Hippocrates Magazine – July/August 1987

 

Raggedy Ann Syndrome: What is the mysterious ailment that knocked the stuffing out of this Nevada  resort community? And where else is it striking?

 

By William Boly

 

INCLINE VILLAGE on the north shore of Lake Tahoe presents itself to the

first-time visitor as a high-altitude Carmel-by-the-Sea, a muted symphony of

cedar and redwood A-frames, upscale condos, and lakefront mansions tucked among

imposing stands of ponderosa pine. For the adult at play, the village offers two

Robert Trent Jones golf courses, an in-town ski lodge, the stylish Hyatt Lake

Tahoe Hotel and Casino, and an assortment of private beaches and boat docks. It

is, by design, a resort community for the well-to-do, dependent for its

prosperity on vacationers and rising property values.

 

Two years ago something happened to Incline Village that was decidedly not part

of the area’s master plan. A mysterious illness struck the town. The sufferers

felt as though they had come down with mono or the flu – sore throats, swollen

lymph glands, aching muscles – only the symptoms wouldn’t go away. For month

after month. One woman said the overwhelming fatigue made her feel like Raggedy

Ann without the stuffing. Soon many of these people began to display a

bewildering array of neurological complications: weakness in the limbs or

partial paralysis, black- outs, vivid nightmares, spatial disorientation, memory

loss. A patient driving into the business district, which has only three

intersections, couldn’t figure out how to get home.

 

“I was afraid I’d be ostracized or run out of town with a scarlet EBV on my chest.”

 

Word of the puzzling epidemic was received in Incline Village about as

cheerfully as might have been, say, a great white shark attack. “Nobody wanted

to hear that you were sick,” says an area resident with the disease. “If they

believed you they didn’t want to get near you; and if they didn’t they said you

were ruining the economy.’* By the summer of 1986 the illness had a name –

chronic Epstein-Barr virus syndrome – and a gathering national reputation. Much

to the dismay of local realtors, ABC TV’s newsmagazine 20-20 came to town. Chris

Guthrie, a young mother with both the illness and fresh good looks to provide a

sympathetic focus for the report, remembers going to the local supermarket at

the time. A stranger stopped her in the checkout line to warn her that she was

going to ruin business around Lake Tahoe and had no right to appear on network

television. “I just felt devastated,” she says. “I was afraid I would be

ostracized or run out of town with a scarlet EBV on my chest. But my strongest

feeling was, if I could help one person out there who was going through what I

had, it would be worth it.”

 

Patients with chronic Epstein-Barr virus syndrome are great ones for taking

their story to the media – with good reason. Until recently the medical

profession by and large refused to recognize their complaints. Sufferers were

written off as hypochrondriacs, given a tranquilizer, told to sit in a closet or

have an affair. Many now feel vindicated because a specific virus has been

nominated as the cause of their troubles.

 

Ironically, however, top researchers in the field are pulling back from the

Epstein-Barr terminology even as the media has come to accept and widely

disseminate it. More accurately, researchers say, they have identified a

widespread “chronic mononucleosis-like illness” in adults that is sometimes

accompanied by high levels of antibodies to the Epstein-Barr virus in the blood.

Whether Epstein- Barr is the cause of the syndrome or a symptom of a more basic

problem – perhaps a new virus on the loose – has not yet been sorted out.

Indeed, just two things appear certain at this stage. First, like pneumonia in

the 19th century or leukemia in the 20th, chronic mono may well turn out to have

several different causes. Second, for an illness with virtually no medical

standing only a few years ago, quite an astonishingly large number of people

suffer from it.

 

How many? The federal government’s Centers for Disease Control doesn’t know and

only recently started planning a surveillance system to find out. “The CDC took

the position very adamantly until 1986 that no such disease existed,” says Ted

Van Zelst of Minann, Inc., an illinois-based foundation that has championed the

cause of chronic mono patients in congressional testimony. The most conservative

view is that there are many thousands of sufferers. But based on the north Lake

Tahoe epidemic, in which at least 200 of the 20,000 residents became ill, some

experts say one percent of the nation may be afflicted – more than 2 million

Americans. Reports from physicians in New York, Boston, Atlanta, Denver,

Houston, and Los Angeles suggest that a low-level epidemic is quietly surfacing

all over the country. Commercial labs describe the growth in demand for the

Epstein-Barr blood test with words like “astronomical.”

