FTD Registry Publishes First Full-length, Peer-reviewed Article in 2022
The FTD Disorders Registry in collaboration with the Association for Frontotemporal Degeneration (AFTD) published its first full-length, peer-reviewed journal article in August 2022.
“Living With Frontotemporal Degeneration: Diagnostic Journey, Symptom Experiences, and Disease Impact” was accepted by SAGE Journals in the Journal of Geriatric Psychiatry and Neurology and published online on August 9, 2022.
Access to the article has been made available for free to the public through a generous grant from AFTD.
The survey was designed to better understand what people affected by FTD consider important regarding possible treatments and participating in clinical trials.
"Published research that highlights the lived experience of FTD and what matters most to people impacted by FTD is extremely valuable,” said co-author Shana Dodge, Ph.D., MA, Director of Research Engagement at AFTD. “The FDA makes decisions, such as approving drugs and weighing the risks and benefits of potential treatments, based on what is meaningful to the people who might take the drug. This data also helps researchers and healthcare providers better understand the impact of FTD."
The article looks at a subset of data from the FTD Insights Survey, which was conducted by AFTD and the FTD Disorders Registry from October 2020 through March 2021. Nearly 1,800 people — including individuals diagnosed with FTD, family members, and past and current care partners — completed the 191-question survey.
The FTD Insights Survey was developed based on input from the FTD Registry, AFTD, as well as clinical and scientific experts on FTD. Core survey questions were developed from existing surveys designed and used by the FTD Disorders Registry. The survey was piloted by people with FTD, their caregivers, and family members, and questions were refined as a result of their feedback.
The Survey covered several broad areas related to the diagnostic journey, disease symptoms and their impacts on daily life, experiences with past treatments, and hopes for future treatments, perspectives on genetic testing, as well as perceptions of research and willingness to participate in future studies.
“This initial data analysis was focused on the lived experiences of those with early to mid-stage FTD,” said former FTD Registry Director Dianna K.H. Wheaton, Ph.D., senior author and corresponding author. “We were so very fortunate that strong community response provided broad representation of the FTD journey and disease subtypes.”
Stage of disease for this analysis was defined as mild (needs little supervision to perform daily tasks), moderate (needs supervision to perform most daily tasks), severe (needs full supervision to perform nearly all daily tasks), or profound functional impairment (can no longer perform daily tasks and has limited mobility and ability to communicate).
“While we are keenly interested in describing the full spectrum of disease impact, we felt an urgency to share data that might be highly applicable to guide current and emerging clinical treatment trials,” said Dr. Wheaton. Individuals diagnosed with FTD, as well as care partners for persons whose disease was within the mild to moderate stage, were included in this subset of 656 survey responses (219 diagnosed individuals; 437 current care partners).
The article tells the story of the diagnostic journey, symptoms, and the impact of FTD on distress, quality of life, and independence.
“Overall, by heeding the perspectives of those living with FTD, we can begin to design more meaningful research studies, provide better care, and develop therapies that improve quality of life,” the article noted.
The full dataset from the FTD Insights Survey is a rich source of information for continued data mining and sharing. Numerous follow-up data analyses are planned.
AUTHORS
In addition to Dr. Wheaton and Dr. Dodge, additional authors of the article include:
- Megan Barker, Ph.D., Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Columbia University Irving Medical Center, New York, NY
- Debra Niehoff, Ph.D., AFTD
- Sharon Denny, MA, AFTD
- Penny A. Dacks, Ph.D., AFTD
- Susan Dickinson, MS G.C., AFTD
- Stephanie Cosentino, Ph.D., Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Columbia University Irving Medical Center, New York, NY; Gertrude H. Sergievsky Center, Columbia University Irving Medical Center, New York, NY; Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY
ABSTRACT
Frontotemporal degeneration (FTD) is an umbrella term encompassing a range of rare neurodegenerative disorders that cause progressive declines in cognition, behavior, and personality. Hearing directly from individuals living with FTD and their care partners is critical in optimizing care, identifying meaningful clinical trial endpoints, and improving research recruitment and retention.
The current paper presents a subset of data from the FTD Insights Survey, chronicling the diagnostic journey, symptoms, and the impact of FTD on distress, quality of life, and independence, in the mild to moderate stages of the disease. Survey respondents included 219 individuals diagnosed with FTD and 437 current care partners, representing a range of FTD diagnoses.
Around half of the survey respondents reported seeing three or more doctors before an FTD diagnosis was given, and a range of prior diagnoses were noted.
Most frequently endorsed symptoms tended to be consistent with clinical characteristics of the specific diagnosis, though there was significant variability in symptoms reported within diagnostic categories as well as considerable overlap in symptoms between diagnostic categories.
Cognitive and language symptoms of FTD were generally most distressing to the person diagnosed, and a loss of independence was endorsed as affecting quality of life.
The distinct perspectives of diagnosed persons and care partners regarding disease impact differed notably for bvFTD/Pick’s disease. Participating independently in a range of activities, within the home, outside the home, and with other people, were reported as challenging for people living with FTD, underscoring the degree to which the lives of these individuals are affected even at the mild and moderate stages of disease.
Overall, by heeding the perspectives of those living with FTD, we can begin to design more meaningful research studies, provide better care, and develop therapies that improve quality of life.