It was a hopping, breakneck-paced Sunday evening in the emergency room. I had a patient’s chart in my hands and a resident next to me, telling me her findings. He has a sore throat, she said. I lifted my pen, ready to cosign the chart, authorize antibiotics, and move on to the next patient. But something in her voice stopped me.
What is it? I asked, immediately alert. Susan was one of the best residents in the hospital, with the sobering knack of usually being right when we disagreed on a diagnosis. If she was concerned, so was I.
I’m not sure, she said slowly, frowning. I think it’s just a sore throat, but he has an odd, swollen lymph node under his chin. She shook her head and shrugged. I don’t know what to make of it. Could you take a look?
I said I would, but before going over I asked a few quick questions: Any fever? Pus on the tonsils?
Temp’s 99.1. Tonsils are clean, she replied.
Is the node soft like an abscess or hard like a tumor?
Neither. Just firm, and a little sore when you press on it.
The information was helpful, but I was really just buying time to think, the way all attending physicians do when smart young residents drop dilemmas on their doorstep.
Mr. Larma was sitting against one of the ER walls, his chair jutting halfway into a bustling hallway. Amid the controlled chaos of the emergency room, we didn’t have the time or the space to give everyone a leisurely, secluded exam--especially if all you had wrong with you was a sore throat.
Even though he’d been waiting several hours, Mr. Larma smiled warmly as I walked over to shake his hand. He had a shiny bald pate, a stocky build, and the bluff, expansive manner of an Italian paterfamilias. If I hadn’t seen his chart, I would have put his age at 60, not 71.
Hello, Mr. Larma, I said, then nodded toward Susan. I know you’ve already told Dr. Chen here some of what the problem is, but you know how we doctors are--we always need to hear it from the horse’s mouth.
Mr. Larma opened his mouth to speak, swallowed with a sharp grimace, and then answered, It’s the throat, Doc. The words were muffled, as if his vocal cords had been wrapped in cotton. Gingerly, he raised a meaty hand to his Adam’s apple. She’s pretty sore.
That tone of voice. Ten fire engines roaring through the ER, sirens clanging, couldn’t have caught my attention as quickly. I had last heard that unique, muffled sound eight years before. But it reverberated as if I’d heard it yesterday.
Deliberately, I asked him the next question.
Is this the worst sore throat you’ve ever had?
Absolutely, Doc. No doubt about it. Again that halting, almost disembodied sound.
His daughter, a fully coiffed but remarkably similar copy of her father, winked at him before piping up. He loves to talk, doctor, but he’s barely said a word all day.
When did the pain start?
Early this morning, she answered for him.
It was now at least 12 hours later, and if my gut instinct was right, we might not have many more to fool around with. I turned to Susan and said, mildly, Let’s have a chat. Then I looked at father and daughter. We’ll be back in a minute, okay?
Mr. Larma nodded appreciatively, as if we’d just taken his order for a gourmet dinner. His daughter sent us off with a cheery You just take all the time you need, doctors.
Susan gave me an expectant look. I didn’t mince words.
I think he has epiglottitis.
The epiglottis is perched midthroat, at the root of the tongue, where the trachea (which carries air to the lungs) branches off from the esophagus (which carries food to the stomach). It combines the beauty of an orchid with the prickliness of a Venus flytrap, and its job is to protect the trachea; it’s a white petal of cartilage that lifts to allow air--and only air--to flow past. Routinely, it opens and closes thousands of times a day, snapping shut with each swallow to keep the delicate lungs free of any food, saliva, or microscopic aggressors you might swallow. It’s the reason you can’t drink and breathe at the same time. But sometimes the protector itself gets attacked by some nasty bacteria, and the resultant swelling-- which we call epiglottitis--can completely close off the windpipe and kill a healthy adult in a matter of hours.
After hearing my diagnosis, Susan looked puzzled, then dubious. You really think so? she asked. At his age? The disease, as we both knew, most often strikes children between the ages of two and five, though it can be found in any age group. And what about the lymph node? That’s not a symptom of epiglottitis. There’s no fever, and he has no trouble breathing. Shouldn’t he, if he really has epiglottitis?
Susan was my favorite devil’s advocate, and I figured that if I could convince her, I could convince a consultant--something I’d eventually have to do if I was right, since the tests to confirm a diagnosis of epiglottitis can be performed only by a specialist.
I agree that there’s not much to go on, I began. But he’s having a lot of pain and it’s not his tonsils--they’re not even red--so it must be coming from farther down. And sure, the hoarseness could be due to simple laryngitis, but then it shouldn’t hurt so much. I really think we’ve got to make sure it isn’t epiglottitis.
Susan just nodded, slowly. No counterargument--a good sign.
I’ve never seen a case.
You just might have now.
