Early Life Respiratory Health Program

Transforming early life respiratory health

The Early Life Respiratory Health Program brings together researchers from the Cystic Fibrosis Airway Research Group (CFARG)and the Respiratory X-ray Imaging Laboratory (ReXIL) to improve lung health from the very beginning of life. Our shared vision is that every child born with cystic fibrosis can live a long, healthy life free from the burden of lung disease.

Our teams combine expertise in respiratory biology, gene and cell therapies, advanced imaging technologies, and translational medicine. CFARG focuses on understanding the biological mechanisms that drive cystic fibrosis lung disease and developing new treatments that address its underlying genetic cause. ReXIL develops innovative imaging technologies that allow researchers and clinicians to visualise lung function in unprecedented detail, enabling earlier detection of disease and more precise monitoring of treatment responses.

By integrating these complementary approaches, our program spans the full research pipeline — from discovering how disease develops, to developing new therapies, and creating tools that monitor lung health in the clinic. This close collaboration enables us to test new treatments, understand how they affect lung function, and accelerate their translation into clinical practice.

Our work is built on strong partnerships with clinicians, engineers, imaging scientists, and industry collaborators, and is supported by national and international research infrastructure including advanced imaging facilities and clinical research networks. Through these collaborations, we aim to deliver new diagnostics and therapies that improve respiratory health for children with cystic fibrosis and other early-life lung diseases.

Cystic fibrosis rat and B-ENaC mouse models

Lung function testing with flexiVent lung mechanics and X-ray Velocimetry functional lung imaging

Synchrotron respiratory imaging at SPring-8 and Australian Synchrotrons

Nasal potential difference measurement

Airway infection models (e.g. Pseudomonas Aeruginosa)

Therapeutics evaluation

Explore the Early Life Respiratory Health Program

Research themes 1
Our people 2
Current projects 3

Our research aims to improve the lives of people with cystic fibrosis by combining fundamental biological discovery, therapeutic development, and advanced imaging technologies. Together, these approaches allow us to better understand how lung disease develops, develop new treatments that address its underlying causes, and monitor lung health more precisely in both research and clinical settings.

Studying disease mechanisms to guide the development of new treatments

Cystic fibrosis lung disease is driven by complex biological processes involving airway cells, inflammation, infection, and structural changes in the lung. By understanding how these mechanisms interact, we can identify new therapeutic targets and improve treatment strategies.

Our research explores:

How cystic fibrosis lung disease develops and progresses
Why disease severity differs between individuals
Why patients respond differently to treatments such as cystic fibrosis transmembrane conductance regulator (CFTR) modulators (e.g. Trikafta)
Biological pathways that may be targeted by new therapies Key research areas
Molecular pathways linked to mutant CFTR
Epithelial–mesenchymal transition (EMT)
Airway remodelling and structural lung changes

Related projects: Fibrosis and Airway Remodelling in CF Lung Disease

Creating treatments that address the root cause of cystic fibrosis

Most current therapies manage symptoms rather than correcting the underlying genetic defect. Our research focuses on therapies that restore CFTR function in airway cells, aiming to halt disease progression or potentially provide long-term cures.

Our goals

Correct or replace the faulty cystic fibrosis transmembrane conductance regulator gene in airway cells
Restore normal airway physiology
Develop durable or one-time therapies

Approaches we investigate

Gene-addition therapies delivering healthy CFTR genes
Gene editing approaches to permanently correct the CFTR mutation
Stem cell-based therapies to regenerate healthy airway tissue mRNA-based therapies that restore CFTR protein function

Related projects: Gene editing towards a universal CF therapy, Enhancing gene therapy for cystic fibrosis

Developing tools to detect disease earlier and track treatment response

Early detection and precise monitoring of lung disease are essential for improving outcomes in cystic fibrosis. Our research develops advanced imaging and diagnostic technologies that allow clinicians to visualise lung function, detect disease earlier, and evaluate new therapies.

