No luck
club: author ticked off about lyme
Amy
Tan, under treatment for late-stage lyme disease, could never have conceived
how hard it would be to get a diagnosis — or how surreal the symptoms
could be
ALS
News
The Washington Post Company
by J.J. McCoy, Washington Post Staff Writer
05 August 2003
It wasn’t until
she began having hallucinations about a naked man approaching her bed that
novelist Amy Tan got really worried.
She first assumed what
she was seeing was her husband, who brings her coffee in the morning. “But
it was the middle of the night,” she recalls, and “he wasn’t
saying anything or doing anything else. He was just coming toward me [before
stopping] next to the bedstand, as though he was turning on the light.”
She took his silence
to mean the worst.
“I thought someone
was dead,” she recalls. “I reached for him, and the image started
to warp as I realized it wasn’t real.”
She sprang to in dread
and started running through her SoHo loft, calling out for him—and
very soon finding her flesh-and-blood husband, Lou DeMattai, calmly watching
TV.
“Oh, thank God,
you’re alive!” she said.
What Tan didn’t
realize is that her experience about 18 months ago was perhaps only the
strangest manifestation of what has since been diagnosed as late-stage Lyme
disease—an illness she believes she had contracted two years earlier
but that continued to cause neurological damage, thanks to several misdiagnoses
by doctors unfamiliar with or even hostile to the possibility.
Even now, after several
months of treatment with antibiotics, Tan says her symptoms range from fatigue
and memory loss to tinnitus (ringing in the ears) and olfactory hallucinations.
“I smelled dead
rat briefly, but over a period of hours” and in different locations.
“It’s definitely weird.”
Other symptoms have
included hair loss, memory gaps, dropping first letters of words when writing
by hand and replacing words with similar-sounding gibberish when speaking.
Most recently she’s experienced vertigo and expects that she’ll
continue to feel some joint pain “as a souvenir.”
Tan’s case dramatizes
the growing, increasingly acrimonious division in the medical community
over the proper treatment and diagnosis of Lyme disease, an illness caused
by a bacterium, Borrelia burgdorferi, that enters the body via the
bite of a deer tick.
There are essentially
two armies in the Lyme war. The larger is the conventional team, supported
by the Centers for Disease Control and Prevention (CDC), the National Institutes
of Health (NIH), the insurance industry, most organized medical groups and
the majority of physicians. Members of this camp embrace a standardized
set of symptoms, tests and treatment. As described on the CDC Web site (www.cdc.gov),
Lyme disease starts with a tick bite and typically involves a “bull’s-eye”
rash—a round, red mark surrounded by a paler ring and, often, a larger
red ring—accompanied by symptoms such as fever, fatigue, headache and
aching muscles and joints. Early on, diagnosis is usually clinical, with
a blood test preempted by symptoms and risk factors for exposure. Standard
treatment is oral antibiotics, usually lasting for 10 days to a month depending
on severity.
The CDC’s Web
site adds that some people with Lyme disease left untreated may experience
“cognitive disorders, sleep disturbance, fatigue and personality changes,”
and that the condition may become severe, chronic and disabling, though
is “rarely, if ever” fatal. The site says some patients may have
recurrent symptoms and require repeated treatments for up to two years.
Then there’s the
opposing side, where other medical practitioners and patients’ groups
(and now, Tan) advocate a broader view of the disease that includes less
predictable symptoms.
The Bethesda-based
International Lyme and Associated Diseases Society (ILADS)
contends that fewer than 50 percent of Lyme patients recall either a tick
bite or a rash. They also claim the CDC’s standard tests often fail
to identify the disease.
Furthermore, they maintain
that the CDC’s standard course of antibiotic treatment is too brief
to provide a lasting cure for many and may even leave the bacteria morphing
and thriving inside the body, where they can wreak permanent neurological
damage.
Gregory Bach, an ILADS
board member with a family practice near Philadelphia, says the standard
methods miss many people with Lyme, exposing them to harm. Among his clients,
“we found that 50 to 90 percent of those tested [by the CDC’s
initial screening method and not diagnosed with Lyme] subsequently tested
positive by other tests.”
Paul Mead, an epidemiologist
for the CDC, says “certainly this is an area of considerable controversy.
There’s no question that some in the community have taken exception
to the criteria for diagnosis developed by expert panels.... Testing is
a complicated issue that we and others are looking into in further detail.
It’d be nice if all the tests were subjected to rigorous validation,”
he says, but questions remain about the tests and lab analysis of those
in the alternative camp.
The reality at this
point “is that there is no answer,” writes physician and Harvard
Medical School assistant professor Jonathan A. Edlow in his new book, “Bull’s-Eye:
Unraveling the Medical Mystery of Lyme Disease” (Yale University Press,
$29.95). “There is something that everyone agrees on: There are these
patients who have classic symptoms. The earlier they’re treated, the
more likely they’ll have a good outcome.”
“Beyond that,”
he qualifies, “you have the divergence.”
