We are the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF). We are a team of doctors, nurses, scientists and researchers who are dedicated to the improvement of the general health of children with CF.
We have two centres, one in Perth, (the newly opened Perth Children's Hospital which has replaced Princess Margaret Hospital for Children) and one in Melbourne (Royal Children's Hospital). Importantly, if you want to discuss any aspect of our research OR have a research idea, speak to a team member about this - we want to make your idea a reality.
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Executive Committee and Scientific Management Committee
AREST CF Executive Committee and Scientific Management Committee
Shivanthan Shanthikumar
Luke Garratt
Yuliya Karpievitch
Kathryn Ramsey
Patricia Agudelo Romero
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Consumer Representatives
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Perth Members
Perth Members
Erika Sutanto
Ingrid Laing
Craig Schofield
Sam McLean
Liz Starcevich
Kevin Looi
Rebecca
Langford
Luke Berry
Emma de Jong
Elizabeth Smith
Alana Harper
Eleanor Ferguson
Samantha Grogan
Sally McCappin
Nick de Klerk
Josh Beeson
André Schultz
Steven Oo
Oded Breuer
Barry Clements
Rachel Foong
Alexia Foti
Grace Pettigrew
Georgia Banton
Thomas Iosifidis
Jaqueline
Macpherson
Harpreet Kaur
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Melbourne Members
Melbourne Members
Louise King
Suzanna Vidmar
Rosemary Carzino
Nadeene Clarke
Phil Robinson
Rebecca McElroy
Natalie Zajakovski
Katherine Frayman
Jo Harrison
John Massie
Julie Smith
Colin Robertson
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Other Members
Other members
Peter Sly
Linda Shields
Tonia Douglas
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Students
Clara Mok (PhD)
Characterising low attenuation regions (LAR) on CT scans in children with cystic fibrosis
Repurpose and validate the use of a novel quantitative CT scoring technique (PRAGMA-CF) in MRI images. Using a mathematical model of the lung, assess how the extent and geographic distribution of CT LARs change over time and correlate these findings with clinical markers of disease. Determine the cause of CT LARs (ventilation, perfusion or combined defect) using functional MRI.
Shivanthan Shanthikumar (PhD)
The aim of my research is to identify epigenetic markers from early life which predict the severity of lung disease at 9 years of age. I will do this by comparing two groups, one with mild lung disease and one with severe lung disease at 9 years of age (based on CT scan results), and I will perform whole genome methylation analysis on broncho-alveolar lavage from early life with the aim of identifying differential methylation patterns between the two groups.
The study involves patients with CF - so the 2 groups of kids will be kids with CF from the AREST CF cohort.
Kelly Martinovich (PhD)
Personalized antisense oligonucleotide therapy to correct CFTR function in Cystic Fibrosis (CF) patients
In 2016 I enrolled in a PhD to test whether Antisense Oligonucleotide therapy is capable of rescuing CFTR function in specific Cystic Fibrosis patients
I aim to develop a new treatment that targets specific CF gene types. The message that the CF gene holds makes the CF transmembrane regulator (CFTR) protein. A faulty gene message results in the CFTR protein not be produced or able to work properly. A non-functioning protein allows mucous build up and infections to establish that are hard to treat. Over time, these infections and the inflammation that follow leads to permeant lung damage.
The treatments we are developing are small molecules called antisense oligonucleotides (AOs). AOs can change the message that is sent from the gene to protein. AOs are made to target the specific site in the CFTR gene where the faulty message is. In selected CF cases, AOs could improve disease symptoms.
I am currently working with Telethon Kids Institute, the University of Western Australia and the Centre for Comparative Genomics to perform my research.
Kak-Ming Ling (PhD)
Innate Immune Response of Airway Epithelial Cells from Children with Cystic Fibrosis to Rhinovirus Infection
Growing evidence has linked respiratory virus infection in Cystic Fibrosis (CF) with lung function decline and pulmonary exacerbation. Currently, rhinovirus is the most prevalent virus detected, predominantly in children with CF less than 5 years old who suffer an exacerbation. Although these children are prone to repeat and prolonged infection, there is no effective targeted treatment. My project will elucidate the mechanisms of viral infection at the primary site of infection, namely the airway epithelium. The first phase of my study will identify dysregulation in innate immune pathways in CF airway epithelium in response to rhinovirus infection utilising RNA-sequencing technology in conjunction with our collaborators (Hancock Laboratory; University of British Columbia). The second phase will validate the proposed candidate pathways and determine their role in regulating innate immune responses during viral infection. The last phase will focus on screening databases of known drugs for desirable interactions with these identified pathways. The anticipated translation of this research is a new area of anti-viral intervention in young children with CF, prior to severe lung disease and irreversible structural damage. By limiting the harmful consequences of viral infection at this early stage, we aim to ultimately reduce viral associated morbidity in CF.
