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Rolensky's Echo - Revealed
When Rolensky returned to the Hospital St. Nicolas from Cange, Sara Gonzalez, a pediatrician who shares the same global health fellowship as Chris Carpenter, awaited Gerdline and Rolensky's arrival. When she was told what Dr. Smith had found on the echo, she wasn't sure what to think.
Back in Cange, Dr. Smith had placed the echo transducer on Rolensky's fontanelle, an area of a baby's head where the bones of the skull haven't yet completely joined together. By looking through the fontanelle, Dr. Smith was able to get a look at Rolensky's brain. "There was a very large free space on the echo that lit up like a Christmas tree," Dr. Smith said.
This area in Rolensky's brain is called a Vein of Galen malformation - a kind of abnormal dilation where lots of arteries converge, creating an abnormal pocket of blood before the veins drain it away. "Because of the large amount of pooling blood, the malformation acted like a suction cup, pulling up blood from the heart," Dr. Smith says. Since so much blood is lost to filling up this space, the heart has to work extra hard. Over time, this causes pulmonary hypertension and shortness of breath, and ultimately, poor feeding and malnutrition.
Sara Gonzalez had
heard of the Vein of
Galen malformation,
but like many other
physicians, did not regularly associate the rare condition with pulmonary hypertension. What she did know was that fixing Rolensky probably meant a very complicated surgical repair to the malformation, a solution that was unlikely to be found in Haiti.
So she reached out to a special group at Boston Children's called the Vascular Anomalies team who perform surgeries for children who have structural problems in the vessels. After they reviewed Rolensky's case, Sara thought they would recommend palliative, or end-of-life care, since the outcome of children with this condition is very poor. But that's not what happened. "The team thought he was a good candidate for surgery and wanted him to come to Boston," Gonzalez said.
With the help of a medical student, Anne Beckett, Sara set Gerdline and Rolensky up for their first trip out of Haiti to the United States. She took a passport photo with her iPhone and ordered an oxygen concentrator for Rolensky's flight. She bought sildenfil - Viagra tablets for the pulmonary hypertension - at a drugstore down the block. "Anne and I got some strange looks while buying it, I'm sure," Gonzalez says.
When Gerdline and Rolensky reached Boston Children's, he was taken into the hands of Dr. Darren Orbach, a calm, experienced interventional radiologist. "The Vein of Galen malformation can either be very simple or very complex, depending upon its architecture - his was more complex," Orbach explains. He reviewed his strategy for the procedure to me: since the malformation is supplied by lots of arteries, called "feeders," the goal is to close these feeders without harming the rest of the brain. Using metal coils or a viscous glue, he can close the largest feeders to prevent blood from filling the abnormal space, effectively closing down the vein of Galen malformation and allowing blood to travel to the appropriate parts of the brain.
Rolensky was intubated and then taken into the procedure room on May 4th. "I closed the largest feeders on the right and left side," Orbach says.
After closing the feeders, the malformation
closed down, barely filling with blood. Suddenly, vessels in Rolensky's brain that had received only a trickle of blood now blossomed on the CAT scan. The malformation was effectively closed.
"He made rapid progress in front of our eyes," says Dr. Mary Mullen, a cardiologist who helped care for him in Boston. "He was weaned off oxygen and started to be able to eat solid foods and clearly became stronger." Tracking Rolensky's weight, he has now reached an almost normal size for his age. His shortness of breath was gone.
Just a couple of weeks ago, Rolensky celebrated his first birthday. When I touch his hand, he grasps my finger and glances up at my face, his eyes full of the kind of curiosity any one-year-old shares. Gerdline lets the interpreter, Nadege, take Rolensky into her lap. Like Nadege, many physicians, nurses, social workers and other health-care professionals have played a role in saving Rolensky's life. Showing you his photograph and telling his story is important because it illustrates how medicine, at its best, can placate human suffering. We are much better at responding to individual stories than processing numbers. But in truth, there are thousands of children like Rolensky in Haiti who do not have his outcome. Many children in the same hospital where Gerdline first brought him blue-skinned and short-of-breath, die of congenital heart disease, cholera, and malnutrition - children whose lives could be saved if resources are appropriately channeled.
Even Gerdline recognizes the difficult reality she'll face returning to in Haiti. "I'm not tempted about going back," Nadege says, interpreting Gerdline. "I have to go." Nadege clarifies what she means: Gerdline wouldn't go back to Haiti if she had a choice. She worries too much about Rolensky's future there.
Chris Carpenter agrees. "Mom doesn't have the food security, she doesn't have the resources or the family supports." However, he is hopeful for Rolensky. "He'll see Sara [Gonzalez] in outpatient clinic. And if he continues to have persistent pulmonary hypertension, we'll bring him back."
Carpenter is optimistic that relationships between American doctors, volunteers in Haiti, and Haitian doctors can help support medical care for Rolensky and other children like him, as long as medical systems in this country continue to make global health a priority.
"Have you seen what Rolensky learned the other day?" Nadege asks me.
I shake my head.
"If you say hallelujah, Rolensky raises his hands above his head."
He turns towards us at the mention of his name.
"Rolensky!" Nadege says. "Hallelujah!"
A moment later, he holds up both of his arms.
If you are interested in donating to support care for children like Rolensky, please visit Partners In Health (www.pih.org).
Rolensky's Echo
This is the case of a real patient. After reading the description of the
case, I invite you to guess the patient's diagnosis in the comments section
below. The answer will be posted Friday.
Only 21 years old, this is Gerdline's first trip to the United States. We are sitting by the Au Bon Pain in Boston Children's Hospital, the crowded atrium awash with activity. She holds her baby Rolensky in her arms. Gentle in her movements, her son's bright eyes open wide when she strokes his cheek. She's quietly agreed to an interview about the events of the past year.
Nadege August, a social worker for Partners In Health, sits with us, interpreting Gerdline's words for me. Gerdline is shy and barely makes eye contact, her gaze drifting around the spacious lobby as though she is still surprised by the circumstances that have brought her into such a large hospital in an American city. Nearby, doctors, residents, and families move in and out of the revolving doors and busy traffic clutters up Longwood Avenue. It's a very different scene than what Gerdline is accustomed to back at home.
