Kawasaki syndrome

by liberal japonicus

This is something I wrote for a local group, with some additions and changes for here. The sotry about my daughter is almost 20 years ago, so everyone is fine.

Perhaps people have been hearing about COVID causing Kawasaki syndrome (though most of the stories now list it as a mysterious inflammatory ailment that is similar to Kawasaki syndrome or have given it the name pediatric inflammatory multisystem syndrome (PIMS)) As my daughter had Kawasaki syndrome about 18 years ago, I thought that I would relate my experience.

So, we were going to the states to take my oldest daughter to see my parents. We agreed to meet in LA and go to Disneyland. Because of timing, we got hotel near the airport with my folks and then I would drive a rental car to Anaheim Sunday morning.

Getting together was magical and it looked like everything was going well. What was astonishing was that I could hear my daughter correcting her English in response to my mom. She was a huge Go Fish fan and would always say the numbers as singular (Give me your six) My wife and I didn't correct her, just modeled the correct one in playing the game and she never seemed to notice. But after the first game with my mom, she clued into her saying plurals and immediately started producing them.

Anyway, we went to bed with dreams of Mickey and Snow White. But when we woke up, my daughter said that her neck hurt and my mom, who was a nurse, noted that her lymph nodes were swollen (symptom 1), so we went to an emergency room near the airport. It was packed, with people who didn’t have insurance but couldn’t afford to take a day off. She was running a fever (symptom 2) and we filled out forms etc. Unfortunately, we had a long wait, so long that she became dehydrated and they had to try multiple times before we realized she was dehydrated. Her lymph nodes were getting worse, and so they recommended we take her by ambulance to a hospital with a pediatric department and an ICU.

Her condition worsened, they would administer aspirin to reduce her fever, which would climb back up and so they put her into the ICU. My daughter stabilized, though began having swelling (symptom 3), though I don’t think the doctors picked up on it as much as we did (and we were even shocked when we say pictures, not realizing how different she looked, and my advice, ghoulish as it may seem, is that if you have a child who is ill, take pictures so it is easier to show doctors what the progression is). They said that she was stable, they didn’t know what to do, and we didn’t either. So we went to our hotel by Disneyland and settled in. After a few days, her temp went thru the roof and we were back in the hospital, but fortunately, a 4th symptom appeared, the ‘strawberry’ tongue. Kawasaki syndrome does not have a test, and can only be clinically diagnosed by the appearance of symptoms (other symptoms are cracking of the lips, rash on the trunk, red eyes (conjunctival injection)) It is especially difficult to diagnose in infants and if patients exhibit 3 of the criteria with a fever, treatment is often recommended.

It’s called a syndrome rather than a disease because no one knows what causes it. I have not kept up with the research, but there is a very strong genetic component, with Japanese and Korean children falling ill much more often, which is one reason why the US doctors did not pick it up quickly. The treatment is intraveneous immune globulin (IVIG), the collected antibodies from blood plasma, which can quickly deal with it. The primary danger of Kawasaki syndrome is the risk of acquired heart disease, so it is important to treat the condition quickly. This parallels with discussions about how COVID damages a number of organs, leaving the patient breathless even after recovery.

I often relate this story because of the genetic component, and people in mixed marriages here in Japan with kids, if they get it when they are back home, the overseas doctors will not be familiar with it and lose valuable time in treating it.

Here are some article, This article in the Lancet points to the occurence in Italy, while this article discusses the condition in the UK and points out that the timing (relatively late in the pandemic wave) suggests that it may be an immune reaction to COVID, also noting that it could provide vital information in vaccine studies. The Beeb has a good general article which has this

most of the children tested negative for coronavirus, but tested positive for detection of antibodies.

"So we really think that the biology of the disease, somehow involves an unusual immune response to the virus," he said.

However Prof Levin said there was "a vast amount to learn" about the reaction, which had only been known about for two to three weeks.

Children appear to be affected up to six weeks after they have been infected with the virus, which could explain the appearance of the new syndrome several weeks after the peak of UK cases.

And the NYTimes article also points out that it is worse, in that IVIG has to be supplemented with steroids. And I found this preprint interesting because the cohort of children studied is 11 patients, 9 of which are male, which also corresponds to observations about Kawasaki syndrome.

Anyway, a post, please feel free to write about information you've heard about Kawasaki, and I'd ask that we keep politics out.

Comments

[Tony P.]

lj,

It sounds like your daughter and my niece may have gone through the same ordeal at roughly the same time and place.

