A More Precise Pacemaker
As cardiologist Kevin Donahue considered the elderly
man's weakening heart, he worried over the possible
downside of installing a traditional pacemaker. True,
his patient had suffered a series of heart attacks
and now courted heart failure, so a pacemaker was in
order, but Donahue knew the constant drumbeat of the
device could put undue stress on a cardiac muscle that
needed only an occasional boost.
And so, Donahue proposed a novel pacemaker he'd seen
introduced at last year's American Heart Association
meeting. Just approved by the FDA, the device would
allow the cardiologist to tailor the electrical pacing
to his patient's actual needs. “This one watches more
than it paces,” he explains. “It only paces the bottom
chamber of the heart when it really has to. When it
detects a pause, it kicks in.” The patient signed on,
and Donahue became the first cardiologist in the mid-Atlantic
region to implant the new device.
Clinical studies have shown that unnecessary pacing
in the right ventricle—the bottom chamber—can increase
the risk of heart failure and atrial fibrillation.
But because traditional pacemakers don't have the ability
to adjust the mode when the ventricles change from
independent beating to needing pacing, the right ventricle
is often paced unnecessarily. The new technology, called
the EnRhythm pacemaker, can be programmed to deliver
pacing pulses to the right ventricle less than 2 percent
of the time. It assists physicians in detecting additional
heart rhythm irregularities, especially in the atrium. “It
actually tells us the percentage of heartbeats that
are paced,” Donahue says—and can painlessly terminate
fast heart rhythms in the atria, the top chambers. “It's
clearly the wave of the future.”
When Age Isn’t the Problem
Old age didn't come gently to Iris D'Ari. By 79, her
thinking had grown fuzzy, she suffered from incontinence,
and her gait was so timorous and shuffling that she
rarely ventured outside. Classic manifestations of
aging, right? Wrong.
Thanks to a physician near her Vermont home who was
enough uncertain of his patient's diagnosis to refer
her to a specialist, D'Ari ended up at Hopkins . There
a neurologist took a look at her test results and suspected
not senility, but a condition called adult hydrocephalus—“water
on the brain” to laymen. Caused by an excess of cerebrospinal
fluid in the ventricles, the condition produces blockages
in the brain that interfere with the body's ability
to reabsorb cerebrospinal fluid, causing the brain
to swell with the liquid.
“It's a condition that can be triggered by any number
of problems—infection, trauma, venous disturbances,
even brain tumor treatment,” explains neurological
surgeon Paul Wang. “But because its symptoms—dementia,
incontinence and difficulty walking—mimic other problems
of old age, there often can be little incentive for
a doctor to probe deeper.” A CT scan showing enlarged
ventricles and brain atrophy could indicate
Alzheimer's or Parkinson's disease, but might also
signal hydrocephalus, so “in the end, this is really
a diagnosis of exclusion.”
To make certain they were on the right track with
D'Ari, neurologists here performed a lumbar puncture,
drained the fluid from her brain and watched what happened.
The results were astounding. Within hours, D'Ari was
walking and thinking as she hadn't for months. The
evidence was in: She was a candidate for shunt surgery.
“Adult hydrocephalus is not something that can be
cured,” Wang makes clear, “but we have several shunt
techniques that can be wonderfully successful in suppressing
the condition.” The method with the lowest complication
rate involves inserting a catheter with a one-way,
programmable valve to move the fluid from the brain
into the abdomen where it is reabsorbed. But if scarring
or blockages make this approach inadvisable, a shunt
also can be inserted from the large jugular vein in
the neck to move fluid toward the heart. These methods
aren't surefire, Wang emphasizes, but a one-in-five
failure rate drops to 6 percent or less after the first
What's so rewarding about shunt surgery is its ability
to lift a fog of confusion and frailty in an elderly
person. For D'Ari, the surgery literally rolled back
the clock. She became lucid and mobile, and for the
first time in years doctors evaluated her as normal.
Consultation with the chief of surgery
Picture the occupants of a vascular surgeon's waiting
room and you're likely to envision the middle-aged
and elderly. In Julie Freischlag's clinic, you'll also
find strapping young athletes who flock to her with
one thing in common: arm swelling and pain so severe
that they're sidelined—permanently, they fear—from
the sport they adore. (> See Minus
a Rib, An Athlete Becomes Whole Again) What
they've got is a syndrome called effort thrombosis.
