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Helen Dalley: Professor Rowe, thanks for joining us.

Prof. Rowe: Pleasure to be here.

Helen Dalley: Can we go back to square one. What is motor neurone disease?

Prof. Rowe: Motor neurone disease is a disease of the central nervous system where special
cells, motor neurones, die, for reasons we dont know. So there are two major classes of motor
neurones, theres the upper motor neurones that reside up here in your cortex, and there are
lower motor neurones that live in your brain stem and spinal cord. The proportion and severity
of the injury to those cell populations produces the symptoms. So if its mainly lower motor
neurone, you get wasting and weakness and twitching and cramps. If its upper motor neurone,
you get clumsiness and stiffness and slowness. So depending on the proportion depends on
how you present clinically. So there are no two patients that present the same way.

Helen Dalley: Do we know what causes it? Do we have any signs or any real evidence?

Prof. Rowe: We actually know that a small proportion of motor neurone disease is produced by
abnormalities in genes, so there are genetic forms of motor neurone disease. Recent science
from here in Sydney has actually contributed to that, so two new genes in motor neurone
disease have been discovered here at the Anzac Research Institute.

Helen Dalley: Would that mean that those genes would be abnormally formed before birth?

Prof. Rowe: Yes. Thats one of the big mysteries. So we know three major genes that cause
inherited motor neurone disease, but inherited motor neurone disease is only a small
percentage of motor neurone disease. So how those genes produce disease 20, 30, 40, 70, 80
years later, we dont yet know. We dont know why a gene abnormality in one person in a family
will present in their twenties, and the same person in the family that has the identical gene
abnormality will present in their seventies. So there are other environmental and biological
modifiers.

Helen Dalley: So when you say modifiers, you mean triggers?

Prof. Rowe: Perhaps triggers. Again, in sporadic motor neurone disease, which is 90% of
motor neurone disease, we dont know what triggers it and we dont have a great handle on
susceptibility. There are some clues genetically and there are some clues about triggers. For
example, its the World Cup; motor neurone disease is a little bit more common in professional
athletes, and theres a study from the Italian A League looking at that, showing that if youre a
professional athlete youre a little bit more susceptible to motor neurone disease.

Helen Dalley: But we dont know why or what is it about a professional athlete?

Prof. Rowe: No. Although there are theories that maybe its effect rather than cause, so maybe
people genetically are predisposed to motor neurone disease who happen to be better athletes.

Helen Dalley: Are there any sorts of professions or classes of people who it might affect, that it
seems to affect more?
Prof. Rowe: The academeology is pretty tricky, but it does seem to be a little bit more common
in people who fly for a living, so commercial airline pilots. There are some other industrial jobs
that are a little bit more common, but youre talking about a very low disease frequency. Its a
disease frequency prevalence of one in 38,000. So trying to pick up clusters of that disease is
very hard from a statistical viewpoint.

Helen Dalley: When does it start afflicting people?

Prof. Rowe: It can start as early as teens and it can present also in eighties and nineties, so I
look after people in their early twenties right through into the eighties and nineties.

Helen Dalley: Again, we dont know why that happens?

Prof. Rowe: No. But we do know that its more common. So in the year 2000, 457 people died
in Australia from motor neurone disease. And in the year 2008, the last year we had statistics
for, nearly 600 people died from motor neurone disease, so thats about a 30% increase in
Australians dying from motor neurone disease. It kills twice as many people as HIV in Australia.

Helen Dalley: So its still relatively small numbers, but as you say theyve been increasing?

Prof. Rowe: 1,400 people its estimated. Theres no true prevalent study yet conducted in
Australia, and were looking to do that. We do have a national registry called the AMNDR, so if
you have motor neurone disease, I would encourage you to join that registry. We have pretty
high penetrants in achieving that, in getting people to join the registry.

Helen Dalley: What is the average life expectancy once youve been diagnosed?

