Frequently Asked Questions
What is the PFF Registry?
The PFF Registry is a distinctive database that that enrolls and follows the care of patients with broadly defined interstitial lung diseases including idiopathic pulmonary fibrosis, connective tissue disease, hypersensitivity pneumonitis, and other diagnoses. The PFF Registry collects deidentified clinical and self-reported outcomes data from patients as well as lung transplant recipients, family members, and caregivers.
Launched in 2016, the PFF Registry initially gathered clinical data from over 2000 patients across the PFF Care Center Network. Looking ahead in 2021, the Registry will expand to included two arms: (1) Direct-to-Community and (2) Clinical. The Direct-to-Community arm features an online portal for participant-reported data. All participants will complete an initial questionnaire on demographics, basic medical history, and lifestyle. A portion of these participants can elect to complete periodic surveys on general health, treatment, and other specific topics such as risk factors, exposure, and oxygen use. All participants will receive regular email communication and quarterly newsletters with updates on the Registry and timely information and education on PF.
The Clinical arm will continue to collect from up to 2,000 patients across the PFF Care Centers. Data includes diagnosis, labs, tests, medical events, and treatments. The Clinical arm also includes questions on COVID to determine participants’ experience with diagnosis and treatment.
How does the PFF Registry differ from a clinical trial?
The PFF Patient Registry is an observational research tool. That means we record participants’ experiences with PF to learn all we can about the disease and how to treat it. Participants don’t have to change their medications or how often they see their physician. It allows us to capture real-world data in a naturalistic manner; learn about actual care patterns; observe subjects for longer periods; and measure and improve quality of care. We also are able to observe a wider group of the PF population.
The Registry is not designed to answer questions about whether one treatment is better than another—that is the purpose of clinical trials. The Registry is helping to answer other important questions like:
- What factors determine who should take anti-fibrotic treatments?
- How does the use of supplemental oxygen change the risk of being hospitalized?
- How do hospitalizations affect survival?
- What role does patient race and gender play in survival?
What are the goals of the PFF Registry?
Research is the pathway to a cure for PF. The Registry’s combination of data from so many patients, including those with less-studied forms of PF, will generate an unparalleled resource for future research into treatments and provide data for improving and reassessing evidence-based care guidelines.
When the Registry was launched in 2016, our initial target was the collection of data from 2,000 patients with all kinds of PF within the PFF Care Centers. This database offers the largest research resource of its kind in support of the following goals:
- Collect medical information to support research that develops new ways to improve the quality of the lives of people with PF
- Create a Registry of patients with PF for participation in additional research studies in the future
- Collect data on the patterns of clinical care delivered at PFF Care Centers, which can improve our understanding of the most effective ways to care for people with PF
As we look ahead, we will continue to track clinical data from the 2,000 patients and also expand to the community to collect data on patients who receive care in community and rural centers, lung transplant recipients, family members, and caregivers. Key aims follow:
What kind of data are collected in the PFF Registry?
The PFF Registry collects clinical information, high-resolution computer tomography (HRCT) scan, and an optional blood sample from PF patients who have consented to participation. Clinical information includes patient demographics, diagnosis, medical history, walk studies, pulmonary function tests, and medical events. We track PF test measurements for DLCO, or the Diffusing capacity of the Lungs for carbon monoxide, and FVC, or Forced Vital Capacity. Participants also complete the following four patient-reported outcomes (PROs) questionnaires at enrollment and at 6-month follow-up intervals: General Quality of Life; Cough; Fatigue Severity Scale; and Shortness of Breath Scale.
What is the PFF Registry Biorepository?
PFF Registry participants may choose to contribute a blood sample to be stored for future research. This blood is divided into plasma, serum, RNA, and DNA, stored in a central lab, and provided to researchers along with key data to study biomarkers of PF, genetic and genomic influences on the disease, and to guide precision medicine studies. When combined with the information in the Registry, these samples will help scientists find new causes of PF, track the effects of PF treatments, improve the ways doctors diagnose and manage people living with PF, and help discover new treatments. Samples are deidentified along with all other clinical data.
