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.”

Ramsey Flynn

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 year.

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.

Jeanne Johnson

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 fixing it.

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 the lungs.

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

> Michael 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 pulmonologist says.

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.”

Michael Levin-Epstein

Let There Be Light—From Artificial Retinas

> The bright disk in this patient's eye may be helping him see.

Wilmer ophthalmologist 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 tuned.

Ramsey Flynn

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 survival.

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 survival.”

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.

Ramsey Flynn

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.”

Michael Levin-Epstein

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 life.

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 healthy.

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.

Mary Ellen Miller

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 blood clots.

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.

Michael Levin-Epstein

Adventures in the Skin Trade

Five 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 a

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 Association, 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.