Researchers are investigating 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. That’s the reality for people living with primary progressive aphasia (PPA), a rare form of dementia that usually begins in middle age and progressively reduces 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 measuring the significant impact of PPA on quality of life and demonstrating 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 such as Alzheimer’s dementia that primarily affects memory in the early 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 is often overlooked in the literature because it can be difficult to gather large groups of people to investigate lived experiences.”

He said it is often overlooked by medical professionals as well, as it is largely underdiagnosed among those with lower socioeconomic status.

Lack of diagnosis can be such a barrier to care for anyone who does not live near a specialist medical centre.’

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

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

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

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

Measuring the impact of PPA on quality of life

To better understand how PPA affects daily life, Rogalski and her colleagues 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 for 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 people with PPA was associated with lower quality of life, particularly affecting areas such as hearing, sensation, cognition and speech.

“It was important to confirm that the HUI, a widely used measure of health across all diseases, captured the essence of the primary damage in these patients,” Rogalski said.

Thomas Hopkins, PharmD, MS, the study’s first author, explained the twofold purpose of the study: to obtain detailed information about the quality of life of people with PPA and to enable direct, objective comparisons between the impact of PPA and that of other diseases . Since the Health Utilities Index is a general metric, it can be applied to any health condition -. even those not similar to 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 drive 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 just as dramatically as many other troublesome 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 the impact of PPA, Rogalski and her colleagues simultaneously enrolled patients in a clinical trial investigating the feasibility of providing speech therapy for PPA via telemedicine. They recently reported the successful enrollment of 95 pairs of participants -? each involving 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.

“We found a way to deliver care that creates a little bit more of a level playing field,” Rogalski explained. “People with PPA do not need to live near a large academic medical center or specialty center to see a specialist and receive treatment.”

This success also paves the way for future research and interventions -? and not only for the PPA.

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

Offering hope even if there is no cure

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

“We’re giving families a voice to share their experiences,” she said.

Despite these research advances, there are still challenges in diagnosing and treating PPA, and no drug or treatment has yet been developed that can cure the disease. However, expanding the understanding of the profound impact of PPA on quality of life and demonstrating the effectiveness of telemedicine interventions are important steps that can encourage patients and families.

“Giving these families hope and practical support is really important,” Rogalski said. “Too many people have had experiences where, even when they find a specialist, that specialist might 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 maximize independence, emotional well-being, self-confidence – all of which can have as many practical aspects to one’s daily life as the possible, while at the same time looking for pharmacological solutions, this is a winning combination.”