"She'll be right, mate." That's the favourite saying, isn't it?
"She'll be right." But she's not right.
He looked at me and he goes "Tania, I'm sorry to say
"but you've got this (bleep) disease, this bastard of a disease."
Every year I manage 50 to 60 deaths
of people that I've looked after for months, years, decades.
I want it to stop.
How would I describe it?
A little bit of us dies every day.
This is a bastard of a disease.
Are you ready? One, two, three!
Thanks to the ice-bucket challenge and the courage of sufferers
like AFL legend Neale Daniher...
My vision is a world without MND. I will get there.
I might be six foot under, but we'll get there.
Australians are perhaps more aware than ever
of the horrifying realities of motor neurone disease.
Every twelve hours, one Australian is diagnosed with MND.
Every twelve hours, one Australian dies from MND.
But two important questions remain unanswered.
Why is it killing so many Australians?
And how on earth do we do something about it?
If we understand the environmental factors
that cause motor neurone disease
this gives us potential toe holds into not only prevention
but we potentially can develop therapies
that slow and stop this bastard.
Neurologist Dominic Rowe is perhaps Australia's foremost expert
on motor neurone disease.
This is the cruellest disease imaginable.
It robs you of your ability to walk, to use your hands, to talk,
to swallow, and ultimately it takes away your ability to breathe
and it kills you.
Motor neurone disease is actually more like a collection of diseases
that attack the nerve cells or neurones that control
and coordinate our muscles.
Eventually these motor neurones degenerate and die.
It will kill as many as 800 Australians this year.
Another 800 will be diagnosed.
It attacks you mercilessly.
It's relentless, from its onset to death,
the average survival is around about 36 to 40 months.
That's with the best medical care available.
At the Macquarie University in Sydney,
Professor Rowe cares for more than 200 MND sufferers,
roughly 10% of all Australians living with the disease.
But in recent years, his work increasingly brings him to Griffith
and the NSW Riverina,
a region famous for its farm produce
that's becoming infamous for being home to a disturbing number
of MND cases.
MND in Australia currently affects 1 in 14,000 in the population.
In Griffith we understand that there are 12-13 patients
currently with motor neurone disease.
Given the population, we think that this is
a 2 to 3-fold increased incidence of MND in Griffith alone.
This afternoon we're going to see Mim Pason.
Mim is the widow of Joe, who was one of my patients,
who died just over a year ago.
I'm so glad you're here.
Some people with motor neurone disease, like Joe,
it affects speaking and swallowing first.
It then affects breathing and this is the form of motor neurone disease
that is the most rapidly fatal.
His farm was, you could say, his second love.
Because he...
I mean, he loved his family but the farm was his pride and joy.
I'm sure he's growing oranges up in heaven
because that was his, that was his passion.
Joe was 69 when we noticed that there was something
not right with him.
I'll never forget the day we were told.
It was on the 12th of November 2014.
He said "Well, Doctor, I've got my death sentence."
We walked out of St Vincent's Hospital
and for about an hour Joe didn't utter a word.
He passed away on the 2nd of September,
so that's not even 12 months.
To me, Joe is still there.
Even though I don't see him.
His shoes, his boots that he had on
when he was on the farm that morning,
they're still on the veranda where he left them.
Took them off and that's where they are.
Joe's motor neurone disease was sporadic, out of the blue,
a 1 in 42,000 chance of developing motor neurone disease
that we think was triggered by the environment.
10% of people with motor neurone disease
is caused by inheriting a faulty gene.
But the 900-pound gorilla
is the 90% of patients who have sporadic motor neurone disease.
Well, I never drank in my life and I never smoked in my life.
Maybe I should have.
Albert Brown has been farming in the Riverina for 60 years.
Last November he was diagnosed with sporadic motor neurone disease.
I reckon I've had it for years before that.
I was laying on the bed and the wife says
it was like water flowing across me back, the way it
I had weight comin' off me. Bones, and skinny all the time.
Shouldn't have been happening.
Years ago I'd lump 180-pound bags of wheat, y'know, sort of thing.
But couldn't lift an empty bloody bag now.