Gerdline and Rolensky live in Arcahaie, a small town on the Western Coast of Haiti. Her brick house has four rooms and is crowded with twelve relatives. Despite the company, she's had little help raising Rolensky. Several times a day, she waits in line at the public pump to bring back water. The same river that gave her cholera two years earlier is the only source of water she has, and even then, the pump frequently runs dry. Although she's nursed him, she's never taken any vitamins or gotten any significant medical advice. After Rolensky was born, he had no pediatrician. Regular healthcare just isn't the custom in Haiti. "Most people don't trust the medical system there," says the interpreter Nadege, who was also born in the island country.
But when Rolensky turned about four months old, Gerdline saw that something was wrong: he had stopped gaining weight. His breaths were laborious and heavy to the point that he was unwilling to swallow. Gerdline left her town and brought Rolensky to the Hospital St. Nicolas, in the province of Artibonite, where a cadre of pediatricians, including some from the United States, were busy attending to sick kids from around Haiti. There, she met Chris Carpenter.
"I'm a global health fellow," Carpenter says. He's modest - at his young age, he has already set up an intensive care unit in the Congo and done work in the Dominican Republic, Guatemala, and Cameroon, and is a physician at Boston Children's Hospital. He was on a medical trip to the pediatric ward at Hospital St. Nicolas in September when Gerdline arrived with Rolensky. "He was very small for his age," Carpenter remembers, describing Rolensky's rapid breathing and blue skin that comes with poor oxygenation. Rolensky was cachectic - his muscles were wasting away. Gerdline and Rolensky were directed into a crowded hallway that was being used as a makeshift intensive care unit. "We were very worried by his condition," Carpenter recalls.
Gerdline wasn't just worried - she was terrified. Around her, the hospital had reached almost double its capacity - babies and young children awaited the attention of doctors, some crowded together on single beds in various stages of distress. Gerdline took a seat on a metal chair, her eyes fastened on Rolensky. At night, she lay on the floor beside his bed, barely able to sleep. "Kids were dying every night," Nadege interprets for me. "I never saw her leave the hospital," Carpenter says. "She was next to him every moment."
Carpenter and his colleagues carefully examined Rolensky. His oxygen saturation was critically low. Using donated oxygen concentrators, they started him on 100% oxygen. Carpenter suspected he had pulmonary hypertension, a kind of high blood pressure that can occur in the vessels between the heart and the lungs. To help treat this, they crushed pills of sildenafil - the generic name for Viagra - which also ameliorates pulmonary hypertension. But Rolensky did not improve much. "He didn't have the energy to cry, or sit. He was nursing, but his weight remained low." They were not able to take the oxygen off - the moment they did, his saturations fell. The team was at a loss as to why Rolensky had pulmonary hypertension. Figuring out the cause would help dictate his treatment. They decided to send Rolensky to a clinic in Cange, where Dr. Frank Smith, a cardiologist from New York, was visiting for a few days to perform echocardiography, a study that images the heart.
"It was a very busy, hot, and crowded day," Dr. Smith says, describing when Rolensky visited the clinic in Cange. "We were seeing twenty children a day, all of whom needed echoes." Ordinarily, echocardiography is not easily available in Haiti, but it's a technology critical in diagnosing heart disease in infants, many of whom suffer from rheumatic heart disease. "He was very malnourished. When I examined him, I was looking to figure out why he had pulmonary hypertension."
Pulmonary hypertension is rare in a young infant. When it occurs, the babies usually have a valve defect or another structural problem in the heart. But Dr. Smith couldn't find any heart abnormality to explain the pulmonary hypertension. Instead, he saw a heart that was working too hard, as though something elsewhere in his body was making his heart pump more than it should. Such heart overactivity can result in pulmonary hypertension. But why was Rolensky's heart overactive?
Sometimes, the key to a diagnosis falls into a medical practioner's mind the way a missing line finally comes to a poet. The process is beyond algorithm or logic but is instead founded on the valuable instinct that grows from the roots of prior cases, moments in a lecture hall, sentences glimpsed in otherwise long-forgotten textbooks. "I put the echo probe on Rolensky's head," Dr Smith says - and there he found the key to the case. Nearly four months after Gerdline had brought Rolensky to medical attention, the puzzle had been solved.
Can you figure out Rolensky's diagnosis?
Photo credits:
Picture 1 (Gerdline and Rolensky) - Katherine C. Cohen, Boston Children’s Hospital
Picture 2 (Hospital St Nicolas, Haiti) - Frankie Lucien, Partners In Health
Picture 3 (Rolensky) - Anne Beckett, Partners In Health
Could the new H7N9 Chinese bird flu reach Boston?
Occasionally, I'll be presenting stories about medicine from around the world. We'll travel to different settings to unravel and explore novel and emerging diseases, newfangled treatments, and little mysteries that shed light on human health, asking local doctors and researchers here in Boston to contribute their expertise. As our world grows more connected, events that affect human health in any country have the potential to involve all of us.
May 28, 2013
You've probably heard about the new bird flu, called H7N9, that has been simmering in Eastern China and has already killed 36 people. Given that Beijing is the second busiest airport in the world, a reasonable fear is that a Chinese flu epidemic could quickly spread if an infected person travels out of the country. Could this flu come to the US and turn into a global pandemic?
It could - but only if the virus spreads easily between people.
Sometime in early February of this year, a middle-aged man living in Shanghai City visited a live bird market. He picked out a seemingly healthy chicken which the vendor slaughtered on site. He brought the freshly killed animal home, where he washed, prepared, and cooked it.
Within 2 weeks of eating this meal, the man's body temperature shot up to 106 degrees Farenheit and he developed chills and was coughing up mucus. By February 20th, he was admitted to a local hospital, where his lungs failed. A week later, the man died in the hospital from this sudden illness.
But here's what's potentially alarming. The man from Shanghai hadn't lived alone. His father and brother stayed in the same house. In mid-February, both of these men developed coughs and high fevers. A week later, the man's father was also dead. The brother, luckily, improved. Both men were found to be infected by a bird virus that we now call H7N9.
Since all three men lived in the same house - can we assume the virus can pass between people? "The investigation by an international scientific team concluded that the evidence so far is not sufficient to say that person-to-person transmission has occurred," says Marc Lipsitch, Director of the Center for Communicable Disease Dynamics at the Harvard School of Public Health.
