Not Bread and Butter
Ask Paul Colombani about the cases he operates on,
and he'll tell you: “We don't do a lot of bread-and-butter
surgery here. More and more, our patients come for
a last-ditch operation.” Which could sound puzzling
until this chief of pediatric surgery describes a case
like 11-year-old Amanda Long's.
Born in Missouri with a rare congenital defect in
which the esophagus fails to develop as a continuous
passage from the mouth to the stomach, Amanda, had
her esophagus reconnected during infancy and a second
repair at age two. But after seven quiet years, “she
would swallow and the food would get stuck at about
the breastbone area,” her mother, Stephanie, describes.
She stopped eating and started losing weight.
That marked the beginning of Amanda's year of five
operations, and hovering on the brink of death. Physicians
at Children's Mercy Hospital in Kansas City tried dilating
the area so food might pass through, but the tissue
became too thin. Then, surgeons found that her esophagus
had fallen down to her spine. They closed her up after
nine hours of exploration. Finally, a 21-hour procedure
to remove her esophagus and replace it with a section
of the small intestine failed. Hemorrhaging, vomiting
blood and unable even to swallow her own saliva, Amanda
was covered with massive infections from the various
lines going into her body.
“But no one would touch her case,” her mother says.
Hospitals all over the country said she would have
to stay the way she was. Finally, Stephanie called
Colombani and described Amanda's situation. His response
was immediate: Staying the way she was wasn't an option.
And so, on Nov. 4, Colombani performed a procedure
known as a gastric pull-up on Amanda that connected
her stomach directly to what was left of her esophagus.
It was a bloody 12-hour surgery during which Colombani
struggled to remove the old, densely scarred graft.
But after it was all over, Amanda spent a mere two
weeks in the Hospital, and for the first time ever,
she had no leaks in her follow-up swallow study.
Since then, Amanda's recovery has been slow but steady.
This fall, she began middle school. And some days,
she says dryly, “I can even eat double quarter pounders
Mary Ellen Miller
The Tiniest Transplant
Johnson, thriving two months after
receiving a new liver.
Liver transplants—even on children—have been around awhile. But last year,
when a two-week-old infant named Brady Johnson showed up at the Hospital weighing
a little over five pounds and in liver failure, pediatric surgeon Henry Lau
knew he was facing a different sort of challenge.
Brady, who had weighed in at five and a half pounds,
had looked fine when he was born in Fairfax, Va. But
just 18 hours later, he turned yellow. Doctors sent
him home, fairly certain the jaundice would disappear.
It didn't. Every time the baby's blood levels were
checked, his bilirubin had gone up. He was suffering
from a severe liver disturbance, but a test for the
common culprit, a blockage in the ducts that drain
bile from the liver, came out negative. For some unknown
reason, Brady simply had a liver that wasn't working.
By the time the infant arrived at Hopkins on May 13,
2004, his white count had soared and his liver had
completely shut down. “I've never seen anyone so yellow
in my life,” his mother, Julie, remembers. Brady's
only hope for survival would be a new liver. He went
on the transplant list as status 1, meaning he was
likely to die within days.
Nine days later, Julie and Eric Johnson learned that
an infant's liver had become available, and pediatric
surgeon Lau was about to begin putting it into their
son. Brady would be the smallest liver transplant patient
in Hopkins' history. “It doesn't get any bigger than
this,” the anesthesiologist told the Johnsons.
Then, as Eric and Julie waited hour after gut-wrenching
hour outside the OR, Lau removed their baby's failed
liver and stitched the tiny donor organ into its cavity.
Ten hours later, Lau came out to tell them Brady had
pulled through, and “prayers wouldn't hurt.”
The baby's recovery from the huge surgery came inch
by inch, but the transformation in his appearance,
Julie says, was instantaneous. “Out came this beautiful
child—pink. My husband called it the “oil change.”
Consultation with The Oracle of Preventative Cardiology
Amid a flood of new ideas for avoiding heart disease
coming from the fall gathering of the American Heart
Association, we've asked the Ciccarone Center's Roger
Blumenthal for a summary.
