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Understanding Primary Progressive Aphasia

Terry Jones from Monty Python puts it like this, “My frontal lobe has absconded.” He has primary progressive aphasia, “PPA”, and experiences increasing problems with communication. These communication problems lie at the centre of this little-known disorder, that CCD researchers in the Aphasia Group are focused on investigating.

PPA is rare syndrome caused by some of the same brain diseases that cause other types of dementia. But PPA goes against our everyday preconceptions about dementia because it doesn’t affect memory or thinking. Instead, PPA affects communication.

The pathology, or the tissue changes inside the brain, can be different in different dementias. This is one reason there are many different types of dementia, and not all dementias are ‘Alzheimer’s Disease’. In fact, ‘Alzheimer’s Australia’ have recently changed their name to ‘Dementia Australia’ to provide a voice for people with all types of dementia. While some people with PPA have Alzheimer’s disease, many others have one of the frontotemporal dementias, a less well-known group of diseases.

Awareness of rarer dementia types like the frontotemporal dementias is growing, however, this week 600 professionals including doctors, scientists, speech pathologists and neuropsychologists met in Sydney, Australia, for the 11th International Conference on Frontotemporal Dementias, to discuss dementia syndromes such as PPA. The conference covered the genetics, brain changes, diagnosis, treatments and clinical care in these conditions. Our team (pictured below) organised a workshop for clinicians and researchers to learn about new approaches to diagnosis and treatment of PPA. There was also a day for carers of people with PPA and other frontotemporal dementia syndromes with information provided by leading clinicians and researchers.

Primary Progressive Aphasia researchers

Public awareness is of PPA is also growing, especially as Monty Python’s Terry Jones has PPA, and Australian Dementia advocate Kate Swaffer, 2017 Australian of the Year (SA), has the ‘semantic’ type of PPA which erodes the meanings of words and objects.

Losing my words not my memory

“Do you feel that you have more problems with memory than most?” one of us recently asked an individual with PPA.

“Oddly, I think ‘no’. Because that’s not affected. I mightn’t be able to express it because I can’t spell it. But I know.”

This answer nicely captures the way people with PPA have problems expressing what they want to say, while their memory for things they have done or things they need to do is not necessarily affected. This is because the areas that are initially affected by the disease are usually in the left hemisphere brain regions that control our communication. This is why individuals with PPA struggle to find the right words, to speak clearly and effortlessly, to string sentences together or to understand others. Some also have difficulty reading and writing. In the long-term, people with PPA usually lose the ability to speak independently. As with most communication impairments, PPA is a hidden disability, because people with PPA often have a difficult time advocating for themselves once their communication is impaired. This is where the advocacy role of speech pathologists becomes vitally important.

Living well with primary progressive aphasia

Terry Jones’ statement above focuses on ideas of loss and of brain changes. However, we are also learning that, once they have dealt with the shock of an initial diagnosis, many people find ways to maintain positivity while living with PPA.

With the right communication support, people with PPA are still able to express themselves, so our research aims to hear the stories of people living with PPA and capture their experiences. Some of the ways people with PPA stay positive include embracing today rather than focusing on the future and keeping busy:

“I think ... getting out and doing things [helps]. And, um, and you have got to work on the basis that it could well be, um, really bad, you know, later.”

“I think it's because I’m healthy physically and, um, and I’m naturally positive so I think, oh, well, you know, I’ve got this [PPA] so I might as well get into it”

Speech pathology support and determination help others stay positive:

“If I didn’t have the, um, the work with [speech pathologist], ah, and I didn’t have information as to what to do and how to move on, that would have been difficult. And I can understand why people get into that situation but, um, I - I definitely had a positive outlook on it and a determination to be busy.”

 

This article was written by Leanne Ruggero, Prof Lyndsey Nickels, and Dr Karen Croot. Keep up-to-update with the latest in Leanne’s research on the lived experiences of individuals with PPA, follow her on Twitter: @Leanne_SLP

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