Boston Medical Mysteries - Questions, Symptoms & Diagnosis [PDF]

The Itching Hour Posted by Dr. Sushrut Jangi February 27, 2014 04:16 PM One night, I woke up itchy. It was around 2 a.m. What a strange thing. Ordinarily, I'm blessed with a capacity for deep slumber and can sleep through everything: rain, thunder, police sirens, and if there were earthquakes here in Boston, I would probably sleep through those, too. That's what made this night particularly odd. The itch started on my legs like toothy insects nibbling at my skin. Then it rose up until the sensation enveloped the rest of me, my stomach, my arms, my face, my scalp. I wasn't an atopic kind of person; never had eczema or asthma or dermatitis to wrist watches, shampoos, or lotions. So I let it go for awhile. There's a book that describes how monks can will away discomfort by accepting it as a neutral sensation. I tried that for five minutes, then started scratching like a gray-back ape. Blissful was the sound of excoriation. Cells were coming off like steel wool abrading the last remnants of debris at the bottom of a pan. For awhile, my body was content. But soon after I stopped, the sensation came back in a wave. The clock read 3 a.m. I stood up and got out of bed. This was ridiculous. There had been other occasions where some symptom - nausea, even unhappiness - had overwhelmed me. But the fact that an itch was causing me distress on such a dramatic scale bordered on the bizarre. I walked to the window and threw it open. The cold winter air rolled in and floated over my skin. In the bathroom I found a tube of moisturizer that I squirted on and rubbed in, but there was no soothing effect. Could I have cried? I could have. I wanted to call someone and tell them what was happening. Everyone - at least in my pocket of the world - was asleep. But I was awake, blundering around the house in search of exfoliating cream, a strong light, and a comb for nits. I actually studied my arm in the mirror, looking for little bugs, at least a culprit to name. We have a word in medicine for such itching: whole body pruritis. But could these words even begin to reflect how potent that itching was? How I wished to set my skin afire to burn the feeling away? How in the depth of the night even a simple discomfort like this could summon clouds of uncertainty and precipitate near existential anguish? We've made a lot of advances in medicine. A doctor can put a tiny stent in your heart and stop a heart attack; we have precise chemotherapies for cancers that were once undefeatable. Yet surprisingly, the most basic elements of the body's suffering - pain, nausea, and in my case, that night of nocturnal itching - remain mysterious, their mechanisms vague and still relatively undefined. Let's take a look at pain. Even though we have the capacity to quantify much of our physiological innerworkings, thus far we have no practical instrument that objectively measures pain. The best we can do is ask a patient to estimate how much pain they feel. In the case of nausea, despite new ways to control this symptom, it remains a much feared side effect of medications, causing patients to drop out of vital chemotherapy regimens, antibiotics courses, or HIV treatments. Itching, described in Atul Gawande's fascinating account of the symptom as "among the most unpleasant physical sensations one can experience," can cause patients such distress that they actually inflict bodily harm on themselves from their relentless scratching. Part of the reason such basic afflictions remain undeciphered has to do with the nature of these feelings themselves. During the course of human evolution, pain, nausea, and the itch became the signals of revulsion from dangerous or toxic stimuli. They are our allies and have been at our sides for thousands of years. Consequently, the circuits that run the programs for these symptoms are deeply and redundantly buried in our brain and body; the ability to manipulate or treat them is far more complex than going after a single target. As a result, many of the diseases that remain relatively intractable to management in our current era - any kind of chronic pain, irritable bowel syndrome, fibromyalgia, cyclical vomiting - are the outcomes of these complex circuits gone awry. Patients that I've seen with such syndromes often sit in the office at their wit's end looking for a solution. Western medicine doesn't yet have it. How can we treat such disorders when we still don't understand their roots - the simple sensations from which they arise? That night, I finally took a hot shower, almost scaldingly so. And while that didn't take the feeling away entirely, somehow sleep came to me. I closed my eyes just as the sun was coming up. An hour or so later, my body felt like my own again. The itching was gone. I've never figured out a cause. And while I've been far sicker than that night, it has left in me a sensitivity that even the most simple and common symptoms - those that we will all at some point experience - are the ones that can be the hardest to explain, to treat, and can leave us the most unhinged. "I'm sorry I've called you in again," a patient told me one night on the cancer ward, after I had been paged to her bedside twice already. "My arm won't stop itching," she said, near tears. "Of all things," she wondered. "It's so silly, isn't it?" she said. The nurse brought in another round of Benadryl. I crossed my fingers.

Questions Doctors Can't Bill For Posted by Dr. Sushrut Jangi January 28, 2014 10:53 AM I draw your attention to the title of this blog "Boston Medical Mysteries" and its subtext "Solve diagnostic puzzles." Oh, yes there is something intriguing in the medical puzzle, as there is in any good mystery. Collecting clues and dismissing the red herrings, watching the story inexorably unfold. The medical mystery often hinges on arriving at a diagnosis, a genre that has catapulted the television show "House M.D." to fame, that runs in the headlines of Lisa Sanders "Think Like A Doctor" in The New York Times, that made the journalist Berton Roueche wildly successful in his carefully researched epidemiology-noir published throughout the 1960s in The New Yorker. Even we doctors, perhaps necessarily so, find time to stop each other in the halls, peddling a tale to a group of bug-eyed residents, "I have a 28 year old male who keeps losing weight," and they lean in saying, "Yes? And then what?" But these mysteries, however intriguing, are only the first layer, a facade of the whole story. The mystery moves beyond diagnosis. Yes, as a physician, I want to know the details of his cough. But I want to know how he's been dealing with the cold this winter. Whether he lives alone or whether his children come home to see him on Christmas Eve. I don't want to know only about the swelling in her hands. But what she has trouble doing because of that swelling. That she can no longer open jars. And what it is that she keeps inside jars - what kind of jams, where exactly she goes, every June, to gather the fruit, and how it is to gather them. Yes, I want to know your travel history. But I want to know what you saw before you fell sick. Tell me about this city that you love, where your parents live, the balcony where you sat with your father and looked out at the water. Why should we care about the answers to these questions? What information do I extract from the details peripheral to your illness? What hides in the periphery is the start of empathy. This is a soft word tossed around in medical school curriculums in danger of being ignored because of its softness, usually practiced by the touching of shoulders and the whispering of buzz phrases like "I know that must be difficult." But those gestures are only a shadow of empathy; they are the customary pleasantries of the profession, the "yes, please" and "no, thank you," of medicine. I am of the camp that true empathy cannot be taught but must be cultivated. Empathy is not soft at all but a difficult exercise in communication. On closer examination, empathy is an intense curiosity for the human condition in all forms of its expression, whether that expression is produced as suffering, lassitude, fear, nostalgia, humor, sadness. Therefore cultivation of empathy is only achieved when the physician opens himself to this spectrum of emotion, a curriculum that moves beyond the science texts into literature, fine art, music, theater, spirituality, and the insights found in both meaningful relationships and solitude. Only then do we truly understand the clues found in the periphery beyond illness, the life into which illness comes. And in that periphery is a far more wondrous mystery than simply solving a case. It is in this periphery where we teach the other what it means to fall ill, to lose faith, to feel stuck, to feel pain, to feel crazy, to grow old, to find grace, to stand up, to forgive yourself, to find peace. And in such an exchange, we open doors, for a moment, into each other's lives. The empathic exchange is the basis for placebo, that strange phenomenon only recently under study in Western medicine, but omnipresent throughout the world in healing. There is no substitute for good doctoring, for possessing knowledge or technical skill. And there is no easy way to code for these peripheral questions on those pink billing slips or allocate the appropriate amount of time for them. But without empathy, we are only treating the patient without healing them. Without empathy, the door into the patient remains locked, behind which may hide the crucial answer that brings a patient's illness to light. This is an art that I am only beginning to learn, that I've witnessed within all kinds of people, not only doctors, not only in those who carry a long list of degrees after their name or produced a great body of research, but in those who have the patience and interest to truly listen. It can be, in some ways, a painful way to practice medicine and live life, to open yourself in this way, but alongside this pain comes an honest understanding of the other's circumstances. So yes, these are puzzles, in the way medical cases are. But step back and see what shapes these puzzles make. They take the myriad forms, however mundane or astonishing, of the lives of real people.