 

What all this means is that chronic mono is bidding fair to become, as Newsweek

magazine recently headlined it, “the malaise of the 80s.” A comparison with AIDS

is probably inevitable – both maladies attack the immune system, trigger a wide

variety of symptoms, and are newly recognized on the epidemiological scene. But

the differences are more pronounced. For one thing, AIDS kills; chronic mono, as

one patient put it, “just makes you wish you were dead.” For another, the

demographics are nearly photographic negatives of one another. Whereas AIDS got

its foothold among homosexual men, intravenous drug users, and Haitian

immigrants, the chronic Epstein-Barr virus syndrome seemed to show an early

appetite for the conventional upper middle class. Two out of three victims are

women. Adults in the physical prime of life are more susceptible than any other

age group. At the outset, doctors treating the disease actually called it the

Yuppie Plague because of its apparent preference for well-educated, ambitious

professional people.

 

Like Sandy Schmidt. Community leader, sports enthusiast, and manager of her

husband’s estate planning business in Incline Village, Schmidt had just finished

running the San Francisco marathon when the illness caught up with her in July

1985. “At first I thought it was post-race fatigue,” she says. “But it was so

exaggerated. I was sleeping 15 hours a day and getting worse. I’d try to stay

up, but just couldn’t concentrate or think or work.”

 

Joyce Reynolds, a bank teller in north Lake Tahoe, contracted the disease more

than two years ago. She has become, in her own words, “a total recluse.” Her

children no longer visit because they are afraid of catching the bug. Even as

simple a thing as going to the movies with her husband can land her in bed for

days.

 

According to a recent poll of chronic Epstein-Barr sufferers, 40 percent have

been forced to leave their jobs or schooling. Marriages fall apart. Depression

is a common complication. To these stresses add the unsympathetic skepticism of

much of the medical community. “Massively overdiagnosed,” “a vogue disease, like

hypoglycemia,” and “wastebasket diagnosis” are just some of the professional

judgments that have found their way into print.

 

These days, however, the debate is switching from whether such a syndrome

exists to how extensive it is and what causes it. Events at Incline Village

helped bring about the change, partly because of some persuasive original

research done there and partly because of the sheer melodrama of the situation.

“It was such a setup,” says one scientist who has studied the outbreak, “this

beautiful paradise of a tourist resort beset by a lurking evil.”

 

The struggle for recognition of chronic mono as a new and possibly widespread

illness is a story of medicine at the ragged edge of the known and the unknown.

And it all begins with a pair of country doctors convinced that something

extraordinary was happening in their practice and stubbornly determined to find

some answers – long after prudence dictated looking the other way.

Dan Peterson was the first internal medicine specialist to set up shop in

Incline Village following the construction of a new hospital there in the early

1980s. As the practice flourished he was joined by Paul Cheney, a lanky,

fair-haired man with a doctorate in physics from Duke, a medical degree from

Emory, and all the intellectual intensity such a combination implies. Young,

intimidatingly bright, and highly trained, the two were building the power-

house practice in town, as other physicians in the area were well aware.

When a few patients with a difficult-to-treat flu began straggling into their

offices in the fall of 1984, Peterson and Cheney didn’t think much of it. “We

were convinced these people had a viral disease,” Cheney recalls, “so we were

very surprised when they didn’t get well, like we kept telling them they would.”

The most obvious diagnosis for patients with swollen lymph glands, aching

muscles, sore throat, and disabling fatigue would be acute infectious

mononucleosis. But the classic blood test for mono, which measures a clotting

reaction, came up negative for almost all the patients. Furthermore, adults

rarely get mono; yet here were dozens of people in their 30s all with the

symptoms at the same time.

 

A list of more obscure alternatives was worked up, screened, and systematically

discarded, including the possibility that what the doctors were seeing was all

in the patients’ heads. “These were people with nothing to gain by being sick,”

says Cheney. “Some of them had been coming in for regular checkups for years. We

knew them as productive, happy, vigorous adults. All of a sudden they got sick

and wouldn’t get well. In some cases they were sweating so much at night their

spouses had to get up and change the bed sheets. That’s just not how

psychosomatic illness looks.”