In short - no - we can't assume they caught it from each other. Instead, the three men may have picked it up from a visit to the local poultry market. Buying fresh poultry from a market is not unusual in China, where stacks of cages sprawl several blocks and contain birds, ducks, quail, and other animals in close quarters, providing ample opportunity for viruses to intermingle and generate new strains, in a process called reassortment. Humans probably pick up these new viruses after exposure to these birds, a risk that greatly increases during the process of slaughtering, preparing, and cooking (but not eating) a chicken.
Consequently, the Chinese government has chased after the suspected exposure by shutting down poultry markets. The results, so far, are decent. The epidemic has begun to wane - the last known case was reported on May 8th.
So thus far, humans don't appear to be spreading the infection to each other at detectable rates. Even if an infected person boards a plane bound for the US, he is unlikely to pass the disease to his fellow passengers.
But neither the US nor the Chinese CDC has put its guard down. There are alternative ways the virus can leave China other than inside people on planes. Since birds infected with H7N9 don't get sick the way humans do, they can silently spread the disease between countries. Some poultry from China are transported into nearby Vietnam. And the virus may be quietly transported by other winged birds that can fly vast distances outside of China.
And what of the three men who lived in the same house that all fell sick? Even if they visited a bird market, isn't it also a possibility they did really catch it from each other?
It certainly is.
The Malady and the Melody: Revealed
Chuck isn't averse to contemporary music, but he can be critical: "Have you heard Mumford and Sons?" he asks me. "They lack all nuance and dynamics," he says. Instead, he's partial to British pop: "the English make pounds and pounds of melody," he says, naming the band Swing Out Sister, whose music he describes as uplifting and positive.
Positivity is crucial, Chuck says, as though reminding himself. Soon after he began to bleed again, he drove to the hospital. A gastroenterologist offered a colonoscopy. Chuck agreed, preparing for the worst. Although the symptoms of inflammatory bowel disease had been quiet over the past decade, Chuck had lost insurance and hadn't had a colonscopy since 2001. The gastroenterologist returned with dismal news. "You have a really large cancer," the doctor told him, and it had already begun to spread from his colon to his liver. A few months later, it would be in his lungs.
Although it is a controversial idea, many specialists believe Crohn's disease increases risk for developing colon cancer; some gastroenterologists will routinely survey the intestine to ensure this process has not begun. Chuck's other symptoms were more certainly side effects from steroids he was taking for Crohn's: prednisone had damaged his teeth, caused a cataract on his left eye, and destroyed his hips, a condition called avascular necrosis, that made walking hard.
Aside from surgery, Chuck needed chemotherapy. "Depending on the drugs," he tells me, "I used to get really sick and uncomfortable from the chemo treatments." It was during one of these infusions that Chuck had a fortuitous meeting. "I met her through happenstance," he says, referring to Lorrie Kubicek, music therapist and director of the HOPES program at Mass. General. Lorrie, who uses harp, guitars, ukeleles, xylophones, and singing to help patients through cancer and other illnesses, suggested that music might help Chuck better tolerate the chemo. "Prior to his infusion sessions, Chuck would go home and search through his collection and would bring in a list of songs and we'd burn a playlist onto CD for him."
"The music washed over me," Chuck says. "I don't take any anti-nausea medicines anymore. Certain tones were like a balm. The music took my nausea away and put me into a dream-like state," he says, almost like he is describing an anesthesia. Chuck mentions several studies that examine music's vibrational properties, and the role of melodic structure in healing. But Chuck doesn't think music works the same way for everyone. "There is no perfect melody," he says, as though he has learned from his obsessive searches and hunts through the record shops in Boston.
For Chuck, music is an intensely personal, spiritual, and familial experience that ties him to his cancer, to his chemotherapy, to his own childhood when he listened to the phonograph with his mother, and even to his grandfather who played alongside his grandmother in Portugal two generations earlier. "Music has lifted me above my physical and psychological problems -- it has been an abiding anchor for me throughout my life."
Through music, Chuck has even lightened the prospect of his own death. "When it happens," Chuck laughs, "I want to have a recessional to a song called Partners in Crime by Jim Brickman. Have you heard it?"
I shake my head.
"There's two things going on melodically in the song. One is a great nostalgic sadness. The other is a gospel-like hopefulness," he says, the two threads of emotion inseparable. "That's how I want to go out."
The Malady and the Melody
In the corridors of the hospital, doctors present cases to each other, leaving out patient names and personal details to focus on the medical facts. But sometimes, a patient's personality, his hobbies, pursuits, and dreams, are inseparable from the course and treatment of his illness.
Meet Chuck - he's 65 years old, a resolute Bostonian who has encountered waves of disease throughout his life. But most importantly, he's a fierce audiophile. "I've never met a man who loves music more than him," his music therapist at Mass General Hospital told me. When I spoke with Chuck, we explored two defining moments in his life - his falling in love with music - and then the emergence of his illness - episodes he remembers with an almost cinematic clarity.
"I was five," he says, impeccably remembering his first encounter with music. "Before school started, my mother would put Django Reinhardt on the phonograph." There were two songs he and his mother loved: Nuages, and Love's Melody. "They were beautiful songs," Chuck says, who listens to them even now.
Like many illnesses, music probably has a genetic component. Absolute (perfect) pitch, for example, tends to cluster in families, the way many diseases do. Two generations earlier, Chuck's maternal grandfather lived on an island off the coast of Portugal, where he played mandolin, the dobro, and banjo. He married a woman who quickly learned to accompany her husband on guitar. "Both my mother and father loved music too," Chuck tells me, painting a picture of a childhood home filled with sound and harmony.
When Chuck entered his early 20s, his tenure in one of Boston's law school was suddenly cut short. One day, when he was twenty-three, he ran to the bathroom in severe pain. Blood poured out into the toilet bowl. "When I looked at myself in the mirror, I looked ashen having lost so much blood." Soon after, Chuck was diagnosed with Crohn's disease, an inflammatory condition of the bowels, although doctors weren't sure whether he might have another related disease, called ulcerative colitis. He dropped out of law school to tackle his disease - but he doesn't remember those days painfully. "I had gone through Motown then, and soon after the Beatles arrived," he says. "I was impressed."