Bill Clinton's surprise bypass got people talking
about how someone with such top medical care could
still require an emergency bypass. What happened?
Clinton's case exemplifies the weakness of relying
solely on the traditional risk factors highlighted
by the Framingham Study. Clinton appeared to be within
acceptable ranges on blood lipids, but he had other
risk factors that were not so apparent. We believe
we can improve risk prediction with some emerging new
factors, including genetics and protein analysis.
Are tests available for these new areas?
Not yet, but two other recently developed tests are.
First is the coronary calcium scan, which can give
us a pretty clear look at areas of atherosclerosis.
We recently studied 2,000 patients with these scans.
Their average age was 50, and many, like Bill Clinton,
showed no evidence of markedly elevated traditional
risk factors. We were surprised at how many had high
levels of atherosclerosis.
The other test is for C-reactive protein, an agent
that indicates inflammation anywhere in the body. A
high CRP reading is now considered a good reason to
treat patients previously deemed at intermediate risk
for heart attack much more aggressively with aspirin
and statin medication.
Is the treadmill stress test still considered valuable?
Yes, but we're learning more about how to interpret
its results. We did a 20-year follow-up on people who
had undergone cardiac stress tests and were impressed
by two key factors. The first was that exercise capacity,
or how long a person walks on the treadmill, is probably
the best determinant of whether that person is going
to have a heart attack or stroke over the next 20 years.
The second stems from heart rate recovery—how much
your heart rate drops in the two minutes after exercise.
(In a healthy person, it should drop by about 50 beats
per minute within two minutes.) It turns out that people
with either below average capacity or below average
recovery rates accounted for more than 90 percent of
major cardiac events over the ensuing 20 years.
Interviewed by Edith Nichols
A Ruptured Aneurysm in the Right Patient
Surgeon Bruce Perler used a new technique to save Henrietta Bartecki
Early last summer, Henrietta Bartecki felt the worst
pain she'd ever known—a terrible piercing on the right
side of her abdomen. “I wouldn't wish it on a dog,” she
says. The 84-year-old widow ended up at Hopkins, the
hospital closest to her tiny row house. By the time
she reached the emergency department, “Man, I was screamin'
and hollerin',” she says.
Bartecki had suffered a ruptured abdominal aortic
aneurysm, a condition so lethal that the massive internal
bleeding kills half of victims before they even make
it to the hospital. Only when the leaking blood happens
to form a clot that seals the hole—thereby giving surgeons
enough time to intervene—do patients make it through.
When Bruce Perler, director of vascular surgery, set
eyes on Bartecki in the emergency department, he estimated
her chances of surviving an open operation were less
than 30 percent. Her age and underlying health problems—hypertension
and heart disease—already put her behind the eight
ball. Her body had also sustained a tremendous shock,
and traditional surgery, releasing the clot to sew
in a graft and repair the aneurysm, would cause her
to lose more blood. “It's about as difficult a surgical
procedure as we do,” Perler says.
Then it hit Perler that this woman might be a candidate
for a new technology in which a graft is advanced up
to the aneurysm through a small incision in the groin,
then mesh-like stents are expanded to hold the graft
in place. The technique had never been performed emergently
here to fix a ruptured aneurysm. In the whole world,
in fact, there had been only a few hundred such cases.
“You have to have the right patient with the right
anatomy,” Perler explains, so Bartecki needed to remain
stable long enough for him to get a CT scan, measure
the size of her aneurysm, and make sure he had the
right graft. “Turns out, we had one stent graft in
house that was just the right size.”
Even after the minimally invasive surgery, Bartecki
spent two weeks in the Hospital recovering, which convinced
Perler that she probably would not have survived a
traditional operation. Today, though, she's back home,
going out for walks and playing bingo with her “lady
“Oh, no, I don't stay home!”
Mary Ellen Miller
Can This Drug Tame Hard-Core Depression?
To help John Helliwell with debilitating depression,
physicians prescribed virtually every available antidepressant.