 

Baffled, Peterson and Cheney began searching for clues in the scientific

literature. Almost immediately they ran across an intriguing pair of articles in

the January 1985 Annals of Internal Medicine. The papers were written by Stephen

Straus, head of the medical virology section of the National Institute of

Allergy and Infectious Diseases in Washington, D.C., and James F. Jones, now

with the National Jewish Center for Immunology and Respiratory Medicine in

Denver. They described several dozen patients who had been referred for

recurrent or persistent illness characterized by chronic fatigue, fever,

headaches, and depression. In both groups the vast majority of patients had

elevated levels of antibodies to the Epstein-Barr virus.

 

Recognizing their patients’ symptoms, Peterson and Cheney ordered the

Epstein-Barr blood tests, which had just become available from commercial labs.

Test after test came back showing high levels of antibodies to the virus, some

very impressively so. “We thought, ‘Aha! we’ve got a bunch of chronic

Epstein-Barr virus patients,’ ” recalls Cheney. But having a name for it didn’t

solve the problem. By June their first patients were no better, and as many as

15 new cases a week were being diagnosed. People from the adjoining towns of

Truckee and Tahoe City were coming in. Soon a pattern of contagion was evident:

a third of one high school faculty, an entire girls’ basketball team, members of

the Hyatt casino staff. “We were horrified,” says Cheney. “We felt like we were

in a nightmare that wouldn’t end. So I did the only thing I knew how to do: I

called the place that’s supposed to figure this kind of thing out.”

 

The Centers for Disease Control in Atlanta is the federal government’s frontline

force against serious public health problems like the AIDS epidemic. Over the

years the agency has also had to check out a lot of false alarms. Keeper of the

gate of medical probity against the latest fad diagnosis, the CDC was skeptical

of the chronic Epstein-Barr virus syndrome from the start – and not without good

cause. As any medical student knows, the virus isn’t supposed to cause the

pattern of illness the people in Tahoe were experiencing.

 

Epstein-Barr is a member of the herpes family of viruses, which brings

humankind chicken pox, cold sores, genital lesions, shingles, and other

ailments. The viruses are tricky. They have a habit of hiding out in the body in

a latent state, then popping back on the attack. The patient experiences an

unpredictable waxing and waning of symptoms. Even in this elusive company,

however, the Epstein-Barr virus stands out as the most eccentric and fascinating

herpes of them all.

 

The key epidemiological fact about the virus is that it shows up everywhere.

Blood samples collected from isolated tribes in the Amazon rain forests have

proved free of measles antibodies but positive for Epstein-Barr. The virus is

easily passed along in saliva, which explains its ubiquity. Oddly enough,

however, the consequences of contracting it vary dramatically from place to

place and culture to culture. In the United States and Western Europe, the virus

causes infectious mononucleosis, that familiar bane of adolescence and college

years. In Africa it is involved with Burkitt’s lymphoma, a fast-growing tumor of

the jaw. Burkitt’s, which affects young boys, looks like a grotesque

exaggeration of mumps, and without treatment promptly leads to death. In Asia

the same virus has been linked to cancer of the nose cavity and roof of the

mouth, especially in older men.

 

The question epidemiologists have long asked is: With the Epstein-Barr virus

distributed throughout the world, why does it lead to such distinct, narrowly

targeted illnesses in various populations and localities? Part of the answer

lies in the company it keeps – what researchers call its causal co-factors.

Burkitt’s lymphoma, for example, appears in a belt across central Africa marked

by low elevation, high rainfall, and warm temperatures – the precise boundaries

of mosquito country. The co-factor allowing the virus to trigger Burkitt’s

lymphoma seems to be a weakening of the immune system brought on by constant

exposure to malaria and yellow fever. The virus’ ally in nose and mouth cancer

is less clear-cut, but scientists suspect a diet heavy in salted and smoked

fish.

 

In the case of infectious mononucleosis, the key co-factor is age. In general,

a toddler exposed to the Epstein-Barr virus will develop antibodies and never

exhibit so much as a sniffle. An older child may have a mild sore throat for a

day or two. But a young adult encountering the virus for the first time stands a

better than even chance of spending a truly miserable month or so in bed with

mono. It seems that the more mature and powerfully developed the immune system,

the worse the reaction.

 

Obviously, if early exposure confers a kind of immunity, conventional ideas

about good hygiene don’t hold up. In Barbados, Indonesia, and Mexico, where

nearly all children have the Epstein-Barr virus in their blood by age six,

infectious mononucleosis is virtually unheard of. But in Sweden, England, and

the United States, where parents are fastidious about the exchange of saliva,

many infants and young children don’t get exposed; as a result, mono is fairly

common among teenagers.