Throughout his thirties, Chuck struggled on and off with his inflammatory bowel disease. But eventually, after years of being on prednisone and other immune suppressants, his disease went quiet. "I had a colonoscopy in 2001. Everything looked great, and I thought maybe I had beat this thing." Aside from working in retail, Chuck had time to pursue music again. He amassed a collection of more than 6000 vinyls, browsing through the local shops in Cambridge and Boston, becoming a regular at Déjà Vu Records and Nuggets in Fenway. "I was looking for melody," he says, as though on a mission.
But starting in 2007, he noticed unusual changes. Once, while walking on the beach with his friend, he remembered being unable to keep up despite being previously active and in good shape. "I started needing a cane to walk, then two canes." Then, he started to lose vision in his left eye and his teeth were discovered to be severely damaged. Finally, in 2009, the bleeding began again.
What was happening to Chuck - and how might music play a role in his treatment?
Is Running the Boston Marathon Good for You?
Most of us have heard of Jim Fixx - the celebrity author of The Complete Guide to Running who got America hooked onto jogging. Ironically, he died of a sudden heart attack at a young age of 52 after one of his daily runs and was found to have extensive blockages in the coronaries supplying his heart.
Every so often, we hear of athletic tragedies - last fall an 18 year old lineman fatally collapsed after a tackle at his team's homecoming game from a heart arrhythmia. And just this weekend, a 24 year old died during a half-marathon in Berlin, according to preliminary reports.
But such dramatic stories - however haunting and unfortunate - don't stop Americans from making vigorous exercise a part of their lives. A decade ago, about 1 million people participated in long-distance races each year. As of 2010, that number has climbed to 2 million. Here in Boston, about 10,000 people registered for the Boston Marathon in 1992; last year that number more than doubled to nearly 22,000 participants. That the popularity of marathons continues to increase is no surprise. Even though Jim Fixx is long gone, the legacy of his lifestyle is apparent in the average Bostonian. On many of the city's street corners we find evidence of this perpetual health renaissance: yoga centers are as common-place as coffeehouses, organic fruits are sold at Downtown Crossing even in the dark of December, and thousands of joggers brave the ice along the Charles River in mid-winter. Recently, Forbes Magazine rated Boston the third-healthiest city in the country. We've begun to believe that attaining good health requires constant, even aggressive attention to the condition of the body. The lifestyle of the marathoner - the long, dutied morning runs, the loyalty to a good diet, and participating in the annual day of reckoning, is a homage to challenging the self and bringing us closer to our ideal health. But is running a marathon - in actuality - good for us? Or are the apparent dangers the media intermittently reports a kind of warning?
To find out, I went to see Dr. Baggish, a young and bright cardiologist at MGH who is also the associate director of the Cardiovascular Performance Program. His clinic is anything but ordinary. The seats in his waiting room, for the most part, are occupied by athletes young and old. Among his patients are marathoners, mountain-climbers, and competitive bicyclists. Many come into his clinic armed with reams of data from their Garmin and Polar Heart Rate Monitors, a collection of physiologic parameters and race performance times exhaustively organized into spreadsheets. "Most of the patients I see are highly motivated," he laughs. But although Dr. Baggish is no stranger to the language of athletics (he has run 30 marathons himself), he is not seeing his patients to compare long-distance times. Instead, he is systematically searching his patients for red flags, early signs that suggest an athlete may be at high risk for heart disease. "Being athletic," he warns, "in no way grants you any kind of immunity."
When the Boston Marathon first began more than a century ago, the doctors of yesteryear were actually really worried. They considered marathoners akin to astronauts or pioneers, crazy people full of bravado who were testing the limits of the human physique. Consider the medical paranoia in this 1903 report from The New England Journal of Medicine documenting the race that year:
The course covers a rolling country, one or two hills, notably in Newton, being extremely long and trying. Each contestant is accompanied by an official guard on bicycle, usually a volunteer from the Ambulance Service of the State Militia. At the end of the race, they were taken in the elevator to the dressing rooms of the clubhouse and immediately examined.
Although we no longer have personal escorts for every marathoner in 2013, the Boston Marathon is still taken seriously by the medical community. A station is set up every mile along the route with supplies for first aid along with cardiac defibrillators. Some of the stations have bags of intravenous saline, EKG machines, and computers that can analyze blood chemistries in seconds. "We have the ability to track every runner, medically," says Chris Troyanos, the medical coordinator for the event. "There are barcodes built into bibs and wristbands. We know when runners check into emergency rooms at any of the Boston hospitals. We know when they check out." The ten hospitals along the route are on high-alert; their emergency rooms declare the Boston Marathon a "mass casualty event," the same category reserved for bioterrorism or natural disasters. Clearly, more than a hundred years later, it appears the medical community is still worried. Why are we so cautious?
Dr. Baggish ran his first marathon in 1997 when he was twenty-one years old, years before he became a doctor. He had no concerns - he says he was blind to the concept of risk. Nowadays, he has changed his perspective a bit. Of all the athletes he sees in his clinic, many - like him - will go on to run dozens of marathons without a problem. But among those athletes, there are a select few who are high risk for experiencing an event. "I've seen many of these people who have gotten into trouble with real heart disease," Dr. Baggish says. He refers to the exercise paradox: that exercise is the best way to reduce cardiac disease, but a cardiac event is most likely to occur during exercise. "A marathon is not inherently dangerous," he says. "But if you are already at risk for a heart attack, the marathon might bring it out."
Okay - then how to figure out who is at risk before such an event occurs? Dr. Baggish shrugs, implying that it's not easy to figure that out. The common symptoms of heart disease - chest pressure, shortness of breath, nausea - are often absent in devoted athletes. Instead, he says, the signs are more subtle. For instance, when Dr. Baggish reviews the performance times and heart rate spreadsheets his athletes so dutifully document, he looks for sudden changes in exercise habits: a sudden drop-off in performance or a new heart-rate trend. Most at-risk athletes do have traditional risk factors, like high cholesterol or high blood pressure. But many will forego getting treated, expecting their vigorous exercise regimens will afford them protection. In other words, athletes often wear a veil of immunity that does not really exist. "These people end up getting missed," Dr. Baggish says.
