People with primary progressive aphasia and their families have unique care needs that a new study finds require specialized support.
A lesser-known form of dementia that primarily affects the language and speech centres of the brain was the focus of a research study led by Vancouver Coastal Health Research Institute researcher Dr. Ging-Yuek Robin Hsiung and Dr. Katharine Davies. The study gathered perspectives from speech-language pathologists on the communication needs of people with primary progressive aphasia (PPA) and their family members, pinpointing areas for improvement in the care delivery of this patient population.
“What we found is that people with PPA and their families have a great need for support throughout the progression of the disease,” notes Davies. “However, right now, care varies significantly between individuals, with much of it inconsistent and lacking in many parts of the province.”
“There is limited guidance in the research literature on what people with PPA and their families need across the development of the disease.”
PPA is a rare syndrome of dementia with a prevalence of approximately three individuals per 100,000. It mostly affects the frontal and/or temporal regions of the brain responsible for the ability to communicate. Onset stems from a variety of neurodegenerative diseases, the most frequent of which being degeneration of the frontotemporal lobe in the brain. However, it can also be due to other dementia pathologies, such as Alzheimer’s disease, or other degenerative brain diseases, such as progressive supranuclear palsy.
PPA typically begins with subtle and often hard-to-detect symptoms, including difficulties with word-finding. Symptoms vary across the three main subtypes of PPA, with people with the semantic variant experiencing difficulty understanding speech or written language and often losing the ability to understand the meaning of words. Individuals with the logopenic variant experience difficulty understanding and repeating long sentences. The nonfluent agrammatic variant results in difficulty speaking fluently and putting words in the correct grammatical order.
Among all three subtypes, individuals in the latter stages of PPA syndrome progression may become mute and lose the ability to communicate with family.
“For people with PPA, interventions provided by speech-language pathologists are the primary support available, especially in the early and middle stages.”
Once a diagnosis is made by a neurologist, individuals are often referred to a public speech-language pathologist through outpatient rehabilitation for a limited number of sessions to discuss their PPA, create a treatment plan and review additional treatment options. While initial sessions are generally covered under British Columbia’s provincial health care plan, additional sessions with private speech-language pathologists are out-of-pocket expenses charged to each patient and unavailable in many regions of the province.
Speech-language pathologists provide both emotional and psychosocial support
Hsiung and Davies’s study involved semi-structured interviews and questionnaires with 14 speech-language pathologists who provided care to adults with PPA in British Columbia. From this data, researchers identified eight categories of communication needs for people with PPA and six for their family members.
Among the four key findings from Hsiung and Davies’s research are the continuous nature of the communication needs of people with PPA and their families throughout the disease continuum and that people with PPA and their family members have complex communication needs that require tailored care.
“Our research revealed distinctive shifts in communication needs along the disease progression that could aid in the refinement of training and supports for speech-language pathologists,” notes Hsiung.
“These findings have key clinical implications for proactive communication care across the continuum of dementia care.”
The third key theme from their research highlights the importance of early diagnosis and early treatment interventions for better patient outcomes. Lastly, speech-language pathologists included in the study highlighted the central role they play as a primary source of support for people with PPA and their families within a broader interdisciplinary care team.
What remains is to determine how best to support their work, such as through expanded funding for their services to people with PPA and their families.”
Resources for people with PPA and their families are available at the Communication Assistance for Youth and Adults (CAYA) website. CAYA is a provincially funded program for people with severe communication disabilities.
