Researchers Reveal Insights into Effect of Early Lung Infections on Cystic Fibrosis

A new Cystic Fibrosis study entitled “Early Respiratory Infection Is Associated with Reduced Spirometry in Children with Cystic Fibrosis” was published in American Journal of Respiratory and Critical Care Medicine by Dr. Kathryn A. Ramsey, part of Graham L. Hall’s group from the Telethon Kids Institute, University of Western Australia, Australia, and colleagues.

Cystic fibrosis (CF) is a genetic disease that affects various organs, primarily the lungs, and is characterized by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which results in regular infective pulmonary exacerbations with opportunistic pathogens. In the United States, patients with cystic fibrosis have an average survival age of 39 years.

In this study, the research team performed a longitudinal study — the first of its kind to analyze results from a significant group of children diagnosed with cystic fibrosis through newborn screening. The study included infants and school-age children with cystic fibrosis, and the researchers found that early lung infections in these patients can lead to long-term conditions such as impaired function of the lung at school age.

“It’s a really important study because it looks at one of the early drivers of lung disease,” said Dr. Ramsey in the press release. “We have longitudinal data where we can track [the children] for 10 years and we can look at what happens early in life and what effect that has at school age where we have a bit more information about what that means for their long-term health.”

The researchers suggest that management of young children with cystic fibrosis through their development with a focus on identifying pathogens should be changed, since there is no standardized protocol to screen for pathogens.

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Dr. Ramsey said that few clinical centers perform bronchoscopy and analyze fluid from the lower airway to screen for pathogenic agents, and those that do screen do so only when the child is ill while some look for these agents in the upper airways.

“What this study suggests is that we should be doing routine [checks for] lower respiratory track infections in all 0-2 year-old infants because it may be able to tell you how they will develop in life.” concluded Dr. Ramsey.

The annual checks for lower respiratory track are limited for children since this procedure has to be done with general anesthetic.

Moreover, Dr. Ramsey added that the risk of lung infections in children with CF maybe decreased if adequate use of prescribed antibiotics is performed and if the children are put under prophylactic antibiotic therapy upon initial diagnosis of CF.

“We found that children that took their antibiotics had better lung function then those that didn’t,” added Dr. Ramsey. “The other thing is you can do is provide targeted antibiotics for the bug that you actually find.”