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.
AREST CF Team
We want to better understand which factors predict lung damage early enough so that we can intervene and prevent lung damage from occurring.