 

Early or late, from the Antibes to Zaire, nearly everyone is exposed to the

Epstein-Barr virus by the age of 30. This viral omnipresence helps explain why

the CDC was so leery of the Incline Village report. After all, you can’t have an

epidemic of a disease when everyone has already been immunized against it.

Or can you? How the Epstein-Barr virus might pull off such a trick is best

explained at the cellular level. The virus infects the B lymphocytes of the

immune system, white blood cells formed in the bone marrow that normally

manufacture anti- bodies against disease. Once infected by the Ep- stein-Barr

virus, B cells proliferate lustily and become immortalized, as the scientists

put it. When the virus transforms a B cell, the immune system responds by

producing a legion of activated killer T cells. These are equipped to identify

the virus- infected B cells and spit out inflammatory agents to destroy them.

Paradoxically, it is the process of getting well at the microbial level that

makes the patient feel horribly sick. During the course of infectious mono, the

immune system is literally at war with itself, T cell attacking transformed B

cell until the Epstein-Barr invasion is eventually brought under control.

The threat is never wholly eliminated, however. A few transformed B cells

harboring the virus – about one in a million – remain present in otherwise

healthy individuals. This post-infection condition has been described as a kind

of armed truce, with a few virus-infected B cells always trying to multiply and

run amok, and the T cells ever vigilant to keep their numbers low. If for any

reason the immune system should fail to react to a flare-up of these infected B

cells, the results can be deadly serious. For example, transplant patients, who

have had their immune systems chemically suppressed in order to prevent

rejection of a newly introduced organ, suffer increased numbers of tumors. Some

of these are masses of Epstein- Barr – infected B cells that multiply out of

control. AIDS patients, whose helper T cells are under attack by the AIDS virus,

are similarly vulnerable to B cell cancers.

 

On the other hand, what happens if the immune system reacts to an uprising of

virally infected B cells, but for some reason fails to master it for months or

years – if war breaks out but neither side wins? As long as this state of

affairs prevails, the patient will feel fatigued, achy, feverish, and depressed,

as though he or she had a perpetual case of mono – which is the clinical

description of chronic Epstein-Barr virus syndrome. Under this hypothesis, the

virus is envisioned as merely the canary in the coal mine of the body. The

fundamental cause of the illness is presumed to be something else, some unknown

agent that damages the immune system and prevents it from reining in the

Epstein-Barr infection.

 

It might be a chemical toxin, a known virus or bacterium, genetic

predisposition, or something entirely new. Or it might be nothing at all –

beyond a case of mass hysteria or confused misdiagnosis by local physicians. It

was the latter possibility that CDC epidemiological investigators Gary Holmes

and Jon Kaplan were leaning toward when they arrived in the Tahoe basin in

September 1985.

 

Eight months later, in May 1986, their report on the situation appeared in the

CDC’s authoritative Morbidity and Mortality Weekly Report, concluding that the

data on the alleged epidemic at Incline Village “neither prove nor disprove” the

Epstein- Barr virus’ culpability. The report questioned the reliability of the

commercial lab test for the virus and pointed out that the test results were

equivocal anyway, because some people with high antibody counts are perfectly

healthy. Blood analysis of a small subset of patients suggested that such

familiar viruses as herpes simplex and cytomegalovirus were equally likely

explanations for the symptoms. The report left the impression that nothing out

of the ordinary had happened at Lake Tahoe. Physicians were urged not to

diagnose patients as having chronic Epstein-Barr virus syndrome until more

“definable and treatable” conditions such as anxiety and depression had been

ruled out.

 

To say that the opposing parties in this controversy hold one another in low

esteem would be an understatement. “I would not take what he was telling you

about patients at face value,” Gary Holmes says of Cheney. “I think that’s true

of a lot of physicians, especially private physicians, who get caught up. They

think they notice something, then they start seeing it everywhere.”

 

For his part, Cheney believes that the CDC came to Incline Village with

preconceived notions. “I think, personally, that after the publication of the

two papers by Straus and Jones and the commercial availability of the

Epstein-Barr blood test in 1985, the CDC was bombarded with reports like ours,”

he says, “They felt the chronic Epstein-Barr virus diagnosis was being overmade,

so they said, well, we’ve got to put a stop to this.”

