Aston University Researchers Working on Life-Prolonging Study for Patients with Cystic Fibrosis

A team of researchers at Aston University are currently working on understanding how bacteria present in children with a diagnosis of Cystic Fibrosis decreases their immunity, enabling other bacteria types to infect their airways.

Cystic Fibrosis (CF) is a life-threatening inherited condition that affects approximately 9,000 people in the UK and 30,000 in the US. In CF, the lungs produce large amounts of thick mucus that block the airways, causing a decline in lung function. Today’s available treatments have led to an increase in the life expectancy of people with CF, however, the disease still leads to a shortened lifespan for most patients.

In the new study, the research team led by Dr. Lindsay Marshall will use a model of a human airway with cystic fibrosis to treat early childhood infections with a range of antibiotics. The aim is to evaluate the degree to which P. aeruginosa can be blocked. The human model was developed at Aston University and can precisely replicate the mechanisms of a human airway and the disease progression.

Dr. Marshall said in a recent news release: “We still do not fully understand the link between the cause of cystic fibrosis and the contraction of cystic fibrosis lung disease. There is a clear need to learn more about the disease process to allow us to develop new and better treatments to keep patients healthy for longer.”

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Another goal of the research, supported by a £99,336 grant from the Human Research Trust, seeks to discover new approaches for reducing the quantity of animals needed in studies replicating respiratory diseases. In 2014, in the United Kingdom more than 115,000 animals were used in studies for conditions such as asthma and lung diseases associated with smoking.

“This project will also allow us to demonstrate how well our layered human cell model replicates CF lung disease and in what ways it could be used to evaluate the effectiveness of future treatments. We hope to use different human cell models in future to examine how the body’s natural defences are altered by a variety of other inflammatory and infectious conditions,” said Dr. Marshall. She concluded that researchers cannot use animals to model the decline in lung function seen in people with CF, since infections found in people with CF and the administration and dosage of therapies to treat CF infections are often incompatible with animal models. “Our human CF model, which contains different layers of cells found naturally in the airways, is extremely representative of what happens in human airways and is both ethically and scientifically an improvement upon current animal models.”