Illustration by Anuj Shrestha

When Lyme Kills

The extremely rare complication you should know about

This story is part of “Tickpocalypse,” a multi-part special report.

JJoseph Elone just felt tired at first, like he wasn’t sleeping well. A 17-year-old high-school student from upstate Poughkeepsie, New York, Joseph was a quiet but popular science whiz and electric-guitar lover who had just finished a summer environmental fellowship at Brown University. He’d spent two weeks studying on campus and hiking in the Rhode Island woods and was still riding high from the experience. It was late July, and life had a carefree feeling to it. “He was really happy that he had this chance to really open himself up,” says Joseph’s brother, Emmanuel. “He knew that as soon as senior year started he was going to apply early to Brown.”

Soon, however, Joseph developed cold symptoms — a cough, a sore throat, head and body aches, digestive problems, and a low-grade fever.

Poughkeepsie is located in New York’s Hudson Valley region, an area known for high rates of Lyme disease. Joseph had seen no signs of a tick, a tick bite, or the bull’s-eye rash that is often the telltale marker of Lyme, but he and his parents decided he should see his pediatrician in any case.

The doctor saw little reason for concern. Joseph likely had the flu, he said. He prescribed the standard regimen — rest, fluids, and the like — and suggested they give it time.

But a few days later, Joseph’s symptoms worsened. He was feeling light-headed and said he was sensitive to bright light. He returned to the same doctor, who ordered blood tests for strep throat, Lyme disease, and another tick-borne illness called anaplasmosis. The results were all negative.

Joseph and his family were aware that the antibodies indicating the presence of Lyme disease can take weeks to show up in tests, but even if Joseph were to develop the disease, they weren’t worried. Joseph was young and otherwise healthy, and Lyme is usually curable in a matter of weeks with antibiotics.

“It just seemed like he had a cold or something, or a little fever, but nothing crazy,” Emmanuel says. “It wasn’t something that you would take seriously in the moment.”

August 4, 2013, was a hot and hazy Sunday in Poughkeepsie. Diane’s 49th birthday was the next day, and the family had planned a barbecue that night. Late in the afternoon, Benedict and Emmanuel were getting the backyard ready while Joseph and Diane drove to the drugstore for cough drops. They had just come back, Diane walking toward the house ahead of Joseph, when she heard her son collapse behind her.

Joseph lay on the lawn, a few steps from the family’s front door, unconscious. Diane began screaming. The others came and started screaming, too — calling to Joseph and hugging him, but he didn’t respond.

EMTs rushed Joseph to a local hospital, where doctors spent several hours working to revive him. His breathing was shallow, his heartbeat erratic. Later that evening, he was transported 50 miles south to Westchester Medical Center. Not long after he arrived, in the early hours of August 5, just three weeks since he had started feeling sick, Joseph was pronounced dead.

It would take at least four months and several incorrect diagnoses to determine the cause of Joseph’s death, but ultimately, examinations revealed the presence of Lyme bacteria in several of his organs, including his heart.

The Elone family, devastated by Joseph’s loss, was shocked all over again. They had never heard of anyone dying from Lyme disease.

AAccording to the Centers for Disease Control and Prevention, the number of confirmed cases of Lyme Disease in the U.S. rose 131% in the two decades leading up to 2017 and 17% from 2016 to 2017 alone. More than 300,000 new cases are now diagnosed annually, and as climate change and other factors trigger a dramatic increase in the tick population, experts expect the disease to become more common still.

If Lyme is detected early, the standard two-week course of antibiotics usually works well, and most people recover quickly. But about 20% of those who contract the disease continue living with its effects for months after treatment. The corresponding diagnosis, Post-Treatment Lyme Disease Syndrome (PTLDS), stands to affect more than two million people in the U.S. by 2020.

There is also a very small subset of Lyme cases in which the disease proves to be fatal. Although such instances are extremely rare, they are uniquely devastating. They may also be underreported — and preventable.

