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Hi, my name is Anna, and I come from a family in which pulmonary fibrosis (PF) appears to be hereditary.
My kind, gentle Grandad died of it in his 70s (I was called home from school to wave to him as they wheeled him out of our house for the last time), and my beautiful, brave Mum died of it at 65 when I was in my 30s. Thankfully, the rest of my family are doing OK, but it’s made the scientist in me ask a lot of questions and want to find the answers.
PF is often described as a ‘disease of premature ageing’, meaning some of the body’s cells start to age sooner than they should. Knowing this, imagine my fascination when watching a sci-fi film one Saturday night, and I hear that ‘telomeres’ are the parts of cells linked with ageing. It set me wondering about my first big question: “What happens if your ‘telomeres’ are not working properly? Could this cause PF?”
I started reading about telomeres and discovered that they’re the end caps of DNA strands. These caps protect the DNA, a bit like how the plastic bits on the ends of shoelaces stop the shoelace from fraying. Millions of the cells in our body divide every day, and to do this, the DNA in those cells has to be copied. Every time the DNA is copied, a little bit of the telomere is lost, and the ‘cap’ gets smaller.
For most people, the telomeres are long enough to last into old age, but for some, they get too short too soon. When they get too short, it can stop the cell from working properly. If this happens in the lungs, it can result in scarring (fibrosis).
I found this so interesting, and with my background in physics research, I wanted to get involved in PF research. I earned a place to do a PhD in PF genetics research at Exeter University. I work with great people, and together we published my first findings in The Lancet Respiratory Medicine. We found that, in some circumstances, short telomeres can cause idiopathic pulmonary fibrosis.
Our research shows that people who get PF might have lower levels of sex hormones than those of the same age. There also seems to be a link between the length of people’s telomeres and the levels of their sex hormones, so it all fits together.
My next big question is whether sex hormones (like oestrogen and testosterone) might help to stop telomeres from getting too short.
Since early 2022, we’ve been running a study to find out if people with PF have low levels of sex hormones. We’re also investigating if we can safely run a trial to increase the level of sex hormones. It’s an enterprise called STARSHIP (Study of Telomeres and Role of Sex Hormones in PF), and we hope it will boldly go far!
After I finish my PhD, I have gained part-time funding from the NHS to continue my research work.
Anna’s PhD research is funded by the Medical Research Council.
If you would like to hear more about Anna's research, you might like to watch her talk at the Talking PF 2023 Webinar: Session 1
Every hour of every day, someone around the globe is working towards a better understanding of pulmonary fibrosis, so we can get closer to stopping this disease. At present, it seems pulmonary fibrosis is linked to a wide range of factors, and as such, there is no simple understanding of why anyone gets the disease.