The No Luck Club
Tan’s story illustrates
how difficult diagnosis and treatment can be for someone whose case does
not conform to the standard course. Tan didn’t realize how tiny the
tick could be—about the size of the period at the end of this sentence—and
her rash never fit the standard description. As bothersome as her months
of joint pain, occasional numbness, headaches and memory lapses had become
by 2001, she accepted numerous medical opinions writing them off as signs
of getting older. (She was 49.) But she couldn’t laugh off the hallucinations
and memory lapses.
Both Tan’s father
and her older brother had died from malignant brain tumors. Her mother had
a brain tumor, too, though hers was benign; she died after having been diagnosed
with Alzheimer’s disease.
“I certainly considered
[that I might be suffering from] Alzheimer’s,” Tan admits, “especially
when I started having memory problems, and hiding mistakes that I’d
made. I thought that must have been what it was like for my mother.”
While her doctors quickly
ruled out either those possibilities, they could not tell her what was wrong,
even after two CAT scans, an MRI and a 48-hour hospitalized fast. She broached
the possibility of Lyme disease, but her doctor “told me three times
he really didn’t think I had Lyme, that we didn’t have it in California,
and that it was rare.”
Tan reminded him that
she and her husband split their time between the West Coast and New York,
but the doctor was unmoved, she said. They left it at that.
A Battle Over Bites
Since Lyme disease
was first detected in the United States in 1975, warnings about the risk
of deer-tick bites have become routine public safety reminders each summer,
along with tips about using sunscreen and avoiding rip tides at the beach.
Though it may not be as in vogue as SARS, monkeypox or even White Nile disease,
Lyme disease continues to proliferate, in its number of victims, geographic
reach and medical controversy.
Cases have been reported
in every state except Montana, with the overwhelming majority concentrated
in the coastal Northeast and Mid-Atlantic states and in Minnesota, Wisconsin
and northern California.
Of the 17,029 cases
reported to the CDC in 2001, there were 608 in Maryland, 156 in Virginia
and 17 in the District of Columbia. The national total increased by more
than 33 percent between 1991 and 2000.
The CDC adds that while
reported cases of the disease have been increasing—it theorizes that
people are being exposed more often to ticks because tick populations are
becoming both more dense and widely distributed—“the disease [remains]
greatly under-reported.”
As the NIH details
on its Web site (www.nih.gov),
the particular spirochetes (spiral-shaped bacteria) associated with Lyme
are notoriously difficult to diagnose and troublesome to treat in their
later phases—not only because they can change their appearance but
because Lyme disease is similar to but separate from other diseases transmitted
by different ticks. For diagnosis, the CDC recommends either of two blood
tests for mid- or late-presenting cases: an ELISA (enzyme-linked immunosorbent
assay) or IFA (indirect fluorescent antibody) test, followed by a Western
Blot test.
The CDC’s position
is that the tests are reliable for late disease; ILADS counters that they
miss fully half of late-stage cases.
Unfortunately, Edlow
notes, both the ELISA and the Western Blot are imperfect, with frequent
false negatives in early testing; in other words, they fail to identify
many patients who have the disease. Since the production of antibodies,
for uncertain reasons, is a little slower against Lyme than against many
other infectious diseases, testing is not recommended within the first month
of suspected exposure.
The ELISA, however,
also routinely presents false positives—identifying disease-free people
as having Lyme. A random sampling of 100 people might—due to bacteria
as relatively benign as those that cause gingivitis—suggest that five
of them have Lyme, when in fact they have gum disease.
This is why the Western
Blot, which produces fewer false positives, is used as confirmation.
Still another problem,
though, is that there’s disagreement about how to properly interpret
a positive Western Blot test. While the test measures which antigens, or
proteins, a patient’s blood contains antibodies to, there remains technical
disagreement over exactly which are significant and to what degree.
The CDC doesn’t
accept as relevant to a diagnosis of Lyme as many bands—measures of
antigens—as private labs like IGeneX (a California lab run by Nick
Harris, a doctor and ILADS board member) measure.
Shared criteria can
also be interpreted differently. Even when the CDC and IGeneX agree on which
bands are indicative, Edlow adds, a patient could test positively to the
blot, “but have gotten [the disease] three years ago. That doesn’t
mean you have active infection, even though the antibodies remain positive.”
“As with most
things Lyme,” Edlow says, “it depends on whose opinion you believe
and how you define Lyme disease.
”There’s
legitimate controversy,” he continues. “While we all prefer unambiguous
results, that’s just not possible the way these are used.”
Life, Interrupted
Tan says she is “not
terribly angry with any of my doctors” for having missed her diagnosis,
though the roster included her primary care physician, an endocrinologist,
a sleep-disorder specialist, two neurologists, a cardiologist and an orthopedic
surgeon. “They were doing the best they could.”
“I’m angry
at the California Medical Association,” she says, “because they
have a board policy that recommends that all Lyme disease patients be treated
with 10 days of antibiotics. I’m angry that they perpetuate the myth
that it’s rare, easily tested and easy to treat.
”I’m mad
at insurance policies.... I’m a typical long-term Lyme patient who’s
seen a lot of doctors and had a lot of expensive tests done,” Tan continues.
