Frequently Asked Questions
What is the PFF Registry?
The PFF Registry is a distinctive database that 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 clinical and self-reported outcomes data from PF patients as well as lung transplant recipients with PF, family members, and caregivers.
A clinical data registry records, analyzes, and reports information about a patient’s health status and care received over time in order to improve health outcomes. Unlike a clinical trial where subjects must follow a specific treatment protocol, a registry captures data from a patient’s usual healthcare and experience via a secure online portal.
The PFF Registry has two distinct data sets: The PFF Care Center Registry, established in 2016, collects medical data from up to 2,000 patients and is administered by PFF Care Centers across the country. The PFF Community Registry collects ongoing participant-reported data directly from PF patients, PF patients who are lung transplant recipients, caregivers and family members.
How does the PFF Registry differ from a clinical trial?
The PFF 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. Unlike a clinical trial where subjects must follow a specific treatment protocol, in a registry, data is captured on a subject’s ongoing care. A registry does not prescribe what should be done or require additional medications or visits. It simply captures real-world data to help researchers learn about actual care patterns, observe subjects, and measure and improve quality of care.
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 has generated an unparalleled resource for research and also provides 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. As we look ahead, we will continue to track clinical data from the 2,000 patients and also expand into the community to collect data from PF patients, lung transplant recipients who had PF, 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) scans, and an optional blood sample from PF patients who have consented to participation. Clinical information includes patient demographics, diagnosis, medical history, and medications and treatments. We also track PF test measurements for DLCO, or the Diffusing capacity of the Lungs for carbon monoxide, and FVC, or Forced Vital Capacity. Participants are asked to 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 are helping 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 the PFF medical team and staff with advisory support from the 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 develops and manages the Registry, 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.
As a researcher, how can I get access to data from the PFF Registry?
Researchers can access the PFF Registry upon submission and approval of a research proposal. Both academic and industry researchers are welcome to apply. Investigators who are not members of a PFF Care Center must include a PFF Registry investigator as a collaborator on their proposal. The PFF Medical Team, PFF Registry Steering Committee, and Scientific Review Committee currently review research proposals on a rolling basis. All proposals are reviewed and scored by a review panel. Investigators of approved proposals will be contacted by a statistician from the data coordinating center 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 firstname.lastname@example.org. For companies interested in utilizing the PFF Registry for research purposes, contact email@example.com to learn more about the application process and fees.
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 firstname.lastname@example.org.
Patient Powered. Data Driven. Research Results.