What is an HRCT scan?
HRCT, or high-resolution computed tomography, is a special type of x-ray scanner that takes very detailed pictures inside the body. These scans are commonly used to diagnose and track progression of PF.
What is DLCO?
DLCO, or the diffusing capacity of the lungs for carbon monoxide, is one of the most clinically important tests of lung function. DLCO measures the ability of the lungs to transfer gas (like oxygen) to the red blood cells.
What is FVC?
FVC, or Forced Vital Capacity, measures how much air you can exhale from your lungs during a test called a spirometry test. These tests are used extensively to track the lung function of patients and changes in this measure are key measures in PF research.
Why is blood collected from patients?
Researchers use the blood samples to study diagnostic and predictive biomarkers. Blood samples include DNA, RNA, serum, and plasma. All information is deidentified. The information is always de-identified, which means that researchers who use it can never learn whose medical data it is.
Who manages the PFF Registry?
The PFF Registry is led by PFF medical team and staff with advisory support from the CCN-Registry Steering Committee, a group of experts and investigators from the PFF Care Centers and other industry partners. The daily operations are managed by the data coordinating center, Statistical Analysis of Biomedical Education and Research (SABER) at the University of Michigan. The SABER team develop and manage the data base, work with Care Centers to ensure proper data completion, and partner with researchers to ensure that Registry data can support their proposed studies. Their support continues throughout the research process, up to and including preparing manuscripts for publication. For more information, click here.
What kind of research on the Registry has been developed so far?
As of December 1, 2020, there have been almost 40 Registry-based research projects that include posters, abstract, and manuscripts on basic/translational research, clinical research, and patient-centered research. To search our list of research projects, click here.
We also launched the study, PRECISIONS, to determine whether n-acetyl-cysteine actually improves outcomes for subjects with IPF. PRECISIONS is funded by a $22M grant from the National Institutes of Health and Three Lakes Foundation. And, earlier this year, the PFF joined forces with Bristol Myers Squibb and several industry partners to form the PROLIFIC Consortium, a new collaboration to develop tests to identify important biomarkers in PF. The biomarkers have been chosen for their potential to predict the disease course of PF and how well a drug will work in a specific individual.
How can I use the PFF Registry's data for my research?
The PFF invites interested clinical and translational investigators to utilize PFF Registry data. Both academic and industry researchers are welcome to apply. Investigators who are not members of a Registry team must include a PFF Registry investigator as a collaborator on their proposal. Researchers can access the PFF Registry upon submission and approval of a research proposal. The Foundation currently reviews research proposals on a rolling basis.
For more information on ancillary proposal studies and to submit a proposal, click here. If you have questions about submitting research proposals, contact us at firstname.lastname@example.org.
As a researcher, how can I get access to data from the PFF Registry?
Interested researchers must include a Registry investigator as a collaborator on their proposal. A list of these investigators can be found here.
All proposals will be reviewed and scored by a review panel. Investigators of approved proposals will be contacted by a statistician from the data coordinating center (DCC) to develop a statistical analysis plan (SAP). In most cases, analyses of clinical data will be performed by the DCC statisticians, following that SAP.
For more information on ancillary proposal studies and to submit a proposal, click here. If you have questions about submitting research proposals, contact us at email@example.com. For companies interested in utilizing the PFF Registry for research purposes, contact firstname.lastname@example.org to learn more about the application process and fees.
Sign Up for PFF Registry Emails
Be the first to know when the PFF Registry opens for enrollment
Your gift to
the PFF Patient Registry will enable us to expand patient enrollment and
provide even greater access to investigators as they search for a cure
Your Privacy Matters
The Pulmonary Fibrosis Foundation Registry considers the protection of your privacy to be one of our most important responsibilities. We have set up strict protocols to protect your personal information. To review our policy, click here
PFF Registry News
To view the latest news and updates about the PFF Registry, click here.
To get in touch with the PFF, please email email@example.com.
Patient Powered. Data Driven. Research Results.