Cystic Fibrosis Risks Increase with Mucins

Image courtesy of UNC Health Care

Researchers from the UNC School of Medicine have found evidence to suggest that patients with cystic fibrosis possess a much greater number of the proteins that forms mucus, known as mucins.

Associate professor at the department of pathology and laboratory medicine, Dr. Mehmet Kesimer, explains what cystic fibrosis, or CF, is and what it can do.

“Cystic fibrosis is a life-threatening genetic disease that primarily affects the lungs and digestive system,” says Dr. Kesimer. “People with CF have a defective gene, and its protein product causes the body to produce abnormally thick and sticky mucus. That is a problem in the lungs; they cannot clear the mucus very well. That is the life-threatening part of the cystic fibrosis.”

With over 70 thousand people across the globe suffering from this disease, UNC researchers have found that the increased amount of mucins the body produces within patients with cystic fibrosis prevents the mucus from clearing through the lungs, which builds and creates inflammation infection, and lung failure. However, if this increased amount of mucins can be reduced, then there is a greater opportunity for better treatments.

“Our study offers simple therapeutic strategies for treating CF lung disease,” explains Kesimer, “for instance, diluting mucins in the mucus layer by simply hydrating agents.”

Using “nebulized hypertonic saline,” a type of sterile salty water, can improve the hydration of the CF airways in order to help in the patient’s mucus clearance to increase lung function. Utilizing these sorts of solutions for patients can provide a much better means of treatment to reduce risk of the mucus build-up.