People with emphysema suffer permanent damage to the walls of the air sacs, or alveoli, of the lung. As the alveoli are destroyed, it becomes progressively difficult to breathe. Margherita Paschini, Ph.D., of Children's Hospital in Boston, wants to find a way to repair the alveoli so breathing is not a constant struggle for people with emphysema.
The alveoli, located in clusters at the end of the bronchial tubes deep inside the lungs, are very fragile. As you breathe in air, the alveoli stretch, drawing oxygen in and transporting it to the blood. As you exhale, the alveoli shrink, which forces carbon dioxide out.
When emphysema destroys the alveoli, the air sacs can no longer support the bronchial tubes. They collapse and cause a blockage, which traps air in the lungs. This reduces the amount of oxygen that reaches the bloodstream.
With a grant from the American Lung Association, Dr. Paschini is studying lung stem cells that have the ability to repair tissue in the lungs that has been damaged. Her work previously found that specific signals sent by neighboring cells direct this stem cell population into forming new alveoli. She is analyzing the signals between the lung stem cells and their environment, in order to understand how these stem cells sense the lung damage, and how they are instructed to selectively replace a specific injured group of cells.
"Understanding this microenvironment in the lung will be critical in finding new cures for emphysema," Dr. Paschini says. "Current treatment doesn't repair the alveoli, although it can slow the process of lung damage. We want to actually reverse the damage, and form new functional tissue that will allow people to breathe more and for a longer period of time."
The need to develop new treatments is urgent, she says. People with emphysema and other types of obstructive lung disease face greater risk from air pollution, which is an increasing problem in developing countries.
Her research is focusing on a molecule called Tsp1 factor, which is involved in directing stem cells to repair alveolar tissue. "We are trying to figure out how that molecule is able to modulate the response that activates stem cells," Dr. Paschini says. "Without that, we are blind."
Page last updated: March 2, 2020