But that's about all I know about that motor neurone.
The bastard of a thing.
Albert lives around 90 minutes' drive from Griffith
in the small community of Lake Cargelligo.
This tiny town is well known for its water sports
and is home to a population of around a thousand.
And yet Albert is one of a least four residents
currently living with motor neurone disease.
There's nothing bright about the beast of the disease.
Buggers your way of life, y'know.
As well as Albert, there's local nurse Suzie Fisher.
The nurse in me made me aware that I had motor neurone disease
a very long time ago.
We know that we're going to not be able to talk,
we know we're not going to be able to swallow or move or do anything.
We know what's ahead, and that's a very frightening thing.
Then there's Tim Trembath.
I was diagnosed early in 2013.
I first noticed a weakness in my right bicep.
It's moved down my arms and my hands and shoulders.
Now I can't do anything.
I've got my wife to help me shower and dress.
I drink through a straw.
I can't lift a cup of coffee or anything like that.
Don Ridley is here representing his father, Col.
He was diagnosed in November, so eight or nine months ago.
He's sort of unwell. He's in hospital at the moment.
He's doing it pretty hard at the moment.
Like Griffith and Lake Cargelligo
there are other towns in the New South Wales Riverina
that have seemingly far higher rates of motor neurone
than the rest of the country.
No one can yet say for certain if it represents
what the medical profession calls a cluster.
But there's no doubting it's a hot spot
at a time where the threat of MND has never been greater.
Over the last 30 years, motor neurone disease
has increased as a cause of death in Australia by more than 250%.
That has to be environmental. It cannot be genetic.
Both the sufferers and the man trying to keep them alive
believe at least some of the answers
lie in the district's greatest environmental asset.
Its water.
Something has happened in the environment in our natural waterways
to change them.
Are they associated with MND? Perhaps.
One of the theories of sporadic motor neurone disease
is exposure to environmental toxins
that selectively target motor neurones.
Some of those toxins may include toxins that are derived
from blue-green algae.
For years now, huge toxic blooms of blue-green algae
have been a common sight in the Riverina.
They've led to many of the lakes and waterways of the district
being closed to the public.
If it's unfit for recreation, is it fit for irrigation?
Blue-green algae is technically not an algae at all.
It's a bacteria.
Cyanobacteria, which can produce one of the suspected culprits
behind MND, a neurotoxin called BMAA.
When exposed to humans, BMAA mimics an amino acid
that our bodies naturally use to make proteins.
There's a whole body of science showing how BMAA misincorporates
into protein to cause cellular dysfunction and death.
This is not just a theory, BMAA is a dangerous toxin.
The blue-green algae theory would fit me fairly well
because I've worked on the river for nearly 30 years.
I've seen really poisonous blooms where it looks like someone's tipped
blue or silver paint on the water.
Lake Cargelligo was actually the first town in the country
to have its water supply prohibited because of blue-green algae
way back in 1990.
I swam in this lake all my life.
And I believe that there is something with the water.
But there are other theories.
The NSW Riverina is prime farming land
and many of its residents have extensive histories
of exposure to a range of pesticides and insecticides.
You get drenched sometimes.
You know, we were spraying, everything was done by hand.
Muckin' around with chemical all my life,
open-air tractors and markin' for aeroplanes goin' over, y'know,
getting covered in chemical.
I recall actually mixing the chemical with my hands.
There was no real WH&S stuff for that.
Put my arm in a drum and stir it around.
This is a very complex environment.
Trying to tease out the different strands that may be involved in MND
is going to be difficult.
But if you want to learn to swim, you don't sit in the bath tub.
But these swimmers may help provide some answers.
At the MND research centre at Sydney's Macquarie University,
scientists are using zebra fish
to test possible causes of the disease.
These factors, these environmental factors
that we suspect may cause motor neurone disease,
we can actually put those into these fish
because they're transparent and we can see the nerves as they grow
in real time in the living animal.
With the use of a fluorescent dye, researchers are able to map
the progress of the disease as the fish develop from egg to adult
in just three days.
The research centre is the largest of its kind in the country
with more than 50 scientists dedicated to tackling MND.