Following disease into our dreams Posted by Dr. Sushrut Jangi January 21, 2014 10:09 AM

A scene from the 2010 blockbuster sciencefiction film, Inception, in which Leonardo DiCaprio's character leaps into a dream to change a person's behavior and extract crucial information. (Picture Courtesy Warner Bros. Pictures, 2010) Boston doctors and scientists use new tools to link dreams and disease There are places doctors can now go where they couldn't go before. In less than a day, a human being can have his DNA entirely sequenced. Colonoscopies collect bacterial samples from deep within the gut. Tiny monitors on the skin upload heart rate and body temperature data to a smartphone. Modern medicine is hunting for telltale signs of health and disease in every piece of data the human body generates. Some of this is sure to be important, but can't we hide a part of ourselves away from the light of examination? Isn't it possible that our body and mind generates ethers and currents that are immeasurable? Dreams go big data I should hope that the dreams we have at night - at least - would remain beyond the grasp of the big data revolution. Nope. If the study of dreams has been historically padlocked, modern methodologies are giving us a new set of keys to peer inside the sleeping brain. Earlier this year, the journal Science reported that a team from Japan was able to guess what people were seeing in their dreams after putting the sleeping subjects through an MRI machine. And novel software makes dream-sharing practical and potentially useful for researchers. An iPhone App called Shadow revolves around the creation of a social network where you can text and upload your nightly visits to la-la land. Some online dream communities, like Dreamboard, store more than 200,000 dreams. Not only are dreams becoming as accessible as tweets, but we may even have the conveniences to edit them as well. Three Bostonians, including MIT alumnus Doug Feigelson, developed a program for smartphones called Sigmund that whispers words into your ear during the time of night you are most susceptible to dreaming. "It's worked on me numerous times," he said. "It's the kind of thing you've probably experienced naturally when you're falling asleep while people around you are talking, and you start dreaming about their conversation." Dreams and disease Since dream content is becoming more available, people are curious if there are clues buried within that hold information about our health. "Oh, yes," said Patrick McNamara, Ph. D., a neuroscientist at the Boston University School of Medicine. "The content of dreams, especially among older adults, can serve as a warning sign for the onset of certain diseases." For instance, people who go on to develop Parkinson's disease and other dementias may physically act out their dreams during sleep, years before they develop the illness. "Some kinds of dementias result in dreams where the dreamer is being attacked by an intruder, and they struggle to protect themselves and their bed partner," said McNamara. Early morning heart attacks may be triggered by high blood pressures and heart rates that accompany the stage of sleep most associated with dreaming. Running from an enemy in a dream is a bit like putting the heart through a stress-test. Consequently, McNamara said that it wouldn't hurt for doctors to ask patients about their dreaming habits. Although the practice of dream-telling might be unusual in Western medicine, it is routinely used in Eastern medical systems to assess the mental status of the patient. "Dreams are faithful reports of a patient's emotional life," said McNamara. "They aren't just fluff, but have a function in the mental economy. If you work with them, they can yield insights about the self." After September 11th, another local sleep researcher at Tufts University, Dr. Ernest Hartmann (who passed away last year) and his colleague Tyler Brezler discovered that people tended to dream more intensely after that date than before the terrorist attacks. In other words, dreams, the exhalations of our sleeping brains, can contain signatures of trauma that we've experienced during waking life. And in the case of post-traumatic stress disorder, dreams may replay the trauma, keeping the memory of the initial event terribly alive. Dreams or nightmares themselves can also plant the seeds of depression or fear. Soldiers who returned from the Gulf War on the dream-triggering malaria drug, mefloquine, described traumatic nightmares that were so vivid, the fear elicited by their dreams carried over when they woke up. But McNamara said you can also entrain your brain to dream happily. "A few studies show that if you can learn to dream pleasantly, you can regulate your mood the next day." Should doctors enter dreams? If happiness and trauma are embedded in the fabric of our dreams, social apps such as Shadow and Sigmund will help investigators like McNamara to link the information in dreams to how we live during waking hours. Some have previously suggested that physical symptoms - such as chest pain - are first noted in a dream, before they are experienced in real life. "We've never really had any statistical power to test that claim," said McNamara. "These big databases will revolutionize dream studies and really demonstrate the clinical relevance of dreams." The day is closer when we may know how dream content may correlate with a person's risk for a heart attack, depression, or dementia. For now, in my own practice, I occasionally ask patients about their dreaming behavior. While their answers alone won't drive me to make any clinical decisions, they are a part of understanding the person holistically. But even as study of dreams advances, they will remain an intensely subjective experience. A dream can make you fall in love again or help you to make a decision. They are a kind of wellspring of individual reflection and inspiration, the realm of the artist and the poet, not just the scientist and the physician. To better understand them myself, I recently decided to keep a dream journal, as McNamara suggested. I set a new notebook on my bed stand and went off to bed. The next morning, even before I opened the notebook, the faint tendrils of the dream slipped from my mind. "Dream recall is tricky," McNamara says. "But you'll get better the more you try it." So I tried again. The next time, the dream came to me in vibrant watercolor. I was walking along the ocean, I wrote, feeling a bit self-conscious. And . . . you know what? I'd rather keep this to myself. What does the world want with my dreams anyhow?

Have we forgotten about our circadian clock? Posted by Dr. Sushrut Jangi December 6, 2013 08:30 AM

