Submitted by Aspergillus Administrator on 23 June 2011
Becky Jones has Cystic Fibrosis (CF) which in common with many other people who live with CF makes her lungs vulnerable to infection. Aspergillosis is a common infection for this group of people (up to 11% of people with CF get aspergillosis), usually in the form of ABPA but this occasionally progresses to the more aggressive forms of aspergillosis such as Chronic Pulmonary Aspergillosis.
No forms of aspergillosis are currently curable with antifungal drugs and treatment usually consists of managing the condition using antifungals and steroids in order to prevent scarring of lung tissue and loss of lung function.
Unfortunately there are increasing cases where the infecting fungus is resistant to all usable antifungal drugs, and in those cases treatment becomes very difficult. Ultimately what is really needed is to replace the diseased tissue with new – a lung transplant.
This strategy has never been tried before as there is a ‘self defeating’ scenario that has to be carefully managed. Someone who has an organ transplant has to take immunosuppressant drugs to prevent rejection of the donor tissue by the patients immune system. This also makes the patient vulnerable to infection and if he/she is already infected with Aspergillus that was thought to make the patient highly likely to become re-infected. Fortunately there have been some advances in the administration of antifungal drugs direct into lung tissue, and if the tissue match is good it might be possible to minimise immunosupression – the stage was set to try to treat a CPA patient in this way.
Becky had extensive Chronic Pulmonary Aspergillosis which had became resistant to all useable antifungal drugs so her infection could no longer be managed. Like many CPA patients Becky would most likely have been treated with steroids to minimise inflammation caused by the aspergillus infection (and so allow her to breathe), which in turn would also make her a little less able to fight the infection – another ‘self defeating’ vicious circle but this time one that relies on the availability of an antifungal that the infection is not resistant to in order to keep the infection in check – as long as that continues the condition is manageable. In Becky’s case there was no antifungal left to use, there was no alternative but to consider a transplant.
To look at Becky in the news reports is to see the hope that this technique, used for the first time to treat CPA can bring. She will now be closely monitored to check for infection and will be given an antifungal ‘inhaler’ to keep aspergillosis at bay. Becky is a pioneer and is leading the way for hopefully many more in the future.
Becky is also a torchbearer for the new £150 000 fundraising appeal for the UK’s foremost aspergillosis research charity The Fungal Research Trust. This appeal aims to provide a new postdoctoral researcher for 3 years to work towards new diagnostic techniques and treatments.
Her personal message is:
” Without the research they have funded, my new lease of life would not be possible. As a young child I coped pretty well with my cystic fibrosis. When I was about 10 I first got Aspergillus infections – which never went away. In my teens, I was often miserable and had lots of steroid medicine. Later balls of fungus were found inside my lungs. Antifungal treatment helped a bit to start with and then stopped working, because my Aspergillus had developed resistance to the treatment. The only way to improve my life was a double lung transplant and receiving intravenous Antifungals. I have made it through the transplant and I can breathe again– please help support the campaign because I have made it through – please help others make it through their difficult times with fungal infection.” Becky’s story
(visit Press release)
The Fungal Research Trust works closely with the National Aspergillosis Centre in Manchester, UK
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