A Recent Discovery Could Help Cystic Fibrosis Patients Avoid Lung Transplants

According to a story from thejournal.ie, a recent study has discovered a new method for bringing down inflammation in cystic fibrosis. The study authors have concluded that the approach could reduce a patient’s overall risk of death and the need for lung transplants. The original study was first published in the American Journal of Respiratory and Critical Care Medicine. The research was led by scientists from the Royal College of Surgeons in Ireland (RCSI).

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 Research

Lung problems in cystic fibrosis are exacerbated by not only frequent infections, but also inflammatory activity. As it turns out, a certain aggressive bacteria encountered in the lungs of patients with this disease actually can cause certain immune cells to alter their metabolic processes. As a result, they release a protein that triggers inflammation. 

The scientists later discovered that patients with high levels of this protein were at a greater risk for death, lung function declines, and needing a lung transplant. With this knowledge, the researchers used a small molecule known as MCC950 in order to bring down protein levels in a lab model of cystic fibrosis. Remarkably, this approach not only reduced protein levels but was also able to bring down the presence of bacteria.

The researchers believe that MCC950 could be a potential candidate for testing in clinical trials for cystic fibrosis. However, further study, perhaps in animal models, will be necessary before reaching that stage.

Check out the original study here.

 


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