The Pulmonary Fibrosis Foundation (PFF) has launched a new initiative in which they hope to capture a far more diverse representation of patients with pulmonary fibrosis (PF) than the current registry allows them to do, a press release from the PFF indicated.
“The existing registry we have – the PFF Patient Registry – is limited to our care centers, which are primarily academic clinical institutions and we have only a few thousand patients within that registry,” Junelle Speller, MBA, vice president of the PFF Registry, told this news organization.
“We wanted to go beyond these care centers and capture patients in community centers, and in rural settings to provide a more complete understanding of patients with this disease and, of course, have a larger sample size,” she added.
“ across all parts of the United States and, most importantly, accelerate the research on PF toward improving earlier diagnosis, treatment, and outcomes for these patients,” Ms. Speller said.
Passive versus active
The PFF Community Registry differs in its structure and purpose from the PFF Patient Registry, as Ms. Speller explained. First, the PFF Patient Registry, established in 2016, is “passive” in its nature in that whatever information is entered into a patient’s electronic medical record or clinical chart on a routine office visit is abstracted and captured in the registry. By contrast, the PFF Community Registry is asking for self-reported data from patients, “so it’s more of an ‘active’ registry and will give us a chance to have a bidirectional connection with participants, provide email updates and newsletters, and give patients an opportunity to participate in future studies within the registry as well as in clinical trials,” she noted.
The two registries still overlap in that both capture demographic data on patients’ medical and family histories as well as any medications patients may be taking, but the Community Registry will also capture information with respect to education, employment, patient-reported outcomes, and quality of life metrics. “It will also let us know how patients feel about continued education on the disease itself and patient participation in support groups,” Ms. Speller observed.
The Community Registry will also collect information from lung transplant recipients who have had PF or any other form of interstitial lung disease (ILD) as well as information from caregivers and family members affected by the patient’s disease. As Ms. Speller noted, both PF and other forms of ILD (of which there are more than 200 types) are all characterized by inflammation or scarring in the lung. “Patients are often misdiagnosed, and it can take months, even years, to identify the disease,” Ms. Speller said.
From there, it can be a very long and difficult road ahead, with no cure in sight, although several antifibrotic drugs do help slow disease progression. Typically, onset is around the age of 60 and symptoms include chronic dry cough, fatigue, shortness of breath, weakness, discomfort in the chest, and sometimes unexplained weight loss. Some patients do have a history of smoking, but not all, Ms. Speller noted. So far, registry data suggest PF largely occurs in White patients.
“We’re very excited about the Community Registry, particularly about reaching into communities that we haven’t been able to reach with our existing registry,” Ms. Speller noted. “The rural population in particular is often underserved, so we are really looking forward to capturing data from these patients as well as those from community centers within smaller and larger cities,” she observed.
“A powerful aspect of the Community Registry is that we can use the information gained from it to understand the experience of individuals living with PF, and how it impacts their lives and those of their families and caregivers,” Kevin Flaherty, MD, steering committee chair, PFF Registry, said in a statement.
“Researchers can also look at the data to better understand fibrotic lung diseases and learn about effective approaches to improve patient care,” he added.
Patients who wish to join the PFF Community Registry can sign up at pffregistry.org. To learn more about PF and its risk factors, readers are invited to visit www.AboutPF.org. More than 250,000 patients in the United States are living with either PF or other types of ILD.
Ms. Speller and Dr. Flaherty disclosed no financial conflicts of interest. The PFF Registry is supported by its founding partner, Genentech, Visionary Partner, United Therapeutics, and its sustaining partner, Boehringer Ingelheim, as well as many donors.
A version of this article first appeared on Medscape.com.
This article was updated 8/8/22.