Now, with the upcoming Boston Marathon next week, Dr. Baggish's vigilance has been kicked into high gear. He offers a set up of tips for every runner. He urges respecting periodicity, or ensuring that runners build up towards the marathon, with equal periods of exercise and recovery. Similarly, he directs attention to warming up and cooling down. "The body hates abrupt starts and stops," he says. On marathon day, he asks runners to adjust their expectations according to what the day brings to them. For example, during last year's marathon, the temperature rose to nearly ninety degrees Farenheit, a situation that prompted organizers to offer participants an automatic requalification for 2013 if they opted not to run in the scorching heat. Most runners ran anyway. "You should be willing to change your routine on marathon day if necessary," Dr. Baggish reminds everyone. Finally, if you're sick with a virus, even something as slight as a cold, he suggests staying at seventy percent of the maximal heart rate.
Despite his suggestions, some will go to emergency rooms or will have trouble during the race. The chances of something extreme happening - like a heart attack - are exceedingly low. According to his group's study, the chances of a runner experiencing a cardiac event during a long distance race is 1 in 184,000. "You're more likely to die in a car crash going to and from the event," Dr. Baggish says. Still - the best thing runners can do to minimize such an event is to avoid a surge of activity during the last few miles of the race. When heart attacks do occur, its often in the last quarter, when participants decide to push themselves harder, a practice that can lead to undue strain on the heart muscle.
The Boston Athletic Association, which hosts the annual race, is obviously aware of the potential dangers of long-distance running. The BAA sends out emails to all participants on how to avoid heat stroke or overhydration. "We even teach the runners to pay attention to each other, so that if someone goes down, they can help start CPR," Troyanos says. "We're the first marathon in the country to do that." This year, in 2013, they have more medical cots than ever before, in part to reduce the strain on the local hospitals. Dr. Baggish will be at the front-lines to help provide medical assistance to anyone in need. But despite his calls for caution, he does not want to discourage the practice of running marathons. "Yes, every so often, there is a tragedy." But such events are still rare. "There is so much good that comes from this lifestyle," he says, his face brightening.
I won't be running the marathon this year - I get shin splits (whatever those are). But I'll be watching. I remember when I was a kid, it was part of my family's tradition to gather at the sidelines of Route 135 every spring with cut slices of oranges in our hands, until a sweaty marathoner swiped the fruit from our palms. When the runners finally arrived around the bend, their inhalations, the drumming of their feet against the asphalt, and their steady exhalations were the sounds of otherwordly machines. I never considered them flesh-and blood human; to me, they were composed of elbows, pivots, tendons and pistons. Now that I've grown up a bit, I've found that the truth is even more surprising: those who run the Boston Marathon each year (some of whom are my colleagues) are regular, disciplined people who often complete the marathon without any serious problems and go on to run several more. Dr Baggish's enthusiasm is encouraging. "I fully believe," he says, "that routine, consistent exercise and training, including training for a marathon or two each year, is the key to long-term health." Maybe one day, I'll try it myself. For now, I think, opening my notepad, I'm okay writing about it.
Revealed: Why was this Boston marathoner suddenly out of shape?
Several people guessed the answer correctly - exitseven7 figured it out first.
"While I was in there, the technician did a test scan first." He starts laughing. "Turns out that was enough for her. She rushed right in after the test scan, and said 'You need to talk to your daughter.' I knew something was wrong. She told me to sit in a wheelchair and not to move. I couldn't believe it. Just an hour ago, I had been driving down the Mass Pike on my motorcycle!"
D's story is not uncommon - a man in perfect health whose life is suddenly altered by unexpected illness. Susan Sontag, the famous writer and critic, forecast the inevitability and randomness of how disease affects each of our lives: "Everyone who is born," she said, "holds dual citizenship, in the kingdom of the well and in the kingdom of the sick. Sooner or later each of use is obliged, at least for a spell, to identify ourselves as citizens of that other place."
Most of us, whether in youth or in older age, will find ourselves grappling with some form of illness and with that territory comes Sontag's unfamiliar kingdom: the waiting rooms of doctor's offices, the dark and the clatter of an MRI machine, the long and dim hallways of a hospital. Such a transition is as dramatic as arriving in a new and unknown country. We respond with a kind of culture shock.
A radiologist called D in the scanning room. "I said - what's up doctor?" D laughs. "But the doctor was totally serious with me. 'This is no joke,' he said to me. 'You're lucky to be alive right now. You've got lungs blocked by large blood clots. If you weren't in the shape you were in, you would have had a really big problem.'"
D had multiple pulmonary emboli - blood clots that usually form in the legs and travel up into the vessels of the lungs. If they are huge, they can interrupt blood flow and cause shock or respiratory failure. Smaller clots can cause breathing problems like D was experiencing; if they are not treated, the symptoms can progress. "I went straight to the hospital and they put me on oxygen immediately," D says. "They started me on a Coumadin," he says, naming the commonly prescribed blood thinner. "I might have to stay on it for life," he sighs.
Pulmonary emboli can happen to anyone. In D's case, they were most likely caused by the long, 12 hour truck ride home from West Virginia to Boston after his motorcycle excursion. "Usually," says Dr. Praveen Akuthota, a pulmonary doctor in Boston, "this happens with prolonged air travel, particularly on very long, trans-oceanic flights that last in the 6-8 hour range. But even just being seated for most of a 12 hour journey puts him at increased risk."
There are other known risk factors for clot formation: cancer, structural problems in the vessels, dehydration, smoking, and certain blood disorders. But immobility, by itself, can be enough. You shouldn't sit in one place too long, Dr. Akuthota says - whether you are at work, driving, or flying. Get up and walk around often. But even then, Akuthota goes on, even if you are mobile and active, you can still get a pulmonary embolus. He points to his chest. I could get one right now.
Thankfully, D's doctor picked up signs that something was critically wrong on the EKG - he had a pattern called right heart strain, which is seen when the heart is pumping harder than usual, sometimes indicative of a clot in the lungs. But things could have gone worse. People can go into cardiac arrest from a pulmonary embolus.
As long as the human body remains complex, medicine will be imperfect, illnesses will strike seemingly at random. There is nothing fair about any of this. But the same flawed mechanisms that bring us into disease sometimes bring us back out again.
D thinks back to the days he used to run marathons. "During the run," he says, "it was a mental game with myself. I had to keep telling myself I could do it, I can do this. I've trained and today's the day to put it all out there."
