Experts believe a pioneering breakthrough by a Manchester hospital research team could pave the way for doctors to better understand and treat adults with Cystic Fibrosis (CF), helping them live longer and healthier lives.
CF is the commonest inherited genetic disease in Europe and the US affecting tens of thousands of people. Although significant advances in care have improved – with some patients now surviving into their 40’s and 50’s – it remains incurable.
For many years the fungus Aspergillus has been known to damage the lungs of CF patients by infecting them and causing an allergic reaction. This two year study, which was led by Dr Caroline Baxter and colleagues at UHSM (University Hospital of South Manchester), shows that 50 per cent of patients had allergic aspergillosis or Aspergillus bronchitis and an additional 15 per cent were sensitised to this common airborne fungus.
Dr Baxter, who did this work as a Clinical Fellow at the hospital’s Adult Cystic Fibrosis Unit explains: “CF primarily affects the lungs. Lifelong chest infections due to multiple bacteria require repeated course of intravenous antibiotics and frequent hospitalisation. Unfortunately, current diagnostic tests for aspergillus disease in CF patients are notoriously imprecise. As a consequence of this failure, choosing the best therapeutic option for treatment has been very difficult, contributing to a decline in lung function.”
Professor Kevin Webb who leads the Centre says the Aspergillus disease has long been a conundrum for CF physicians. “We face steroid therapy and the risk of diabetes if we treat, or worse lung function if we do not. Aspergillus also impacts on transplant options for patients, curtailing life expectancy. Dr Baxter’s work is pivotal to CF care for the coming decades, as we strive to enable patients to live a normal life for as long as possible.”
Professor David Denning, who is Director of the National Aspergillosis Centre and Professor of Medicine and Medical Mycology at UHSM adds: “This ground breaking research has opened the door to a more informed therapeutic management of Aspergillus disease in CF patients and a greater understanding of its origin and development. As antifungal treatment of these complications tends to be long term, with or without steroids, better studies of the impact of antifungal therapy can now be done.