And Freischlag is the rare surgeon who's bold about
Can you describe this injury for us?
People (often very athletic ones) come to the emergency
room with a swollen arm and think a bug bit them. But
when they raise their arm, the pain is terrible. The
problem belongs to a group of disorders known as thoracic
outlet syndrome, which can be complex and somewhat
confusing. Most cases affect the nerves that pass into
the arms from the neck, but a tiny percentage of cases
involve arteries, and in about 3 percent of patients,
there's obstruction of the veins from a clot. It's
easily misdiagnosed, but without proper treatment,
there's a risk the clot will go somewhere else, like
How does it happen?
Many of the people I see with effort thrombosis are
heavy-duty athletes who get very big neck muscles—especially
the subclavian and scalene anticus anterior muscles—and
end up pinching the vein.
Is this common?
No, it only affects less than 1 percent of athletes,
but I've helped major league and college pitchers,
tennis players, football players, weight lifters, surfers.
I consult on about 200 patients a year who have thoracic
outlet syndrome, and about 15 percent to 20 percent
have venous problems. They come from up and down the
East Coast, from New York to Georgia . I've done several
cases from Israel .
What's your draw?
We're very aggressive with treatment. We give tPA—tissue
plasminogen activator—which actually dissolves the
clots. Afterwards, we operate to remove the subclavian,
the scalene muscle and the first rib. Two weeks later,
we dilate the vein to make sure all the scar tissue
is gone. Then we put patients on blood thinners for
about a month. A lot of people tell these athletes
that they won't be able to go back to their sport,
but we find that, because they're so motivated, they
usually rehab fine.
Interviewed by Mary Ellen Miller
Cystic Fibrosis: No Longer Just for Kids
Boyle with a young-adult patient
Like a lot of physicians, Michael Boyle talks diet
to his patients. “I tell them to go to McDonald's for
lunch, and then stop in at Burger King on the way home,” this
Boyle specializes in cystic fibrosis. And because
this deadly, inherited disease throws off salt/water
balance and damages pancreatic function, for CF sufferers,
eating high-fat, high-sodium foods is a must.
Boyle's interest in CF began rather accidentally a
decade ago, when he stood in for a fellow resident
in the pediatric CF clinic. At the time, CF was still
a childhood disease, since few victims lived beyond
their teens. But medical advances were offering new
hope, and Boyle anticipated a need for adult specialists.
During four years of fellowship training, he focused
on the disease. Then in 1999, he founded the Johns
Hopkins Adult CF Program
His timing was impeccable. Today, the median life
expectancy for CF patients is up to 35, and the Center—considered
one of the finest research and treatment facilities
in the country—treats more than 160 adults. Two more
pulmonologists, two nurses, a dietitian, a physical
therapist and a social worker have joined the staff.
Adult patients face huge challenges. They may spend
a couple of hours a day inhaling medicine and receiving
physical therapy to clear their airways of thick mucous.
And 80 percent of those over 18 develop a chronic lung
infection that requires aggressive treatment.
“And yet,” Boyle says, “many go to college, begin
careers and start families.”
His own research focuses on analyzing why the disease
attacks its victims so differently. Some suffer from
lung problems so severe they require a transplant by
the time they're teens. Others live well into adulthood
with relatively healthy lungs.
“Cracking this riddle,” Boyle says, “is the holy grail
in CF. That's what our team is pursuing.”
Let There Be Light—From Artificial Retinas
bright disk in this patient's eye may
be helping him see.
Julia Haller implanted “artificial retinas” into eight
patients going blind from retinitis pigmentosa and
says they've all done beautifully. Haller performed
the procedures on patients ranging in age from their
20s to 50s as part of her participation in a three-way
trial initiated by a Chicago group.
“From the earlier Chicago study,” she says, “people
were already getting larger visual fields. Now, the
first signs from our patients here look promising.”
The technology—which relies on a 2-millimeter-diameter
silicon computer chip that is surgically implanted
in the eye—appears to benefit from electrical microcurrents
within the tiny chip itself, which is 25 microns thick.
The chip contains 5,000 microscopic solar cells that
convert light energy from images into electrochemical
impulses that stimulate the remaining functional retinal
cells of RP patients.