Our aims

Detect lung disease before irreversible damage occurs
Monitor disease progression and treatment response
Enable personalised treatment strategies
Evaluate new therapies in clinical trials

Technologies and methods

X-ray Velocimetry (XV) functional lung imaging
Synchrotron-based airway imaging (SPring-8, Japan)
Lung imaging studies at the Australian Synchrotron
Development of next-generation imaging techniques such as dark-field imaging

Related projects: XV imaging for early lung disease detection, AI-powered lung imaging analysis

Associate Professor Martin Donnelley

CFARG/ReXIL Director

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Dr Nathan Rout-Pitt

Cystic Fibrosis Airway Pathophysiology Lead

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Dr Alexandra McCarron

Cystic fibrosis gene and cell therapy lead and Future Making Fello

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Dr Ronan Smith

X-ray imaging lead

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Dr Nikki Reyne

Animal models lead

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Understanding how lung disease develops is essential for designing better treatments. Our research investigates the biological mechanisms driving cystic fibrosis and develops innovative therapies to prevent infection, limit damage and improve long-term health.

Fibrosis and airway remodelling in CF lung disease

Chronic inflammation and infection in cystic fibrosis can trigger structural changes in the airway, including fibrosis and airway remodelling that progressively impair lung function. This project investigates the biological pathways that drive fibrotic signalling in the CF lung, with the goal of identifying targets for therapies that prevent or reverse airway damage.

Nanomedicine to treat chronic lung infections

Bacteria in cystic fibrosis lungs form protective biofilms that resist antibiotics. We are working with collaborators (Dr Sha Liu, Adelaide University) to develop and test Phage-LumenGel, an inhalable nanomedicine that penetrates mucus, releases bacteriophages that attack bacteria, and activates antibacterial molecules on demand. This triple-action therapy aims to eliminate infections and reduce reliance on antibiotics.

Understanding CF therapies during pregnancy

CFTR modulator drugs have transformed cystic fibrosis care, but their effects during pregnancy are not well understood. This project (A/Prof Elena Schneider-Futshik, University of Melbourne) studies how exposure to these medicines before birth may influence the development of cystic fibrosis pathology, helping guide clinical decisions and improve health outcomes for mothers and their children.

Our gene and cell therapy research aims to correct the underlying genetic causes of lung disease. By developing new delivery technologies and genome editing approaches, we are working towards treatments that could halt disease progression—or potentially cure it.

Enhancing gene therapy for cystic fibrosis

Gene therapy has the potential to restore the missing CFTR gene in airway cells, but delivering therapies deep into the lungs remains challenging. This project develops new viral vectors and airway preparation strategies to improve gene delivery and uptake, helping bring effective airway gene therapy for cystic fibrosis closer to clinical use.

Gene editing toward a universal CF therapy

Many cystic fibrosis treatments only work for specific genetic mutations. We are working with collaborators (Dr Fatwa Adikusuma, Adelaide University) to develop a next-generation gene editing strategy that inserts a functional CFTR gene directly into the genome. This mutation-agnostic approach could enable a single treatment that works for all people with cystic fibrosis.

We develop advanced imaging technologies to better understand how lungs function in health and disease. By combining physics, medicine and artificial intelligence, our work aims to detect lung disease earlier and provide clinicians with new tools to guide treatment.

XV imaging for early lung disease detection

X-ray Velocimetry (XV) is a breakthrough imaging technology that maps how air moves through different regions of the lung during breathing. Unlike conventional lung tests, XV can detect early disease and can be used in young children. Our research with industry partner 4DMedical aims to translate this technology into routine clinical care to improve diagnosis and monitoring of respiratory disease.

AI-powered lung imaging analysis

XV functional lung imaging produces enormous amounts of data from every breath. We are developing artificial intelligence tools to transform these complex datasets into clinically meaningful biomarkers. This work will improve interpretation of lung imaging, reduce radiation exposure by removing the need for CT scans (Prof Tom Drummond and A/Prof Kris Ehinger, University of Melbourne), and accelerate global adoption of XV imaging technology.

Contact the Early Life Respiratory Health Program

Location
Cystic Fibrosis Airway Research Group and Respiratory X-ray Imaging Laboratory
Adelaide University
Respiratory Medicine, Level 6 Gilbert Building, Women’s and Children’s Hospital 72 King William Road, North Adelaide, SA 5006