"Night shifts really affect me," Jamie Horton tells me. A nurse employed on one of the busiest floors of a Boston hospital, Jamie typically works two weeks of day shifts before switching to a row of nights. The sudden change in schedule palpably affects her health. "I typically refer to it as feeling like I am hung over for days. I get sicker much easier," she says. In the dark winters of Boston, when ambient light is low and the days are mercilessly short, her discomfort is often magnified: "It's already dark when I wake up and only sunny for a very short time before I go to bed. As far as my mood goes, I am miserable." But Jamie's getting enough sleep: this isn't straight up sleep deprivation. What bothers her is the abrupt change in her sleep-wake cycle: one day she goes off to work during sunrise, another day she goes to bed at dawn. Her schedule, however unpalatable, is not uncommon in this country: nearly 10 percent of the American workforce participate in disorienting, rotating work schedules that lead many shift workers to describe symptoms of malaise, depression, or inattention, which can, at their worst, impair workers' performance. Just earlier this week, a Metro-North Railroad train derailed in the Bronx, killing four passengers and injuring more than 70. It was reportedly operated by an engineer who "nodded off" moments before the crash. While sleep deprivation may be to blame, so may be the consequence of his early work shift. That morning, his shift began around 5 a.m., before sunrise. Two hours later, he was still "in a daze. I don't know what happened," he reportedly told investigators. Jamie's symptoms during her changes in the shift and the train accident in the Bronx do not come as a surprise to Dr. Frank Scheer, who has made studying the physiology of shift workers part of his research focus. "Do you know about the suprachiasmatic nucleus?" he asked me. Dr. Scheer directs the Medical Chronobiology Program at Brigham and Women's Hospital. His question is a valid one: I don't think most doctors know an answer when we are evaluating a patient's health. The suprachiasmatic nucleus, Dr. Scheer describes, is our master clock. A tiny part of the brain (about the size of a grain of rice) located just behind the eyes, the master clock is a fleshy, pink network of neurons linked by a series of timed chemical reactions instead of cogs, sprockets, and second-hands. Aesthetically, it's an unconventional time-piece but a functioning clock all the same--this bundle of neurons wakes us up, tells us when we're hungry, adjusts our hormones, regulates our body temperature, and reminds us when it's time for bed. Hundreds of other clocks, located throughout the body's cells, respond to the master clock the way a symphony follows the lead of a conductor. All fine when the master clock works--but what keeps the suprachiasmatic nucleus running on time? Daylight. The moment we open our eyes, little photons from a sunray sail through the chambers of the eye, striking the film-like retina and sending a message to the master-clock: "Time to reset!" the sun says. Eventually, after a series of such mornings, the master clock adjusts to the solar cue. The body's peripheral clocks then adjust to the change in the master clock. But now imagine Jamie's schedule--her internal clock has no time to orient to the earth's light and dark cycles; consequently, her master clock is thrown out of wack, and with it, all of the body systems it helps to regulate, which disrupts mood, digestion, and even menstruation. "If you are doing rotating shift work, the internal body clock just can't keep up," Scheer says. Living and working on an irregular schedule, Scheer says, is akin to jet lag. It's like suffering the effects of changing time zones without leaving the country. What's worrisome is when the body's clocks stay unsynchronized chronically, and lots of systems begin to go haywire. Over time, "people can develop glucose intolerance, high blood pressure, or suffer cardiovascular events. They may even have trouble losing weight," Scheer warns. Exactly how glucose intolerance or high blood pressure could result from a disrupted circadian clock is still being worked out. The field of chronobiology is mysterious, if not previously hokey, in the medical community. But lately, surprising findings are emerging: several groups have found that some medications, including chemotherapies, may be more effective when administered at specific times of the day. A diagnostic test can give varied results when blood is drawn in the morning rather than in the afternoon. "We're used to thinking of ourselves as computers," Janis Anderson says, a Harvard psychologist who studies the effect of light on human biological rhythms. "But we can't be just turned on and off. We operate on a cycle," she reminds me. Perhaps in the era of Starbucks and smartphones it's easy to forget that human beings don't come with an on-off switch. For the last tens of thousands of years, we have carried out our lives under the watchful eye of the sun and the dark expanse of the night sky. "The best thing to do," Scheer says, "is to maintain regularity. Try to wake up, take your meals, and sleep regularly, even on weekends." A corollary of his advice: listen to your body - tune into its cycles rather than working through them. It's good advice. Things will always get in the way of course: shifts, deadlines, families, even recreation. But - as much as you hate to hear it - our body and mind are tethered to the goings-on of the natural world. Here's the alternative. Some laboratories have managed to destroy the master clocks of rats. These animals wake up whenever. They sleep haphazardly. They don't really like to rest. Many get sick, even growing tumors faster than normal animals. The rats work their exercise wheels indiscriminately, no matter if it's night or day. Look at them running ceaselessly. Remind you of anyone?

Diagnosed - The woman in the chair Posted by Dr. Sushrut Jangi November 15, 2013 08:44 AM This is the conclusion to the medical case presented on Monday, which can be found here: http://www.boston.com/lifestyle/health/mysteries/2013/11/the_woman_in_the_chair.html

Why would it be difficult for someone to get up from a chair? Standing from a seated position -- especially without using your arms at all -- means relying on the muscles of the leg, particularly the quadriceps. These are the large, meaty muscles of the thighs. The quadriceps extend the knees, let us walk, run, squat, jump, and rise out of a chair. Take out the quadriceps and standing up from a chair is next to impossible. Consider what happens to young boys afflicted with muscular dystrophy, a genetic condition that causes a key muscle fiber to malfunction and that results in atrophied quadriceps. Affected children often have to get on all fours and must use their arms to "walk themselves up their legs." Grace doesn't have muscular dystrophy (such a disease would not blossom in late adulthood) but her symptom - using her arms to push herself off the chair into a standing position -- is a compensation method similar to those used in boys with dystrophy. Dr. Steven Greenberg, a neurologist at Brigham and Women's Hospital, has made a career specializing in diseases that affect the muscles. He knows that lots of different conditions can cause a person to have weakness in specific muscle groups, such as the quadriceps. The causes may be as varied as inflammation in the muscle, Cushing's syndrome, an underactive thyroid, and even certain infectious agents -- including a parasite that comes from eating undercooked pork. Certain medications, including statins, can cause the muscle to break down, leading to weakness and muscle pain. Differentiating among these causes usually means looking at the affected muscle under a microscope with special stains. "Dr. Greenberg told me they wanted to biopsy my muscles," Grace says. "They ended up taking some tissue from my thigh." Soon after obtaining the tissue, Greenberg had a diagnosis. He was able to rule out statin-induced muscle damage and other kinds of muscle diseases. "When he told me I would need a cane, a walker, or a wheelchair for the rest of my life, I broke down and cried." Grace's disease is one that Greenberg has been studying for years: it's called inclusion body myositis. The cause of this mysterious disease is unknown but appears to involve some combination of muscle getting injured by the immune system and other causes of muscle degeneration. "Next to the injured muscle tissue, we often see lots of immune cells, and they are probably causing a lot of the damage. But sometimes, some of the muscle fibers appear to die without any immune cells around," he says. Inclusion body myositis is a rare disease, although it is one of the more common causes of degenerative muscle disease in those above 50. "Up to 70 people per every million may have the disease. It's mostly seen in Caucasians. It's virtually unheard of in Africa and certain parts of Asia, but that might be changing. There have been more cases in Japan recently." Usually, Greenberg says, people with inclusion body myositis have a slow progression of symptoms, and like Grace, it may take months or even more than a year before they decide to come in to see their physician. "People have difficulty gripping objects, opening jars, turning a key to get into the house. They might have trouble getting out of chairs and bicycling. Later on in the disease, people will complain of their knees buckling." Doctors didn't know what to make of Grace's rash, although some thought about a disease that mimics inclusion body myositis, called dermatomyositis, which can cause a rash above the eyelids that has the pinkish-purple color of the heliotrope flower. But Grace described the rash on her legs, and the biopsy was not consistent with dermatomyositis. Given the difficulty with diagnosing the disease, Greenberg has been searching for a way to help identify patients with inclusion body myositis. Recently, his research group developed a new blood test, which looks for an antibody, called anti-cn1A, that appears to react to a component of muscle. The blood test comes back positive in more than half of patients with the disease. Unfortunately, even if the disease is accurately diagnosed, there are no effective therapies. Many of the afflicted require a wheelchair within a decade or may suffer serious injury or even death from falls or respiratory problems. Recently, there have been some glimmers of promising drugs on the horizon. Novartis has developed a compound called BYM338 (bimagrumab) that stimulates muscle growth. The drug might be useful in other diseases that cause muscle breakdown too, such as in the muscle wasting that often accompanies cancer or HIV. Two months ago, bimagrumab received special status from the US Food and Drug Administration to undergo expedited review that may help get it to patients faster if clinical trials show continued benefit from the therapy. Last year, Grace herself was on a trial using this new agent, but she thinks she was probably getting the placebo. "I didn't get any of the side effects," she says. "I've heard the drug's side effects include stomach cramps and acne, and I didn't get either of those." Some of the other people she knew in the trial noticed increases in their muscles. "That didn't happen to me." Grace is hoping to qualify for another clinical trial. She's called Novartis, and she's also writing a letter asking the company to grant her compassionate access to the experimental drug. Meanwhile, Grace has found other ways to try to help herself. She has visited the Osher Center at the Brigham, which focuses on holistic health. "I've been taking over-the-counter supplements, one called coenzyme Q that helps build muscle." Chairs are still a problem for her. "Yesterday, I was at my temple and I got up once from my chair without a problem. The second time though, I couldn't do it. A young man who does the catering came over and helped me. People are always around to help. I still have trouble with chairs," she says, "but I'm not embarrassed anymore."