“The chip itself doesn't give vision,” explains Gislin
Dagnelie, one of Haller's research associates. “But
we think the microcurrents released by the chip may
be causing neighboring cells to release chemical messengers
that improve the health of the remaining rods and cones
in the center of the retina, one quarter of an inch
away from the chip itself.” Haller also speculates
that when the foreign body is implanted in the eye,
a healing response ensues that rejuvenates tissue quality.
Is this technology feasible? “Yes,” says Haller. Does
it help? “There are encouraging signs. What's still
unknown is how much it helps and how long it will last.” Stay
Buying Time For Ovarian Cancer Victims
The much-awaited results of oncologist Deborah Armstrong's
latest ovarian cancer study confirm that chemotherapy
delivered directly into the abdominal cavity offers
advanced-stage patients a better chance for long-term
Already, three years ago, authorities took note when
Armstrong reported that stage III patients whose tumors
had been reduced to less than 1 centimeter—and who
were treated with a solution targeted directly at postsurgical
tumor sites via a catheter into the abdomen—fared better
than those who received the standard IV chemotherapy
regimen. (Study patients showed no disease progression
for an average of 24 months, compared with 19 months
for the other group.) And though the new treatment
elicited more toxicity during therapy, according to
Armstrong, the patients' quality of life equalized
shortly after the treatment was complete.
“It meant,” Armstrong explains, “that a higher concentration
of the chemo drug was reaching the site of the tumor,
but not the surrounding normal tissues.”
At that point, the National Cancer Institute's Edward
Trimble said, “We eagerly await the data on long-term
Those data are now in. And though the study still
hasn't been published, Armstrong feels comfortable
revealing that the treatment appears to add another
1.5 years of life to stage III patients. What's more,
when combined with the other therapies at the Ovarian
Cancer Center , Robert Bristow, the director, points
out that this achieves a new average life expectancy
of more than five years. “A big step forward,” he says.
Dangers of New Technology
|> Lisa Maragakis helped discover the reason for a sudden spike in infections.
When Trish Perl took a look at the number of bloodstream
infections in the pediatric intensive care unit during
the final quarter of last year, she knew something
was decidedly wrong. In children who'd had catheters
inserted, there had been more than 17 infections per
1,000 catheter days, compared to a norm of only five
per 1,000. What made the numbers especially frustrating
was that PICU staff were in the midst of a campaign
to decrease such infections and had been paying extraordinary
attention to how they inserted and monitored catheters.
Perl, director of Hospital Epidemiology and Infection
Control, launched a probe but found little to go on.
Then, last summer, a break arrived in the form of a
new infection control nurse, Karen Bradley, who'd heard
similar cases discussed at national meetings. The common
denominator had been a state-of-the-art technology
called a positive pressure mechanical valve, or PPMV.
Unlike earlier ports that attach to IV lines, the new
PPMV featured a mechanical valve that allowed access
without using a separate needle.
With this news in hand, the team of investigators
headed straight back to the PICU and discovered that
the unit had indeed switched to the PPMV at exactly
the moment the infection rate started climbing. Perl
quickly alerted the whole Hospital, the FDA and the
Centers for Disease Control and Prevention, and by
last February, all PPMVs in the Hospital had been removed.
In the PICU, bloodstream infections dropped to baseline
rates as soon as the unit returned to its earlier equipment.
Lisa Maragakis, an infection-control specialist here,
says she isn't sure exactly why the PPMV increased infections.
She speculates, though, that the device may not completely
flush out existing blood products, allowing dangerous
organisms to grow. But there's a moral to this story,
Maragakis says. New technology needs careful monitoring. “Just
because a device is state-of-the-art doesn't mean it
can't cause problems.”
The Persistent Patient
Dave Rutstein, who had successful surgery for lung cancer—with his wife Rena.
