You've probably never heard of Lemierre's syndrome. Many doctors haven't heard of it either and that can be a problem. A century ago, Lemierre's syndrome all too frequently led to small unmarked stones in graveyards. And then, thanks to antibiotics, it practically disappeared. But Lemierre's may be making a comeback, a possible unintentional result of the push to reduce antibiotic prescriptions. It is a terrible disease. I should know. It almost killed my daughter.
It started off as just another sore throat. My daughter is 16, and, like many teenagers, can be somewhat dramatic. So when Sarah told me one morning in August that her throat was "killing" her, I didn't worry. I thought it served her right. She had gotten home way past her curfew the previous night. But she complained so much I called her doctor. It was the Friday before Labor Day weekend, and they were booked with back-to-school shots and checkups. I needed to take Sarah to the urgent care clinic instead. The doctor there noted that her tonsils looked mildly infected and ran a strep test. Negative. We went home with drops for her earache and something called Magic Mouthwash the doctor said would soothe her sore throat. Sure enough, she perked up enough to go to West High's first football game of the season that night, painting a big yellow W around her newly pierced belly button (sigh).
But the next morning, Saturday, she woke up sick again. Her sore throat was now joined by a headache Sarah called "the worst in my life." She also had a fever. I figured she had the flu, and a good nap was in order. I left her under the covers with some juice and headed for a family hike. Several hours later, I got a worried call from my aunt. Sarah's fever had shot up to 104 degrees, and she was shaking so hard she was panicked and having trouble breathing. My aunt took her to urgent care again. This time, the clinic did some blood tests. They were fine. I started to wonder if my daughter was a tad hysterical.
Over the rest of that holiday weekend, my daughter's symptoms got more confusing. She'd complain of crushing headaches one minute and perk up the next. She'd be so hot, her sheets were sticky with sweat, and then start shaking with chills even under her down quilt. Late Sunday night I drove her to the emergency room. The clerk behind the desk took one look and handed her a mask. The bars had just emptied, and a fellow waiting next to us had gotten his nose busted in a brawl on State Street. He started bawling while his friend bellowed for help. The nice but frazzled doctor who checked Sarah made sure she could bend her neck, told us she didn't have anything scary like meningitis, and moved on to his other patients. He sent us home with a painkiller he said should help.
It did, until she threw it up the next day, Monday. And kept vomiting everything else I gave her. By then she was complaining that not just her head, but the left side of her neck hurt. Again, we went to urgent care. Again, they sent us home. Tuesday was her kid sister Ollie's first day of kindergarten. Sarah had wanted to walk her to the bus stop, but all she could do was walk to the bathroom and lay her head on the toilet seat.
Enough. I didn't care what all the doctors and tests said. Now that the holiday weekend was over, I could finally take Sarah to the doc who knows her best. As Sarah walked into the exam room, leaning on me, Susan Ehrlich took one look and just "felt," she told me later, she was seeing something more than a kid with a sore throat. "She looks terrible, doesn't she?" Ehrlich asked her nurse, who agreed. Ehrlich has cared for children for more than 25 years, long enough to develop keen instincts and long enough to learn to trust those instincts. "Medicine is not just a science. It is an art," Ehrlich said later. "Ill-appearing young lady, pale," she wrote in Sarah's chart. She ordered more blood work.
The results that came back that afternoon were frightening. My daughter's platelet count had plummeted over the past three days from a normal range of 150-450 to 56, and other figures were off, too. We had our first clues. Something more than a mere sore throat and adolescent angst, but what?
Two things can wreak that kind of havoc on a kid's blood cell count: cancer and infections. Ehrlich ordered X-rays to check for masses in my daughter's chest. And then she called Greg DeMuri, a pediatric infectious diseases expert with the UW American Family Children's Hospital. Ehrlich teaches her medical students that one of the most important things they must learn to say is "I don't know." She didn't know exactly what was going on with Sarah and she needed help. Infectious diseases specialists are medical detectives. They are the reinforcements pediatricians and primary care doctors call on when they can't figure out what's making their patients sick. "We're the fever guys," DeMuri says.