 

Nonetheless, it wasn’t long before the news spread that a pair of federal

investigators had come into town inquiring about a mysterious disease. A version

of the story broke in the local biweekly, the North Lake Tahoe Bonanza, and

within a few days Peterson and Cheney were fielding alarmed calls from San

Francisco papers asking about an out- break of AIDS at Lake Tahoe. To clear the

air, the two held a press conference at the Hyatt.

 

An enterprising reporter rang up the other physicians in town for their

reaction. Not only had these doctors not seen any evidence of a fatigue

syndrome, they found it very unusual that all the cases were showing up in a

single practice. The newspaper reports, they said, had caused undue concern

among tourists and local residents. “There has to be something wrong with

Peterson and Cheney’s diagnosing procedure,” one of them concluded.

 

In fact, many of the fatigue sufferers had been to other area doctors in the

first place. “Peterson and Cheney believed we were sick,” says Irene Baker, one

of a group of local teachers with chronic mono. “That’s why they got all these

patients.” Publicly the polite skepticism of the CDC and open disavowal of local

physicians undermined Peterson’s and Cheney’s credibility. Privately, Cheney

admits, there were long periods of self-doubt. “We’d sit down at the end of a

day and I’d say, ‘Dan, could we both be deluded at the same time? Are we seeing

this in everyone because we’re attuned to it?’ If enough people who know more

than you do tell you your diagnosis is garbage, then you start to believe it.

The thing that kept pulling us back, though, was our patients.”

 

Gerald Kennedy, who teaches auto mechanics and drafting, had a nearly flawless

24-year attendance record at Tahoe-Truckee Unified High School. That’s

fortunate, because the accumulated sick leave has helped support his family for

the last two rocky years. “It’s been a new experience, I’ll tell you,” he says.

“People are skeptical about it. After a while you feel a little better and you

start question- ing yourself. That’s when you try doing something – maybe drive

out, sit by the lake in a lawn chair and fish for a couple of hours – and pay

the penalty for the next week. It’s like riding a roller coaster.”

 

“Yeah, or a picket fence,” says Baker, who teaches at the same high school.

Following a year of bed rest, Baker can now struggle through a day in the

classroom. She has managed this much through sheer perseverance and with the

help of a young daughter who has taken over the household chores. “My life right

now revolves around work and rest.” She spells it out: “There’s no F-U-N.”

After a year and a half with the illness, Sandy Schmidt began to feel better in

November. The longest well period before that was a month. She now feels her old

energetic self, and has even begun to run again. “I’m still a little guarded

about making blanket statements that it’s gone forever,” she says. There have

been too many disappointments for that. But she’s encouraged.

 

Schmidt, Baker, and Kennedy represent the spectrum of recovery in the Lake

Tahoe patients. About a third of the 200 people diagnosed over the last two

years or so consider themselves well again. The majority are “cycling” – going

through periods of relatively good health followed by relapses. And a final

group – 15 to 20 percent of the original – are as sick as ever. The range of

symptoms is enormous, everything from prolonged sleeplessness, low-grade fevers,

and intense headaches to alcohol intolerance and sensitivity to bright light.

Some patients have taken to wearing portable stereo headphones around their

homes to drown out a perpetual ringing in their ears.

 

In one support group for Tahoe patients, there have been four miscarriages, a

case of total paralysis requiring months on mechanical ventilators, and a B cell

lymphoma. Most consistently, however, patients complain about a drastic decline

in mental acuity – an inability to concentrate, remember names, think. “Of all

the things I’ve lost,” a patient fired from her job and divorced by her husband

wrote Cheney, “I miss my mind the most.”

 

Even with their problems, the Tahoe patients are fortunate in one respect: They

readily found a pair of doctors who believed their ailments were more than

psychosomatic. Widespread skepticism on the part of doctors may explain how the

early stereotype of chronic mono sufferers got started. “Well-educated

high-achievers would be the people least likely to take no for an answer,” says

Anthony Komaroff, a professor of medicine at Harvard University. “But in our

experience we’re seeing people from all walks of life: lots of non-Yuppies,

blue- collar workers, blacks as well as whites.”

 

Recently, chronic mono sufferers stopped getting mad at their doctors and

started getting even – with their own organization, medical advocates, and

lobbying effort. “It’s a grassroots kind of thing,” says Van Zelst of the Minann

foundation. “The patients are literally having to slug it out with the medical

profession.”

