Tasmania has the highest incidence of Cystic Fibrosis (CF) of any state in Australia, with approximately 110 people with CF. One of the most dramatic impacts of CF is a dysfunctional immune response – this includes a hyper-inflammatory response that often proves ineffective when faced with ongoing respiratory infections. The associated impact on health and wellbeing for those living with CF can be significant.
Local research undertaken by Dr Louise Roddam and her team, in collaboration with researchers at the University of Sydney and funded by the Royal Hobart Hospital Research Foundation, has recently explored the widespread nature of immune dysfunction in people with CF. Most particularly, the team has defined the nature of dysfunction at the greatest level of detail compared to any studies undertaken to date. As a result of this analysis, they have observed a difference in important regulatory immune cells amongst people with CF when compared to the wider population. Notably, this confirms a negative impact on lung function and also reflects lung tissue damage.
This finding is important because this investigation can now be used to continually monitor lung function and disease progression in people with CF. In advancing the techniques used by Dr Roddam and her team, future analyses can now also be undertaken using only very small volumes of peripheral blood (about half a teaspoon), far less than through previous approaches.
Coupled with these steps forward, this new insight into immune dysfunction amongst those living with CF could also enable a shift in care towards a greater focus on treating immune defects while also better targeting infection. Importantly, this will ultimately decrease the overall impact of CF in Tasmania! The new approach offers a means of evaluating the effectiveness of medication used to respond to CF and immune dysfunction, further aiding the management of this condition.
As part of the team’s investigations, they also identified a widespread degree of immune dysfunction in the parents of CF children enrolled in the study, noting that these parents were not living with CF themselves. While we already know that 1 in 25 Australians are CF carriers, this research suggests that these folk also have subtle changes in their immune cells which may potentially influence susceptibility to the development of a number of disorders. While the clinical consequences, if any, are yet to be fully investigated, this research provided a basis for future exploration.
To progress this research, Dr Roddam’s team has developed an ‘antibody panel’ to assess immune cell dysfunction further. But until recently, the wider studies undertaken in Australia have focused on using specialised antibodies that have only been available at the University of Sydney and which rely on the use of analytical equipment that is not currently available in Tasmania.
To overcome this challenge, a new project, jointly funded by the Royal Hobart Hospital Research Foundation and Cystic Fibrosis Tasmania, aims to modify and optimise the antibody panel so that local researchers can begin steps to further profile immune cells. This investigation will not only use more widely available antibodies, but also a locally developed instrument at the University of Tasmania.
We’re delighted to know that this project will deliver multiple benefits! It will build local research capacity and strengthen research ties between the RHH, UTas and CF Tasmania, while also opening up new opportunities for Tasmanian people with CF to participate and benefit from local research. It is also anticipated that local expertise in this analysis will increase the likely success of future collaborative research applications with other institutions across Australia, advancing CF research and its impact even further.
Paula Wreidt, Executive Officer of Cystic Fibrosis Tasmania says that she is “Thrilled with the developments that have been able to progress through the partnership with the Royal Hobart Hospital Research Foundation that would have otherwise not have eventuated, and looks forward to the next phases of this crucial study for Tasmanians living with CF.”
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