The fungus Aspergillus fumigatus is frequently cultured from the sputum of people with cystic fibrosis (CF). Yet clinicians face great uncertainty as to how harmful Aspergillus is, and if it is, how to identify those patients requiring specific treatment. We need better ways to detect Aspergillus infection in the lungs of people with cystic fibrosis. We also urgently need ways to identify those people with Aspergillus who will be most at risk of lung damage if untreated.
In about 60% of people with CF, Aspergillus is detected in their sputum. Aspergillus infection is reported to be associated with worse lung function and increased exacerbations. Although common in children too, we know very little about how and when children first pick up the infection, nor how much impact this has on their lungs.
An infection with many forms
Part of the problem is that Aspergillus infection in CF can take several different forms. In some people it causes an intense allergic response that can cause progressive lung damage (also known as ABPA). In others it causes infection in the airways that seems to result in more sputum and chest symptoms (Aspergillus bronchitis). In others, it causes an asthma-like picture of wheeze.
We don't have simple methods of identifying how a patient will respond to Aspergillus infection, nor what it will mean in the long term. The treatments we have are also difficult, as they may be poorly absorbed in CF, and without better understanding of how the conditions develop it can be hard to determine when treatment has been successful.
Aims and objectives
To revolutionise the management of CF-related Aspergillus diseases we aim:
- To define specific measurements of disease progression and outcome in a consortium-based detailed analysis of 1,000 people with cystic fibrosis.
- To develop and test a way of identifying which patients will benefit most from treatment.
- To prevent inappropriate use of antifungals and steroids, improve lung function and quality of life and reduce hospital admissions.
Our objectives are:
1. Clear description of Aspergillus disease in children and adults with CF using existing and new tests of fungal infection and to establish the effect of these infection types on lung disease.
2. To understand how Aspergillus causes lung disease and identify new targets for future treatments.
3. To define and test ways of selecting those patients who will benefit from anti-fungal treatments.
Academia, industry, patients and the Trust
The ASPerCF consortium is a collaboration between four academic institutes, the Cystic Fibrosis Trust, three industrial partners and a patient representative.
Members of the consortium are: Dr Darius Armstrong-James, Dr Elaine Bignell, Prof Debby Bogaert, Prof Rosemary Boyton, Dr Siobhan Carr, Prof Jane Davies, Prof Graham Devereux, Prof Stuart Elborn, Dr Tim Felton, Ms Katie Gathercole, Dr Alex Horsley, Prof Andrew Jones, Prof Amanda Lee, Prof Graeme MacLennan, and Prof Adilia Warris (chair).
We submitted our full research plan to the Medical Research Council in June this year and will be informed about the outcome at the end of November 2017.
Please do share with us your ideas about our research plans by using our twitter account @Asper_CF and have a look at the video above to find out more.
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