Jack Engle was 84 when a dry, hacking cough and nagging fatigue prompted a visit to his physician. After several appointments and numerous tests, he was diagnosed with Idiopathic Pulmonary Fibrosis, commonly called IPF.

Getting acquainted

“I’d never heard of it before,” says Engle, who quickly followed his doctor’s advice to “finally stop smoking.”

IPF, according to the National Heart, Blood and Lung Institute, is a disease in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The formation of scar tissue is called fibrosis. When the lung tissues thickens, lungs don’t properly move oxygen into the bloodstream and other organs don’t receive the oxygen they need.

Since IPF symptoms resemble other lung conditions, it can sometimes be difficult to diagnose. But signs to look out for in addition to the fatigue and cough Engle experienced, include shortness of breath, joint and muscle aches and an unexplained loss of weight. One notable red flag is also a widening of fingers and toes—called “clubbing.”

Getting ahead of IPF

There are no hard and fast rules, since cases vary from person to person. However, genetics are thought to factor into a higher incidence of contracting IPF.

While there currently is no cure, IPF treatments often include corticosteroids, sometimes in combination with other drugs that suppress the immune system, as well as oxygen therapy, pulmonary rehabilitation and, in some cases, a lung transplant. The most important actions to take upon diagnosis that can improve and extend your lifespan include quitting smoking, eating healthy, getting flu shots and seeking ways to reduce stress. And of course, be sure to make regular appointments with your physician.

There is good news on the horizon. Research breakthroughs are helping doctors learn more about IPF, allowing them to better diagnose the disease. Several medicines are currently being examined to slow the progress of IPF, especially in regard to the formation of scar tissues.