The ultimate aim is to try and make a fish
which develops MND quite rapidly
and we can then treat lots and lots of fish
with different compounds and drug libraries to find a compound
that may give us a cure for motor neurone disease.
We still don't have a smoking gun.
Indeed, in sporadic motor neurone disease it's highly likely
that we're going to have more than one factor involved
in the production of the disease.
Dom Rowe also believes that understanding
the environmental factors involved in sporadic motor neurone disease
may help unravel some of the mysteries
of the genetically inherited form.
It's possible that the same factors that are involved
in sporadic motor neurone disease
may be involved in the production of familial motor neurone disease.
But we don't have the data to answer that question.
This is a disease that's going to affect all of us
and it's something that has frightened us ever since.
Back in Griffith, sisters Tania and Michelle
have already seen twice before
what MND does to a person.
We didn't realise that it was genetic,
especially when Pop was diagnosed, we didn't realise
until Mum was diagnosed that, okay, there must be a connection.
She burst into tears and I said "You know what this is, don't you?"
And she said "Yep" and I said "Is this what Pop had?"
and she said "Yes".
The symptoms of their mother Karen's form of motor neurone disease
were aggressive.
It started in her mouth,
so within a couple of months of being diagnosed
Mum couldn't talk
and within six months she couldn't eat, couldn't swallow.
Karen died within 12 months of being diagnosed.
Dom Rowe helped care for their mother.
So in 2011, when Tania realised something was wrong,
she knew where to go.
How you going? Not so good, huh?
When Dominic diagnosed me
he called me into the room and he looked at me and he goes
"Tania, I'm sorry to say but you've got this (bleep) disease,
"this bastard of a disease."
And then just grabbed my hands and then he cuddled me.
By the time of their mother's diagnosis,
the sisters had already started families of their own.
Both have children.
They also have another brother and a sister.
All live in the shadow of this disease.
The risk of Tanya inheriting that faulty gene from her mum Karen
was 50-50, and unfortunately Tanya drew the short straw.
Given there's four siblings in our family
and with Tania being diagnosed
we're just assuming that there's going to be at least one other
that's going to be diagnosed
and we don't know which one it's going to be
and we can't find out either.
Dom and his team have yet to isolate the particular faulty gene
that has caused Tania and Karen's motor neurone disease.
I've already been tested for four and they've all came back negative.
And it costs a lot of money to get tested but there's another one
that they've just found, another gene,
and now they're going to test me for that.
I want to find the gene for my children.
Because I know one of them's going to get it.
So I want to know the gene.
Dom and his team also want to know
why Tanya developed symptoms so young.
Tanya's first symptoms began five to six years ago,
in her 30s.
Her mum, Karen, her symptoms only began in her 60s.
Some of the factors may well be in the interplay
between the environment and genes.
There are no two patients with motor neurone disease
who are the same.
Tanya fortunately thus far
has a slow and indolent progression of this bastard.
I want to get to 18 years.
That's the longest in Australia so far, is 18 years.
So, that's my aim.
Beat that one, get in the record books.
(Laughs)
Tania and Michelle are now the driving force
behind a local support group
as well as fund-raisers for Dom's clinic.
Motor neurone research has experienced a revolution in funding
in the past few years and it's paying off.
Macquarie is about to launch separate drug trials
for people with sporadic and familial MND.
It's early days, but Dom Rowe believes
with these and further drugs still in development,
there is finally potential to slow
or perhaps even stop this bastard of a disease.
This is personal, you know.
Yesterday one of my patients died.
A 26-year-old mother.
I hate this disease with a passion.
I want it to stop.
For the Riverina sufferers, a cure may be too much to ask for.
But hope is always welcome.
It's not going to happen in my time,
it may not happen even in the generation after me,
but after that, with research,
they will be able to find a cure for this disease.
Well, I hope they figure it out for future generations.
It won't do me much bloody good,
but it might keep you goin' a bit longer.
I don't want anyone to be diagnosed any more
and told "See you later go home, you're going to die."
I just don't want that any more.
I just want to have some bit of hope.
Captions © SBS Australia 2016