 

The National Chronic Epstein-Barr Virus Syndrome Association in Portland,

Oregon, enrolled more than 11,000 members in its first year, and according to

president Gidget Faubion, it doesn’t hesitate to mobilize them. When James F.

Jones’ funding for research on the disease at Denver’s National Jewish Center

was held up by slow moving medical review panels, Faubion’s group sent out an

alert. Two thousand members wrote Anthony Fauci, director of the National

Institute of Allergy and Infectious Diseases, to express their impatience. The

money appeared.

 

As this example illustrates, the chronic mono sufferer’s chosen weapon is the

pen. Houston pathologist William Hermann wryly suggests that polygraphia –

voluminous letter writing – be recognized as a symptom of the disease. So

effective has this campaign been that in its latest funding bill Congress

specifically instructed both the CDC and the National Institutes of Health on

steps it wants taken with regard to chronic Epstein-Barr virus syndrome –

including more research and a surveillance network to report on the incidence.

 

After the early public relations debacle with the CDC, the rehabilitation of

Peterson’s and Cheney’s reputations began when Harvard’s Komaroff agreed to

visit Incline Village in February 1986. Komaroff’s team conducted a thorough

epidemiological study: They drew blood, interviewed patients, and searched for

alternative causes and patterns of spread. At the same time they looked into an

outbreak of fatigue in the nearby ranching town of Yerington, Nevada.

 

Komaroff’s conclusion was unequivocal: Something very real had taken place in

both communities. During the interviews he noticed what he came to regard as a

signature for the illness – the high proportion of patients who named a definite

date of onset, a date that marked the transition in their lives from good health

to misery. “You hear that once or twice and say, ‘Gee, that’s strange,’ ” says

Komaroff. “But when you hear it 50, 100, 200 times and you add in the sore

throats, swollen glands, low-grade fevers – which are hardly a reflection of

anxiety – it seems overwhelming to me that this is evidence of organic illness.”

Komaroff’s support helped Cheney establish ties with several other East Coast

researchers. The fruits of these collaborations were recapped at a medical

conference in Austin, Texas, in November. Cheney reported on a variety of

sophisticated lab tests showing that chronic mono sufferers’ immune systems were

impaired. Among the most disturbing results were a series of pictures

demonstrating brain damage in many of the patients from north Lake Tahoe.

 

A magnetic resonance imaging scan allows a look at the soft tissues of the body

as clearly as an x-ray reveals the bones. The scans from Lake Tahoe show what

look like small holes in the brains of 45 of the 80 chronic fatigue patients

examined – bright white spots against a gray field. Most of the spots are small

and well-defined, not like the larger smudges associated with multiple

sclerosis. Some- times their location corresponds closely to the patient’s

neurological symptoms. For instance, a young secretary who previously had a

clear brain scan discovered one day that she could no longer pick up the phone

with her left arm. A subsequent scan turned up lesions in an area of the brain

that is responsible for motor control on the left side of the body.

 

A battery of tests administered by several California neuropsychologists

yielded results equally suggestive of physical – not psychosomatic – disease.

Patients were asked to perform simple tasks that measured manual dexterity,

long- and short-term memory, and ability to solve visual problems and arrange

numbers and letters in proper sequence. A blindfolded patient would be asked to

put round, square, or triangular blocks through a board with matching holes

using the right hand, then the left.

 

“What we found were spectacular differences,” Cheney told the Austin

conference. A patient able to do the task easily with one hand couldn’t get a

single block in with the other. “This kind of discrepancy is not characteristic

of any psychological disease.” If Cheney insists on this point, it’s because he

and Peterson have had it questioned so often by infectious disease specialists

to whom they have referred patients.

 

From February to April 1986 Cheney called the CDC regularly to report the new

findings on the Incline Village patients. None of it was included in the May

1986 article on the syndrome in the Morbidity and Mortality Weekly Report. In

fact, Holmes told the Los Angeles Times later that month that the agency planned

no further study of the matter. Shortly thereafter the CDC’s public affairs

office turned down a request from 20-20 for an on-camera interview on the

subject.

 

But Cheney wouldn’t accept that the case was closed. Because of Incline

Village’s new notoriety, he was receiving a steady stream of inquiries from

fatigue sufferers around the nation. As an exercise in ad hoc epidemiology, he

decided to plot the date the illness struck each of 185 current sufferers who

had contacted him from 35 states. The resulting graph describes a flat line from

1953 to 1977 followed by a steeply rising curve.

