Imagine gradually losing the ability to express yourself — not because you've forgotten the words, but because they simply won't come out. This is the reality for individuals living with primary progressive aphasia (PPA), a rare form of dementia that usually begins in middle age and increasingly impairs language abilities over time.
Researchers at the University of Chicago Medicine are working to illuminate the struggles of those living with this condition and pioneer accessible treatment models. They recently published new studies that measure PPA's significant impact on quality of life and demonstrate the feasibility of international telemedicine interventions — research that could reshape care delivery and inform policy decisions.
Understanding PPA
PPA is a unique neurological condition that primarily affects language skills, setting it apart from more common and well-known forms of dementia like Alzheimer's dementia that primarily impacts memory in the earliest stages.
"PPA is a relatively rare dementia," said Emily Rogalski, PhD , the Rosalind Franklin PhD Professor of Neurology at UChicago and a leading researcher in the field. "It's often overlooked in the literature because it can be difficult to assemble large groups of people to survey lived experiences."
She said it's frequently overlooked by medical professionals as well, going undiagnosed particularly among those with lower socioeconomic status.
"Lack of diagnosis can be such a barrier to care for anyone not living next to a specialized medical center," she said.
A feature that makes PPA especially challenging is its early onset.
"These individuals are at a different life stage from late-onset Alzheimer's dementia patients," Rogalski said. "They're often still in the prime of their working career; they may have young children in the home."
This can mean PPA not only affects the patients but also has significant implications for their families, family relationships and economic stability.
Measuring PPA's impact on quality of life
To better understand how PPA affects daily living, Rogalski and her collaborators conducted a study using the Health Utilities Index (HUI), a standardized tool that measures various aspects of well-being, including physical abilities, emotional health and cognitive functions.
Unsurprisingly to those familiar with the disease, the results showed that PPA has a moderate to severe negative impact on patients' health-related quality of life. The results also showed that greater language impairment in individuals with PPA was linked to a lower quality of life, particularly affecting domains such as hearing, sensation, cognition and speech.
"It was important to confirm that the HUI, a commonly used health measure across diseases, was capturing the essence of these patients' primary impairment," Rogalski said.
Thomas Hopkins, PharmD, MS , the study's first author, explained the study's twofold purpose: obtaining detailed information about quality of life for individuals with PPA, and enabling direct, objective comparisons between the impact of PPA and that of other diseases. Since the Health Utilities Index is a generic measurement, it can be applied to any health condition — even those dissimilar to dementia, such as cardiovascular disease or cancer.
"When it comes to policy-making and government allocation of resources, it really is important to have these broadly applicable measures so better decisions can be made," Hopkins said.
This quality-of-life data can inform crucial decisions including research funding and prioritization, insurance regulation, disability coverage and more. Now armed with concrete evidence that PPA can impact the lives of middle-aged adults just as dramatically as many other disruptive diseases, researchers, patients and families can advocate for more resources and support.
Expanding access to care with telemedicine
Even while working to establish objective measurements of PPA's impact, Rogalski and her colleagues were simultaneously enrolling patients in a clinical trial exploring the feasibility of delivering speech-language therapy for PPA via telemedicine. They recently reported the successful enrollment of 95 participant pairs — each comprising a PPA patient and their primary caregiver — from four countries, demonstrating that remote recruitment and video chat intervention are viable options for overcoming geographic and socioeconomic barriers to treatment.