While she could afford to spend more than $50,000 on diagnostic tests before
learning what was ailing her, ”I’m luckier than most—there
are many who have it far worse than I do.”
”I know people
who’ve lost homes, everything they have. I’m mad at the arbitrary
saying that it’s two weeks’ treatment regardless of what’s
happening to you.”
Nearly half a year
before Tan’s hallucinations began, she had returned home after a four-month,
trans-oceanic book tour for her novel “The Bonesetter’s Daughter.”
The persistent fatigue plaguing her before she started her itinerary had
become life-consuming.
The day-long sleep
that she first wrote off to jet lag didn’t suffice; in the weeks that
followed, she would sleep for 12 to 20 hours a day. Even more disturbing
were the trembles that punctuated her sleep, waking her every two or three
hours with what she called “Dolby Digital Syndrome”—a feeling
of constant vibration, like reverberation from a bass-heavy stereo.
When she shortly began
waking each day with a sore neck, Tan bought a new buckwheat pillow. She
tried another type, and still another, adding everything from Tempur-Pedic
foam to a U-shaped neck roll.
As the weeks turned
into months, the mental toll turned from an extended writer’s block
to an inability to maintain concentration or short-term memory.
“By page three
or four [of her writing], I was unable to recall anything and had to begin
anew,” she says. At the dinner parties, “I often couldn’t
keep up with the repartee.... I nodded and laughed at the moments when everyone
else did.”
She decided to consult
a psychiatrist for the first time in nearly 20 years; the doctor in turn
suggested she also get a complete medical workup.
Finally, after comparing
what she knew with what other patients reported on the ILADS Web site (www.ilads.org),
Tan discovered Rafael Stricker, a Lyme specialist in San Francisco.
Stricker says Tan’s
“clinical symptoms and history were very suggestive: striking psychiatric
problems and hallucinations, which you can see with Lyme disease.”
He tested her with
a Western Blot, and had it analyzed by IGeneX.
Stricker says Tan has
tested positively on the Western Blot five times in a row. Among the 16
antigen bands that IGeneX identifies, her tests “have been pretty uniform,”
with positives for seven or eight bands each time. She has consistently
tested positive for two of the three bands the CDC considers accurate indicators
of Lyme, he says. He considers her diagnosis conclusive.
Stricker points out
that as a public health agency, the CDC is interested mainly in surveillance
and less in clinical applications of tests. “They’re not as concerned
in missing something, while I want a test that works for my particular patient’s
diagnosis.”
The CDC’s Mead
confirms that the agency’s analysis of the Western Blot requires a
minimum of two of the three bands for a positive diagnosis, and that “this
is a surveillance-case definition,” which is useful for tracking the
disease but may not be sufficient for a specific patient.
“It’s a balancing
act,” he says. “It’s true that to a certain extent [we] have
different objectives. A clinician can treat, just in case, to help his patient.
Nevertheless, there can be problems with over-treatment [with long-term,
powerful antibiotics]. Overall it’s not helpful to treat someone without
a disease.”
Stricker recently changed
Tan’s antibiotic medication to Flagyl (metronidazole) from doxycycline.
He says recovery is usually progressive: “Usually when they feel better,
they stay better, though there’s possibility of relapse. Sometimes
it requires months of treatment for a complete cure,” he says. “Sometimes
it requires years.”
That Tan’s answer
hadn’t been discovered through the CDC’s diagnostic protocols
doesn’t surprise Edlow. After all, he says, the Borrelia burgdorferi
spirochetes “are weird bugs. Part of the way that they evade the immune
system is to morph, or change themselves to appear like a new bug to the
immune system. Or they don’t. Or else patients have one or another
or more than one at the same time. It gets kind of tricky.”
Tan meanwhile argues
that Lyme disease patients can’t afford to wait for the science to
inch its way forward. Her imagination still reels at the thought she was
tested for both syphilis (after 29 years of marriage) and Lou Gehrig’s
disease before her doctors considered her for what the CDC terms “the
leading cause of vector-borne infectious illness in the U.S.”
“I’ve never
met a single person in my lifetime who had ALS, but they tested me for that
before Lyme disease,” Tan adds. “It’s not the way the medical
community should be dealing with these very real problems that people are
having.”
Imputing her symptoms
to age strikes her as no more logical.
“Did most baby
boomers lose their hair in clumps? Did [they] read e-mails and respond at
length, then have no memory of doing either? Were they amazed to read unfamiliar
pages of stories they had evidently composed?... Did they become lost in
their own neighborhoods, unable to recognize any familiar landmarks, too
mortified to ask for help?”
She concludes in an
essay from her upcoming book “The Opposite of Fate”: “I am
in this for the long haul, with treatment that will likely last for years.
I won’t feel safe until the scan of my brain and blood tests on my
immune system return to normal, until the Western Blot is negative for Lyme
disease, and my myriad symptoms are gone... By having Lyme disease, I have
automatically been drawn into the medical schism over both its diagnosis
and treatment.”
“I now know what
is the greatest damage that Borrelia has caused: It is ignorance.”
© 2003 The Washington
Post Company
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