 

Statistically speaking, the results are not significant, since the data was not

collected in a random fashion. Nevertheless, Cheney has ventured a tentative

interpretation. The flat line, he says, is consistent with chronic mono as

viewed by early investigators like Jones and Straus: a rare, isolated disease

long present in the human population. The rising curve, on the other hand, may

reflect the appearance of a new infectious agent. It’s a bold speculation, and

there are alternative explanations for the curve. For example, if the disease

lasts no more than a few years in most cases, people stricken recently should

always outnumber current sufferers who got the disease five or ten years ago.

But Cheney favors the new virus thesis, partly, he admits, because he is privy

to inside information.

 

What may prove to be the turning point in this medical mystery unfolded at the

National Cancer Institute’s Laboratory of Tumor Cell Biology last fall. It was

there that Robert Gallo and his co-workers announced they had identified a novel

herpes virus, christened HBLV (for human B lymphotrophic virus), in six patients

suffering from a variety of lymph cancers and blood disorders.

 

Because the herpes viruses wreak a lot of havoc, the discovery posed a series

of pressing questions: Is this virus a new player on the scene, or just a newly

discovered one? How is it transmitted and how contagious is it? What illnesses

might it cause? Before long Cheney had the federal scientists interested in

Incline Village. He summarizes the situation neatly: “They had a virus looking

for a disease, and we had a disease looking for a virus.”

 

The involvement of human B lymphotrophic virus in the Incline Village epidemic

is far from proven, but there are grounds for suspicion. For one thing, as its

name implies, the new virus seeks out and attacks the same niche in the body as

the Epstein-Barr virus – the B cells of the immune system. This suggests that it

is ideally situated to function as a co-factor, perhaps by upsetting the balance

of power between the immune system and the la- tent Epstein-Barr infection

present in all of us.

 

So much for theory. The proof of this pudding will be in the blood. By the end

of 1986, Gallo’s lab had tested 700 blood samples from around the world in an

effort to find out the range of their viral . discovery. The test is

painstakingly slow and not completely accurate; nevertheless, the results so far

are provocative. They suggest that HBLV is markedly less widespread than the

Epstein-Barr virus; that HBLV may be much more prevalent in parts of central and

eastern Africa – regions where Burkitt’s lymphoma is common – than in the United

States; and that within the United States it is distributed very unevenly.

 

Taken together, these provisional findings point in a consistent direction:

HBLV is indeed a relatively new arrival in this country, and like the AIDS virus

it probably came here from Africa. As for the blood samples at Incline Village,

published but unauthorized reports say 60 percent of the chronic mono patients

there have tested positive for HBLV, as opposed to 30 percent of the general

population in north Lake Tahoe. Gallo prefers not to traffic in numbers yet.

 

Like some other researchers in the field, he is cautious because he suspects

chronic mono will turn out to have more than one cause. But he will say this:

“My feeling is that HBLV is a very hot candidate to be involved in a portion of

what is now being called chronic Epstein-Barr virus syndrome.”

 

Pressure from Congress and the discovery of the new herpes virus have changed

the CDC’s lukewarm institutional interest in the chronic mono syndrome. Last

fall, CDC investigators did a telephone survey of internal medicine specialists

in Nevada and Georgia to determine how many were seeing long-lasting monolike

cases. The investigators wanted to find out if the glut of local publicity over

the Incline Village outbreak had skewed the perceptions of Nevada physicians.

The results were virtually identical: Forty percent of doctors contacted in both

states reported treating at least one patient meeting the CDC’s chronic mono

description. “That’s a pretty frightening number,” says Holmes. “It’s certainly

enough to make you feel justified in doing a lot more study.”

 

Nonetheless, Holmes is the only person at the CDC working on the issue on a

regular basis. “On any given day, it’s second or third on my list,” he says.

Like Jones and Straus, he believes that the Incline Village outbreak and what

the media is calling chronic Epstein-Barr virus syndrome is not a new

phenomenon. “There have been syndromes almost identical to this reported in the

literature going back to the 1930s,” he says.

 

The list of examples is impressive: at Los Angeles County Hospital in 1934; in

Akureyri, Iceland, in 1948; and at Royal Free Hospital in England in 1955, to

cite just a few. The names have changed (epidemic neuromyasthenia, vegetative

neuritis, benign myalgic encephalomyelitis), but some of the symptoms – and

especially the protracted course of recovery – remain remarkably constant. As

has a tendency to disbelieve: All these outbreaks have been described in medical

journals as examples of mass hysteria.