DeMuri met us that night at the urgent care clinic and examined Sarah, who kept nodding off. DeMuri has a gentle manner that belies his ferocity for hunting down organisms. He gets lots of calls about mysterious ailments, and often, he says, they turn out to be not that mysterious, just run of the mill infections. One of his guiding theories is Occam's Razor, which basically means that the simplest explanation is usually the correct one. He tells his medical students: "If you hear hoof beats, don't think zebras. Think horses." But from the start, Sarah's case suggested a more exotic villain, he said, partly because she had recently traveled to both coasts and out to her grandparents' home in Spring Green, which exposed her to a handful of mosquito- and tick-borne viruses. These became immediate suspects. Others included leukemia, meningitis and encephalitis. DeMuri and Ehrlich sent us to the hospital and ordered a raft of tests and procedures to be done immediately. They also started Sarah on antibiotics for a possible infection.
What worried both Ehrlich and DeMuri that night even more than the alarming lab results was how sick their patient looked and acted. "It was like the Battle of the Bulge," he said, comparing the war he feared was raging inside Sarah's infected body to the deadly World War II skirmish. We had little time to lose. If we couldn't track down what was wrong with her in the next one to three days, the bad guys were going to win. "She would have succumbed," he later told me.
We checked into the hospital that night, and for the next hours and days our lives became a blur of tests and procedures and exams. Packs of medical students came into Sarah's room, asking the same questions and doing the same exams. Afterward they would tromp out to the hall, where they would discuss what they had seen and what it could mean. We didn't mind. We hoped their ideas and observations would provide key clues. Sarah's case was a real puzzle, said Jennifer Mosher, the first-year resident who was assigned her case. A thoughtful, soft-spoken native of Casper, Wyo., she cheered Sarah up one day by chatting about cowboy boots. That first night, Mosher was convinced that Sarah had mononucleosis, partly because of the spongy lump on the left side of her sore neck. Mosher and many others who examined Sarah figured it was a swollen lymph node, a classic symptom of mono.
Toward midnight Sarah got a spinal tap. A doctor stuck a huge needle into her back and pulled out fluid from her spinal cord. It can be one of the more painful pokes in a hospital, yet Sarah's headache was so bad, she barely noticed. The spinal fluid was clear, which ruled out potentially fatal meningitis and encephalitis. I held Sarah tight during the procedure. It had been a long time since I had held her hand for that long. It felt so fragile. That night her shakes, which I learned were called rigors and were caused by her fevers, made the bed rails rattle.
A white-coated lab worker drew blood from Sarah - more tubes than I could keep track of. The 26 lab workers in the microbiology department are in charge of identifying which of the thousands of possible species of bacteria, viruses, fungi and parasites are infecting patients. At the UW hospitals, the lab runs about 470 such tests a day. "Nobody hears about us, but we are the micro detectives," said Carol Spiegel, the director of clinical microbiology at the University of Wisconsin Hospital & Clinics. "Every patient is a mystery, and we put it all together." Various tests were run on Sarah's blood. One of the most important was a blood culture. About two tablespoons of her blood were put into two bottles full of broth, a soup for bacteria. Then the bottles, a blue one with oxygen inside and a purple without, were put into an incubator, which looked like a rectangular refrigerator and kept tilting the bottles back and forth.
Normally cultures can take 24 to 48 hours to grow; this one grew in 12.8 hours, a sign of how deadly it was. On Wednesday, Sept. 3, around 10 a.m., an alarm went off in the lab indicating that bacteria was growing in the purple anaerobic bottle. Sue Brakob was on duty then, and she immediately pulled the bottle out of the incubator and put a smear onto a slide. She then used a procedure called Gram staining, which has been around for over 100 years, to stain the slide with purple dye. All bacteria will pick up the violet dye, but when you rinse them with iodide, some bacteria will wash out and become colorless. They are then stained red and pink. These are gram negative bacteria. Spiegel put Sarah's slide under a microscope, and discovered a cluster of bright pink gram-negative rods. The lab paged the resident on duty.
