There was once a time where a diagnosis of Primary Progressive Aphasia (PPA) was felt to be rare. More and more I have found people being referred to me with this exact diagnosis. Is it a combination of better identification? Better tools in the healthcare world that can help to identify this progression early on? And what do we do with this information?
PPA is a devastating disease process. It is a form of Frontotemporal dementia that begins with the gradual deterioration of speech and language function. Deterioration is caused by structural and physiological abnormalities in the left hemisphere of the brain, where language-related centers are focused. Generally, cognitive functions like memory, visual-spatial processing and planning, and personality are preserved up into the late stages of the progression. In many cases, the individual is able to drive, participate in their activities of daily living, and even use technology (like their computer or iPhone). Individuals with PPA progressively lose their ability to speak, often beginning with observable word-finding difficulties, then progressing into impaired ability to formulate a complete sentence. There may also be the presence of dysarthria of speech (weakness, which may sound like slurred speech), or apraxia of speech (incorrect use of the sounds). The aggressiveness of the disease is high, with an average life expectancy of 6-8 years after the onset of symptoms, and 4-7 years once diagnosed. As one could imagine, this is incredibly devastating and terrifying to the individual and to their families.
Aphasia due to stroke is a common diagnosis. Aphasia refers to an impairment of language that can affect comprehension or understanding, expression and thought formation, and reading and writing. In the setting of a stroke, a speech pathologist can work to repair the damaged areas by increasing neural connectivity between the non-damaged areas of the brain. Often these connections are strong enough to solidify the language that is remaining and can improve the individual’s language, word-finding, and speech production. In PPA, although this work can be done, due to the degenerative process of the disease, it is a constant uphill battle, and planning for the inevitable loss of language is required.
Speech-language pathologists are keenly adept at helping to manage this illness. Like any progressive disease, PPA is marked by different stages, with each stage requiring assessment, intervention, modifications, and education. Speech pathologists understand the changes in the brain and the progression of the disease. This not only helps the individual maximize their language and communication abilities during each stage but helps the family and caregivers understand the changes and provide their loved ones with the necessary care and support.
What can you expect from skilled speech therapy for PPA? Speech intervention in the early stages of the illness may focus on the impairment itself, which may address language and word-finding, vocabulary, or speech production and output. Intervention in the middle stages of the disease process may introduce other communication approaches and augmentative and alternative communication (AAC), such as gesturing, drawing, and writing, or creating communication books. This is a key time in discussing goals of care and plans for the patient’s future needs. The individual with severe deficits may require the use of a high-tech AAC or speech-generating divide (SGD) to effectively communicate at all. Identification of this need should occur in the early to middle stages of the illness, where learning for programming and the use of a device can be better addressed. Many SGDs offer a feature for “voice banking,” where the individual can record the words and phrases on their device in their own voice. This is a beautiful way for the individual to stay connected with their loved ones as their disease progresses.
With any disease process, early identification, diagnosis, and intervention are necessary. The earlier PPA is recognized, the more effective the interventions will be. Unfortunately, some doctors do not always identify the need for speech-language therapy in this disease process, especially in the early stages, which results in individuals seeking out therapy support far later into the progression. At this time, many opportunities for improved quality of life and connections may have been lost. A speech-language pathologist trained in the treatment of neurodegenerative diseases can provide support, collaboration, and structure. A clear baseline can be identified, as well as areas of strength and areas at risk for decline, and the estimated level the individual is in their process. This information is key in assessing the progression of the disease and helps track where the person is in their process so the right supports can be recommended. Most importantly, the individual and their family have another support they can lean on as they navigate decisions, which helps improve calm, acceptance, and quality of life.
Useful Links and References for More information:
National Aphasia Association https://www.aphasia.org/
The Association for Frontotemporal Degeneration https://www.theaftd.org/
The Alzheimer's Association https://www.alz.org/
American Speech-Language-Hearing Association https://www.asha.org/