Together we can transform the lives of people with pulmonary fibrosis
We have partnered with the James Lind Alliance and Imperial College London alongside a Steering Committee including patients, carers, family members and clinicians and our wider community to identify the Top 10 priorities for future research into pulmonary fibrosis. These priorities will help set future priorities for generations of researchers in the UK and across the world, and ultimately take us closer to finding a cure.
We are excited to announce the Top Ten Progressive Pulmonary Research Priorities are:
- How can the diagnosis of PPF (progressive pulmonary fibrosis) be improved in terms of accuracy and the time taken (screening programme, early signs and symptoms that could be detected in primary care, blood markers, imaging, biopsy, artificial intelligence, etc.)?
- Can new treatments other than pirfenidone and nintedanib slow, halt or reverse the progression of PPF?
- What can be done to improve the speed and accuracy of PPF diagnosis in primary care (e.g. training, integration of case-based studies in GP training, awareness campaigns)?
- What is the best time for drug and non-drug interventions (pulmonary rehab, oxygen therapy, psychological support) to start to preserve quality and length of life for patients with PPF?
- What are the best ways (drug, non-drug and aids) to treat cough in PPF?
- Would early treatment delay progression, lung function decline, and improve survival in PPF?
- Which therapies will improve survival in PPF?
- What treatments (drug, non-drug and aids) can reduce breathlessness and phlegm production in PPF?
- To what extent do different interventions (pulmonary rehab, oxygen therapy, psychological support) impact length of life in patients with PPF?
- Can new treatments for PPF be developed with reduced side effects? Does how the drug is delivered (e.g. oral, nebulised, through a vein) affect potential side effects of the drug in PPF?
How did we reach these priorities?
The Priority Setting Partnership took nearly 2 years to complete and involved people with lived experience of pulmonary fibrosis and health care professionals throughout the process.
Thank you to everyone who took part. We hugely value your experiences and the influence you will have on future research and the lives of anyone affected by pulmonary fibrosis
Louise Wright, CEO Action for Pulmonary Fibrosis
- A steering committee was established, including patients, carers and clinicians to help guide the project.
- Survey 1: People affected by pulmonary fibrosis and healthcare professionals were invited to tell us what they felt the unanswered questions were in PF research. Over 600 people responded with more than 2000 single questions and statements! These statements were carefully analysed and grouped in themes leading to a shorter list of questions. We checked against the existing evidence, and refined the questions to include only those unanswered by scientists.
- Survey 2: We invited people affected by pulmonary fibrosis and healthcare professionals to prioritise the shortlisted questions in order of importance. Over 800 people told us what their ten priorities would be from the longer list. The most popular priorities were taken forwards to the next stage.
- Shared decision making 2-day workshop: A group of over 20 people affected by pulmonary fibrosis and healthcare professionals worked together to prioritise the order of the shortlisted priorities for research. This was a rigorous and collaborative process. After 2 days of intense discussion, listening and learning, they decided on the Top 10 priorities in progressive pulmonary fibrosis research.
Why is the question I chose not included and does this mean it won't be answered?
Deciding upon the final Top 10 research priorities was incredibly challenging. The number of unanswered questions highlights the continuing desperate and unmet needs of people living with pulmonary fibrosis. Questions that are not included in the Top 10 remain very important to be answered. All the identified unanswered questions will be available for researchers to see on the JLA website and the complete findings of the process will be shared widely.
Deciding between the different priorities was really difficult. How do you choose between prioritising helping patients with a debilitating cough or severe breathlessness? – everything feels urgent.
Priorities for research are normally defined by funders, researchers and industry. Some of the questions that did not fall within the JLA Top 10 are already general priorities for funders, for example, understanding the biological basis of diseases. However, we know that significant increases in investment in PF research are needed to improve our understanding of the disease so that we can find a cure.
We will continue to campaign, raise awareness, and work with researchers to ensure everyone affected by the disease has a better future. You can learn more about our priorities for funding research here.
What happens next?
We are taking action by investing £600,000 in pulmonary fibrosis research. We will be spreading the word about these priorities, including through publication in journal articles, presentations at conferences, and sharing across our social channels. These priorities will help us identify gaps in funding and support our decisions in campaigning and awareness raising. Over the next few years we will review our progress and ask ourselves - which questions are now being answered in research as a result of this work? Where are there still gaps? We will ensure there is a strategy for investing time and funding across the research priorities with a focus on collaboration and innovation.
What else can I do to take action for pulmonary fibrosis?
- Share the priorities with your support group or others that you know are affected by pulmonary fibrosis
- Share with your healthcare professional / consultant
- Find out more about our research