The words of a marathoner are the same sentiment I've heard from patients who have been affected by illness; these are the words every fighter carries close to his heart.
Stay tuned for a follow-up post which will address the current medical controversies, risks, and benefits of running marathons. The 117th Boston Marathon will be run next month, on Patriot's Day.
Why was this Boston marathoner suddenly out of shape?
This is the case of a real patient. After reading the description of the case, I invite you to guess the patient's diagnosis in the comments section below. The answer will be posted Friday.
Why Was This Boston Marathoner Suddenly Out Of Shape?
FULL ENTRY
Next month, nearly 30,000 people will run the annual Boston Marathon, although D isn't planning on running it this year; he's still recuperating.
For a long time, people have known that long races can be traumatic to the body.
During the running of the first Boston Marathon in 1897, doctors waited at the sidelines, anticipating that runners might collapse or suffer permanent heart injury. Perhaps they were reminded by the case of a famous Athenian athlete: around 500 BC, a man named Phidippides ran twenty-six miles across the Plains of Marathon to announce to the city of Athens that the Greeks had triumped over the Persians. Soon after delivering the message to the joyful city, he fell dead, presumably from cardiac strain. Even now, 115 years after the first Boston marathon, many hospitals prepare for a mass influx of patients, anticipating that runners will come into emergency rooms with heat exhaustion, dramatically low sodium levels, and dehydration.
Mr. G walked into a mini-epidemic
Isles of White in a Sea of Red
Sometimes, physicians describe a finding as pathognomonic, which means that the finding almost certainly suggest a diagnosis. A good example are the white spots seen on the inside of the cheeks, called Koplik spots, which are quite specific for measles. However, pathognomonic signs are rare. Usually, a diagnosis is riddled with uncertainty. "I took one look at his legs and saw a brilliant erythema," Dr. Golding says. Erythema - a deep, red color of the skin - stood out among Mr. G's signs and symptoms. Woven through the dark red was a faint tracery of a lighter color that Mr. G had referred to as a "fishnet" in appearance. One of Dr. Golding's colleagues, who was from the Phillipines, remembered hearing about a tropical rash that presented as "isles of white in a sea of red," a pattern suggestive of dengue hemorrhagic fever.
Dr. Golding thought this was possible, especially since Mr. G had fevers and a headache, typical of the disease. Although the rash was not pathognomonic, he used it as an anchor to a possible diagnosis. He sent Mr. G's blood to a lab to get him checked for dengue. A few days later, the test returned positive.
Vectors
Dengue hemorrhagic fever has a scary name. But hemorrhage - or bleeding - is rare. Most often, people develop high fevers, terrific headaches, and bone and muscle pain. Because of these symptoms, some people call dengue "breakbone fever." Eventually, the virus is tackled by the immune system and the host gets better. In a small number of cases, people fall very ill and can even die. The disease is caused by a flavivirus - a family of pathogens that cause similar illnesses including yellow fever, West nile, and Japanese encephalitis. Most of these viruses are spread by mosquitoes. In the language of epidemiology, since mosquitoes carry the disease from human to human, they are vectors; we are the hosts. But dengue is not spread by the kind of fat, muck-water mosquitoes we are used to in Boston.
Instead, says Dr. Sharone Green, an expert in flaviviruses who works at Umass Memorial Medical Center, "dengue is spread primarily by Aedes aegypti which are different from the Culex mosquito we have here." The dengue mosquito is tiny, and is often found singly, rather than hovering in clusters like the Culex of New England. "Most people don't know if they've gotten bit." The dengue mosquitoes also feed all day long. "Many people tend to put on mosquito repellant and put up nets at night," says Dr. Green. But that's not enough to protect against the dengue mosquito who feeds at any hour. Perhaps most disturbingly, Aedes loves to live indoors. The moment it bites a person who has dengue, the mosquito remains infected for life. Consequently, a single insect may infect an entire household. "Dengue transmission is very focal," she says. "When cases occur in Puerto Rico, they are often found in little clusters of homes and neighborhoods."
Hosts
So Dr. Green isn't surprised to hear that Mr. G lived in the same house as someone who might have had similar symptoms. A few days after Mr. G had received his diagnosis, he received an email from D - the woman whose husband had fallen ill in Puerto Rico. In her email, she described the disease that had afflicted M.
Hi,
Yes,he (M) had it has as you left. After you left the numbers of people with the dengue skyrocketed. The goverment has increased awareness and people are really taking care of themselves. Here some neighbors in [name of the community omitted] have even a group in facebook to inform about it. We have had always had a (dengue) season but not this long. After you told me I have been telling my guests about it and that they protect themselves with Off spray, mostly in the afternoons and night that they come out. Climate change has made what has always been our dry seasons rainy seasons.
I hope it changes soon.
During his trip to Puerto Rico, Mr. G had walked into a mini-epidemic that had risen around him. Clusters of people in San Juan soon became infected. According to the CDC, reports of dengue in Puerto Rico in December of 2012 were above traditional epidemic levels.
What happened throughout the region, had occurred on a smaller scale within the house where Mr. and Mrs. G had stayed. "It's possible the same infected mosquito had bitten [the host] and the patient," Dr. Green postulates. [see CDC graph]
Printed with permission from the CDC
Every Place Has Something
Although every country hosts numerous diseases, tropical countries often have more opportunities for travelers to get sick than other environments. Dr. Green recommends that anyone traveling to a warm or tropical climate should go to the travel clinic or visit their physician to get advice about the kinds of illnesses that exist in the country.
The classic reference doctors use to track illnesses globally is a book published by the Center for Disease Control called the CDC Yellow Book http://wwwnc.cdc.gov/travel/page/yellowbook-2012-home.htmhttp://healthmap.org/en/. Other references include the Health Map, founded by Boston Children's Hospital in 2006, that follows epidemics across the world in real-time by surveying official disease reports, accounts from social media, newspapers, and magazines. The project is even currently reporting on the active outbreak of dengue that Mr. G experienced during his recent trip. The same research group has even discovered that what people search on Google may help predict where nascent epidemics are emerging. Even D's email mentions a facebook group that helps monitor symptoms in the neighborhood. Epidemiology detectives now use footprints on the web and in social media to help track new epidemics of disease the moment they arise.