The woman in the chair Posted by Dr. Sushrut Jangi November 11, 2013 04:43 PM This is the case of a real patient seen in a Boston hospital. After reading the case, I invite you to think through the facts and try to determine a diagnosis in the comments section below. The answer will be posted Friday. "I had just come back from Israel," she tells me. "Oh, it was a very exciting trip! Everyone was trying to talk to me in Hebrew, even though I didn't know the language myself. It was a beautiful place and I met a lot of interesting people. But," she goes on, "I do remember being tired a lot. The hardest part was getting into and out of the bus." That's when things started, she says. "I had a rash on my legs. I remember that, on the front of my legs, both sides. It didn't go up to my thighs. I went to see my doctor." "What did they do for you?" I ask her. "I don't remember what we did about the rash. It just went away. It was all very strange. I knew, even back then, that I had some kind of situation." Little things, she says. The most ordinary things can warn us, scare us, transform us during illness. Grace (as I'll call her) is 80 years old, but you wouldn't know it. She has a lively and vibrant voice and manages the house by herself. "People say everyone in my family looks younger than they are," she laughs. She lives in Newton and has raised her two children in Brookline. Little things kept happening to Grace after she returned from her trip in 2005. "For me, it was chairs," she says. "I'd be sitting in a chair, at a restaurant, at a movie theater, with other people. But when it came time for me to get up, I had a hard time. I would look around and compare myself to other people. I'd see other people my age standing up from a chair without a problem. They had no difficulty. Chairs! I couldn't understand why they were giving me such a hard time!" "What was hard about it?" I ask her. "What were you feeling when you tried to get out of the chair? Did it feel like your legs were heavy, like lead?" "No," she replies. "Nothing like that. Not like they were asleep. I just felt like my legs were going on me. Like they were going to buckle. Sometimes, I'd start to stand, and halfway up I couldn't do it anymore. I couldn't go the whole way. I would sit down again. Sometimes I thought I was going to drop to the ground. I noticed that certain chairs made it easier. For instance, when the chairs had arms. I could support myself that way, push myself up. Or if the chair was higher up. Like a barstool. So I started putting a cushion under me. That made it easier to stand up." So it was your legs, mostly. Your leg muscles that had suddenly gone weak. She agrees with that. "But was there anything else? Did you notice your arms were weak too? Did you have trouble combing your hair?" "No. But there were a few other things," she recalls then. "I had a few falls. The worst fall, I was in my house. My feet gave out. I fell. Fractured my hand. My ankle would just go on me. I started to feel unstable walking." Were you dizzy? Trouble breathing? Trouble swallowing? No, again. "Oh," she says. "I used to be on Lipitor. You know, for high cholesterol. Then I started getting pains in my quadriceps. That can happen from Lipitor. So they stopped that. They switched me to simvastatin." Grace does know her diagnosis now. But still, the most ordinary, familiar places feel like uncharted terrain. "I went to the Newton public library recently. I went to get out of the car, and I just started sliding. A woman saw me. She had a little child with her, but she helped me up." “And later that day," Grace says, "I went to a CVS. I was in an aisle, pushing a shopping cart, and I just fell to the ground. I sat there on the ground, crying, thinking my life was over.” Can you figure out what was happening to Grace?

Turning yellow - diagnosed. Posted by Dr. Sushrut Jangi October 18, 2013 05:20 PM This is the solution to the diagnostic mystery I posted Monday: http://www.boston.com/lifestyle/health/mysteries/2013/10/turning_yellow.html? comments=all The diagnosis: Dr. Edward Ryan, the director of Tropical Medicine at Massachusetts General Hospital, led the infectious disease team that consulted on Jean's case. After drawing his blood and preparing a smear on a glass slide, the team saw swarms of parasites had infected almost a third of his red blood cells (see figure in prior post). Jean was diagnosed with severe malaria, caused by the parasite Plasmodium falciparum and spread by mosquitoes in many tropical countries. This disease moves rapidly, and if diagnosed late, can be fatal. Several clues point to his diagnosis. The first is a high and recurrent fever. A patient returning from a malaria zone with fever should always be evaluated for this disease. American physicians frequently neglect to do so, especially since the disease may not announce itself until weeks after a person comes home from vacation. The yellow color of the skin, called jaundice, occurs when the parasites destroy red blood cells and release a molecule called bilirubin, a bright yellow pigment that seeps from the circulation into the skin. The malaria parasite also causes tiny clots to form throughout the blood vessels; one by one, the organs may be starved of oxygen. The kidneys can fail. The brain, as it receives less oxygen, causes people to become drowsy and lethargic. The same tiny clots can shear platelets as they try to move through the circulation, causing their counts to fall, as they did in Jean. Malaria kills 1 million people every year worldwide; the incidence of the disease in the United States is highest among travelers returning from abroad. Half of these travelers, like Jean, are visiting family and friends in a country they consider home. Although Jean's parents might have some immunity to the disease, their children may not. "This is not a case of bad parenting," Dr. Ryan says. "Instead, it's simply not on the radar screen for families or even primary care physicians. Families need to take appropriate precautions when traveling to malaria zones, which may include using mosquito netting, DEET-containing insect repellants, and medications." The federal Centers for Disease Control and Prevention's website (http://wwwnc.cdc.gov/travel/destinations/list.htm) lists the necessary precautions people should consider before traveling to a particular country, even if it's a country that the family considers home. Given Jean's progressive symptoms, the team had little time to waste. The doctors started Jean on intravenous quinidine, a drug that poisons the malaria parasite. They also called the CDC in Atlanta to ask for the most potent malaria medicine available in the United States - artemisinin. Unfortunately, because the Food and Drug Administration hasn't approved this drug, hospitals don't carry it. Instead, artemesinin is made by the military at Walter Reed National Military Medical Center and stockpiled by the CDC throughout the country. In Jean's case, a shipment of the medicine was flown from JFK airport to Boston, reaching Jean when he most needed it. "In general, we try to get artemisinin to patients who need it within seven hours of the telephone call," says Paul Arguin, chief of the Domestic Response Unit in CDC's Malaria Branch. That's commendable for a drug that no company makes, that no hospital stocks, and that the FDA has not yet approved. After receiving artemisinin, Jean's blood was cleared of the parasite. Had recognition or treatment of the disease been delayed longer, the outcome could have been far worse.

Turning yellow Posted by Dr. Sushrut Jangi October 15, 2013 04:57 PM This is the case of a real patient seen in a Boston hospital. After reading the case, I invite you to think through the facts and try to determine a diagnosis in the comments section below. The answer will be posted Friday. Jaundice, or the yellowing of the skin, usually first shows up in the whites of the eyes, or in the skin just underneath the tongue. One winter, Jean came into the hospital emergency room with yellow skin. He had been a healthy and active 7-year-old-child. Recently, Jean had accompanied his family on a trip to their home country of Ghana. They visited relatives, ate home-cooked food, and drank local water. During their one-month stay, none of them felt anything was wrong. Two weeks after Jean came back to the United States, he noticed his muscles had started to ache all over his body. One night, he became feverish, sweating through his clothes, his temperature rising to near 102 degrees Fahrenheit. His family brought him to his pediatrician, who discovered the child had a sore throat. Given the weeks of fever, sore throat, and body ache, the pediatrician suspected that Jean had a case of infectious mononucleosis, and started him on amoxicillin. At first, Jean got better on the antibiotics. His fever came down and his muscle aches improved. But a couple of days later, his temperature climbed again. It had now been more than two weeks since they had returned from Ghana. Jean stopped eating; he could not bear to swallow the antibiotics. He was moaning in discomfort and felt so weak that he could not get out of bed. His family called for an ambulance and he was rushed to the hospital. In the emergency room, his blood pressure was noted to be very low, and his heart was fluttering rapidly. The medical team started him on intravenous antibiotics. The boy drifted in and out of sleep, waking occasionally to nod his head. He was jaundiced: his skin had turned yellow. His platelets - the cells that prevent bleeding - were 10 times lower than normal. The ER deemed him critically ill. A blood test (results shown below) confirmed his diagnosis. What was Jean's disease - and how would you treat him? Results on blood smear: Picture courtesy: Perrine Marcenac & Dan Milner, Harvard School of Public Health

A breast disease that wouldn't go away - diagnosed. Posted by Dr. Sushrut Jangi September 13, 2013 07:01 AM This is the solution to the diagnostic mystery I posted Monday, in which a woman kept returning to the doctor with an apparent breast infection that wouldn't go away. Dr. Lester and her colleague flipped through textbooks, searching for a solution to the puzzling case of the woman whose breast disease wouldn't go away. The patient had finally come to the surgeons and pathologist with a concern for an abscess, but Dr. Lester didn't find any signs of infection. Instead, the pathologists found keratin, a protein made by a type of skin cell called a squamous cell, that gives skin its almost waterproof-like quality. Squamous cells and keratin are not found deep in the breast tissue, but Dr. Lester observed them lining the ducts that ordinarily carry milk. Why were there cells that looked like skin cells in the breast ducts? The answer to this question is a grand mystery, one that goes further than the particulars of this case.