In medical school, our professors used to thunder about the do- not-miss-this-on-peril-of-your-soul diseases. One of the trickiest, and most lethal, is epiglottitis. An adult’s trachea at the level of the epiglottis is less than half an inch in diameter; it doesn’t take much swelling to cut off the airflow in such a narrow pipe. In children it’s even smaller, barely wider than one of their pinkie fingers. Mercifully, the disease is rare. But when it does occur, it can be deadly.
Epiglottitis is treacherous not because it lacks symptoms but because they’re so commonplace. The hallmarks are sore throat and fever: What could be more innocuous? By the time stridor develops--the tight, organ-pipe whoop that’s caused by trying to breathe through a severely narrowed trachea--it’s almost too late. Unless a plastic tube is immediately inserted past the inflamed, swollen cartilage, the trachea will be closed off and air will no longer be able to get to the lungs.
So if Mr. Larma really was suffering from epiglottitis, we had to get moving. I told Susan to take him for a lateral neck X-ray--a view from the side that’s the first step in diagnosing the disease. Then I’ll make some calls and see how hard it is to get hold of an ear-nose-and-throat guy on a Sunday evening, I added with a slight grimace.
While I had told Susan my provisional diagnosis, I didn’t tell her why I felt so certain. I didn’t tell her about the last time I’d heard that timbre of voice. Diagnosis by prior example is a lousy form of medical reasoning; it usually means a doctor doesn’t know enough to consider similar, yet distinct, diagnostic possibilities. But with some prey, the scent never fades.
One memorable night during my internship, contrary to all the laws of medical probability, I’d seen not one but two cases of epiglottitis. It began innocently enough. A worried mother called me early on a Saturday afternoon. Her two-year-old was running a fever and complaining of a sore throat. I asked some routine questions. Was she drinking well? Yes. Any problems breathing? No, no problem. It didn’t seem like an emergency to me, so I told the mother just to keep an eye on her daughter. Four hours later, I called back to check on her. The child just wasn’t herself, said her mother, and the fever was climbing. I met them at the emergency room: a towheaded, strong-limbed child lay flat on her back, eyes half-closed, ominously indifferent to the hubbub swirling around her. She seemed to have been drained of her last drop of energy. A blood count and cultures, spinal tap, and chest X-ray gave me no clues.
Finally I examined her throat. I’ll never know what made me save this routine maneuver for last, but when I did look I was startled. Popping up behind her tongue was a red, beefy epiglottis. It didn’t make sense. Kids with epiglottitis, I’d been told, are usually sitting up and drooling, with their chins tilted forward to fight for air. They don’t lie flat. But even though the case was unusual, it was clear that by putting a tongue depressor down the child’s throat I’d broken a cardinal rule: never, ever, provoke an infected epiglottis. Like a Venus flytrap gone mad, the slightest pressure--even something as innocuous as the touch of a tongue depressor on the back of the throat--can send the epiglottis into an irreversible and instantly fatal spasm.
Feeling both lucky and sheepish, I approached the pediatrics resident. I think I just saw a hot epiglottis, I said. He raised his eyebrows, skeptical, and went to see for himself. Yup, he said, stick in hand, peering into her throat, looks red to me too. Then he flinched. I guess I shouldn’t have done that, he mumbled. Gingerly, as if backing out of a minefield, we tiptoed from the room.
A breathing tube is routinely put down the throat of any child diagnosed with epiglottitis, since the airway is so narrow that any swelling at all will inevitably close it off. So we immediately called the anesthesia team. Within half an hour, the little girl had been put under, intubated, and pumped full of antibiotics. Relieved--and feeling very lucky that misleading symptoms and two impetuous young doctors hadn’t caused any harm--I drove home. There was a message on my answering machine: Call the adult ER. You have a patient with a bad sore throat.
No way, I thought as I climbed back into my car. Not two in one night.
The young woman sitting in the exam room didn’t appear sick. I said hello. As she tried to answer, a beseeching look came over her face just before it contorted in pain. The hello she finally uttered was muffled and throaty. After that, each swallow or syllable brought a fresh spasm of agony, as if a branding iron were pressing on her throat. I carefully looked at her tonsils--no sign of infection. Her temperature was only 100.8, and her lateral neck X-ray showed no obvious thumbprint: normally the epiglottis is a fine, feathery structure in an X-ray, but when swollen it looks thick and pudgy, like a thumb.
I was feeling cautious after my close call earlier in the day, though, so I called the ear-nose-and-throat consultant anyway. Even after a long, painstaking exam with a laryngoscope (a narrow tube that lets a doctor peer beyond the tongue at the epiglottis and vocal cords), he was unsure whether the epiglottis was inflamed. Knowing neither of us would sleep that night if we sent her home, we admitted her to intensive care and gave her broad-spectrum antibiotics.
