Will, the intern assigned to Fast Track, was looking puzzled. Fast Track is where the worried-but-well in the emergency room are sent so that they can be quickly seen and treated.
"I dunno…” Will said.
"You dunno what?" Checking the "to be seen" rack, I leaned past Will and saw that, as always, he was way behind. Charts were stacked everywhere. He was the slowest intern we had.
"It's not right…" Will told me.
"Will," I replied pointedly, "you've got patients to see."
He persisted: "Larry told me to discharge this kid, and it's just not right." Larry was the attending physician. "This kid is sick."
"Will, move." He looked down, frowning.
"Okay, show me."
Together we walked to room 4. A boy of about 5 lay in bed with his worried-looking mother sitting beside him.
"Well, well," I said in a jolly tone. "Howdy, partner. How's it going?"
The boy didn't seem to have the strength to look up at me. His eyes were "injected"—pinkeye-looking. His lips were lipstick-red and chapped.
This kid was sick. "What's the story here?" I asked.
"He has a fever," his mother said.
"For how long?"
"Five days. We were here yesterday. They said he was okay, but the fever hasn't gone away."
I looked down at the boy's hands. They looked like little sausages—red, swollen, covered with a rash. I peeked into each ear—nothing but earwax.
"Say ahh…" His throat was parched and beefy red, and his tongue was swollen. Pikes. I went on mechanically to his neck. He had multiple enlarged lymph nodes—"shotty nodes" (as in "buckshot") is how we describe it. Shotty nodes don't point toward any particular disease. Most sick kids have clusters of swollen nodes.
Fever, ruby-red lips … lots of lymph nodes… Something was bubbling up, something too amorphous to name, something bad. I pulled the warm white sheet down and surveyed the boy's naked chest and belly. There it was: a "desquamating rash," peeling red skin where summer swim trunks would pinch. I went back to the swollen little red fingers. Mentally, I ran down what I was seeing: red eyes, red hands, a strawberry tongue, a peeling rash. What, what, what? I dug my stethoscope out of a pocket and donned it. Lungs clear. No heart murmur.
I slipped for a moment into some ER lecture in a long-ago classroom. I could hear the slide projector whirring, and I could even see, projected in cobalt blue, a slide featuring a list of clinical signs—this boy's signs—on the white screen in front of us tired residents. But I couldn't make out the diagnosis. Thinking fever, fever, fever, I looked back at the boy—and then it came, one of medicine's many laundry lists col-lapsed down to a single diagnosis.
Kawasaki disease! It had to be. During my training we residents were lectured to death on Kawasaki disease, even though it is rare—about 3,500 cases a year in the United States. I had learned the list: fever, red eyes, shotty lymph nodes, exanthem (skin rash), enanthem (mouth rash).
Will cleared his throat, and I looked up out of my reverie into the mother's pale, worried face.
"So," she said, doubtfully, hopefully, "I can take him home?" Hopeful be-cause going home meant he wasn't that sick, doubtful because she knew better.
"Kawasaki!" I whispered to Will and gave it a moment to sink in. I could see him walk through that list of signs. "Kawasaki!" he echoed.
"Just a second," I told the mother. "If you could give us a moment?' I backed Will out the door.
The fact was that after coming up with those five signs I had pretty much exhausted my knowledge base. We needed to know more.
As recently as a year ago I would have pulled out a tattered, out-of-date text on emergency medicine. This time I went straight to the computer. Within a couple of clicks I had the world's literature on Kawasaki disease.
The syndrome is a disease of childhood, most often seen in children younger than 5. It occurs sporadically all over the world, but its highest incidence is in Japanese children. The key feature of the syndrome is a severe, un-remitting inflammation of the blood vessels, probably caused by an overreactive immune response. But what revs up the immune system so much?
Nobody knows for sure. Researchers propose the culprit is a "super-antigen" that provokes a far more ferocious immune response than, say, your simple, garden-variety influenza virus. The literature suggests several possible risk factors, including Japanese descent, infections caused by retroviruses, high socioeconomic status (a rare disease-trait), or residence near a body of water.
Early deaths are from heart inflammation, and 5 percent of untreated patients die. In untreated survivors, the inflammation can cause ballooning coronary blood vessels, called aneurysms, and scarring of the coronary arteries. The result: a 6-year-old kid with a heart attack.
If treated early, most children with the disease recover with minimal aftereffects. Treatment involves infusions of gamma globulin (antigen-fighting material from human serum) and humble aspirin. The gamma globulin is just a dose or two, and a 2-gram-per-kilogram injection works wonders. A high dose of aspirin is given daily until the patient's fever goes down; then a lower dose is given over weeks or months, de-pending on symptoms. Both gamma globulin and aspirin block the damaging effects of an overactive immune sys-tem. Simple and effective treatment—as long as you nail the diagnosis.
And how do you do that? There aren't any tests, no million-dollar work-up. The diagnosis is strictly clinical. You have to hang your hat on the five signs. And, no question, they were all there in room 4.
We called the pediatric referral hospital; Will talked to the on-call resident, who panicked.
"They say they want him medevac-ed," Will said.
"Well," I said, startled. "It's not exactly as if seconds count here."
"Oh, great," Daphne, the charge nurse, grumbled. "Now we are medevac-ing patients out of Fast Track."
I sat in front of the computer screen, studying the expert consensus on what this boy's future held. With treatment, the risk of severe complications is less than 1 percent. Still, the weeks he faced in the hospital would be filled with fevers, chills, achy joints, sleepless nights, blood draws and IV sticks, X rays, scans. I thought of myself as a child and hoped for a moment of good. Perhaps this little boy would someday remember this as "the day I got to ride in a helicopter."