Dave Rutstein is as meticulous about his health as
he is about his law practice. The 60-year-old Washington
attorney, who was general counsel of Giant Food for
22 years, jogs, does yoga and gets a physical each
spring. Four years ago, at the usual “very boring annual
hour” with his internist, Rutstein asked if he should
have a heart scan test, something he'd read about in
The Economist. His doctor seemed indifferent to the
idea, but Rutstein's diligence ended up saving his
The test revealed a sound heart, but some of the 39
resulting pictures inadvertently captured a portion
of the left lung. Something was there. But since Rutstein
has never smoked, his doctors told him he was 95 percent
safe. Nonetheless, he sought a second opinion on how
to proceed, and one name kept recurring: Stephen Yang,
chief of thoracic surgery at Johns Hopkins.
When Yang looked at Rutstein's pictures, he lowered
the odds to about 70 percent safe. “One of the reasons
I went to law school was because I wasn't very good
in math,” says Rutstein, “but I could understand that
I had a one in three chance of cancer.” Yang explained
matter-of-factly that to push forward, whether the
results were cancerous or not, Rutstein would need
major surgery. There was no possibility of biopsy.
“My mindset was, if it was not cancer, then I was
putting myself through needless pain and recuperation,” says
Rutstein. “But if it was cancer and I waited, there
could be horrendous results.” And so Rutsetein took
Yang's next opening and was lying on the operating
table within a week of his first appointment.
On the day of surgery, a fairly relaxed group of Rutstein's
family and friends felt sure that everything was fine,
right up until they heard the words “lung cancer” from
the surgeon. Yang had removed his patient's left lower
lobe, including a dime-size lesion, a rare form of
cancer called bronchioloalveolar carcinoma often found
in nonsmokers. And though this kind of tumor occasionally
develops at multiple sites in the lungs, Rutstein's
lab results came back clear. He needed no radiation
or chemo. Since that day, July 5, 2001 , he has been
Today, Rutstein says he's heard from a lot of physicians
that few people would have chosen surgery with his
odds and health profile. He's glad he did.
Minus a Rib, An Athlete Becomes Whole Again
After surgery, Jad Vonderheid checks his time at his final meet for U MASS.
Jad Vonderheid first noticed the trouble in his right
arm during his junior year at the University of Massachusetts
. Vonderheid, a star swimmer for the Division I school,
suddenly found himself with a limb that was swollen,
painful and turning blue. An ultrasound revealed two
Jad was diagnosed with a rare disorder, called “effort
thrombosis,” associated with repetitive limb exertion.
Also called Paget-Schroetter syndrome, the condition
involves compression and injury to the main (subclavian)
vein as it passes through the thoracic outlet, the
tight, triangular path between the neck and chest.
The vein often gets pinched by the first rib and scalene
muscle against the collarbone.
Jad's was a classic case. Just brushing his teeth
caused his arm to discolor. Surgery to remove his rib
and trim the muscle was an option, but physicians painted
a sobering picture. He could end up in constant pain,
never able even to throw a ball or lift a box.
Jad opted instead for blood thinners to dissolve the
clots, but found no relief. He stopped swimming, didn't
attend the championship meet that year and fell into
a depression. “You have no idea how discouraged we
were,” recalls his mother, Betsy Vonderheid, of Annapolis
Then, late one night, Jad called his parents asking
for help—he wanted the operation. The Vonderheids decided
to go outside their health plan to consult with vascular
surgeon Julie Freischlag, chief of surgery at Johns
Hopkins. And at last they heard something positive.
Freischlag told Jad, “I can make you whole again. You
can be normal.” (> See
interview with Dr. Freishlag)
“My son said, Sign me up,” his mother remembers, “and
I started to cry.”
On August 2, 2004 , Freischlag cut under Jad's right
arm to remove his rib and chunks of his scalene muscle.
He was discharged the next day. Two weeks later, his
venogram showed that his injured subclavian vein was
forming a new path of collateral veins almost as big
as the original. Following physical therapy, he returned
to school for his senior year and began training.
Slowly Jad built up to an hour of practice a day (one-fifth
the norm) and made the conversion from distance swimmer
to sprinter. At his final home meet last January, he
swam his lifetime best times in the 50-yard freestyle
and the 100-yard butterfly while his teammates and
onlookers screamed encouragement. In February, when
the UMass Minutemen won their fifth consecutive A-10
title, Jad shaved another 0.14 seconds off his 50 free.
