MJD Foundation

Independent Living - Magazine Publication

Independent Living - Magazine PublicationVolume 25 No 2 - July 2009

MACHADO JOSEPH DISEASE - Starting a New Foundation
Nadia Lindop
Libby Morgan

Far, far away there is a tiny town on a tiny island in the Gulf of Carpentaria. The Island is called Bickteron Island and the town is ‘Milyakburra'. To get there from Darwin, you must first fly the 1.5 hour flight to Groote Eylandt, and then take a charter flight on a single engine Cessna another 20 minutes over cerulean blue waters to Bickerton Island.

It's a beautiful island, part of the Groote Eylandt archipelago in remote north eastern Arnhem Land. This is the traditional ‘country' of the Warnindilyakwa people. They are Ancient and beautiful places,....but they harbour a tragic secret story. It is a secret of ‘shame' and ‘blame'....it is the story of the struggle of this proud and ancient people with an old and deadly foe, a fatal inherited disease called Machado Joseph Disease.

If you live at Milyakburra, you are amongst a population of around 200 indigenous Australians. The island's coastline is spectacular with some of the most beautiful cliff lines in Australia. Being sub-tropical, it rains heavily for around 4 months of the year. The rain is a welcome relief from intense year round heat, but if you live at Milyakburra, and you are disabled, that same rain on the unsealed roads makes a wheelchair trip to the local shop unfathomably difficult.

Machado Joseph Disease is a cruel and complex disease, robbing those who have it of their independence and dignity. It occurs in families, often starting in late adulthood with each child of an affected person at a 50% risk of developing the disease themselves, almost always at an earlier age than their parent. It is an old disease, some strains are thought to be up to 4000 years old, and its worldwide spread has been attributed to the trading activites of Portuguese and Chinese sailors. Although rare in mainstream Australia, the incidence of MJD in these communities is the highest in Australia. In some of the Groote and Bickerton families carers struggle to support three generations concurrently.

It is a terrible story, but it is not unique to Groote. The disease also affects several other Indigenous Arnhem land and Central Australian communities, occurs in small numbers in other regions and is known on every continent on earth.

What is unique to this remote and isolated region are some formidable barriers to providing high needs support, conducting appropriate research and providing quality care. Isolation and distance, and the attendant costs, combined with complex kinship and language systems and a transient professional population have meant that access to equipment, services and facilities found elsewhere requires significant and co-ordinated resources over many years. The MJD Foundation was established in May 2008 to meet these needs.

Libby Morgan and Nadia Lindop from the MJD Foundation visited the Independent Living Centre NSW in Sydney in 2008 to gain an understanding of the latest Assistive Technology available. ILC staff spent many hours understanding both the challenges of providing practical equipment in such a remote area of Australia, along with the physical needs of those suffering with MJD and their families.

Some of the issues related to the remote location are:
• Prolonged monsoonal conditions ‘The Wet'
• Cyclonic conditions
• Unsealed and poorly maintained roads
• The length of time (and enormous cost) to get items shipped to Darwin, then on barges to remote locations
• Lack of options for care, installation and maintenance of equipment in communities

These harsh environmental conditions necessitate a focus on functionality and durability of the Assistive Technology utilised.

Key AT challenges relating to Machado Joseph Disease and its high incidence in the area are:
• Gradually decreasing mobility, requiring incremental mobility aid provision (wheeled walker => wheelchair)
• Gradually deteriorating communication capacity
• English as a Second Language (ESL)
• Overcrowded living conditions and poorly maintained homes
• A highly mobile disabled population
• High % of population affected
• Multiple generations affected in the same family, hence carers may themselves be experiencing early symptoms of MJD.

Just a few of the specific challenges include the following:
Mobility
Standard issue government wheelchairs are provided with an orientation to durability and cost containment. They are sturdy and require little maintenance, however in very remote locations such as Milyakburra where roads are unsealed and the wet season sets in for 4-5 months at a time, they are difficult to manoeuvre in muddy and sandy conditions. Although able to be folded, standard issue wheelchairs are also cumbersome, and in an area where the primary transport means is costly light aircraft charter, the necessity for two seats to be taken up by a wheelchair that does not fit into the cargo bay is both expensive and inconvenient.

The MJD foundation is currently experimenting with a range of temporary portable and all terrain wheelchairs to assess their suitability and is working with suppliers and service providers such as SEAT to come up with innovative and practical solutions to these and similar problems. The ultimate aim will be to incorporate items that meet needs into the Territory Independence and Mobility Equipment (TIME) scheme and providing items additional to these according to individual need.

Self Care
Due to the degenerative nature of the condition, self care capacity is progressively impaired. Wet areas require modifications and adaptations such as rails, seated showering, hand held showering devices and non slip products.
In the latter stages of the disease, bowel and bladder function is significantly compromised. The ensuing incontinence is very difficult to manage and wet areas in homes bear the cumulative burden of heavy use by multiple members of the family with the disease. This necessitates more frequent monitoring and upgrades.

In addition, housing conditions in remote indigenous communities are notoriously poor, and overcrowded, and some elements of cultural practice mean that Individuals may move between family members often. Therefore a disabled person, may live in several residences across the year. This necessitates multiple modifications and is costly. This makes sourcing appropriate temporary alternatives a priority.

Accessibility
Similar issues apply to general housing access. Whilst the MJD Foundation has been trying to fast-track the building of ramps in houses, a quick and practical solution has been to purchase a range of portable ramps, gutter ramps, and curb ramps that the whole community can use as needed. There are currently a range of these being trialled in the Groote Eylandt and Bickerton Island communities.

Communication
In the most progressed stages of Machado Joseph Disease people are unable to communicate either verbally or with sign. The attendant distress to themselves, their families, carers, health and community members is immense. The MJD foundation is currently trialling AT communication devices in a number of locations in the affected communities including the local health clinic and flexible aged care centres. The efficacy of these will be assessed in a study to be conducted by the University of Queensland over the coming 2 years.

The needs of this community of people are significant and will increase exponentially over time. The nature of Machado Joseph Disease and the locations where it is currently being experienced pose significant and ongoing challenges to meeting these needs. The MJD Foundation looks forward to continued collaboration with the ILC and other relevant organisations to meet these needs.

For more information on the work of the foundation, please visit www.mjd.org.au