Most of the drugs had no effect. Some worked briefly.
Others launched him into his other nemesis, mania (even
though he was also on a mood stabilizer). Helliwell,
who was putting his wife and teenage son through what
he describes as “holy hell,” finally rounded up the
whole slew of meds and swallowed the lot.
Luckily, the 43-year-old Baltimorean was resuscitated.
Then, shortly afterward, he happened on an ad for a
Hopkins study led by psychiatrist Jennifer Payne testing
a different kind of medication in people suffering
from depression, who seemed impervious to known antidepressants.
Payne was studying the drug riluzole, a neuroprotective
agent that's typically used to slow the progression
of ALS, or Lou Gehrig's disease. She had been reading
data suggesting that excess glutamate—the brain's most
widely distributed neurotransmitter—might play a role
in triggering depression. One of riluzole's main effects
is to inhibit the release of glutamate. Payne's double-blind
trial would ask patients with bipolar disorder, like
Helliwell, to continue taking a mood stabilizer while
they also took either riluzole or a placebo.
Helliwell became one of Payne's first enrollees. At
the end of his eight weeks in the study, he says he
wasn't surprised to learn that he'd been taking riluzole,
not placebo. The steady improvement in his mood and
his sense of well-being was so obvious that he elected
to continue the regimen. “It's the longest any drug
has worked for me,” Helliwell says. “After years of
curling up in a ball on the sofa, I get up every day
and move forward.”
Mary Ann Ayd
Let's Meet... Luca Vricella
“I love the anatomy, the attention to detail,” Luca
Vricella says about his
job as a pediatric cardiac surgeon. “Technically,
I think it's one of the most wonderful disciplines
in surgery. All the instruments are small, the field
is small, the patient is small.”
Vricella, who was born and raised in Rome, comes from
a family of physicians, including his father, two uncles
and his twin brother, who is a plastic surgeon. His
own decision to become a pediatric cardiac surgeon
occurred while he sat watching an open heart operation
on a small black and white TV. “I thought, This is
just absolutely fantastic.” He was 10 and never looked
During medical school, Vricella spent summers in the
United States dabbling in American medicine. By the
time he graduated, there was no doubt that he would
leave Italy for the rest of his training. “I thought
the hands-on experience was just fantastic, the way
they gradually brought you up to very high-level, complex
Four years ago, as Vricella wound up his residency
and fellowship, Hopkins' chief pediatric surgeon Duke
Cameron recruited him for the faculty here. Describing
why the thrill of his specialty remains as strong as
ever, Vricella marveled recently, “Dr. Cameron and I just operated on
a baby that was 2 days old, 2.8 kilos, and she's just
about to go home doing well. I like the fact that it's
the chance—how can you say?—to help a life that's developing.
We establish a relationship with these families and
patients, and it's a beautiful thing.”
Mary Ellen Miller
Ball and Socket Switch Makes an Arm Work
Mary Boody has no trouble raising her
With a single misstep on winter ice in 2003, Mary
Boody crashed onto her shoulder and destroyed her right
rotator cuff. Even after Boody underwent surgery to
repair these vital muscles and tendons that stabilize
the shoulder joint, followed by physical therapy, she
remained in nonstop pain. Unable to lift her arm, she
couldn't so much as comb her hair. Finally, her Eastern
Shore physician referred her to orthopedic surgeon
Edward McFarland at Hopkins.
Most minor rotator cuff tears can be treated nicely.
But as recently as two years ago, patients with the
major damage of an “end-stage tear arthropathy” like
Boody's had to resign themselves to ongoing disability.
The reason, McFarland says, is that even traditional
joint replacement only works if a substantial portion
of the rotator cuff is functioning. Otherwise, patients
emerge much like a marionette with broken strings:
sometimes in less pain yet missing the means to raise
the affected arm much above their waist.