Mary Ellen Miller
More Than PMS
Oh, you're just being emotional!” Psychiatrist Karen
Swartz has stopped counting the number of female patients
who've told her their doctors have dismissed them with
some version of that phrase. “It not only does them
a disservice,” she says, “but writing off what may
be serious mood disorders as ‘just hormones' is also
dangerous.” It's just one more reason why Swartz and
colleagues opened Hopkins ' Women's Mood Disorders
Center last fall.
The clinic centers on the common ground between hormones
and mood disorders—namely pregnancy and postpartum
disorders, menopause-related disorders and premenstrual
dysphoria disorder (PMDD). Women with major depression
or bipolar disorder—both marked by gender differences—are
also seen by Swartz or colleague Jennifer Payne, psychiatrists
who co-direct the center.
Some of the center's reasons for being are sociological. “Many
women, for example, are less likely than men to acknowledge
depression because they feel whole households depend
on them,” Swartz says. And dealing with depression
during pregnancy is dicey. Some women you just monitor,
Swartz says. For others, not treating depression puts
them and their babies at risk—low birth weight or other
problems. “We walk a fine line,” she says.
Adventures in the Skin Trade
years ago, when a couple of School of Medicine faculty
members launched an online image database for dermatology,
it seemed unlikely that they would turn themselves
into Hugh Hefner offshoots. DermAtlas, the name Christoph
Lehmann and Bernard Cohen came up with for their site,
was meant as a resource for doctors and patients seeking
information about skin conditions. Then the site collided
with the X-rated Web.
Cohen and Lehmann first hit on the idea of an online
dermatology resource after a chance meeting in a hallway.
Cohen had the imagery. Lehmann had the technical skills.
Their purpose was clear, their intentions were noble.
Doctors across the world would have access to thousands
of visual case studies. A patient with a lesion could
compare it to DermAtlas photos to see if he might
need treatment. “We thought we were putting a medical
textbook online,” Lehmann recalls.
By 2003, however, Lehmann started noticing unusual
traffic patterns. On one day alone there was a 40
percent jump in the number of visitors. Why the spike?
Had the American Academy of Dermatology alerted its
members to the existence of a brilliant new resource?
Had acne.org sent traffic their way? Not quite.
After a little digging, Lehmann discovered that the
visitors had actually come upon DermAtlas through
a site called gorgasm.com. Suspicious, he clicked
through and, at that moment, surrendered his Web-surfing
virginity, stumbling into the Internet's equivalent
of the curtained-off room in the local video store.
Almost one-fourth of all Internet searches, it seems,
are hunts for nude photos, and with a name like DermAtlas,
porn prowlers thought they'd discovered a gold mine.
Now, to be clear, Lehmann states, “None of the images
we have on the DermAtlas Web site are even close to
pornography.” But that hasn't stopped surfers from
perusing it for the wrong reasons. Further analysis
revealed a disproportionate number of queries related
to genitalia. And the porn-seekers brought another
kind of unwanted attention. One watch group filed
formal complaint—since dismissed—accusing the DermAtlas
developers of trafficking in child pornography.
Once they'd gotten over their disbelief, Lehmann,
Cohen and an “infomatician” named George Kim responded
like true scholars and planned an article. The result,
which will appear in the Proceedings
of the Annual Symposium of the American Medical Infomatics
addresses the logistical, ethical and even legal strains
that porn-seekers place on medical image-based libraries.
The researchers have also taken steps to prevent
further misuse and built what Lehmann calls a “crude
but effective filter.” At this point, he's still manually
entering the sites he wants to block. Though he'd
love to automate it completely, that won't be simple.
For one thing, not all the unwanted visitors are
focused on genitals or breasts. “For a while,” Lehmann
says, “we were a really big site for foot fetishes.” The
police work isn't just a matter of flagging crude
search terms, either. The group doesn't want to turn
away patients just because they use slang instead
of the correct anatomical terms.
That said, there are giveaways. When someone searches
for “foreign body in vagina”—which has happened—it's
clear they're not interested in dermatology. They
either need to get to an emergency room or a very
different sort of Web site.
The filtering effort is important, and the group
hopes that their paper will alert other well-intentioned
developers to the perils of publishing image- based
medical content online. But for the most part, they're
focused on improving DermAtlas, which now has upward
of 28,000 visitors a day from around the globe. “We
want this to be the biggest, baddest dermatological
resource on the Web,” Lehmann says.