It’s Rare Disease Day on Thursday 28 February. As the Australian Bureau of Statistics advised the Royal Commission into Aged Care Quality and Safety recently dementia will become the leading cause of death in Australia by the mid 2020’s, you could be excused if you thought dementia did not have any relevance to Rare Disease Day.
In fact, the opposite is the case. Most people will have heard of the most common type of dementia in Australia, Alzheimer’s disease. They may even be able to name the other three most common causes of the symptoms of dementia.
However, what many people don’t realise is that among the more than one hundred conditions that cause dementia, there are many rare conditions.
These conditions are so rare that symptoms or identifiers are often misread as something else, making these rare dementias hard to diagnose.
Take for example one of the rare dementias, posterior cortical atrophy (PCA), also known as Benson’s syndrome. Whereas Alzheimer’s disease is more common in older people and usually causes impairment in memory first, PCA tends to develop in people aged 50-65 and first affects vision. It is not that the person’s eyesight is failing. PCA causes an impairment in the way the brain interprets what the eye is seeing. In fact, the memory of a person diagnosed with PCA is mostly unaffected until the latter stage of the disease.
Someone who has been living with PCA for 10 years, since the age of 55, is Dr Glenda Parkin, former school teacher, university lecturer and principal of St Stephen’s School and Penrhos College.
For Glenda, PCA is a cruel and debilitating disease that has now robbed her of almost all her visual capability. She is now functionally blind and requires assistance with many daily living tasks.
Her husband and full time carer, Bronte, says Glenda still maintains remarkable insight into her own condition.
“Glenda is still socially aware and wants to engage in as much as possible with friends and family. She enjoys all forms of music and loves to sing,” he said.
“Although unable to see clearly, Glenda also attends an art therapy program for people living with dementia at the Art Gallery of WA.”
Bronte says unlike people who are technically blind and perhaps can still manage to ‘read’ via Braille, Glenda’s cognitive decline has meant that normal coping strategies designed for people who are blind or with a major visual impairment have not been workable.
Since Glenda’s diagnosis in 2010 and her consequent premature retirement, and Bronte’s early retirement in 2011 to provide Glenda with full time care, the couple has enthusiastically embraced the advice of Glenda’s neurologist to travel to the parts of the world she wanted to see before it was too late. While lengthy overseas travel is now curtailed, they travel frequently to see their two adult sons who live and work in Melbourne.
“I realised early last year after our youngest also moved to Melbourne for work the importance to Glenda of being able to continue to have quality time with both of our sons and to be able to give them a hug,” Bronte said.
Glenda pronounced early after her diagnosis that she had spent her career as an educator, and wanted to continue in that role, albeit she would now educate about dementia.
“Educating about dementia makes me feel as though I’m still alive, I have a purpose,” said Glenda.
Glenda and Bronte have tirelessly advocated for dementia for several years, both in Western Australia and nationally. Glenda represented Western Australia on a national dementia advisory committee and Bronte served as a director on the Board of Alzheimer’s WA. They have also been willing participants in the media and as speakers, giving people living with dementia a voice.
For their efforts, the couple was awarded the WA Volunteer Award in the Aged and Community Services Australia Excellence in Aged Care awards in 2017.
Glenda’s cognitive impairment is caused by a rare disease. Her life-long commitment to education and her love of life, family and friends is inspiring, and also rare.
We can only hope that in the years ahead we make progress in understanding the contributing factors to cognitive decline and the disease conditions that cause it, both the common and the rare.
In the meantime, we should be inspired by rare people, support those with cognitive impairment, treasure good health and be grateful for each day we have.
If you are concerned about dementia, please contact Alzheimer’s WA on 1300 66 77 88.