Now, however, McFarland has begun performing a novel
three-hour operation that returns many of these patients
to a freer life: He literally switches the positions
of the shoulder's ball-and-socket assembly. By turning
the scapula into the shoulder joint's ball and the
upper end of the humerus into its socket, he taps the
strength and mechanics of the large, triangular deltoid
muscle covering the shoulder. The switch allows the
arm to move forward, backward and sideways, while providing
the joint with stability. Patients begin moving their
arm in a day or two and need pain medication less than
a week. Three months of physical therapy builds strength.
“Dr. McFarland explained that I wouldn't be able to use
my arm the way I could before my injury,” Boody says, “because
the prosthesis wasn't designed that way. Still, I have
great use of my arm again and no discomfort.”
The Brodsky Approach Saves a Life
Rob Brodsky and Mark Strome, whose severe blood disorder is cured.
Mark Strome felt like one lucky guy as he drove his
silver Maserati toward his mother's birthday party
in June 1999. Every financial publication had recently
touted his skill in creating one of the world's most
successful hedge funds. But on that June morning, the
Los Angeles financier got the first signal that his
future may have hit a roadblock—a nosebleed that wouldn't
Skipping the party, Strome drove straight to his physician,
who cauterized his nose. When the bleeding kept on,
the doctor ordered tests and found that Strome's platelet
count was 1,000 out of a normal 200,000. “Go immediately
to the hospital,” he directed. “And don't bump your
head or you'll die.” A week later, Strome learned he
had severe aplastic anemia (SAA), an amazingly rare
disease in which the body's immune system renders the
stem cells incapable of producing red and white blood
cells and platelets. Death usually comes in one to
Strome's LA hematologist suggested standard treatments—bone
marrow transplant or the “gold-standard” drug, ATG
(antithymocyte globulin). In Strome's case, no suitable
donor appeared for him to have a bone marrow transplant,
so he went on the drug. But ATG leaves patients wide
open to side effects like infection, hormonal imbalance
and osteoporosis. Up to half also have a relapse or
develop some type of malignant blood disease. Strome
followed the script. First, he improved and then the
SAA came back. By August 2000, he'd run out of options.
And at that crucial moment, a psychiatrist friend surfing
the Web discovered Rob Brodsky.
Brodsky, a 44-year-old Johns Hopkins hematologist,
has been achieving extraordinary results with SAA patients
by treating them with a novel protocol. First, he gives
them massive doses of the immunosuppressant cyclophosphamide,
which stuns the bone marrow and sends already low blood
counts plummeting to zero. Then, he waits as patients'
decimated immune systems miraculously begin “rebooting” and
return to what Brodsky describes as a “healthy, virgin
state, like that of a newborn child.” Once that happens,
the person's chance of developing SAA again becomes
Two weeks later, Strome was in Brodsky's office hearing
that the likelihood he could be cured was about 70
percent. But he would need to endure 12-hour days of
drug infusions plus debilitating side effects. His
treatments began the next week, followed by terrible
nausea and a lung infection. But by Halloween of 2000,
Mark Strome was back in LA, working out five times
a week. Today, he is completely healthy.
Since then, Strome has donated $2 million to Brodsky's
SAA research. “I used to be the Lone Ranger,” Strome
says. “I thought I could solve everything by myself.
Now, I'm alive, but the Lone Ranger is dead.”
The Schizophrenic Mind Becomes Clearer
Breathing heavily and carrying multiple bags, Akira
Sawa raced into a classroom full of students last fall
and apologized for being a “not so well organized person.” But
Sawa, a neurobiology researcher who once practiced
psychiatry in his native Japan, has impressed his fellow
scientists as just the opposite. Over the last couple
of years, using a highly systematic approach, he's
made notable strides in determining the genetic basis
To get to the root of the disabling psychiatric condition,
Sawa and his lab group have been zeroing in on a gene,
named DISC1 (short for Disrupted In Schizophrenia 1).
