University Seeks Patient, Caregiver Help to Define Future PF Research

Scientists at the University of Nottingham, in the U.K., are asking pulmonary fibrosis (PF) patients — many of whom donate “gifts” of cells and samples — and their caregivers to help define the institution’s future research goals.

These researchers, from the university’s faculty of medicine and health sciences and the Nottingham Biomedical Research Centre (NBRC), are asking people with PF, as well as their caregivers and healthcare professionals, to complete a short survey.

Their aim is to get direct patient and caregiver input in determining which research questions in PF they should tackle in the future.

“This is an opportunity for people with pulmonary fibrosis and their carers to have their say on our research plans for years to come,” the researchers wrote on the survey webpage.

Titled “Understanding Patient Priorities for Scientific Research on Pulmonary Fibrosis,” the short survey should take respondents just 5–10 minutes to complete.

The investigators’ work at the University of Nottingham has sought to identify the signals and cell behavior that contribute to PF — and which, therefore, could serve as potential therapeutic targets — using cells and lung samples donated by patients with the lung-scarring disease.

These samples “are valuable gifts that have helped us to do our research for many years,” the investigators wrote.

“We want to know what patients and their carers think about the research we should do in future, to make sure that we are using these samples in a way that is important to them,” they wrote.

In particular, the university’s Respiratory Research Unit has been focused on integrin signaling, activation of TGF-beta 1 — a key mediator of tissue scarring (fibrosis) — and extracellular matrix stability.

The extracellular matrix is a network of molecules found outside cells that provides structural and biochemical support. However, in people with PF, extracellular matrix components are overproduced and the matrix itself is unusually stiff.

Integrins are a class of proteins involved in the adhesion of cells to each other and to the extracellular matrix, controlling a wide variety of cellular processes. Abnormal integrin signaling has been associated with several diseases, including those related to tissue scarring, and some integrins are known to activate TGF-beta 1.

The short survey, which first collects participants’ demographic data, asks respondents to classify the importance of five research questions on a scale of 1 to 10, with 1 being “not important” and 10 being “very important.”

The questions are:

• Why do small amounts of lung scarring sometimes grow, resulting in pulmonary fibrosis?
• Why do lung cells in people with pulmonary fibrosis behave differently to those in healthy people?
• How do signals from physical changes like scarred lung and stretch from breathing cause pulmonary fibrosis to get worse?
• How do different kinds of lung cells talk to one another to make areas of lung scarring grow?
• How can a person’s surroundings affect their genetic code and cause changes that lead to pulmonary fibrosis?

Participants do not need to provide any contact details and will not be identifiable in any analysis of the survey’s results, the researchers said. The university may use the information generated in applications for research funding and also may publish the survey results on its websites and social media channels.

“We will use your survey answers to help to decide which research questions to try to answer with our future research,” the investigators concluded.