Tip:
Highlight text to annotate it
X
NARRATION 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 ů At that point I'd never really heard much ů 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.