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30 Speak Out August 2015 Speech Pathology Australia
BRANCH NEWS
NORTHERN TERRITORY
I FIRST BECAME aware of Machado-
Joseph Disease (MJD) while flying on a
twin-engine plane to Groote Eylandt for
a site visit. Groote Eylandt archipelago
is situated in the Northern Territory
on the western side of the Gulf
of Carpentaria, approximately
600km south-east of Darwin. The
Warnindilyakwa people are the
Traditional Owners of the Groote Eylandt
archipelago region and are referred to
by their language name, Anindilyakwa.
My family moved to Groote Eylandt in
January 2014 for my husband’s job,
and because of my background and
interest in rehabilitation, I joined the
MJD foundation as a volunteer
speech pathologist in April that year.
MJD is an inherited, autosomal dominant
disorder, which means each child of a
person who carries the gene has a 50%
chance of developing the disease. This
progressive neurological disorder occurs
due to a fault in a chromosome that
causes the production of an abnormal
protein. This protein causes nerve cells
to die prematurely in the cerebellum.
The mutation is typically expanded
when it is passed to the next generation.
This is known as the “anticipation
effect” whereby the symptoms will
appear approximately 8–10 years earlier
and are more severe with successive
generations.
The damage to the cerebellum initially
causes muscular weakness and
progresses over time to a total lack of
voluntary control and very significant
permanent physical disability, including
dysphagia and dysarthria/anarthria.
Progression to dependence occurs
over 5 to 10 years and most people
are wheelchair-bound and fully
dependent for activities of daily living
within 10–15 years of the first symptoms
emerging. At this time, there is no
known cure for MJD.
Previously known as “Groote Eylandt
Syndrome”, the effects of MJD have
been known to the Aboriginal people of
this region for at least four generations.
Although it is impossible to predict the
number of people who will develop
MJD there are currently around 550
people alive today thought to be “at risk”
of developing the disease across the
Top End.
I started this work, with a resounding
interest in clients with MJD and the
potential of regular speech therapy to
improve and/or maintain the speech
and swallowing function of these
patients. Weekly therapy focuses on
breath support, voice projection and
safe swallowing strategies.
I am fortunate to work within a
multidisciplinary team and our therapy
is client-centered, holistic and functional.
Sessions are often conducted “on
country” (fishing at the beach, bush
walking) which helps to keep clients
engaged and ensures therapy
techniques are meaningful. When
we are at the beach and the wind is
blowing and the waves are crashing,
you definitely need to “yibina ayakwa”
(loudly talk).
Working with the MJD clients
has been both personally and
professionally rewarding. It has taught
me to acknowledge and respect the
connection that indigenous Australians
have to their families, their culture,
their communities and their country.
You can find out more about MJD
and the MJD foundation at
MICHAELA JACKSON
Speech Pathologist
“Yibina Ayakwa” – Loudly Talk
Michaela and Joyce doing voice exercises on the beach.