Researchers explore the impact of primary progressive aphasia on quality of life

Imagine gradually losing the ability to express yourself -; not because you’ve forgotten the words, but because they just won’t come out. This is the reality for people living with primary progressive aphasia (PPA), a rare form of dementia that usually begins in middle age and progressively affects language skills over time.

Researchers at the University of Chicago Medicine are working to illuminate the struggles of people with this condition and pioneering accessible treatment models. They recently published new studies measuring the significant impact of PPA on quality of life and demonstrating the feasibility of international telemedicine interventions -; research that could reshape healthcare delivery and inform policy decisions.

Understanding PPA

PPA is a unique neurological disorder that primarily affects language skills, distinguishing it from more common and well-known forms of dementia such as Alzheimer’s disease, which primarily affects memory in its earliest stages.

“PPA is a relatively rare dementia,” says Emily Rogalski, PhD, the Rosalind Franklin PhD professor of neurology at UChicago and a leading researcher in the field. “It is often overlooked in the literature because it can be difficult to bring together large groups of people to explore lived experiences.”

She said it is also often overlooked by medical professionals, and goes undiagnosed especially among people of lower socio-economic status.

Lack of diagnosis can be such a barrier to care for someone who does not live near a specialized medical center.”

Emily Rogalski, PhD, the Rosalind Franklin PhD Professor of Neurology at UChicago

One feature that makes PPA particularly challenging is its early onset.

“These individuals are at a different stage of life than patients with late-onset Alzheimer’s dementia,” Rogalski said. “They are often still in the prime of their careers; they may have young children at home.”

This may mean that PPA not only affects the patients, but also has significant consequences for their families, family relationships and economic stability.

Measuring the impact of PPA on quality of life

To better understand how PPA affects daily life, Rogalski and her collaborators conducted a study using the Health Utilities Index (HUI), a standardized instrument that measures various aspects of well-being, including physical skills, emotional health and cognitive functions.

It will come as no surprise to those familiar with the disease, but 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 delay in individuals with PPA was associated with lower quality of life, particularly in areas such as hearing, sensation, cognition and speech.

“It was important to confirm that the HUI, a commonly used health measure for diseases, captured the essence of these patients’ primary disability,” Rogalski said.

Thomas Hopkins, PharmD, MS, the study’s first author, explained the dual purpose of the study: to obtain detailed information about the quality of life of individuals with PPA, and to allow direct, objective comparisons between the impact of PPA and that of other diseases. Because the Health Utilities Index is a generic measure, it can be applied to any health condition; even those that don’t look like dementia, such as cardiovascular disease or cancer.

“When it comes to policymaking and government resource allocation, it’s really important to have these broadly applicable measures so that better decisions can be made,” Hopkins said.

This quality of life data can inform critical 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 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 as they worked to establish objective measures of PPA’s impact, Rogalski and her colleagues were simultaneously enrolling patients in a clinical trial examining the feasibility of delivering speech therapy for PPA via telemedicine. They recently reported the successful registration of 95 pairs of participants; each consisting of a PPA patient and their primary caregiver -; from four countries, demonstrating that remote recruitment and video chat intervention are viable options to overcome geographic and socio-economic barriers to treatment.

“We’ve found a way to provide care that creates a little more of a level playing field,” Rogalski explains. “Individuals with PPA do not need to live next to a major academic medical center or specialty center to connect with a professional and receive treatment.”

This success also paves the way for future research and interventions; and not just for PPA.

“We see our approach as a potential model,” Rogalski said. “We think the framework we have can be adapted and used to support advocacy and interventions for multiple different dementia syndromes and conditions.”

It offers hope even if there is no cure

Rogalski points out that care partners play a critical role in studies like these, providing vital insights into the daily challenges and diverse needs that span family dynamics and life situations.

“We give families a voice to share their experiences,” she said.

Despite these advances in research, challenges remain in diagnosing and treating PPA, and no drug or therapy has yet been developed that can cure the disease. But broadening understanding of PPA’s profound impact on quality of life and demonstrating the effectiveness of telemedicine interventions are meaningful steps that can encourage patients and families.

“Providing hope and practical support to these families is very important,” Rogalski said. “Too many people have been through experiences where, even if they find a specialist, that specialist may say, ‘There’s nothing we can do. It’s a terminal diagnosis.’ But finding a cure isn’t the only way we can help people. Maximizing independence, emotional well-being and self-confidence can all have many practical aspects to everyday life while we search towards pharmacological solutions, that is a winning combination.”