Each weekday morning I walk down to Front Beach clad in Speedos ( now there is a scary sight!!) board shorts and T shirt, sweat beading along the hairline and slithering south the moment flesh hits humidity. Beneath the bat laden mango tree I watch the thousand strong population vibrate, squeak and squirt a tarry black poo adding to the soggy air smells of tropical vegetation and then there is a walk along the wave line where hermit crabs scuttle around in witch hat shells and I watch out even more religiously for would be loitering crocs. Armed with zip lock bags the collection of sea glass, hieroglyphed shells and lumps of gorgeously shaped coral grows and daily I tread the paw printed sand…a dynamic duo of pit bulls… back to the Alyangula swimming pool. 25 metres of blue water, coconut palm shade and lounging chairs looking out on the Carpentaria Gulf I am often there on my own and sometimes sharing the pools with mums and children, bronzed toddlers confidant in the paddling pool and leaping with water winged abandon into the warm water.
One morning as I loll in the shade with a book by the pool, two four wheel drives pull up in the car park. A young Aboriginal boy with long curly hair, aloha patterned board shorts and swimming goggles launches into the pool while an older man is pushed onto the grassy surrounds in a wheelchair accompanied by an Aboriginal woman who stretches out on a lounger in the shade and dozes, her arm shading her eyes, and a trio of non Aboriginal Australians wearing ‘rashies’ with the MJD logo. Short cropped hair, a smile as wide and glowing as a new crescent moon and Ray Charles dark tinted glasses the wheelchair man is helped from his chair into motorised chair lift that lowers him into the pool and the arms of perhaps a physio and they ease through a range of watery exercises and floating. The gentle immersion into which he sinks with an even bigger grin burying his face for what seems like a very long breath must surely provide the warm weightlessness it does for all who enter the blood warm embrace of the clear watered pool.
He is muscle withered and wheelchair bound and the victim of an hereditary neuro-generative disease, one of the family of neuro-generative diseases including Huntingdon’s Disease. Until 1995 this condition was known as Groote Eylandt Syndrome until the discovery of the gene for MJD and Groote Eylandt Syndrome was confirmed as MJD.
So what is MJD?? Named, the M and the J that is, for the two families Machado and Joseph of Portuguese/Azorean descent who were two of the original families described with the symptoms of this disease in the 1970s it is prevalent among people of Portuguese/Azorean origin and in a staggering 1 in 140 on the Azorean Island of Flores and one can only imagine what that ratio is or will be on Groote with its tiny population.
It’s nasty and as yet untreatable and occurs because of a chromosomal disorder producing an abnormal protein which causes premature death in nerve cells. The cerebellum damage causes muscular weakness which progresses with time culminating ultimately in complete failure of voluntary control and a permanent physical disability. Children of those carrying the defective gene have a 50% chance of developing the disease and even more terrible is that the mutation expands when passed to the next generation…’the anticipation effect’…resulting in symptoms appearing 8 to 10 years early and more severe and so it continues. While travelling through the two main towns on Groote I see people in wheelchairs and learning more about MJD realise that the age of those people will be younger and younger with each generation. So how did this become a Groote Eylandt problem?? The spread of the disease to Arnhem land has been linked so far, though research and investigation continues, to the activities of Portuguese sailors who traded and sailed to northern Australia in the 16th century and the trading relationships between the Arnhem Land Aboriginals and the Macassans who in turn traded with the Portuguese. A centuries old story of twins good and not so good told the world over where trade and goods and ‘new technology’ is undermined by an invisble infiltrator.
‘The MJD Foundation is a charity, established in 2008 to seek to improve the quality of life for Indigenous Australian Machado Joseph Disease sufferers and their families in Arnhem Land and beyond’, and more can be learned at this link, http://www.mjd.org.au specific to MJD and Groote Eylandt and Arnhem Land and beyond. Do read Gayangwa’s story about her life in a family affected by MJD and as a carer too and her speech at the MJD Foundation launch on the link..it really is inspiring… and how insidious MJD is and how it affects the families on Groote.
Back in the pool with the man with water bubbled hair, Ray Charles sunnies and enormous smile still in place has floated and waded in the pool supported by loving, caring hands and arms while watching the aloha boardshorted boy swim, dive and splash. This is my only opportunity to see the MJD Foundation at work in a tiny part of what they do but on one of our outings onto muddy pooled roads I see the blond bobbing ponytail of one of the pool trio running a similar muddied road through the country that has supported life for such a long, long time.