Jen Mosher got the call while she was eating lunch in the cafeteria. She didn't believe it at first. Gram-negative rods are highly unusual and highly virulent. "It threw most of our theories right out the door," she recalled. "It didn't fit with anything we thought we had." The medical team quickly switched Sarah to the antibiotic Flagyl, which a search of medical literature suggested was potent against the kind of bacteria Sarah had.
While the news baffled the young residents, it made a lot of sense to Sabrina Wagner, a hospitalist who had only started working at UW a month earlier. Almost immediately she suspected Sarah's constellation of symptoms might not be mono or cancer or even a bug-borne illness, but a weird and rare disease called Lemierre's syndrome. Lemierre's is named after the French physician who published a study about this vicious killer in 1936. He described a fatal condition in which bacteria from infected tonsils invades neighboring blood vessels of the neck, where it forms an infected clot that seeds the body's organs and joints with poison. Wagner had come across a Lemierre's patient a few years earlier during her residency in Chicago, and so, luckily for us, it was on her radar. Since penicillin mostly vanquished it some 40 years ago, many other doctors have not even heard of Lemierre's, much less seen it. Until recently, there have been about 160 known cases in all of the U.S.
DeMuri was hot on the trail, too. There are only two places without oxygen deep in the body where gram-negative rods flourish: the gut and the neck. Sarah had been complaining about pain in her abdomen. So doctors ordered up a round of MRIs and X-rays to check for an abscess there. Negative. That left Sarah's sore neck as the most likely scene of the crime. By the next morning, Thursday, when they met at grand rounds, Wagner and DeMuri looked at each other and whispered: "It's Lemierre's."
Soon there was proof. CT scans revealed that the lump in Sarah's neck that residents had believed was a swollen lymph node was actually a 3-inch-long infected thrombus. The blood clot was blocking her left jugular vein. And in a laboratory lineup of petri dishes, the gram-negative rod bacteria had been painstakingly coaxed into growth. They turned out to be the dreaded Fusobacterium necrophorum, the usual villain in the illness described by Lemierre 80 years ago. Sarah's rigors, fevers, aches and sore throat were classic symptoms.
But had we caught the infection in time?
I looked up the diagnosis on the Internet. Caught and treated early, it is rarely fatal these days, thanks to antibiotics. But there can be serious complications. I found a Web site about a 17-year-old athlete in St. Louis named Justin Rodgers who was killed by Lemierre's earlier in the year. It took doctors more than a week to take his symptoms seriously, and in that time the infection attacked his lungs. He died three weeks later. The photos of him on the Web site skipped from a shot of a handsome, strong young man playing polo to photos of his comatose body sprawled on a hospital bed, hooked up to machines.
"It's going to get worse before it gets better," Ehrlich warned me.
Sarah sensed our uncertainty. She hadn't spoken much since she got really sick, but she asked a doctor shortly after her diagnosis what was going to happen. "I read 'Death Be Not Proud' this summer," she said. "Am I going to die?"
One of the most deadly things about infections is how quickly they can spread. E. coli can double in less than 30 minutes, and while nobody knew the rate at which the fusobacteria were multiplying in Sarah's blood, it was obvious from her X-rays that they moved fast. In the two days between the first set of X-rays Ehrlich ordered, looking for lymphoma, and the second set taken at the hospital, a white cloud indicating infection and fluids had taken over most of her lungs. Her breathing was choked not only by nodules from the clot, but by her body's inflammatory response.