A breast disease that wouldn't go away Posted by Dr. Sushrut Jangi September 9, 2013 11:43 AM This is the case of a real patient seen in a Boston hospital. After reading the case, I invite you to think through the facts and try to determine a diagnosis in the comments section below. The answer will be posted Friday. A visit to a pathologist would be very different from seeing a regular doctor: there is no waiting room, no examination table, nor a nurse to take your height and weight. Instead, you travel to the pathologist in bits and parts -- a slide of your skin, a smear of your blood cells, or perhaps a sliver of bone marrow. The pathologist then tries to identify patterns in the tissue structure, searching for the telltale footprints of a diagnosis. "This is what drew me to pathology," said Dr. Susan Lester, chief of Breast Pathology at Brigham and Women's Hospital. "I got to sit in an office, surrounded by textbooks, with an aim to observe, notice, and classify disease." But for the pathologist, like for any clinician, diagnoses aren't always straightforward. One day, during her pathology residency, Dr. Lester received a breast biopsy from a woman who kept returning to the doctor with signs of a painful breast abscess. A breast abscess, or collection of pus in the breast tissue, is not in itself unusual -- almost 10 percent of lactating females develop inflammation of breast tissue and can go on to form an abscess, usually colonized by swarms of Staphylococcal bacteria. What was odd about this case was that the patient wasn't lactating -- she was an older woman. Moreover, these infections usually get better with antibiotics. This patient did not; after each course of antibiotics, she came back with the same symptoms. Dr. Lester had heard about cases like this before. Sometimes, when patients get re-infected by the same bacteria over and over, doctors suspect that patients might be infecting themselves to get attention, either willingly or subconsciously, a psychiatric condition called Munchausen's syndrome. But that kind of diagnosis was a last resort - a patient with recurrent illness might also mean a clinician is repeatedly missing the same boat. Dr. Lester hoped she could help crack the case by finding a diagnosis hidden in the tissue, even though she would probably never meet the ailing patient. "Sometimes," she acknowledged, "pathologists do become a little divorced from the patients we are trying to help." After spending all day looking at slides, she said, it becomes possible to forget that the tissues belong to real patients, who days later, may receive a serious diagnosis from a clinician because of the pathologist's diagnosis. So while beginning a new case, she gently reminds herself: "Just imagine this diagnosis could be for your mother or for your child." Under the light of a microscope, Dr. Lester carefully examined the slides processed from the patient's biopsy. In the tissue, she didn't see any swarms of bacteria that you might find seeding an abscess. Instead, she saw clusters of lymphocytes, plasma cells, and giant cells -- the kinds of cells that accumulate in an inflammatory response. But that wasn't enough: she needed a specific finding that might reveal a cause for the woman's recurrent disease. She showed the slide to a colleague, who noticed a puzzling finding: some of the normal glands that ordinarily line the milk-producing ducts of the breast near the nipple had transformed to resemble the cells of the skin, and they were making a substance called keratin, the dry, scaly stuff that comes off nails and skin surfaces. "He knew he had seen this pattern before, but he couldn't remember the exact significance," Dr. Lester said. She saw him pulling textbooks off his bookshelves and scientific papers out of his old case files. They paged through images and case descriptions. These were the kinds of mysteries that had brought Dr. Lester to this field. "You need to have a good visual memory, attentiveness to detail, and an intense curiosity about finding the cause of disease," she reflected. Together, she and her colleague provided the patient's clinicians with a diagnosis. This is what she saw under the microscope:

What was happening to this woman?

Picture Courtesy: Textbook - Diagnostic Pathology: Breast (Published by Amirsys)

The "Right" Treatment for ADHD: Revealed Posted by Dr. Sushrut Jangi August 23, 2013 04:28 PM This is the conclusion to a case about a child diagnosed with ADHD I posted last week (http://www.boston.com/lifestyle/health/mysteries/2013/08/medicating_children_for_attention-deficit_hyperactivity_disorder_adhd.html). The child's family and physician were making decisions about how to treat this disease. Many readers voted that starting an ADHD medication and behavioral therapy together might be a good way forward. Her doctor agrees with this approach. "A lot of judgement happens the day I talk about starting medicines for young children," Dr. Chan says. Most parents have already tried numerous other routes, such as behavioral therapy which is frequently recommended first. But behavioral therapy alone is hard to implement. "It's hard to access and there's not too many families who can actually carry it out," Chan says. "If you're a single parent working multiple jobs, its really hard to fit the time to take your child regularly. It's a huge time investment." J's parents tried the behavioral therapy route and they worked hard at it. But he wasn't improving. Dr. Chan is more than familiar with the culture of fear that surrounds ADHD medications, but she feels these fears are overinflated. Consequently, children who might benefit from being on medicine get delayed treatments, which can have harmful social effects. "Children in his class already know that he's different, so they react to him differently. Children with ADHD start getting negative feedback from their peers early on." Dr. Chan feels that this is one potential justification for starting medications early. "These medicines can help children get out of cycles of negative-feedback. And we're not condemning children to medicine for the rest of their lives. They can be started as a trial, and then stopped down the line." Frustrated, tired, and hoping for a solution, J's parents listened to Dr. Chan and made the choice to begin medication. Within weeks, J's teachers noticed a startling difference. His behavior reports at school, which used to be mostly reds and yellows - a sign of inadequate performance suddenly transformed into greens. His parents were stunned. "The effects of these medicines are fairly immediate," Chan says. "You can pretty much tell within a day or two if they are going to work." But the key, Dr. Chan says, is not just using medications. Once a child has proven to benefit from an ADHD medication, the child is more likely to work together with a parent or a teacher to learn strategies that will lead to longer-lasting behavioral changes. Then, when the child is off medication, he retains these useful behavioral patterns. The diagnosis, Dr. Chan stresses, must be accurate before starting a child on ADHD medications. Sometimes, other conditions masquerade as ADHD, such as insomnia, anxiety, depression, autism, seizures, lead poisoning, or even child abuse. When I ask her if she feels that dramatic rise in ADHD rates is real, she pauses. "I think overdiagnosis of ADHD occurs but so does underdiagnosis. There are children out there getting medications who shouldn't be, and there are children who are not receiving medical therapy who should be." But despite this phenomenon, she agrees that its possible something else is going on in our society, something that is driving up ADHD rates in children. "We are requiring younger and younger kids to be in more structured academic environments. There's a lot of kids who are 3 or 4 or 5 years old who are just not ready to be quiet and do that kind of intensive work." Schools have curbed recess and made lunches 20 minutes long; at home, kids are exposed to high amounts of television, Ipads, and the internet providing continuous novel stimuli that might induce neurologic changes that foster shorter attention spans. "The jury is still out on this, but people are studying these kinds of environmental changes now," Chan says. Not every child with ADHD will benefit from the medicine, or even the same kind of medicine. Every diagnosed child requires a unique combination of treatments, whether they are medications, behavioral therapy, school accomodation or special education services. And every medical decision is intensely personal, shaped by experiences of the child, parents, and the physician. Recently, at a party, I met a couple raising their four year old daughter that made me realize how difficult diagnosis and treatment of ADHD in children must be, especially when childhood is a period of such intense activity, growth, and change. The young girl, I noticed, had a lot of energy and was running around the room in circles. During dinner, she sat in the corner with an Ipad glowing, the screen throwing colorful lights and sounds across her face. "The Ipad keeps her quiet," her mother whispered. But I worry, she went on, that she can't keep quiet without it. What will we do when she starts school? Do you think she's normal? she asks. Or do you think she's developing a larger problem?

