Kawasaki Disease Video

Kawasaki Disease Video

It’s an infectious disease that literally breaks the hearts of thousands of children worldwide, but we know surprisingly little about it, not even its cause.

Dr Michael Cheung
What the infection is? Don’t know. Why some people are more prone to it? Don’t know. And why people react to it and have a lot of damage to the coronary arteries, we don’t really know.

Dr Jane Burns
Kawasaki disease is a mystery that has grabbed the imagination of paediatricians all over the world.

NARRATION
Medical research races to keep up as it spreads to every continent, and in many countries, it’s on the rise.

Dr David Burgner
And fascinatingly it is increasing with rapid industrialisation. So as countries move from a rural to an industrial economy, there seems to be an explosion, if you like, of Kawasaki disease.

NARRATION
It was first diagnosed in Japan by Dr Tomisaku Kawasaki in 1967. Now 88, Dr Kawasaki still hopes to reveal the secrets of the disease that bears his name.

Mark Horstman
After more than forty years of research, Kawasaki disease remains a mystery. We just don’t know if it’s a virus, or bacteria, or something else. But we do know it crosses oceans to be the most common cause of childhood heart disease in the developed world, including Australia.

Boy
Yes! Ooh!

NARRATION
It’s hard to believe that a few months ago, five-year-old Josh was gripped by pain and fever. After a week of getting worse, his mum rushed him to hospital.

Luisa Henderson
By then I had to carry him, because he couldn’t walk, and his eyes were bloodshot, and he still had a temperature of, it was now thirty-nine point something. They admitted him straight away.

NARRATION
Josh is one of two hundred cases of Kawasaki disease recorded in Australia each year – mostly children less than five years old. If undetected in childhood, Kawasaki can lead to heart disease later in life.

Dr David Burgner
It’s more common than meningococcal disease, and children with Kawasaki disease have very high fevers, up to forty degrees, and the fevers go on for days. So you need to have a fever for four or five days before you can actually make the diagnosis. They often have a bright red rash, red lips, red eyes – bloodshot eyes, swollen and painful hands and feet. And the older children will have a swollen gland in the neck, often.

NARRATION
When the visible symptoms have faded away, the real worry is left hidden here, around the heart. Ironically it’s the body’s own defence system that does the damage. The disease attacks the coronary arteries that supply blood to the heart muscle by triggering an overreaction of the immune system. Cells of the immune system invade and inflame the artery walls. How to stop the damage? Dampen down the immune response, as quickly as possible.

Dr David Burgner
The only proven treatment for Kawasaki disease at the moment is immunoglobulin, which is antibody taken from blood donors, so it’s the sort of clear part of the blood, not the red part of the blood.

NARRATION
Used within ten days of the infection, the immunoglobulin dramatically reduces the risk of heart damage and makes the fever disappear.

Luisa Henderson
And that’s exactly what happened, they treated him and within twenty-four hours he was a different child. His bloodshot eyes had almost gone and his temperature had come right down to normal.

NARRATION
But that’s not the end of hospitals for kids sick with Kawasaki disease. The next visit is an echocardiogram, an ultrasound to check for swollen arteries called aneurysms.

Dr Michael Cheung
The goal of the treatment is to protect the heart. We don’t want the coronary arteries damaged, we want the heart muscle to be working as well as possible for a long life.

NARRATION
In five per cent of cases, the treatment fails. This is an x-ray image of an aneurysm in a child, the potentially fatal impact of Kawasaki disease.

Dr Michael Cheung
Yeah, unfortunately this one was probably the worst one that we’ve seen here for many years. And what you can see here is a very enlarged coronary artery through that section over a long distance. It’s a bit like a river, if you’ve got a river bend, and a bit of a, a bank there, then the water swirls around it. Little clots may form there, and if those little clots then go out into the periphery and into these smaller blood vessels, it may cause temporary blockage of the blood supply, and cause the heart muscle to suffer because of that.

NARRATION
And that can require surgery, something that Cameron Mates and his mother Shirley know all too well.

Mark Horstman
Here you are with heart man.

Shirley Mates
Hearty Heart, they call him.

NARRATION
Back in 1994, Cameron caught Kawasaki disease and spent his seventh birthday in hospital.

Shirley Mates
Out of that he had this massive aneurysm in his heart, which … At that point I’d never really heard much … you don’t sort of associate heart disease with children, really.

Mark Horstman
What’s that big black ball there?

Cameron Mates
That’s the aneurysm that you can see, and a typical coronary artery is three to four millimetres, and mine is eighteen millimetres.

Mark Horstman
So it’s really swollen up?

Cameron Mates
Yes, it is. And eight millimetres is what they consider as a giant one.

Mark Horstman
What do they call yours?

Cameron Mates
A humongous one.

NARRATION
By the time he was fourteen, young Cameron needed an adult-sized coronary bypass operation to save his life.

Dr David Burgner
I’m particularly interested in why some kids get sick and others don’t, when really kids are exposed to the same bugs and triggers all the time.

Shirley Mates
I think there’s a lot more out there, probably years and years ago there was a lot more that’s just never been diagnosed.

Mark Horstman
It looks like whatever causes Kawasaki disease is spread throughout the population. So the question is this – why do so few people catch it, when so many are exposed?

Dr David Burgner
There’s good evidence that genetics plays a major role in deciding or determining who gets Kawasaki disease. For example, we know that Kawasaki disease is twenty times commoner in the Japanese than it is in Caucasians in Europe and in Australia. But when Japanese families move to the US, which is a low-incidence country, their children have an incidence that is as high as it is in Japan, in fact it’s slightly higher.

Mark Horstman
Now there’s a surprising twist in this story. New research has found not the cause of Kawasaki disease, but perhaps what it’s carried by – the wind.

Dr Jane Burns
There are no examples that I know of in human medicine where there has been trans-oceanic transport of a human pathogen carried by dust particles on the wind.

NARRATION
Dr Jane Burns is speaking at the international conference on Kawasaki disease. What she has to say in a lecture called ‘Blowing in the Wind’ stuns the medical community.

Dr Jane Burns
This will be a revolutionary new concept in transmission of human disease.

NARRATION
Over the last forty years, when north-westerly winds blow over Japan, there are spikes in Kawasaki cases. That’s remarkable in itself, but when the winds from Asia connect with winds crossing the North Pacific, there are epidemics on both sides of the ocean at the same time.

Dr Jane Burns
If it’s something that lofts up into these wind currents over the plains of Central Asia, and then can drop out of the sky on children in Japan, but then can also follow these wind portals across the Pacific Ocean, and then rain down on children here in the United States, and particularly here in Southern California, then it must have some kind of particle mass.

NARRATION
Atmospheric scientists are flying high-tech sampling instruments above Japan in the hope of isolating what those particles might be.

Dr Jane Burns
If it’s in fact true that infectious agents can move around the planet on dust particles, that would also open up the possibility that anthrax or influenza or other infectious agents might also move around the planet that way too.

NARRATION
This is a detective story with plenty of suspects. Dave Burgner has a hunch of his own.

Dr David Burgner
I think it’s more than one bug can act as the same trigger, and actually I suspect there might be two bugs acting at once.

NARRATION
Research continues, but the answer to this mystery is elusive. Whatever the cause, getting the correct diagnosis as quickly as possible is crucial.

Dr David Burgner
And if you get fobbed off by your doctor or your emergency department and they haven’t thought about it, as the fever gets beyond four or five days, just get them to consider Kawasaki disease.

NARRATION
In the shifting winds of a changing world, a child’s heart could depend on it.