Joseph Elone’s death in 2013 occurred the same year the CDC released a report of three other deaths related to Lyme. The discovery of the four cases, a small but nevertheless unusual number, led to further inquiries that uncovered numerous additional instances in New York state that had previously gone largely unnoticed, leading experts to wonder if Lyme deaths might actually be more common than previously thought. The new focus on Lyme deaths also shined a light on a cause of the fatalities called Lyme carditis, raising questions about doctors’ awareness of that condition, and the screening procedures used to detect it. Advocates say those questions have yet to be resolved.

“One preventable death is one too many.”

Timothy Sellati, a veteran Lyme researcher and the chief scientific officer of the Global Lyme Alliance, which funds research and awareness programs, says the number of deaths associated with Lyme carditis are underreported. His group and others have begun calling for revised methods of diagnosis to detect the condition earlier. While the overall number of deaths from Lyme disease remains small, “One preventable death is one too many,” he says.

GGiven the absence of swift and reliable testing to detect Lyme disease, confirming its role in any death is a slow process. At first, all experts could say was that something had weakened Joseph Elone’s heart, compromising its function. The cause of death initially recorded was myocarditis (an inflammation of the heart muscle) and meningitis (a bacterial infection). Another tick-borne illness, Powassan encephalitis, was also suspected at one point.

It was only in December when reports surfaced that tests had found Lyme spirochetes, or bacterial residue, in Joseph’s heart tissue. Sure enough, in March 2015, a prominent medical journal confirmed that Joseph’s liver, heart, lung, and brain tissues all contained evidence of Lyme. The official cause of Joseph’s death was Lyme carditis.

Lyme carditis occurs when Lyme disease bacteria invade the tissues of the heart, disrupting electrical signals between the organ’s upper and lower chambers. That, in turn, causes an abnormal heart rhythm and a problem known as “heart block.” Heart block can be mild, but it can also become serious very quickly. Patients typically experience many of the symptoms characteristic of Lyme disease — fatigue, coughing, headaches, and the like — plus cardiovascular or pulmonary symptoms like light-headedness, fainting, shortness of breath, heart palpitations, or chest pain. Like Lyme itself, the condition is generally treatable if detected early. (Depending on the severity of the case, people are given oral or intravenous antibiotics; in some instances, they may need a temporary pacemaker.) But if Lyme carditis is not treated in time, it can be fatal.

According to the CDC, Lyme carditis occurs in approximately 1% of Lyme cases reported to the agency; in one 2008 epidemiological study, the condition was named as the main cause of death among patients who die from Lyme disease. A 2009 study of 207 pediatric patients with Lyme disease found that 16% had Lyme carditis. All three of the additional deaths cited in the CDC report issued the year Joseph died were linked to the condition.

The other three victims were two men and one woman, the youngest 26, the oldest 38. In November 2012, one of them, a Massachusetts man, complained of malaise and muscle and joint pain in the weeks before being found behind the wheel of a car after it veered off the road. In July 2013, a New York state corrections officer who had also worked as a landscaper was hanging laundry on his front lawn when he clutched his chest in pain, staggered, and dropped to the ground. He had experienced chest pain on and off for at least several days, his wife later said, but had not seen a doctor. That same month, just a few days before Joseph’s death, a Connecticut resident collapsed after more than a week of complaining of occasional shortness of breath and anxiety. In all three cases, Lyme carditis was found posthumously.

When Joseph Elone became known as the fourth case of death from Lyme disease in the space of a year, reporters from the Poughkeepsie Journal combed through 1.2 million death certificates from non-New York City counties in New York state searching for more fatalities that might be linked to Lyme. They found nine Lyme-related deaths over 13 years. Not all of those cases involved Lyme carditis, and the details of each weren’t analyzed.

The CDC has officially identified just nine cases of fatal Lyme carditis in the United States between 1985 and 2018, but if one newspaper can find nine Lyme deaths in one state in a shorter period of time, some experts believe the real number may be higher.