The "Right" Treatment for Attention-Deficit Hyperactivity Disorder (ADHD) Posted by Dr. Sushrut Jangi August 20, 2013 09:23 AM J was only 6 years old, but his parents came to their pediatrician with a difficult question: should we start J on medications for possible attention-deficit hyperactivity disorder? Dr. Eugenia Chan sees families struggling with this question all the time. She's a pediatrician at Boston Children's Hospital who directs the attention deficit hyperactivity disorder (ADHD) program in the Development Medicine Center. Many parents come to her afraid of ADHD medications - after all, we frequently hear stories that validate these fears. For instance, a recent report from the Substance Abuse and Mental Health Services Administration (SAMHSA) reported that ADHD medications sent 23,000 young adults to the emergency room last year in drug-related emergencies: that's a four-fold increase compared to 2005. ADHD medications have been linked to anxiety, addiction, and rarely, psychosis. A popular story in the New York Times a few months ago reported on the suicide of the college student Richard Fee, who hanged himself after prescriptions for his ADHD medication expired. While such tragedies are rare, abuse of ADHD medications in the college setting are not uncommon: almost 35 percent of students have been estimated to use these medications to provide short-term bursts of energy to get them through rounds of finals. Colleges have begun cracking down on ADHD prescriptions to unmask abusers. Furthermore, with sales of stimulants reaching $9 billion last year compared to $4 billion in 2007, and almost 14,000 new prescriptions written monthly (a three-fold increase compared to five years ago), ADHD medications have become stigmatized, their use surrounded by a culture of fear and abuse, a culture parents are largely aware of and consequently do not want their children to enter at a young age. The option of medicating children for psychiatric or neurologic conditions force parents to face a difficult choice. For some parents, the decision takes a long time, especially when the diagnosis may not be clear. Dr. Chan met J when he was six, but his parents and teachers had noticed problems far earlier. "Even when he was 2 years old, he was restless and easily worked up," Chan says. By the time he started kindergarten, J's teachers raised red flags. They called home and told the parents they were concerned about his impulsivity and occasional aggression. His parents had heard similar complaints from others before. A year earlier, when J was first enrolled in preschool, the teachers had asked his parents to pull J out of class because of his disruptive behavior, a "solution" that is unfortunately not uncommon. Frustrated, J's parents took him to behavioral therapy, where a psychologist recommended they try a program of regular, consistent activities (keeping a daily schedule, limiting choices, using time-outs) to ensure that J got the direction he needed to move past his impulsiveness. But by the time he was almost 7, nothing had changed. J's parents were exhausted. Every few months, they got calls from teachers describing a litany of his difficulties: trouble following directions, inability to sit still, and trouble focusing his attention. "I met with this family several times," Dr. Chan describes, "and very quickly realized he met criteria for ADHD." She introduced the idea of starting a medicine to supplement his behavioral interventions. The drug she would use - called methylphenidate - has been approved as a first-line agent for the treatment of ADHD for children as young as 6 years old. Swallowed or chewed, the medicine prolongs the effect of the neurotransmitters dopamine and norepinephrine on the nerve cells in the brain, providing a stimulating effect that can foster renewed focus. But since the brain is still evolving during childhood, many parents fear that stimulants may disrupt normal neurologic development and create dependency. Consequently, J's father was against starting medication for J. "His father told me that when he was his son's age, he couldn't focus either," Chan says. It was only with time and sustained effort, J's father said, that he learned to grow out of it. "Many parents want to find an answer other than medication. Because when parents make a choice to start one of these medicines for their child, there isn't a single day they don't second-guess that decision. Will this affect my child's appetite, his sleep, will it change his personality? Will it make him a zombie?" Dr Chan says. Most often, she goes on, parents feel they are condemning their child to a lifelong drug. "I've only had a handful of parents walk in who say, 'I want to medicate my child.'" The conclusion to this case will be posted Friday.

Clover Food Labs Experiment with Transparency during Recent Salmonella Outbreak Posted by Dr. Sushrut Jangi August 6, 2013 12:51 PM One rainy morning, I meet Ayr Muir at Cutty's, a breakfast-and-lunch outfit across from Clover's temporarily shuttered Harvard Street location. Even in that early hour, Ayr, the CEO of Clover Food Labs, asks for a few leaves of Swiss chard on top of his wheat bagel. "I wanted a real name," he says, when I ask him about his restaurant's namesake. "Something memorable, easy to say, that would evoke the things we are trying to do." He drinks from his tea and takes a bite of his bagel. "There's the luck element of the name, of course," he says. Unfortunately, bad luck has come to Ayr in recent days: Clover Food Lab has been linked to dozens of cases of Salmonella in the Boston area this past month. Customers who fell ill ate one of two sandwiches: the chickpea fritter or the egg and eggplant. A cadre of epidemiologists from Massachusetts descended on Clover over the past few weeks to perform an exhaustive investigation of their food inventory, employees, and operations. "What can you tell me about the outbreak?" Ayr asked the state department when he first got a phone call from them three Fridays ago. Up until that moment, his restaurant had never been linked to food-borne illness. "Where could the Salmonella be coming from?" he wondered. Food-borne illness is more common than we like to think. In the United States, nearly 50 million episodes occur yearly (on average, one of us will have food poisoning every three to four years). The most-wanted bacterium responsible for food-borne disease is Salmonella. But when we hear about a Salmonella outbreak, the story is usually touted as an exotic and rare event, a departure from the usual causes of food poisoning. It shouldn't be so. Salmonella is ubiquitous. After all, it's name has nothing to do with contaminated fish (the bacteria was named after the veterinary surgeon, Daniel Salmon); Salmonella-associated disease is almost a routine cause of food-borne illness from sources as varied as peanuts, mangoes, dry cereal, sprouts, papayas, and eggs. Such frequent cases often go undiagnosed and outbreaks aren't heavily advertised. The state investigates, a restaurant quietly turns off its lights, and after people have recovered, the doors open again. But Clover has responded to this outbreak differently. "The day after that phone call from the state, I started to blog about it on our website, so that everyone, including our customers, would know what was happening." Broadcasting the outbreak is an unusual move. The major federal agencies that regulate food safety including the FDA, the USDA, and the CDC historically have been tight-lipped when food outbreaks begin. And, as reported in the Columbia Journalism Review earlier this year, a fragmented food regulatory system has caused significant struggle for the media to report food outbreaks as they occur. For example, in 2009, the CDC began investigating a national Salmonella outbreak but headlines didn't hit newspapers until nearly three months later. The consequences? People get sick while outbreaks are still occuring; the media and the public, unfortunately, are often left in the dark. A graduate of MIT with a degree in Material Science, Ayr thinks critically and scientifically about food, and he wants us, as a society, to start thinking thoughtfully about food as well - when it's good - and when something goes wrong. On his website, he advertises that Clover has "no back-of-house," meaning he wants the process of food preparation and distribution to be completely transparent. So, just a day after he got a call from the state, Ayr notified the public. "We learned late Friday there is a Salmonella outbreak in Massachusetts," he wrote on his blog. "Some of the confirmed cases ate at Clover." Immediately, he described an action plan, the possible foods that might be responsible, and the changes he anticipated in the coming days. In perhaps the most intriguing experiment at Clover Food Lab thus far, Ayr is giving us a real-time window into an outbreak as it occurs, giving us updates on the last-known exposure and preliminary results of the state's tests ("so far, everything has come back negative.") The gutsy response to the outbreak is a fulfillment of the promise that should exist between a food provider and the public, one that other restaurants would be wise to emulate. It's a move that brings us, the public, up close to the realities of food, both to its dangers and its benefits, rather than fostering the illusions that marketers try to sell us. Elizabeth Hohmann, a physician and an associate professor of medicine at Harvard Medical School who researches Salmonella, also advocates for the public to try to better understand what they are eating. "People want their food to be perfect, but it is not, and it won't ever be. Even with the best suppliers, handling, and elimination of cross-contamination, outbreaks will still occur, even in the best restaurants," Hohmann says. "Food," she reminds us, "is not in any way sterile! In our culture, there's a huge disconnect between our perception of food on a plate, and what food really is." Ayr is hard at work trying to repair that disconnect between us and the food that we eat. "People have said to me, 'wait a minute, you take vegetables that came raw from a farm, and you carry those through your kitchen, to the restaurant, and then you make food?' And I say, yeah. Then they ask 'Have you ever been to any of those farms?' and I say, yeah, we've been to all of them." Keeping outbreaks behind shuttered doors is not only a safety hazard but it prevents the public from thinking critically about food. "It's so hard to change that culture," Ayr says. But I think Clover Food Lab is opening doors that have been traditionally locked. The restaurant's mostly organic, local, vegetarian food challenges the routine of processed foods and centralized distribution and asks us to put thought into what we eat outside of our homes and what we bring back to our kitchen counters. Perhaps Clover will have to do some work to regain our trust in the short-run. But we should be impressed with their response to this outbreak. "I expected a lot of negativity, so we braced ourselves," Ayr says, when he decided to go public. But that negativity never really came. Most are already back onboard with Ayr's overall mission. "Would you go back to Clover now that it's reopening?" I ask Hohmann. She doesn't even hesitate. "Absolutely." Recently, Ayr got an email from one of his customers. I ate at your Brookline location, the woman wrote. I think I was one of the victims, since I had vomiting and diarrhea. But I'm okay. When are you planning on opening a location in Framingham? Picture: Re-opening day at South Station, courtesy Lucia Jazayeri