Samuel Montgomery (PhD)
The Role of Hypoxia and Necrosis in Sterile Neutrophilic Inflammation in Children with Cystic Fibrosis
Samuel is a fourth-year PhD student who focuses on an important but poorly researched area in CF; early life inflammation in the absence of infection. Specifically, his research aims to investigate the IL-1R inflammatory pathway in CF and the mechanisms surrounding inflammation arising from cell death following airway hypoxia and viral infections.
Daniel Laucirica (PhD)
Early life airway infections in children with Cystic Fibrosis
My research focuses on early life airway infections in children with Cystic Fibrosis (CF). The goal of my project is to assess innate immune responses of the CF airway epithelium to infection by a variety of CF associated pathogens, and connect these responses to functional changes in airway neutrophil activity observed in vivo. One of my key interests is modeling pathogenic interactions in CF airways that may enhance microbial dissemination, predisposition to further infection, and exacerbate epithelial immune responses. In addition to contributing new knowledge on early life airway infections in CF, results may also inform new strategies for the management or prevention of airway inflammation in CF lung disease.
Peter Van Mourik (PhD)
Generating 3D organoid as a cell model for drug discovery and therapy in cystic fibrosis
Intestinal organoids can be used to study the function of the CFTR-protein in patients with Cystic Fibrosis, and to predict whether someone will respond to CFTR-modulating drugs. We aim to study the effect of drugs in intestinal organoids of persons with rare CFTR-mutations to identify persons that might benefit from treatment.
Wolfgang Haehr
Matrixmetalloproteases in cystic fibrosis airway repair
The airways are covered with a thin lining called the epithelium. In cystic fibrosis this lining may have a defective process of repairing when damaged. Matrixmetalloproteases are a family of enzymes that can influence the process of repair and has been found increased in the cystic fibrosis lung environment. However, the celluar origin of these proteins as well as their activity during repair is yet to be interrogated in the epithelium.
Andrew Vaitekenas
Renee Ng (PhD)
Phage Therapy for Children with Cystic Fibrosis Pseudomonas aeruginosa Lung Infection
Chronic bacterial infections are one of the main contributing factors to permanent lung damage and loss of function in the lungs of people with Cystic fibrosis (CF). Mucus build up on the airway epithelial cells results in an ideal breeding ground for opportunistic bacteria like Pseudomonas aeruginosa to grow. Pseudomonas aeruginosa easily develops resistance to the antibiotics typically used to treat it. Presently, intravenous antibiotics are given over an extended period of time, sometimes at very high concentrations which can have significant side-effects including kidney damage. With little investment into discovery of novel antibiotics by the pharmaceutical industry, there is an urgent need to explore alternative therapeutics. In my project, I will be revisiting the use of bacteriophages, or “phages” to treat Pseudomonas aeruginosa bacterial lung infections in children with CF. Phages are bacterial viruses that use specific bacteria as a host to grow which in turn, kills it. Phage therapy is not a standardised treatment in Australia and many other western countries due to insufficient evidence about their use and effects in humans. However, phage therapy has been commonly used in Eastern Europe as treatment. We hypothesise that specific phages will show high activity against bacteria that cause chronic lung infection without detrimental effects on the body. Ultimately, my project aims to establish a screening tool for high throughput phage assessment and develop a potential therapeutic pipeline to treat affected children.
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Alumni (past team members)
AREST CF Governance Structure
Research conducted under the auspices of AREST CF complies with the highest scientific and ethical standards, the Australian Code for Responsible Conduct of Research and principles of good governance. AREST CF is a collaborative research group. Membership comprises of the Executive Committee and the Scientific Management Committee within the AREST CF governance structure.