Researchers learned that DISC1 has something to do
with schizophrenia several years ago when they studied
a Scottish family with the disease peppered throughout
and discovered that many members had a mutation in
this gene. Sawa took this knowledge to the next step:
Because scientists also knew from autopsies and MRIs
that people with schizophrenia possess misshapen neurons
and have abnormally large brain ventricles, he began
looking for a link between these defects and mutant
First, he created one set of neurons in which the
DISC1 wasn't functioning and then he compared the behavior
of these neurons with another set in which the gene
was at work. What Sawa observed was amazing. The neurons
with inactive DISC1 dawdled, while those where the
gene was active moved in a swift and orderly fashion.
The neurons with inactive DISC1 were also misshapen.
Once Sawa was certain of DISC1's role in schizophrenia,
he went on to “knock down” the gene in laboratory mice.
This, it turned out, actually induced the malformed
neurons and large brain ventricles that appear in the
human form of the disease. Now, Sawa is examining the “schizophrenic” mice
more thoroughly to try to pinpoint other markers. Discovering
these traits, he reasons, could help diagnose the condition
and lead to drugs to treat it.
With such stunning successes in hand, Sawa is ready
to start comparing living neurons from schizophrenia
patients with neurons from healthy people. He extracts
olfactory epithelium cells (a little tissue from the
nose) from willing patients in a 10-minute outpatient
procedure and starts analyzing. “What's so thrilling
about all these experiments,” Sawa says, “is that they
keep reminding us how precisely our brains are organized—which
perpetually makes us appreciate the beauty of the human
A Newscaster Rejoins the World
|>Newscaster Bob Turk on the air and hearing again.
For more than a decade, Baltimore newscaster Bob Turk
battled increasing deafness. At first, he managed with
hearing aids. But by late 2004, even the most expensive
models were failing him: Hearing in his right ear had
plummeted to less than 15 percent, and what speech
he caught in his left ear kept breaking up. No longer
able to understand the news anchors on the air, Turk's
responses could sound confused. “After all those years
at the station,” he says, “I didn't know if they'd
Finally, Turk went public about his hearing loss in
a broadcast last spring, and a coworker asked when
he planned to do something about it. He decided right
then to look into a cochlear implant.
Unlike hearing aids, which merely amplify sound, cochlear
implants bypass impaired inner-ear structures and stimulate
the auditory nerve directly via an electrode array
surgically embedded in the cochlea. The devices have
been well publicized in children, but adults with acquired
deafness account for about half of Hopkins patients.
Most people don't seem aware, says John Niparko, director
of Otology and Neurotology, that adults can do well
with a cochlear implant. They benefit from their already-established
ability to comprehend speech.
When the unit is activated a few weeks after surgery,
Niparko explains, the patient's new perception of sound
isn't automatically understandable as speech. The external
components of the device, a sound processor, microphone
and transmitter—must be individually mapped and fine-tuned.
Further, patients must be highly motivated to relearn
how to interpret incoming signals. “There's a big difference
between having your sound sensitivity restored and
being able to listen effectively,” Niparko says.
But Bob Turk surprised everyone. “The minute my implant
was activated,” he reports, “it was like I was in a
radio booth with a headset and mike. Right away, I
heard and understood everything. And the next week,
I was back on the air. If you ask me, John Niparko
is a miracle worker.”
Mary Ann Ayd
Baiting the Hook
For Ivan Borello, a researcher at the Sidney Kimmel Cancer Center , who's been trying for awhile to teach the body to fight off cancer, the last few months have been heady. Working with two kinds of human T-cells—which come equipped with the power to identify cells foreign to the body and mark them for destruction—Borello and his team discovered that one group was “smarter.”
For the study, the researchers used only T-cells that had been primed to attack the bone cancer myeloma. They took some from the bone marrow, however, and the rest from the blood. When they tested the kill-rates of both T-cell groups, they found the bone marrow cells outperformed their blood counterparts by more than 90 percent.
“Now we have evidence that educating T-cells in the bone marrow may be the most effective way to get an anti-tumor response,” Borello says.
The next step will be to test the activated marrow
T-cells in a few myeloma patients, both alone and in
combination with a myeloma vaccine. What's especially
exciting, though, is that the researchers believe that
patients with other blood, bone marrow and solid tumors,
such as breast cancer, may also benefit from this type