“I think they are underreported,” says Renu Virmani, a cardiac pathologist who has studied Lyme carditis deaths and runs a cardiac research organization in Maryland called CVPath Institute. Her suspicion, she says, is based on the fact that Lyme carditis occurs in one out of every hundred Lyme cases, but medical examiners rarely consider testing people who die from sudden heart failure for Lyme. “Nobody thinks of doing that until after you made the diagnosis,” she says. The only deceased patients who are tested for Lyme posthumously are people who tested for Lyme while they were still living, she says. And since it is well-established that many Lyme cases go undiagnosed while the person is still living, it stands to reason that at least some fatalities may happen with no one knowing.

“It’s very unusual for a medical examiner to make the diagnosis of Lyme disease,” Virmani says. “Very unusual. And I’m sure there are people who die of Lyme disease.”

LLyme carditis isn’t the only source of Lyme-related deaths. People with underlying health conditions can be overcome by Lyme disease, and suicide is a risk for PTLDS patients who become severely debilitated or depressed. But those risks are relatively well-known.

Lyme carditis, on the other hand, often goes overlooked. The condition is so rare that most people and doctors are barely aware of it, if they’ve even heard of it at all.

After Joseph Elone’s death and the report of the three other Lyme carditis deaths in the same year, officials in Duchess County, New York changed their public health advisory to doctors weighing Lyme as a cause of illness. Now physicians are told to ask about cardiac symptoms in possible Lyme cases and to check cardiac cases for possible exposure to ticks and symptoms of the disease. (Forty-two percent of the pediatric Lyme carditis patients identified in the 2009 CDC study had advanced heart-function problems.)

Lyme prevention advocates want standards like those adopted universally. Doctors in the United States currently follow guidelines for Lyme diagnosis set forth by the Infectious Disease Society of America, but a newly formed group of physicians called the International Lyme and Associated Diseases Society has developed a more aggressive screening approach. It involves more than one blood test, and faster treatments for patients seen to be more at risk, and above all a set of criteria that encourages doctors to move more quickly based on their own professional judgment, even when tests come back negative. Under the older IFDS guidelines, advocates say, some doctors felt penned in by the criteria, concerned about professional blowback for treating cases in which there is no confirmation by a blood test or no classic bull’s-eye rash.

“I think the problem with the IDSA guidelines is they operate from the notion that all patients are going to respond to antibiotics the same way and a relatively short course of antibiotics will cure all patients,” says Sellati of the Global Lyme Alliance. Such a rigid, uniform approach may not permit doctors to be more aggressive when they believe the circumstances warrant it. The new recommendations issued by ILADS, Sellati says, are more nimble. “They want to provide more deference to the physician’s experience, especially if they see a lot of Lyme disease patients. Using a combination of their experience, seeing large numbers of patients, and the broader symptoms that could be associated with Lyme disease, they’re allowed to sort of think outside the box.”

He suggests parents not be alarmed about Lyme carditis, but to be aware of it, “especially if a patient has other heart abnormalities, like congenital heart disease or other things that could impair their heart’s function.”

AAfter his son’s death, Benedict Elone called for increased awareness of Lyme’s fatal potential and for more to be done to prevent Lyme deaths. “Be it Lyme or be it Powassan — people need to know that it can kill a strong, physical guy,” he told reporters. “There is something in our environment that can kill somebody that fast. We really need to know about it, study it, and find some solutions.”

In front of the TV cameras, he spoke lovingly about his son. “He’s a kid who knew who he was,” Benedict said. “He had all kinds of ambition. Teachers couldn’t stop speaking well of him.”

And then he dissolved into tears, repeating the same phrase again and again. “I just miss my son,” he said. “I just miss my son. I just miss my son. I just miss my son.”

This story is part of “Tickpocalypse,” a multi-part special report.

Author of the #1 NYT best-seller “Hidden Valley Road,” an Oprah’s Book Club selection, now available in paperback from Anchor Books.

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