Participants sought: Can exercise replace chest physiotherapy for people with cystic fibrosis?

Participants with CF sought for virtual discussion group on
how best to test if exercise can replace airway clearance.
See more: Study flier

Can exercise replace chest physiotherapy for people with cystic fibrosis? Partnering with consumers to address a research priority

People with cystic fibrosis (CF) have indicated that finding out if exercise can replace daily physiotherapy (airway clearance) is an area of research priority for them. However, the best way to test whether exercise can replace airway clearance is not clear. 

We are running a study to understand the thoughts and experiences of people with CF

Gene-product therapy a success in cystic fibrosis treatment: review

Professor John Wilson AM is using gene-product modulating
therapy for treatment of patients with cystic fibrosis

by Anne Crawford

Gene-product modulating therapy – treatment addressing gene abnormality by acting on the proteins arising from the gene rather than trying to correct the gene itself – has been attracting considerable scientific attention in recent years.

Monash University’s Professor John Wilson AM, who is using this approach to treat patients with cystic fibrosis, recently reported on the therapy in a comment piece published in Gene Therapy Nature.

Science competition "CF's Got Talent!" streaming live from London 26 Sep 2019

Dr Anh Thu Dang

One of our researchers, Dr Anh Thu Dang, is competing in the UK based Cystic Fibrosis (CF) Trust science competition "CF's Got Talent!" next week, 26 September. The event will be livestreamed on CF Trust's Facebook page. She'll be talking about damping lung inflammation by tweaking the gut microbiome, and her title is: ‘Short chain fatty acids as CF therapeutics against pulmonary infections’.

Anh Thu is a post-doctoral research assistant based at Monash University in Melbourne, Australia, in Professor Benjamin Marsland’s lab. They are working on one of the programmes within the CF Trust's SRC ‘Restoring balance in the gut for less inflammation in the lungs’, led by Leeds-based Professor Daniel Peckham. 

Fascination with 'bugs' leads to cystic fibrosis research

Dr Katherine Langan presenting at the
2016 CCS postgraduate symposium

by Anne Crawford

As a child, Katherine Langan was intrigued by bugs – but not the type you caught in jars. Dr Langan recalls being fascinated by infectious diseases since she was about 12 witnessing the advent, then spread of HIV/AIDs in Australia. Even then she wondered what could be done about it. 

Dr Langan’s interest in ‘bugs’ took her to Liverpool to study tropical medicine in 2011 and to Bangladesh to see first hand the effects of malaria in one of the country’s major teaching hospitals. The hospital was an eye-opener: there was no running water, no electricity for much of the day, patients had to pay for everything they used and were washed and fed by their families. Dr Langan watched as malaria killed young people who arrived too late to be effectively treated.

Now an infectious diseases specialist at The Alfred undertaking a PhD at the Central Clinical School, her preoccupation is with Pseudomonas aeruginosa, specifically in people with cystic fibrosis.

Follistatin promising for cystic fibrosis treatment

Lung scan showing airways obstructed with mucus
Image: Guy Viner

Cystic fibrosis (CF) is the most common life-limiting genetically acquired respiratory disorder. Patients with CF have thick mucus obstructing the airways leading to recurrent infections, bronchiectasis and neutrophilic airway inflammation culminating in deteriorating lung function. Current management targets airway infection and mucus clearance, but despite recent advances in care, life expectancy is still only 40 years.

Researchers from the Monash Department of Allergy, Immunology and Respiratory medicine investigated whether activin A (a proinflammatory protein associated with excessive mucus secretion) is elevated in CF lung disease and whether inhibiting activin A with its natural antagonist follistatin retards lung disease progression. They found that activin A levels are elevated in CF and provide proof-of-concept from an experimental model for the use of the activin A antagonist, follistatin, as a therapeutic in the long-term management of lung disease in CF patients.

Reference: http://www.nature.com/icb/journal/vaop/ncurrent/full/icb20157a.html

High rates of bowel cancer in cystic fibrosis: Dr Ilana Gory, Department of Gastroenterology

Dr Ilana Gory, a PhD student in the Department of Gastroenterology, reported on her research at the Australian Gastrointestinal Week. Her topic was "Cystic Fibrosis is associated with an increased risk of colorectal malignancy and adenomas". As a practitioner, she and her team at Alfred Health had anecdotally observed high rates of cancer in patients with cystic fibrosis. Her scientifically based study found that patients with cystic fibrosis (CF) may be up to ten times more likely to develop colorectal cancer.