AREST CF seeks to improve the detection, prevention and treatment of early respiratory disease in young children with cystic fibrosis in order to improve clinical outcomes and quality of life for patients and their families.
To better understand mechanisms underlying early lung disease
To define early risk factors associated with lung damage
To develop new and innovative ways of assessing early lung disease
To define early clinical outcomes that can be used to assess severity of lung disease and response to treatment
To develop therapeutic strategies designed to prevent or delay the onset of lung disease and to minimise the progression of lung disease
The Perth and Melbourne clinics run a unique early surveillance program for infants diagnosed with cystic fibrosis following newborn screening. A comprehensive assessment is undertaken at diagnosis and annually (close to the child's birthday).
Some of the measurements include:
Measurements of inflammation and infection in the lung
Chest scans that show us detailed lung structure
What have we learnt so far?
Inflammation begins early in life and may be present even though we can't detect an infection
Decreased cystic fibrosis transmembrane conductance regulator (CFTR) function is associated with increased pulmonary inflammation
Infants can have lung damage and infections even though they have no apparent respiratory symptoms
Lung damage is more common than first thought in young children
Most children have at least one pulmonary infection in the first 6 years of life
1/3 of children who experience a pulmonary infection in the first 6 years of life have an infection with Pseudomonas aeruginosa
Lung function in preschoolers is worse in children with respiratory symptoms and pulmonary infection
These observations show the need for interventions to begin early in life to prevent irreversible lung damage.
The measurements we make in the first few years of life will allow us to better understand which factors predict lung damage by school-age and which tests best reflect the progress of lung disease early in life. This information will greatly facilitate intervention studies and aid disease monitoring by providing valid clinical endpoints that can be used to determine change in clinical status.
Telethon Kids Institute is the sponsor of BANK CF and is responsible for the oversight of the biobank.
AREST CF Executive Committee
The AREST CF Executive Committee (EC), comprising three study Principal Investigators, the AREST CF Program Manager and a consumer representative, oversee the research, including the biobank and database(s). The EC meets quarterly to discuss:
Strategic plans and directions for cystic fibrosis research
Financial aspects of the program
Membership of the AREST CF Scientific Management Committee
The AREST CF Scientific Management Committee
The AREST CF Scientific Management Committee (the SMC), comprising researchers invited by the Executive Committee, the AREST CF Program Manager and two consumer representatives, monitor, review and report on the management and operation of the Biobank and activities pertaining to resources. The SMC meets monthly to discuss sample and data governance, ongoing studies and issues pertaining to publications.
Responsibilities of the SMC in relation to the Biobank are to:
Monitor the operations of the Biobank for compliance with the applicable domestic law, ethics, guidelines and international instruments
Monitor compliance with standards for the physical and electronic security of:
Biological and genetic data that is generated from the samples, and
The information technology system for the Biobank.
Report to the Executive Committee on compliance or otherwise.
Review the Biobank’s documentation, including but not limited to:
Standard Operating Procedures (SOPs),
Material Transfer Agreements (MTAs),
Be responsible for access to samples from the biobank and corresponding data for external and internal researchers via the Scientific Management Committee (SMC) request workflow.
The cystic fibrosis consumer reference group provide input into respiratory research, including the structure and use of the biobank. This helps to ensure that the participant’s rights and expectations are considered and respected.
The AREST CF Operations group, comprising a data manager, biobank manager and project officer, led by the program manager, manage the day to day operations of the Biobank. They ensure that the biobank operation is consistent with best practice, with input from the SMC. Members of this team have significant experience and appropriate qualifications and competence in the collection, curation, processing, storage and cataloguing of biological specimens and associated data.
The Respiratory Centre Cystic Fibrosis Biobank (BANK CF) is available for the purposes of advancing health outcomes and medical research in cystic fibrosis. To achieve this outcome, AREST CF will invite and approve, if thought fit, new research proposals submitted by Telethon Kids Institute researchers, MCRI researchers and stakeholders, and third-party researchers (to be reviewed by the AREST CF SMC).
Audits of samples and associated data are performed in accordance with guidelines for biobanks and databanks. Regular reports will be submitted to Ethics Committees as per local requirements.
The AREST CF team will audit samples and data routinely and expect to engage an independent auditor in the future with the aim of an external audit every 3 years, unless required by the result of the first audit, to be done more frequently.
A member of the biobank research team can be contacted, by calling Telethon Kids Institute on (08) 6319 1000, to answer any questions you have about the use of your child’s samples in research.
All research in Australia involving humans is reviewed by an independent group of people called a Human Research Ethics Committee (HREC). The ethical aspects of this research have been approved by the Perth Children’s Hospital HREC. BANK CF will be carried out according to the National Statement on Ethical Conduct in Human Research (2007). If you have any concerns and/or complaints about BANK CF, the way it is being conducted or your child’s rights as a research participant and would like to speak to someone independent of the project, please contact The Executive Director of Medical Services at PCH on (08) 6456 2222. Your concerns will be drawn to the attention of the Ethics Committee who is monitoring the study.