Helping Patients Get a Breath of Fresh Air

“The lung is the only organ that's continuously exposed to the outside: 12 breaths a minute, multiply that by 60 for an hour, times 24 hours, 365 days a year,” says Ross Summer, MD, professor of critical care medicine in the Jane and Leonard Korman Respiratory Institute.

With each breath, we sample our surroundings—one of cigarette smoke, car exhaust, and airborne microbes—exposing the lungs to micro-injuries or “insults,” and resulting in a continuous process of repair. Sometimes this healing goes awry, leading to pulmonary fibrosis, the scarring of lung tissue, which can be caused by a combination of any number of genetic and environmental factors.

For Summer, who studies a vexing form of the disease called idiopathic pulmonary fibrosis (IPF), the issue is particularly pressing. “People are living fine and then all of a sudden get a diagnose of IPF,” Summer says. “Within five years, they're gone.” Each year there are 30,000 new cases of IPF in the United States, a number he expects to grow as the population ages—unless someone solves the riddle.

Summer’s team at the Korman Institute believe the source of IPF lies in decreased metabolic activity, leading to cells that are metabolically lethargic and unable to perform their various roles. One metabolic process that he believes is particularly compromised is lipid synthesis, which is essential for maintaining the inner and outer membranes of cells. A slowdown in their production means the never-ending process of insult and renewal doesn’t take place as planned, and the lungs instead form scar tissue.

With this in mind, Summer is putting his head together with other Jefferson investigators from many different backgrounds, inspiring solutions and devising new ways to jump-start healing.