Investigators
A/Prof Kathryn Ramsey, Prof André Schultz, Dr Luke Garratt, Dr Yuliya Karpievitch, Alana Harper, Craig Schofield, Crystal Bourke, Kitty Obando, Tabitha Cleary
Project description
Bronchiectasis is a chronic lung disease characterised by chronic wet cough, recurrent hospitalisations, and progressive lung damage. Despite the increasing prevalence in Australia and significant burden of the disease, bronchiectasis remains an under-recognised, under-treated, and under-researched respiratory condition.
Bronchiectasis often starts in childhood and early treatment is critical to reduce the lifelong burden of disease. However, the only approved treatment for bronchiectasis is long-term antibiotic use, which is not sufficient to prevent disease progression.
The number one priority for consumers and clinicians is to find new ways to improve treatment for bronchiectasis. Yet, clinical trials of other treatments (e.g. hypertonic saline, mucolytics) have had mixed results and failed to show an overall clinical benefit. We hypothesise that there are subgroups of paediatric bronchiectasis patients with distinct pathobiological mechanisms driving their disease progression.
Study design
This prospective, longitudinal cohort study will examine airway sputum, lung function, and clinical surveillance data of children with bronchiectasis attending Perth Children’s Hospital. Specifically, we aim to:
- Characterise disease mechanisms in childhood bronchiectasis through comprehensive assessment of airway mucus, inflammation, infection, and single cell sequencing
- Identify subgroups of individuals with distinct underlying disease mechanisms
- Determine which subgroups are associated with worse clinical outcomes
Identification of patient-subgroups will allow for personalised clinical management of bronchiectasis and clinical trials targeting treatments at the individuals most likely to benefit. Effective, personalised treatment for bronchiectasis in childhood will alter the course of the disease, improve long term outcomes, and reduce the significant burden of the disease.
Eligibility
All children with bronchiectasis attending Perth Children’s Hospital (0-18 years).
For more information, please email bxresearch@thekids.org.au.
Funders
NHMRC and WA Child Research Fund
External collaborators
Mark Nicol (University of Western Australia), Anne Chang (Menzies)