By Deborah Borfitz 

September 21, 2022 | In July, the Pulmonary Fibrosis Foundation (PFF) launched the PFF Community Registry to begin collecting data directly from patients in the U.S. with pulmonary fibrosis (PF) and interstitial lung disease (ILD)—together with their nonprofessional caregivers and biological family members—as well as lung transplant recipients previously diagnosed with PF or ILD. The new database will complement an existing PFF Patient Registry established in 2016 tracking medical data from 2,000 patients served by PFF Care Centers nationwide, according to Junelle Speller, MBA, vice president of the PFF Registry.   

Relative to the over 250,000 individuals in the country estimated to be affected by PF and ILD, the Patient Registry represents a fraction of the data that might be gathered to understand the true impact of the diseases, says Speller. PF and ILD are also rare conditions and, as such, the data pool is relatively small without a dedicated effort to capture as much of the available information as possible to paint a clearer picture of those diseases. 

There is no enrollment ceiling with the PFF Community Registry, which takes data collection beyond the four walls of the Care Centers and into additional settings and geographies, Speller says. After only a month of its public launch in mid-July, the Community Registry had close to 500 enrollees from most states in the country, accomplishing one of its goals in terms of geographic diversity. 

Pivotal to the creation of the original Patient Registry, and growth and development of the Community Registry, is pulmonologist Kevin Flaherty, M.D., steering committee chair of the PFF Registry. "Data collected from the PFF Community Registry provides researchers with a better understanding of the experience of individuals living with or affected by PF and, in turn, a unique look at how the disease impacts the lives of their caregivers and family members. The information gathered will help researchers gain valuable insights into fibrotic lung disease and effective methods to improve patient care for years to come." 

While clinical registries have been a growing phenomenon in the rare disease space dating back to the 1970s, community registries where patients self-report to a database—requiring both their trust and ongoing engagement—are newer to the research scene. 

Collection Methodologies 

Individuals in the Community Registry will be surveyed at baseline and then every six months thereafter regarding any new diagnoses, medication changes, acute disease exacerbations, trips to the hospital, and other changes to their health and healthcare, says Speller. The questions are aligned with those from the Patient Registry to allow comparisons between what is reported by healthcare professionals and how patients perceive their diagnosis and care.   

For both registries, patients consent to participate, she says. They also directly answer survey questions about their quality of life, including the impact of various symptoms such as cough and shortness of breath. 

Questions are asked of caregivers and family members regarding the impact of a loved one’s disease on their wellbeing, adds Speller. “There is sometimes a family linkage with pulmonary fibrosis through various generations and, even within one generation, there may be... [multiple relatives] with the same disease, so we want to look at that data as well.” 

Community Registry enrollees will also be asked additional questions about their participation in support groups and their educational needs and preferences, she continues. The PFF will look at some of that data with an eye toward identifying opportunities to better serve patients. 

But the big difference between the Patient and Community registries is the collection methodology, says Speller. Instead of the information coming largely through clinicians at the PFF Care Centers and extracted from patients’ medical records by an on-site clinical coordinator, data in the new Community Registry is being captured 100% from patients as well as lung transplant recipients, caregivers, and family members.  

The initial enrollment questionnaire takes an estimated 45 to 60 minutes to complete, she says, and intended to be no longer than necessary for the capture of as much relevant data as possible. Follow-up surveys should take much less time. “We’re looking at an annual effort in terms of time to be about two hours.”  

Research Purposes 

Information from the PFF Patient Registry already has been or currently used in over 40 studies, reports Speller. Among these are studies on the characteristics of patients selected for transplants or associated with supplemental oxygen use; outcome differences based on sex, race, or smoking status; and the effects of anticoagulants on survival in patients with PF and ILD. 

Potential research purposes for data in the new Community Registry include patient perceptions about their care and diagnosis and the experienced burden of caregivers, as well as investigations specific to lung transplant recipients.  

The expanded dataset will open opportunities for researchers to develop sub-studies focusing on a particular cohort of patients—individuals who have had military experience, for example, or those with a specific type of PF (e.g., idiopathic pulmonary fibrosis), she continues. Optional surveys, beyond the broader biannual ones, would be pushed out as needed to the relevant populations. 

A multi-pronged recruitment and retention campaign around the Community Registry includes invitation-to-join emails and mentions in newsletters sent to the roughly 46,000 individuals in the PFF database as well as social media posts with information and a call to action, the PFF Registry microsite, and engagement with PFF support groups and ambassadors, says Speller. Printed one-pagers have also been produced for distribution to patients, lung transplant recipients, caregivers, family members, researchers, and other groups. 

Engagement efforts are focused on providing participants with follow-up data and updates on the status of the registry and the research projects that their information sharing has enabled, she adds. A biannual newsletter is also planned where the PFF will provide more detailed coverage on the Community Registry and perhaps profile some of researchers.