 

As for HBLV’s role as a possible new pathogen, Holmes is curious about the

possibility but stymied. The CDC has been unable to obtain samples of the virus

from Gallo’s lab. “We’ve tried repeatedly but we’ve been rebuffed,” Holmes says.

“We have been kept completely in the dark on it.” For the time being the CDC’s

main effort is to help formulate a definition for the syndrome that researchers

in the field can agree on.

 

One key issue is duration. Some researchers say the illness can strike and

abate within six months; others insist that it’s not chronic mono unless it

lasts two years. Another issue concerns how much weight, if any, to assign to

the Epstein-Barr blood test as opposed to physical symptoms. Once it has a clear

definition, the CDC plans to set up surveillance networks in four or five states

by this fall.

 

The Epstein-Barr virus lobbying group is not concerned that the discovery of

HBLV may have left it plumping for the wrong virus. “We don’t care if they label

it CEBV, HBLV, or XYZ,” says Faubion. “All we know is, we’ve got a lot of

suffering people with identical symptoms. There’s a national epidemic of immune

system dysfunction and viral disorders in progress which until recently the CDC

has been more interested in covering up than doing something about.”

The organization is working to gain enough recognition for the fatigue syndrome

so that victims can readily qualify for disability benefits. Until now

relatively few have qualified, and then only after going through a long, slow

process. In the mean- time, medicine can offer little in the way of a remedy.

The antiviral drug acyclovir and injections of immune globulin have been tried,

with mixed results. “There are people who claim to have therapies and charge a

lot of money for them,” says Straus, who has done studies on patients in

treatment. “But there’s not one that works very well.”

 

That leaves the chronic mono sufferers hoping for a cure and learning to live

with the limits of the disease. At Lake Tahoe, after being unable to conceive

for a long time, Chris Guthrie is now about to deliver her second child. “I’m

very excited,” she says. “I felt my life had been on hold for several years.”

Guthrie is bothered by partial loss of strength in her right arm and is easily

tired. She says that blood tests show she has high levels of antibodies to the

new HBLV virus. And since she cut back her medication to protect her developing

baby, she has had a constant strep throat that makes her voice scratchy and

hoarse. But perspectives change over time. “If that’s the worst that happens,”

she says, smiling, “that’s nothing.”

 

Paul Cheney has learned that medical prophets have a tough audience in their

hometowns. After he presented his slide lecture at an Incline Village Chamber of

Commerce luncheon last July – complete with the results of lab work,

psychometric tests, and brain scans – a member of the audience remarked on his

way out, “Sounds like a bunch of hypochondriacs to me.” The rumor in town was

that Peterson and Cheney were waxing fat off the profits from the specialty lab

work they were ordering, when quite the opposite was true; they subsidized the

costs with tens of thousands of dollars out of their own pockets. Cheney’s

children had some verbal barbs thrown their way at school. “Maybe we violated

some law of nature that says one does not do research projects on viruses in

resort communities,” Cheney observes. In January he accepted an offer to

practice with the Nalle Clinic in North Carolina and left town. The move has

reinforced his belief that the chronic mono illness is a national phenomenon: In

his first six weeks he diagnosed nine cases of the syndrome.

 

But his thoughts drift back to the Tahoe patients. Lying awake in the early

morning hours, Cheney wonders if HBLV will turn out to be a “big trick,” another

canary in the coal mine like the Epstein-Barr virus, disguising yet another

agent or pathway of the disease. “Although,” he adds, “I keep telling Tony

Komaroff, nature cannot be that sadistic.”

 

Perhaps the main lesson of Incline Village is that, if not sadistic, nature is

certainly resourceful. A few years ago Americans may have believed that the days

of contagious disease were numbered. Small pox. Polio. Tuberculosis. One by one,

medical technology could be counted on to eliminate the remaining scourges from

the face of the Earth – or at least from sanitized and well-regulated industrial

societies such as our own. The surprise appearance of the deadly AIDS epidemic

shattered that complacent view. The discovery of HBLV underlines the lesson. We

are part of a natural world, where evolutionary change – sometimes swift and

sharp – goes on apace. Incline Village got the brunt of the publicity; the

nation got the disease.

 

William Boly is a contributing editor.