According to a story from Medical Xpress, a recent study conducted by researchers from the UNC School of Medicine has illustrated that the accumulation of sticky, abnormal mucus, along with corresponding inflammation, is the primary trigger of lung damage in patients with the rare disorder cystic fibrosis. While infections of the lung are a common symptom of the disease, the findings illustrate that the mucus itself, not infection, is most responsible for lung damage and declines in lung function.
About Cystic Fibrosis
Cystic fibrosis is a type of genetic disorder which can have impacts throughout the body, but it is most characterized by the build up of abnormally thick, sticky mucus in the lungs. This mucus becomes a fertile breeding ground and habitat for potentially infectious bacteria. Many patients must take antibiotics for much of their lives. This disorder is caused by mutations of the CFTR gene. Symptoms of cystic fibrosis include progressive decline in lung function, lung and sinus infections, coughing up mucus, fatty stool, poor growth, infertility in males, clubbed digits, and digestive problems. Treatment includes antibiotics and medications or procedures intended to maintain lung function. Lung transplant is an option when lung function declines severely. Life expectancy ranges into the 40s and 50s with good care. To learn more about cystic fibrosis, click here.
About The Study
A critical area of cystic fibrosis research that has been lacking is the understanding of how lung damage is initially triggered in young children born with the disorder. These findings indicate that the mucus itself is responsible, which could inform treatment strategies for kids with cystic fibrosis. Early intervention with treatments that can thin out the mucus could allow for significant delay in symptom onset and disease progression.
The findings were based on fluid from 46 child patients which was compared alongside samples from 16 children with different breathing issues not related to cystic fibrosis. The fluid from cystic fibrosis patients contained much greater amounts of mucus debris and significantly less bacteria. The study also found that cystic fibrosis patients still had heavy amounts of mucus in areas that had not been damaged, but early signs of inflammation were present. Meanwhile, infectious bacteria were less prevalent.
The original study was published in the academic journal Scientific Translational Medicine.
