Idiopathic pulmonary fibrosis (IPF) is a rare condition with no known cause and no effective treatments besides a lung transplant. Current therapies focus on slowing progression of the disease but patients quality of life is still severely affected. It is seen most frequently in adults. IPF is the most common form of fibrotic lung disease and unfortunately, it’s also the most severe. About 20% of those with lung fibrosis have IPF.
IPF causes major damage to the interstitium in the lungs and the blood vessels throughout them. Specifically, it causes scarring and stiffening of the interstitium. IPF is progressive, resulting in fatigue, aching muscles, shortness of breath, dry cough, and other debilitating symptoms. Most patients lose their life to respiratory failure within five years of their diagnosis.
Two new grants awarded to the Translational Genomics Research (TGen) Institute, totaling 6.1 million dollars, will support new research into this condition with a high unmet need. Hopefully, it will lead to new effective therapies for patients. Ultimately, researchers believe this new investigation will be extremely valuable not only for determining the cause of IPF, but of all other forms of lung fibrosis as well.
Newly Funded Research
Nicholas Banovich is an Assistant Professor at the institute. He first became interested in IPF research when his own friend developed the disease. He knew there had to better way to help patients and he was determined to find it.
The first grant was given from the NIH to both the TGen institute, Vanderbilt University (VUMC), as well as the Norton Thoracic Institute. This grant is focused on determining the cause of IPF and it totals 3.5 million dollars.
The second grant was given to VUMC and TGen from the Department of Defense. It is focused on examining genomic factors associated with other forms of pulmonary fibrosis. This grant totals 2.6 million dollars.
The aim of the research is pretty straightforward- determine the cause of IPF at the molecular level. That has been one of the biggest setbacks so far in terms of developing new therapies. For if researchers don’t understand the underlying cause of disease it is extremely challenging to determine the most effective way to treat it.
What they plan to do is create a comprehensive list of the characteristics which are different between healthy lungs and IPF lungs. To do this they will conduct single cell sequencing. Single cell sequencing allows them to see the gene expression within each individual cell. From here they can identify their function and how they vary between healthy and sick patients.
The samples for this study will be taken from patients who are receiving lung transplants. Volunteers will donate tissue from their scarred lungs to be studied. In this way, patients are able to contribute firsthand to research. Researchers explain that patients know that even if their research does not result in an effective cure for them, it could drastically alter the lives of future patients. Their donation is truly an invaluable gift to science.
The hope is that 1) by understanding the molecular changes which cause IPF, patients will be able to be diagnosed faster and 2) once diagnosed, there will be better treatments available for patients.
Thanks to this new financial contribution, researchers will be able to begin working on this influential research which could drastically alter the lives of those living with IPF currently in addition to those who will be diagnosed in the future.
You can read more about this new research here.