… Not The Motorcycle

Among all the stories and articles dealing with Covid-19 there are sprinkled individual reports of a disease in young children who’ve developed inflammation of blood vessels in locations all around the body. Some of these kids have died of heart-related problems, and many of them are Covid-19 positive.

There is mention of the disease Kawasaki syndrome in some of these articles, because of many similarities between the two conditions. The pic below is of a child with Kawasaki syndrome, and he is obviously displeased with his diagnosis.

This disease was first described in 1967 in Japan, later spread across the globe, and as of today we still don’t know its cause. Which is what is so intriguing about the newer syndrome and its relationship to a specific virus.

I have a Kawasaki story. Well, two of them, really.

The first one is very short. My first motorcycle was a 400cc Kawasaki street bike. I loved it and would have kept it forever but one day I saw a Honda Gold Wing on the dealership sales floor and she twisted my mind and I followed her and left that marvelous KZ400 behind. I wonder whatever became of her.

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The second story follows.

I was living and working in the Upper Peninsula of Michigan when I first read about Kawasaki syndrome. That was in 1974, and the article was in the journal Pediatrics. Part of the disease’s presentation was a fairly dramatic rash, and the idea that babies with this condition were dying of “heart attacks” was alarming. I looked at the color plates accompanying the reports, and mentally filed them away.

Initially all of the cases were in Japan, but then there were reports from the Philippines, later Hawaii, and finally in 1976 there were kids on the west coast with the disease. It had traveled and been tracked making its way across the Pacific Ocean, and behaved much as an infection would.

And then there came a day in 1977 when I walked into an exam room in my office in remote Hancock MI and saw a child who had presented with a florid skin rash and a fever. My first reaction was “What the hell is this? I’ve not seen anything like … wait … yes, I have seen that rash somewhere before.” So I dug out the articles and yep, this child turned out to be the first case of Kawasaki syndrome diagnosed in the Upper Peninsula, sitting right there in a small room in the middle of the continent.*

By then several things were known about the syndrome. First, that unless you had heart involvement you were going to be miserable for a week or two but would most likely make a complete recovery. And second, if the vessels in the patient’s heart were inflamed, treatment with intravenous gamma globulin was very effective in reversing these lesions.

Since the UP was a pediatric cardiologic desert, I immediately sent the boy downstate to Ann Arbor, where they found that his heart was involved and the child was soon treated with IVGG, with a happy outcome.

So I will watch the present situation with both old and new interest.

*[This story is not told to point out how wonderful a clinician I was, although that is certainly true and I will be the first to admit it, but that the information necessary to make such a diagnosis was available to physicians even in remote and unlikely locations.]

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When I was a pediatric resident, on Saturday mornings we attended Dr. Good’s Rounds. Dr. Robert A. Good was one of the few true geniuses that I’ve met, and each week we would present him a case as an unknown and try to stump him. We never did.

On one occasion, after the resident had laid out the clinical aspects of a case, Dr. Good began: “Well, the most interesting part of this child’s problems is the presence of those vascular lesions …” and he stopped to look around at our group. He began again: “By the way, you do know that eventually all diseases will be found to be infectious in origin, don’t you?” We nodded dutifully, even though of course we’d never heard such a statement before.

Kawasaki syndrome reeks of being infectious in origin, and the recent Covid experience only adds to that odor. So why haven’t we found the cause of Kawasaki syndrome after all this time? Because we need a new flashlight, obviously.

Let’s say that at some time in your life you went camping. Exactly an hour after everyone has settled down and is sleeping, you wake to find that you require the sort of comfort that a privy can provide. You reluctantly leave your warm sleeping bag and turn on that little easy-to-carry flashlight with the anemic amount of light that it provides and make your way to the outhouse, tripping over every root and rock in the path because you can’t see them clearly.

But you learned something from that negative experience.

So when you return to civilization, you make a trip to a hardware store and buy the biggest, brightest flashlight you can afford, and the next time you make that chilly trip to the toilet you see everything. The roots, the rocks, the raccoons, something ominous that slithered away into the underbrush – it’s all out there. Technological progress has improved your life.

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It’s the same in medicine.

As a junior medical student I was required to attend a series of lectures on physical diagnosis. We were learning the art of eliciting information through touching patients’ bodies, listening to their hearts and lungs, and asking them to perform certain tasks. All terribly important stuff. Skills and knowledge that had been basically unchanged for a hundred years.

One day, after an hour of talking to us about stethoscopes and the alteration of the sounds you hear caused by diseases of the lungs, and of the art of percussion of the chest (the tapping while listening), the lecturing pulmonologist paused.

“Now,” he said, “I must tell you that everything I have talked about so far this morning is not worth the diagnostic value of a single chest x-ray.” And he closed his notebook and left the room.

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The discovery of x-rays was a revolutionary thing. It was the newest and best flashlight of 1913. But what amazing ones were to follow – the CT scan, brain scan, PET scan, MRI, etc. And that was just in the radiology department.

Each time there is a technical advance, we learn new stuff. Not just about what we might have been studying at the time, but other things as well. We’re unfortunately accustomed to the term collateral damage, as when a weapon kills innocents along with the intended target.

Well, there is such a thing as collateral learning as well. This occurs when a tool is developed and all of a sudden uses are found for it far from the original plans.** As an example, all of the diagnostic testing now being done for Covid-19 uses technology that didn’t exist when I started out in medicine.

Each time we get one of those new flashlights, people begin immediately shining them everywhere and oh, what things we learn. But so far, no new beam to shine on Kawasaki syndrome, not yet.

**[Steve Jobs was particularly conscious of this phenomenon. When he first presented the iPhone, he knew that it was a remarkable technical achievement, but that neither he nor anyone else could know how it would eventually be used. That was where we came in. And who could have imagined how useful it has become?]

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BTW, as long as we’re on the subject, you do know what the world’s most powerful diagnostic instrument is, don’t you? And it’s been around for a thousand years?

It’s the retrospectoscope. With it you can look backward in time, and declare “I knew it all along!” to everyone who is within earshot and you believe may be susceptible to your unmerited self-praise.

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