It was two days before we had proof that we’d done the right thing: that’s when her blood cultures came back positive for Hemophilus influenzae, a bacterium that, despite its confusing name, is responsible not for cases of the flu (which is actually caused by a virus) but for most cases of childhood meningitis and epiglottitis. Happily, by then both she and the little girl (whose blood culture had also come back positive for the bug) were out of danger.
Eight years had passed since that Saturday. In that time I’d treated thousands of sore throats but not another case of epiglottitis-- until now.
Twenty minutes after our somewhat one-sided chat with Mr. Larma, Susan had his lateral neck X-ray in hand. It looked suspicious but not definitive. I didn’t care. My old enemy had never left very clear prints. Mr. Larma needed to be checked with an endoscope--an improved, longer version of a laryngoscope. In a hurry, I called Dr. Robertson, whose name headed the list of ENT specialists on call.
His answering service picked up the phone. He’s on vacation, a disembodied voice told me.
Okay, then who’s covering for him?
I’m not sure. Why don’t you try Dr. Bernstein?
I tried Dr. Bernstein. He asked for a quick description of the case. Despite my best effort to make Mr. Larma’s plight sound dramatic, his only response was, You’re sure it’s epiglottitis?
No, I’m not sure, I answered, beginning to get annoyed. That’s why I want him endoscoped.
Listen, I’d be glad to come in, but I’m right in the middle of dinner, I’m not really on call, and I’m quite a way from the hospital. Why don’t you try Dr. Wallace? He might be closer by.
I tried Dr. Wallace.
Hmm, no fever and no respiratory difficulty? he said. You’re sure it’s epiglottitis?
Once again I went through the whole song and dance.
Tell you what, he said. Why don’t you see if the ENT resident is still in the hospital? He gave me a pager number to try.
I glanced over at Mr. Larma, still sitting quietly in the hallway with his daughter, still looking as if he had all the time in the world. But I knew he didn’t.
No one answered the pager number. Then Dr. Wallace called back.
No, I answered curtly, letting a touch of exasperation seep into my voice.
Okay, listen, he said. I’ll page the senior surgical resident and tell him where to find the endoscope so he can get started. (The endoscope is kept locked up in a special suite, available only to the ENT specialists, surgeons, and gastroenterologists trained to use it.) I’ll be in as soon as I can.
I let Susan know what had happened, and together we continued to wait. It took another half-hour. I attended to three or four more patients but kept a continual watch on Mr. Larma. He seemed to be holding his own. Finally the surgical resident arrived, dressed in scrubs and holding the long-awaited endoscope.
He’s over there, I said.
The three of us whisked Mr. Larma to our surgical room, where the surgeon removed the endoscope from its case. The quarter-inch-thick tube has an outer skin the color and texture of electrical tape. The resident fiddled with some knobs on the handle, and the tip of the tube bent preternaturally, like an alien proboscis straight out of the tavern scene in Star Wars. Mr. Larma seemed unperturbed.
This will feel a bit uncomfortable, sir, the resident explained. If it gets too bad, just say the word and I’ll stop.
You go right ahead, young man, Mr. Larma smiled, coolly eyeing the wriggling nozzle aimed at his nose.
After squirting novocaine jelly in the left nostril, the resident gently pushed the scope in, little by little, then stopped. He gave a low whistle and said, There it is. No doubt about it.
He offered me the eyepiece. I got my anatomical bearings: the base of the tongue, the opening of the esophagus. Then I saw it--half- buried by engorged, cherry-red laryngeal tissue was Mr. Larma’s epiglottis, twice its normal size. I passed the scope to Susan.
I guess now I’ve really seen a case, she said softly.
Luckily, Mr. Larma’s trachea wasn’t dangerously blocked, so we didn’t have to create an airway. Instead we admitted him directly to intensive care and blasted him with antibiotics, all the while keeping an intubation set open at his bedside, just in case.
Thirty-six hours later I paid a visit. Mr. Larma was sitting up in bed, looking genuinely tempted by his hospital breakfast.
Hey, Doc, how ya doin’? he boomed.
A moment of pleasant surprise: it was a completely different voice. Baritone. I’m fine, thanks. So that’s what you really sound like! You had us scratching our heads the other day.
Well, Doc, looks like you hit the nail on the head anyway, he laughed.
The following day I went to drop off an instrument at the ENT office. While there, I ran into the chief of the department, who had taken over Mr. Larma’s case. Since things had turned out so well, I decided not to mention how much easier it had been to convince Susan of the diagnosis than to persuade his colleagues to confirm it. Instead I simply asked him, So what did you think of Mr. Larma?
He became immediately stern, pushing a pair of bifocals up on his nose. Obviously epiglottitis. The whole area was infected. His voice dropped to finger-wagging range. You know, you people down in the ER have to be very careful. It’s terribly easy to miss one of those. Nightmare if you do.
I practically laughed out loud, but stifled it. What could I say? Just that some voices are much more evocative than others.