Study Shows Children with Cystic Fibrosis Develop Lung Bacteria Earlier than Others

Over 1,000 individuals in the United States are diagnosed with cystic fibrosis (CF) every year. The condition affects over 70,000 individuals worldwide and is most common among children of North European descent. CF is triggered by a dysfunction in the CFTR gene, which causes the fluids within the lungs to become mucus-like (thick and sticky). The mucus blocks the ducts and passageways in the body, thereby leading to persistent bacterial infections, malnutrition, inflammation of the airways and eventually, lung damage, and respiratory failure.

More about the study

Researchers from the University of North Carolina School of Medicine report that bacterial changes within the lower respiratory tract during the first years of a child’s life may indicate the progression of lung disease. The study, called ‘Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children’, was published in the journal PLoS Pathogens. Findings suggest that the onset of lung disease in children with CF can be predicted and the use of preventive therapies, such as hypertonic saline, is necessary. “Lung symptoms in kids with CF are likely due to an increased burden of bacteria. This implies there’s an opportunity for early intervention that could dramatically increase the quality of life for these kids,” said Matthew Wolfgang, senior author of the study and associate professor of microbiology and immunology at UNC Chapel Hill.

Since a majority of studies on CF have been previously performed on older children and adults, the UNC research team aimed to target a younger group. The researchers examined the bacterial content of 46 bronchoalveolar lavage samples gathered from young children with CF (ages 3.5 months to 5 years). Each child had previously undergone a bronchoscopy to determine the structure of their lungs and the procedure was done as part of the Australian project, known as AREST CF. The AREST CF project focuses on targeting the treatment and prevention of CF lung disease in children under the ages of 7 (as identified through newborn screening). The research project aims to extend the age at which children with CF begin to experience deterioration of lung health, as well as improve both clinical outcomes and quality of life for them and their families.

More about the AREST CF project

The main objectives of the AREST CF project are to define the early risk factors of lung disease, to better understand the mechanisms of early lung disease, to develop new approaches to treating early lung disease, to predict early clinical outcomes in accordance with the severity of the disease and responses to treatment, and to develop new treatments designed to prevent lung disease or delay its progression.

Based on the gathered findings to date, it has been noted that:

• Inflammation begins in the early stages of life and it may be present even if an infection has not been identified
• Newborns can experience infections and lung damage even if they show no apparent signs of respiratory problems
• Most children experience at least one pulmonary infection in the first 6 years of their lives
• Over 30% of young children who experience a pulmonary infection in the first 6 years of their lives have an infection with the bacterium Pseudomonas aeruginosa

Read on to learn more about this recent study and what it could mean for the future of cystic fibrosis diagnosis, analysis, and treatment.