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, until the age of six, or when they can expectorate sputum, including:
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Measurements of inflammation and infection in the lung
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Lung function in infants and preschoolers
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Chest scans that show us detailed lung structure
From the program we have found that:
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Inflammation begins early in life and may be present even though we can't detect an infection
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Decreased cystic fibrosis transmembrane conductance regulator (CFTR) function is associated with increased pulmonary inflammation
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Infants can have lung damage and infections even though they have no apparent respiratory symptoms
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Lung damage is more common than first thought in young children with 67% of children having gas trapping in the first year of life and 40% of children have bronchiectasis by the age of four
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80% of children having at least one pulmonary infection in the first 6 years of life and 50% of these children are asymptomatic at the time of detection
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36% of children who are infected in the first 6 years of life have an infection with Pseudomonas aeruginosa. The median age of first infection with P aeruginosa is 26 months and early acquistion is a major risk factor for bronchiectasis. P aeruginosa can be eradicated with aggressive treatment with 77% of infections eradicated after a single treatment cycle
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Lung function in preschoolers is worse in children with respiratory symptoms and pulmonary infection
The program has introduced into routine clinical practice:
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Lung function in infants and preschoolers
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Measurements of infection in young children
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Measurements of lung damage using scans in young children
These observations show the need for interventions to begin early in life to prevent irreversible lung damage.
The measurements we will 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.
Click on the following links to find out more information about different sections of the program:
Bronchoalveolar lavage
Chest computed tomography
Lung function
Epithelial cells
Early detection and eradication of Pseudomonas
Others
Inflammatory mechanisms underlying lung disease
Epithelial function and development of lung disease
Genetic determinants of lung disease
Bacterial contribution to lung disease
Evolution and assessment of structural lung damage
Evolution and assessment of lung function
Last updated 11 November 2008