Prof. Rowe: Helen, it varies depending on the form of motor neurone disease that you have,
and for many years weve known that there are different types of motor neurone disease. But
again, two neurologists down in Melbourne have been critical in looking at the different survival
depending on the form of motor neurone disease you have. If you have bulbar motor neurone
disease, so motor neurone disease that principally affects your swallowing and speech first,
because it also affects your breathing, your survival is on average somewhere between 20 and
30 months. So its worse than most forms of cancer. Yet there are some forms of motor
neurone disease, a rare form called primary lateral sclerosis, where the median survival is out
around ten years.

Helen Dalley: Are there any treatments, even for that time, to alleviate the illness? Because
there must be a fair amount of suffering with such rapid deterioration.

Prof. Rowe: Yes, and one of the major improvements in care in motor neurone disease has
been coordinated care. So we can double peoples survival just by coordinating their care well.

Helen Dalley: Really?

Prof. Rowe: So principally making sure that people are well hydrated and have adequate
nutrition. One of the things that happens is problems with breathing through the night, so we
look for that and treat it even before people have symptoms with non-invasive ventilation, so like
a CPap mask for snoring. That dramatically improves peoples survival. Its been shown in
randomised control trials. Unfortunately thats still not supported under Medicare; patients have
to pay for that therapy themselves. There are some medications. Theres a medication called
Riluzole or Rilutek which improves survival a little bit, and there are other experimental drugs
that are looking very promising in motor neurone disease.

Helen Dalley: I guess the reason that you need an awareness day is because a lot of people
dont know or understand enough about it, and youre trying to raise funds. Tell us about your
new facility at Macquarie University in Sydney, because its a brand new hospital at the
university, isnt it?

Prof. Rowe: Yes. So next Saturday a 180 bed hospital opens up just up the road at Macquarie
University, so its a hospital that was the genesis of the university. Its a new medical school
thats aimed at post-graduate teaching rather than under-graduate teaching.

Helen Dalley: So a very exciting prospect?

Prof. Rowe: Very exciting, yes.

Helen Dalley: Why? Partly because of the funding, isnt it?

Prof. Rowe: Yes. So this is very novel. This is a university owned hospital, its a not-for-profit
hospital. Its not part of the Department of Health, its not part of the state government.

Helen Dalley: So state governments had nothing to do with it? And the university and the feds
raised the money?

Prof. Rowe: Yes. Our motor neurone disease clinic as we get up and running will reconstitute.
I ran for ten years a multidisciplinary motor neurone disease clinic at North Shore, but moving
over to Macquarie we will start that again in the near future.

Helen Dalley: So thats up and running now in your neurology department. Carers of people
with this illness, just in our few remaining minutes, must be extremely pressed. Is there enough
support both in the community and from government levels for carers?

Prof. Rowe: As always, yes and no. Theres a very effective organisation, the Motor Neurone
Disease Association of Australia, and the Motor Neurone Disease Association of NSW, and
each state has an association. They are very, very effective in carer support and supplying
equipment and political lobbying to obtain better access and better care for patients with motor
neurone disease and also the carers. Because if the carer falls over, the person with motor
neurone disease is in a lot of trouble, much more trouble. So its very important. This disease
is seriously the worst disease that you could imagine. It robs you of your ability to move, to talk,
to breathe. And it leaves all your special senses intact, so your vision, your thinking, your
hearing, your sensation are all intact.

Helen Dalley: So we really need a lot more money invested into research, all that sort of thing?

Prof. Rowe: We do, and theres the Research Institute of Australia, which is part of MND
Australia, and we give out around about a million dollars a year to Australian researchers in
post-graduate scholarships, graduate scholarships and also seed funding that translate into NH
and MRC projects.
Helen Dalley: Professor Dominic Rowe, well leave it there. Thanks very much for joining us for
Global Awareness Day for Motor Neurone Disease.

Prof. Rowe: Appreciate it.