Dying, Fast and Slow: The pesticide poisonings in India Posted by Dr. Sushrut Jangi July 24, 2013 08:54 AM I'm not used to seeing public health stories about India in American newspapers - but last week, the tragic deaths of 23 North Indian children after they ate tainted school lunches in the agricultural state of Bihar was prominently featured in the New York Times, CNN, the Washington Post, NPR, and the Boston Globe. Such extensive American media coverage about a health tragedy in a poor, rural state in India surprised me. Obviously, the death of children arouses our compassion, but unfortunately, such events occur so regularly throughout the developing world that most end up receiving notably less attention. Consider an event in Nigeria three years ago: a medical team belonging to Doctors without Borders ventured into northwest Nigeria to conduct an immunization campaign for children but instead found farming towns mysteriously devoid of children altogether. The team found families engaged in subsistence gold mining, a process that released dust laden with unbelievably high concentrations of lead. Partnering with local agencies, the Centers for Disease Control, and the World Health Organization, researchers determined that the lead exposures were among the highest in the world - and that such exposure had killed more than 400 children under age 5 and left more than 2,000 children with permanent disabilities. But when I asked some colleagues about these two stories - the 23 dead in India, and more than 400 dead and 2,000 disabled in Nigeria - most were very aware of the school-lunch story but had no familiarity with the second. The reason for this discrepancy, I suspect, rests on the speed of the two tragedies. Moments after the Indian children noticed a funny taste in their food - apparently from a potent insecticide -- many developed stomach cramps and vomited; within hours, some were critically ill or had died, even before reaching the hospital. Within a day, news agencies around the world were alerted to the crisis. On the other hand, in Nigeria, the lead poisoning epidemic has evolved over months to years, and the consequences of lead poisoning - brain damage, paralysis, deformity - have taken a long time to reveal themselves. Consequently, the Nigerian story, although tragically important, has been more difficult to tell in our current rapid pace of media coverage. Without reminders, the slow-moving public health crisis tends to slip under our radar. Even in the recent news about the poisoned schoolchildren in India, a slower story hides in its shadow. "You've heard about the cancer train?" asks Amit Khurana, head of the Food Safety and Toxins Program at the Center for Science and Environment in New Delhi, India. Nightly, he says, a train departs from a farming district in Punjab, taking passengers to a hospital in the bordering state of Rajasthan. The train is nicknamed "marizon ki train" or "train of the ill": its seats routinely fill with cancer patients, both young and old, afflicted with all manner of malignancies. The reason for high cancer rates in this rural farming community?

And here, again, is the crux of the problem in a slow-moving disease process: the longer the time-lag between a potential exposure and its health consequence, the harder it is to track, to prove, and to report. "So a lot of people in India remain unaware of chronic pesticide exposures," Khurana explains. "A few years before I took this job, I didn't know about it either." Now, Khurana is intimately familiar with chronic pesticide poisoning in India. Over the past decade, his NGO has found signs of high pesticide concentrations throughout the Indian marketplace. More than 17 brands of bottled water tested in New Delhi, for instance, contained almost 40 times the standard limit of pesticide concentrations. Similarly, in Pepsi and Coke bottled in India, the group found 30 - 36 times the recommended levels of pesticides. Although two governmental bodies have since formed to try to regulate the registration, sale, and use of pesticides in agriculture, the implementation of such regulations has proven difficult. Monocrotophos, the chemical found in the school in Bihar, is banned in the United States and the European Union, but farmers in India routinely spray it on rice paddies even though the government has restricted its use. "What a farmer does has got nothing to do with the regulations of the state or central government," Khurana says. "He is more likely to be influenced by the sales representatives of the pesticide companies. Most farmers aren't even aware of the dangers of pesticides, how much they should use, or how long they should wait before bringing a sprayed crop to the market." In the United States, we've been pretty good about regulating pesticides. Production and use of highly toxic organophosphates - including monocrotophos - have been curtailed. Acute pesticide poisonings, like the one in Bihar, are rare in this country. But the possibility of slow, chronic diseases from low-level absorption of pesticides are still possible. "There have been several prospective studies that show a relationship between pesticide levels in pregnant women and lower IQ levels in children," says David Bellinger, professor of neurology and environmental health at the Harvard School of Public Health. In other words, he agrees with the idea that low, chronic pesticide exposure can adversely affect human health over time, with accumulating evidence connecting pesticide exposure to neurotoxicity in children and cancer risk in adults. Given more effective regulations in the United States, chronic pesticide exposure is less of an issue in our country, but Bellinger says that "we still have a way to go." Meanwhile, in India, this slow poisoning continues unchecked, and with a large pesticide industry lobby, no effective government regulations have been set into place. I ask Khurana: if I go into any market in an Indian city and I buy a piece of fruit or vegetable, what are the chances that it will contain significant levels of pesticide? "One hundred percent," he says. Maybe all this reporting of the tragedy in Bihar, I tell Khurana, might push the government to make changes, so this doesn't happen again. "Maybe," Khurana hopes. But the Indian media, he says disappointingly, has already moved on.

Why was Wendy paralyzed? Revealed Posted by Dr. Sushrut Jangi July 19, 2013 05:00 PM This is the answer to the diagnostic mystery I posted earlier this week about a patient named Wendy.