She was "third spacing," which means that her overstressed veins were leaking fluids all over the place. Nurses were constantly giving her bags and bags of IV nutrition and medicine and painkillers, but she wasn't drinking or eating or hardly urinating. She had gained nearly 9 pounds since admission, but it was all trapped fluids. Her face was puffy, and her abdomen swollen. All of this also made breathing difficult. When she tried to sit up or swivel out of bed onto the commode, her heart rate and respirations jumped, her oxygen levels fell, and she coughed and gasped for breath. She needed extra oxygen pumped into her nose, and albumin, a protein to help with absorption and shock, added to her IV. At night, I lay awake watching the red and blue zigzags on the screen of the telemetry machine that monitored her vital signs.
But then on Friday night - three days after admission - I told some of her friends they could visit. And something amazing happened. Suddenly Sarah was sitting up in bed smiling and talking while the girls braided her hair and filled her in on all the gossip. The nurse let the kids raid the refrigerator for Popsicles and graham crackers, and seeing what good medicine the teens were, she also let them stay past visiting hours. Sabrina Wagner told me later that the kind of transformation we saw in Sarah that night is typical of pediatric patients - and one of the reasons she loves working with them. They are unpredictable and resilient.
That weekend was a turning point. Sarah went from throwing up blood clots and green gastric juices to eating a bag of Sour Patch Kids. Her blood work improved. Clearly the antibiotics were the reinforcements her body needed. When Sarah complained it was unfair she had such a freaky illness and had to miss the first weeks of her junior year of high school, I was secretly relieved. Before she had been too sick to complain. I knew I was getting my dramatic teenager back when, frustrated by all the tubes that made going to the bathroom by herself impossible, she declared, "I can't do this anymore! I want it to end! I want to die!"
Sarah's nurses and doctors started describing a different kid in her medical chart. The numbers she gave when asked to rate her pain on a 1 to 10 scale were sometimes 3s and 4s instead of just 7s, 8s and 9s. "Interactive, making jokes," they wrote in their notes. "I didn't realize how much she wasn't herself until she became herself, and starting answering our questions with spunk and attitude," Jen Mosher said later.
On Sept. 11, after 10 days in the hospital, Sarah was discharged.
Before we left, I had to practice giving IV treatments to a plastic model called Chester. Her first days at home, I gave Sarah antibiotic infusions every six hours. We had one scare a few days after discharge when she became violently ill and started exhibiting all the symptoms of Lemierre's. It landed her in the hospital again, but luckily turned out to be only an infection in her IV line.
Sarah still has the blood clot in her neck. She gives herself heparin shots twice a day in her stomach, which is covered with bruises. She likes to gross out her friends by letting them watch. We hope the shots will get rid of the clot, but nobody really knows for sure. Lemierre's is such a rare disease, and there are few studies on how to treat it. And nobody official is keeping track of it, unless you count Justin Rodgers' sister, Tammy Valencia, in Missouri, who has started a Web site to honor his memory and spread awareness about the return of what many experts call "the forgotten disease."
Just last month, the Centers for Disease Control started looking into reports that the illness is sneaking back. DeMuri says he knows of at least two other cases of Lemierre's in Madison this year, compared to just a few over the last 16 years. Whether this is just a statistical blip or evidence of a true comeback remains to be seen.
One of the local cases involved a 17-year-old wrestler from LaFollette High School who got sick not long after Sarah was discharged from the hospital. It took a week of visits to doctors, clinics and an emergency room before Terrance Barmore was hospitalized at Meriter and diagnosed with Lemierre's. He is doing fine now. In an effort to increase local awareness, some of those who treated Sarah and Terrance are using these cases to teach others to recognize when a sore throat is not just a sore throat. When there is a zebra in the emergency room, as DeMuri would say. "Lemierre's is so easy to miss, but now it will pop into my mind quickly as a possibility." Jen Mosher said. "Sarah will be with me forever."
Sarah won't forget all of the people who took care of her, either. Thanks to them, we tracked down a killer. "I'm so happy I'm back!" she said when she got home from the hospital. Me, too. Even if we're bickering about curfew again.