Using novel ways to diagnose and track illness can help travelers and natives prevent transmission early. Since dengue mosquitoes are day-biting, Dr. Green recommends frequent use of repellent and even treating clothing with permethrin. Choosing light or beige colored clothing is preferred over bright colors, which tend to attract mosquitoes. And, she says, "if [you] are sleeping in the house with someone with suspected dengue, [you] should be sure to use spray and consider using a bednet at night."
Many physicians, unlike Dr. Golding, may not recognize dengue when they see it. Patients should be aware of the diseases prevalent in a country so that if they fall ill when they return, they can bring it to the attention of the doctor.
Mr. G has since recovered. The rash has faded and his fevers are gone. I don't want people to be paranoid when they travel, he says. But, as Dr. Sharone Green reminds us - "every place has something going on."
A Host in Puerto Rico
This is the case of a real patient. After reading the description of the case, I invite you to guess the patient's diagnosis. The answer will be posted Friday.
Mr. G's photograph of the El Yunque Forest in Puerto Rico
"When we first arrived, [M] looked pale and leaned heavily on the wall for just a few moments, not saying much," he said. The man's wife, D, seemed unaffacted. She was gracious and talkative, and soon, Mr. and Mrs. G were settled into their new home.
The next day, Mr. and Mrs. G stepped out into the city. They strolled down the Plaza del Mercado de Rio Piedra, a busy marketplace that bustled with natives and tourists. Vendors hawked cigars, old books, candles; food stalls sold cut coconut, giant avocadoes, and meat smoked on the open flame.
On December 7th, the couple traveled to the slopes of the Sierra de Luquillo mountains, descending into the tropical rainforest called El Yunque [see photo that Mr. G took]. "The weather was gorgeous," Mr. G recalled. The air was moist, but not humid; the sky threatened rain, but none fell. The river that ran through the rainforest stood still. Ferns and palms grew alongside the path, some reaching a height of 50 feet; the canopy was thin enough to make out crescents of blue sky and the wings of colored birds. A few giant snails clung to the underside of the sparse underbrush.
By noon, the couple had descended deep into the rainforest, where they broke for lunch. "We sat in an open-air pavillion built by Boy Scouts. She ate sardines. I had a banana, dried dates and some nuts," Mr. G said. During that hour, he remembered, something had bit him, four or five times. He guessed it was a mosquito. "I thought the bites pretty weird at the time. It wasn't very buggy around there." On the trip, they hadn't seen many bugs or animals at all -- just clouds of gnats, little lizards that scampered underfoot, and the birds in the trees.
The couple hadn't forgotten the health of their host. Between trips to old San Juan, a swim at Guanica, and a hike through the Dry Forest nearby, they saw that M's condition was improving. One evening, he joined them on the veranda in conversation. The illness he had seemed to have passed.
But just as M had recovered, on December 10th Mr. G awoke in the middle of the night in a profuse sweat. Although he had no thermometer, it was evident that he had broken out in a fever. As the night pressed on, strange and recurring dreams occupied his mind. "A dream replayed over and over and into the day -- some delusion about a mass I had in my stomach, with little hairy projections I had to snip," he said, without laughing, as though remembering the potency of the dream. The delusions and fevers were persistent.
Even a short trip to a botanical garden with his wife exhausted him. Moreover -- he had developed a wincing headache that came and went nearly every minute -- unusual in a man who never had headaches at all.
FULL ENTRY
What caused Mrs. C's chronic diarrhea?
In a previous post, we asked you to try to diagnose our patient, Mrs. C. What follows below is the actual diagnosis, but visit the previous entry if you want to see what folks guessed.
This winter season in Boston, we've been struck with an early and particularly vile spate of influenza cases that have filled hospitals to capacity and have Boston residents scrambling for vaccinations. What's unusual about this year's flu is that in addition to respiratory symptoms, many are suffering from gastrointestinal distress, including vomiting, abdominal cramps, and diarrhea.
The symptoms of this flu virus resemble those of another bug that sweeps through the city nearly every winter. That bug is norovirus, a fast-moving and highly infectious virus that causes 24 hours of vomiting and often violent diarrheal episodes. But both the flu virus and norovirus usually get better: these are examples of acute diarrheal illnesses, and for the most part, such viruses and their cousins are to blame for short-lived cases of diarrhea. Since these bugs can't be treated, doctors usually let acute diarrheal episodes resolve on their own, without aggressive treatment or further investigations.
Or was it something more sinister?
Chronic diarrhea is an entirely different circumstance. Diarrhea needs to last more than four weeks before it can be considered chronic. Mrs. C more than meets the definition -- she's had diarrhea for probably more than a decade. In developed countries such as the United States, people who have diarrhea off and on over a long period of time often suffer from irritable bowel syndrome (IBS), a disease that leads to an overactive and sensitive gut that may squeeze more often than it should. However, what raised flags for Mrs. C's gastroenterologist, Dr. Jacqueline Wolf at Beth Israel Deaconess Medical Center, was that these episodes started affecting her at night. Because stress is thought to be related to IBS, patients with this condition rarely present with diarrhea at night, when stress levels drop.
FULL ENTRY
Not an ordinary case of traveler's diarrhea
This is the case of a real patient treated at a Boston-area hospital. After reading the description of the case, I invite you to submit a comment guessing the cause of the patient's symptoms. The answer will be posted Friday.
"I was a nurse for 39 years," she says, "but that didn't help me figure this out at all." Mrs. C has been haunted by debilitating symptoms for more than a decade -- since her mid-50s, she says. She's been seen by several doctors and has wracked her own brain for answers, considering various exposures, angles, and ailments.
Mrs. C is a lively, resilient, and adventurous woman. "My husband's job often required him to travel," she says. "We were chaperones along with another couple for a group of 20 teenagers on a trip to Haiti. The other lady had severe dysentery while there. She thought it was from eating salad containing unwashed lettuce. She's the one where I first heard the expression about passing 'cut glass' with diarrhea. That's how I'd describe my episodes as well."
Hunting for a Diagnosis - Revealed!
Your Differential Diagnosis:
Among the many answers submitted, many of you guessed the correct answer - lead poisoning. The first person to guess correctly was patches2. Congratulations!
Almost as many people guessed lyme disease as an alternative diagnosis; other common responses included syphilis and diabetes.