Some years ago at the hospital where I worked, a crying woman came into the emergency room with her husband, who had woken up in the middle of the night with high fevers and chills. By morning, he had developed a purplish rash that spread down his legs. When he arrived, he was clammy, delirious, and unresponsive. We started him on intravenous fluids and antibiotics, but within hours of reaching the emergency room, he was dead. I had never before seen a man die so precipitously from illness. The blood drawn from his veins grew out a bacterium called Neisseria meningitidis, an organism that can enter the spinal fluid and cause a swift, and sometimes fatal, meningitis. Did meningitis explain Wendy's sudden decline? Probably not. Wendy was confused, and people with meningitis usually have a normal mental state because the disease affects only the tissues surrounding the brain. Consequently, patients with meningitis may have pounding headaches, a stiff neck, light sensitivity, and high fevers. But if the infection spreads into the tissue of the brain, a person may begin to behave bizarrely, hallucinating, shouting, or becoming lethargic or comatose. We call this encephalitis, which can be caused by a number of viruses, the most famous culprit in Massachusetts being the West Nile virus, which was found in 5 percent of mosquitoes last year and caused a record number of 33 human cases in a single season. Numerous other viruses can cause encephalitis too, but many of these are difficult to find in the spinal fluid - our testing isn't perfect. So lots of cases of viral encephalitis end up going undiagnosed. So did Wendy have some kind of encephalitis? The picture almost fits - encephalitis can cause problems with motor function, potentially explaining the immobility of the lower legs. Had a neurologist not asked for a repeat MRI, viral encephalitis probably would have been her diagnosis. The MRI of Wendy's brain illustrated white hyperintensities, a finding that can also be seen in normal people. However, in patients with symptoms similar to encephalitis, such brain lesions can represent a rare illness, called acute disseminated encephalomyelitis, or ADEM. Unlike meningitis or encephalitis, ADEM is not directly caused by an infectious agent. Instead, ADEM is the result of damage caused by the body's own immune cells, which target the tissue of the brain. Nobody really knows why the body's immune system goes awry in ADEM, although some feel a prior infection, or even vaccination, may incite the immune cells to attack. Treating ADEM means shutting down the angry immune system, usually accomplished through the administration of steroids. That's what Wendy got soon after the MRI was read. Just two days after the steroids were given, she opened her eyes. Despite the suddenness of the onset of ADEM in Wendy (this illness causes dramatic decline in a matter of days) the effects of the illness lasted far longer. While Wendy recovered her ability to speak, and breathe, and eat on her own, the disease had caused significant damage to her brain and spinal cord, rendering her quadriplegic at the time of her discharge from the hospital. It was only following prolonged and aggressive treatment at Spaulding Rehabilitation Hospital, that nearly two months after the illness had begun, she was able to stand, walk, and - with great effort, attempt a jog down the hospital corridors. A sudden illness - one that began in a matter of days - reached its grasp around Wendy's life weeks, months, even years after it first began. The impact has had such resonance on Wendy's perspective that she has catalogued her story in a book, titled To Get Back Home. The disease has erased the memory of the first few days she fell ill and much of the time she spent in the hospital; the details of her story have been reconstructed through her interviews with her doctors, family, and loved ones. I've noticed stories of illness are rarely told by only one narrator; whenever I walk into a hospital room and ask the patient what happened, family often jumps in - "No, mom, tell them about your hand," or "That's not what happened, Dad!" Like memory, the impact of disease is shared. It is this sharing that can make illness so terrible and recovery so meaningful. Wendy Chapin Ford recounts the entire story of her illness in the book she has authored, "To Get Back Home: A Mysterious Disease: A Fight for Life."

Why was Wendy suddenly paralyzed? Posted by Dr. Sushrut Jangi July 16, 2013 12:17 PM 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.

Over the past century, chronic diseases - diabetes, high blood pressure, and cancer - have become more common, while sudden, thunderclap illnesses are less frequent. But when an acute illness does occur, a comfortable landscape is jarringly altered: one moment, your sister, mother, husband is sitting beside you at the dining table, and the next, he or she is unconscious, in a hospital gown, in an unfamiliar bed. Such illnesses don't give anyone time to prepare for change; we are forced into circumstances, and the repercussions can last far longer than the course of the disease. Just ask Wendy. One Monday morning, 42-year-old Wendy woke up and found she couldn't walk. Wendy and her husband Bruce lived in one of those pretty oceanside towns that dot the rocky arc of the North Shore of Massachusetts. They had a wonderful, happy life. Wendy worked downtown, her office high up in the glassy Hancock Tower that gave her an expansive view of Boston. A summertime tennis player, a wintertime skier, and a year-round active mom (she had a seven-year old son, a three-year-old-daughter) Wendy considered herself lucky, blessed, healthy. But one day in spring, Wendy came down with some kind of flu. "I spent that weekend resting," Wendy would say later, but Bruce noted that she had barely gotten out of bed at all. The lethargy was unlike her; Bruce thought it would pass. By Sunday night, however, things had only gotten worse. She had developed intermittently high fevers, her forehead was clammy, and Wendy had begun to say things to Bruce that were increasingly non-sensical. He wondered if she was hallucinating. "If you're not better by tomorrow," Bruce had said, "we're taking you to the hospital." On Monday morning, Wendy called to her husband, in shock at the inability to coordinate her legs. Devotedly, Bruce helped her to dress. Then, he lifted her out of bed onto his back and carried her downstairs. As he carried his wife out to the driveway, his concern doubled. "Is that a new car?" Wendy asked him, when she laid eyes on the old Buick they had owned for years. He drove her straight away to the hospital downtown. Wendy was rushed into the emergency room, where physicians inserted a long needle into the space between her lower vertebrae to test the fluid around her spinal cord. They were hunting for the source of her fevers and paralysis, wondering if her brain harbored infection. Soon enough, the laboratory called back with the results. Her spinal fluid was full of white blood cells - markers of inflammation -- but in Wendy's case, the doctors found no bacteria. Nevertheless, they started her on antibiotics. "Which ones?" Bruce asked the doctor, who replied: "All of them." Over the next few days, despite the cocktail of antibiotics, Wendy's condition deteriorated. When her name was called, she woke only briefly, babbling incoherently. Soon, she stopped following instructions altogether. Her lower legs remained immobile. Four days since that Monday morning, she stopped opening her eyes, unable to wake. As the doctors stood around Wendy's bed in the intensive care unit, they had reached a standstill: her diagnosis was in question, treatments had no demonstrated effect, and Wendy's life hung in the balance. Such standstills, which occur often in medicine, require reframing the story. Crazy ideas are entertained. Old clues are reviewed under new light. At the suggestion of one of the neurologists, the team decided to repeat an MRI of her head. What had been an ordinary scan the first time around had transformed into a new film: a brain scattered with white blotches that heralded a promise of a diagnosis and a change in Wendy's treatment. Can you help out Wendy's medical team? What was her diagnosis, and what was the treatment? Below: MRI similar to Wendy's, which shows white hyper intensities in the brain. Photo courtesy Dr. Dapaah-Afriyie, Miriam Hospital, Rhode Island.

Rolensky's Echo - Revealed

Posted by Dr. Sushrut Jangi June 14, 2013 12:00 PM This week's Medical Mystery The Hospital St. Nicolas, where Rolensky spent four months receiving oxygen therapy, is a public hospital in Haiti that serves much of the province of Artibonite. Under the auspices of Zanmi Lasante, the sister organization of the Boston-based NGO Partners In Health, the hospital charges about 50 Hatian Gourdes (or about $1.25) for pediatric consultation with nearly all medications covered by this fee. Such low costs attract families from throughout Haiti - and furthermore - that fee is waived for children under five, HIV patients, and families who are unable to pay. 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 Posted by Dr. Sushrut Jangi June 11, 2013 10:05 AM 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:

Could the new H7N9 Chinese bird flu reach Boston? Posted by Dr. Sushrut Jangi May 28, 2013 11:49 AM 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 midFebruary, 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 Posted by Dr. Sushrut Jangi May 17, 2013 12:00 PM 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 Posted by Dr. Sushrut Jangi May 13, 2013 07:00 AM 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? Posted by Dr. Sushrut Jangi April 8, 2013 03:13 PM 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: 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? Posted by Dr. Sushrut Jangi March 22, 2013 04:47 PM Several people guessed the answer correctly - exitseven7 figured it out first. A few days later, D found himself in a CAT scanner per his doctor's instructions. "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? Posted by Dr. Sushrut Jangi March 18, 2013 09:26 AM Why Was This Boston Marathoner Suddenly Out Of Shape? 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 Posted by Dr. Sushrut Jangi February 11, 2013 07:00 AM 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.

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]

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."