2013 study in the journal Emerging Infectious Diseases, their health “severely impaired” by the infection. The boy fared better. Hospitalized and treated, he was, by ten months, meeting the milestones of infancy—babbling, crawling, walking with help. But there were indications that the virus had left its mark, as it does in half of cases. Brain scans showed he suffered a loss of brain tissue, called encephalomalacia. A form of calcification was also seen in a part of the baby’s brain called the basal ganglia, a change usually detected only in scans of the elderly.

  Discovered in 1958 after the death of a five-year-old Canadian boy, Powassan has been growing in recent years, showing up in states beyond the US Northeast, like Tennessee and Minnesota, and at unexpected times of year. The Connecticut boy had been bitten in the month of November, an outgrowth of increasingly balmy autumn weather, his case a harbinger. By 2013, the virus was reported in mammals in Alaska and Siberia, what a paper in Emerging Infectious Diseases called “rapidly changing Arctic environments.” As blacklegged ticks spread on the heels of a warming climate and other environmental changes, so will Powassan, a Yale University expert on tick-borne disease, Durland Fish, predicted in 2015. “Until a few decades ago, it was only transmitted by a tick species that does not commonly bite humans,” Fish said in an interview with Yale News. No more. “This recent change in the ecology of Powassan virus has caused concern within the public health community.” In 2017, Powassan was reported in up to 5 percent of blacklegged ticks in Wisconsin and New York, while Connecticut deer with antibodies to the virus rose precipitously, from one in four in 1990 to nine in ten in 2009. Like that other new bug, Borrelia miyamotoi, Powassan can be passed from mother tick to her thousands of babies. That makes larvae infectious from their first blood meal and potentially spreads the pathogen to the mammals—mostly mice but maybe people—they tap first.