In Massachusetts, many of us are familiar with Lyme Disease, a tick-borne illness that usually presents with the characteristic rash along with fatigue, headaches, muscle pain, and joint pain. Many readers felt this patient could have the later stages of lyme disease, which can present with nerve pain and arthritis. However, lyme disease is unlikely to cause hearing loss and digestive problems; foot drop would be a very rare presentation. Neurosyphilis can present in a variety of ways, but usually patients complain of meningitis - headaches and a stiff neck. Diabetes can present with a painful neuropathy as this patient describes - even when blood sugar levels are only in the pre-diabetes range. However, this patient's other symptoms and lab results suggest the leading diagnosis.
The Diagnosis:
Mr. M, over a long time period, had developed hearing loss, memory problems, constipation, numbness and tingling in his feet, and foot drop. While some of these problems could be attributed to age, the simultaneous onset along with the more unusual symptoms in his feet suggested a possible unifying diagnosis.
Foot drop, when it occurs on both sides of the body, suggests a systemic disease. Alongside the numbness and tingling, it suggests that Mr. M has a condition that is affecting his nerves, which we call neuropathy. Other than diabetes, other causes of neuropathy include alcohol (which he doesn't drink), or a vitamin B12 deficiency (his levels are normal). Other, more rare causes, may occur from toxic agents. In this situation, the doctor gathered an exhaustive history and found it remarkable for Mr. M's significant exposure to metals as a machinist - though his doctor ruled that out as a cause because he was long retired - and his interest in gun sports.
"I use regular shotgun shell bullets," Mr. M says. "As the BBs come out of the shotgun shells, you end up breathing in mist. And those pellets are made out of lead."
So his primary care physician checked his lead level with that extra blood test -- and it was sky high. "Ideally, lead levels in the blood should be less than 10 [mg/dL]," says Dr. Rose Goldman, a professor in the Department of Environmental Health at the Harvard School of Public Health. Mr. M's lead level was near 50.
Lead poisoning -- a condition that we often think of in children -- occurs in adults too. Frequently, symptoms develop chronically, especially in people with occupations or hobbies that expose them to lead over time. "Cities like Boston and Baltimore are full of old, wonderful buildings we like to renovate," Goldman says. "Construction workers who deal in demolition or who scrape bridges are exposed to old paints that contain high levels of lead." Other at-risk groups include plumbers, police officers, car mechanics, and gas station attendants. People who take Ayurvedic herbal medications, paint, remodel homes, or do pottery work can also be exposed.
Symptoms of chronic lead poisoning may be vague. Commonly, patients present with abdominal pain, constipation, headaches, and trouble concentrating. They may develop nerve damage, such as wrist drop or foot drop, or even hearing loss.
Gun ranges, especially indoor, may have poor ventilation systems and patrons may inhale lead; preparing bullets increases this risk.
"Even outdoor shooting ranges, where a lot of people stand in close quarters on hot, muggy days, can develop high levels of lead," Goldman continues. "People who shoot guns should always wash their hands afterward."
Since getting his test results back, Mr. M has stopped going to the range for now, until his levels come back down to normal. Already, they've dropped below 40. He is hoping his symptoms will get better. He has also told his friends to get tested, but they are resistant. "They don't want to stop shooting," he says, but hopefully they'll come around.
Do you have your own medical mystery?
Send me a description of your own case and I will consider writing about it. E-mail me at sushrut.jangi@gmail.com.
Hunting for a Diagnosis
This is the case of a real patient treated at a Boston-area hospital. After reading the description of the case, I invite you to submit a comment guessing the cause of the patient's symptoms. The answer will be posted Friday.
"Nah, I was never a big game hunter," Mr. M tells me. "Sure, when I was a kid I used to go shooting a lot. Mostly birds, pheasants, quail. On rare occasions, deer."
Mr. M is 74 and has lived just outside of Boston his whole life. He's a retired machinist, who worked with nylon, Teflon, and silicon. A delightful conversationalist, he tells me that other than having high blood pressure, he's been healthy. It's only in the past couple years that he's noticed strange symptoms that haven't gone away.
"So I've done my little bit of hunting," he explains. "But now I just shoot clay." For the past 28 years, Mr. M has enjoyed various gun sports, shooting as many as 20,000 rounds per year, preparing his own bullets, and usually firing at clay disks that burst open the moment the bullet makes contact. "Whenever I shoot, I'm always careful to wear ear protection. But I don't think it's been working."
Mr. M has noticed his hearing has profoundly worsened in both ears over the last two years. His wife often has to say things twice. He didn't think much of it, and figured it was part of normal aging. After all, he's had some trouble with his memory and digestion too. "I started drinking prune juice, which helps a little bit," he admits.
But what's troubled him most is that he started to have unusual sensations in his feet. "Three years ago, my feet started becoming very, very cold," he says. "I went to see a podiatrist, who recommended a [calcium-channel blocker] called nifedipine." At first, the medicine worked. But a few months later, the soles of both his feet began tingling. The sensation creeped up the side of his leg and he noticed painful, aching cramps. "I started to have some trouble walking," he says.
He went to see his primary care physician, who performed a careful physical examination. Almost immediately, his doctor noticed Mr. M was slapping his feet when he walked, a finding called "foot drop," which reflects weakness of the muscles or nerves of the lower leg. (Watch video below for an example of left-sided foot drop.)
During foot drop, the ankles and toes have difficulty moving upward, such that the forefoot drops during walking, as seen in this patient?s left foot. You can notice that the patient raises his left leg up while he walks to prevent the forefoot from dragging. Mr. M developed foot drop on both sides. Usually, this reflects a systemic disease process, rather than individual nerve injury. (Video courtesy of Larry Mellick, Medical College of Georgia)
Mr. M also had trouble feeling vibrations and light touch on his feet and ankles. His primary care physician ordered several blood tests, all of which came back normal.
Lab Results:
Hemoglobin A1c 6.0 (normal 4.5-5.9)
Vitamin B12 596 (normal 200-900)
Hematocrit 43.6 (normal 42-54%)
Before Mr. M left the office, his primary care physician, on a hunch, added on one more blood test. A few days later, this test came back positive, and his doctor immediately called the patient, and then reported the finding to the Department of Public Health.
What did Mr. M's blood test reveal?
Click on the "Comment on this story" link below to post your guess and your reasoning. I'll post the answers on Friday.