My 23-year-old niece had Kawasaki when she was 3 or 4 in Santa Barbara. I was not there; I'll have to check with my sister for more details. But one thing I do remember is my sister telling me about an episode in the hospital: the doctors had my niece on some medication which they decided to stop or change; the nurse, on coming in to chuck the remainder of the original in the trash, said something like "Well, there goes $10K". Could have been IVIG, I suppose, or maybe the replacement was.

I know it took a day or two for the docs to decide it was Kawasaki. And I know they said the main concern once the crisis passed was the possibility of heart disease in the future.

So far, my niece has had no heart issues. She was a strenuous dancer (ballet and hip-hop) through high school and college, rising to captain of the BU Dance Team (NCAA champions; there's an actual tournament in Florida every year) and thus the most physically fit relative I have.

I can't help adding: my brother-in-law was a young Assistant Professor of Chemical Engineering at UCSB at the time, with presumably excellent health insurance, for I don't remember the cost of my niece's treatment having been an issue.

May your daughter and my niece both live long and prosper,

--TP

[John Thullen]

May all daughters, and sons, be healthy and live long lives.

And provided gold-plated healthcare.

[JanieM]

Thanks for such a detailed account, lj.

It's not the same thing, but a story of dealing with the health care system in relation to a mysterious illness reminds me of some of the ins and outs of my mother's ailments and treatments over these last few months -- lots of confusion, utterly fragmented strands of care, non-optimum dealings (on the part of generally kind and well-intentioned providers) with someone who was almost completely blind, deaf (tho with hearing aids), and getting increasingly unable to process information. The system is a nightmare. (These impressions are mostly from my sister, who had the most direct involvement and is a retired medical professional herself.)

I'm also reminded of the long-term challenges faced by someone I know with chronic pain issues. This is an excellent book about dealing with some of these challenges when faced with your own health care decisions, but it's hard to put the recommendations into practice without the help of a very strong advocate who isn't addled with pain/sickness at the moment. (As I know from direct experience.)

Glad to know your daughter, and TP's niece, went onward with no long-term consequences.

[liberal japonicus]

Tony, curious (if you don't mind) but any Japanese or Korean ancestry for your niece?

Janie, I cut out a big chunk of writing about dealing with the health insurance, though that experience has me pretty cynical about how the US handles health care.

[JanieM]

lj, I'm glad you left the health insurance side of it out of this one. The health care system is complicated enough--partly because of the insurance system, of course, though not entirely--and an interesting/rich enough topic in its own right.

Anyhow, if you'd made it about insurance too, you could hardly get away with saying "no politics in this thread." ;-)

I don't know how many times during this past half-year with my mom's situation I found myself saying, "I don't know what people do who don't have a Vicky in their family" (stipulating that my sister's name isn't really Vicky).

She could talk on an equal footing with the people treating my mom, she's knowledgeable, competent, smart, practical, and able to hold her own and insist on stuff she thinks is important. And all those qualities were needed, like when records didn't get where they were going and tests were about to be repeated needlessly, specialists weren't talking to each other, there was a different hospitalist from the one two weeks before, decisions had to be made that my mom wasn't really mentally competent to make, and so on.

I'm trying to think about how all that is being affected by the COVID-19 crisis, but the mind quails.

[Tony P.]

lj,

None.

Well, I can vouch for 3 generations back; her grandparents could vouch for 3-4 generations before that; and it's extremely unlikely for umpteen generations before that, because Alexander the Great's army only made it as far as Afghanistan.

But Greeks have been sailors since before they were Greek and it's not absolutely impossible that one of her remote ancestors, serving on some Ottoman or Byzantine ship perhaps, had commerce with some Japanese or Korean woman in some exotic port. It would take a pretty far-fetched scenario on top of that to introduce Japanese or Korean "blood" into my niece's lineage.

On the other hand, I would have thought it far-fetched, when I was in Tokyo in 1987, to have to choose between two Greek restaurants for a night out with my Japanese friends. The place we chose, which was so "authentically" Greek that it had boxes of plates specifically for smashing stacked up in the back corner, turned out to belong to a Japanese retired merchant mariner who had fallen in love with Greek culture on his travels.

Also, having recently watched a 1962 Greek movie that included a fleeting scene in a Japanese restaurant in Athens, I can imagine that Greeks and Japanese have not been as entirely isolated from each other as one might think.

Still, despite these musings, I can say with high confidence that my niece has no claim to Japanese or Korean heritage in any ordinary sense.

--TP

[wj]

Unexpected Asians tucked away in a European ancestry is far less likely than the odd European hidden in a Japanese family tree. Which would likely only surface when a mixed marriage (probably in the US) turned up some kids with blue eyes. That's when you realize that, pre-Sakoku (say early 1600s) some Scandinavian sailor on shore leave in Nagasaki....