Everyone should be able to breathe clean air with healthy lungs, yet sadly far too many people continue to gasp for breath because of lung disease. In the UK, one in five people develop some form of lung condition in their lifetime. The challenge of developing better diagnostics, treatments and cures is one that faces us all, and the British Lung Foundation (BLF) is here to provide hope, help and a voice to everyone who is left breathless by a lung condition.
Our understanding of how different types of lung disease develop has improved in recent years, which is accelerating the development of better care for more and more people. For example, innovative approaches to understanding how lung cancer develops and changes over time is paving the way for new treatments that will hopefully replicate the success seen in treating other cancer types. Efforts by governments across the UK to ban smoking in cars and in public places has reduced people’s exposure to second-hand smoke, helping to clear the air for everyone to breathe easier in their daily lives.
Although advances have been made in diagnosis and treatment, too many types of lung disease remain without adequate care or cure. To take just one example, idiopathic pulmonary fibrosis (IPF) has no cure and limited treatment options, with 3 years the average length of time that patients have left to live after their diagnosis. With 20% of all deaths in the UK resulting from various forms of lung disease, it’s never been more urgent for lung disease research to receive the priority and support that it deserves.
Since the BLF was formed over 30 years ago, we have spent over £30 million on research grants for a wide range of forms of lung disease – focusing our efforts on diseases which receive much less attention, like mesothelioma, IPF and chronic obstructive pulmonary disease (COPD). We are committed to funding outstanding research which offers real hope to patients right across the UK – research supported by the thousands of generous donations we received from people whose lives, or the lives of their loved ones, have been devastated by lung disease.
Personalised approaches to tackling IPF
Idiopathic pulmonary fibrosis (IPF) causes scars to form on lung tissue over time, which hardens the lungs and makes breathing more and more difficult. It is not clear what causes IPF to develop, but what is clear is that it blights the lives of 32,500 people every year in the UK. Understanding the biological processes that lead to the scarring of lung tissue, and ultimately preventing, halting or reversing them, is therefore a top priority in tackling this horrendous disease.
With advances in genomics and DNA technology, we have unprecedented opportunities to look at the full range of genes which are expressed in patients with IPF and understand the role our genes play in the cellular processes which cause scar tissue to form and spread. Professor Louise Wain, our BLF Chair at the University of Leicester, has analysed the genomes of patients with IPF in the UK and found that the A-Kinase Anchoring Protein 13 (AKAP13) gene is more strongly expressed in patients with IPF. Since AKAP13 is already understood to be involved in tissue fibrosis, there is an exciting opportunity for new drugs to be targeted to inhibit this protein, and therefore the tissue fibrosis pathway in the epithelial cells of the lung. In turn, this could halt the development of scar tissue and offer a new treatment for patients with IPF. Analysing and understanding the genome, and the patterns of gene expression associated with respiratory disease, has uncovered new and, in some cases, unexpected factors at play in the development of lung disease. Understanding how and why the genome operates in the way that it does is crucial in finding clinically effective treatments for IPF and other forms of lung disease.
The goal of personalised IPF treatment is within sight, aided by a growing understanding of the biological mechanisms which causes scar tissue to form in the epithelial cells in the lungs. Professor Toby Maher, another of our BLF Chairs, has made important strides in understanding the underlying cellular processes that causes IPF to take hold. Professor Maher has extensively studied the presence and variety of bacteria in the alveoli of patients with IPF. Examination of patients with IPF has shown that they have higher numbers of harmful bacteria in their lungs, which helps us understand which proteins are manufactured by the body in response. Professor Maher has also identified elevated levels of epithelium-specific proteins in the blood of patients with IPF – a finding which enhances our understanding of the role of epithelial cell damage in the development and worsening of symptoms of IPF. This research paves the way for new treatments which can tackle the underlying processes that drive IPF and eventually will bring forward the day when IPF is no longer the death sentence that it is currently.
Alleviating inflammation from bronchiectasis
Bronchiectasis occurs when the lungs become permanently enlarged, which results in abnormally high levels of mucus forming in the lungs. Inflammation in the airways, caused by infections facilitated by the increased mucus production, can cause permanent tissue damage. Although not usually fatal on its own, the excess mucus that builds up in the lungs from bronchiectasis can leave people vulnerable to other serious infections, like tuberculosis and pneumonia. BLF statistics suggest that 210,000 people are living with bronchiectasis in the UK, with many cases going undiagnosed for several years. Professor James Chalmers, BLF Chair at the University of Dundee, is seeking to change the prospects for people living with bronchiectasis by examining the phenotypes and bacterial infections associated with bronchiectasis, so that treatments can be targeted more effectively.
Professor Chalmers’ research has also shone a light on the impact of bronchiectasis on quality of life for patients and how their living environment affects their ability to cope with their disease. By analysing the detailed medical records of patients in Dundee and Perth and comparing it with data on air pollution levels in the city, Professor Chalmers has demonstrated that high air pollution levels correlate with increased admissions to hospital and GP practices in NHS Tayside. This research has made a real difference to the public understanding of the dangers of air pollution to public health and is shaping the public debate on measures to reduce air pollution in towns and cities in the UK. Making a compelling case for curbs on polluting vehicles to be the priority of governments across the UK is now one of our top priorities, thanks to the high-quality evidence provided by the scientists we fund.
Building a world where everyone can breathe clean air with healthy lungs will continue to be a major challenge in the years to come. Thanks to the promising efforts of our researchers, the future looks much more hopeful for people with lung disease, although there is much more work to be done to find new treatments and cures. With the continued generous support of our patients, supporters and donors, we will strive to find safe and effective cures for all forms of lung disease, so that everyone can breathe easier for generations to come.