John Morthanos recalls sitting in his pulmonologist’s office with his wife, Paula, listening to his doctor tell him he had idiopathic pulmonary fibrosis and, unless he received a lung transplant, two years to live: “I was hoping she hadn’t heard my death sentence.”

John’s case

Idiopathic pulmonary fibrosis, or IPF, is a rare disease where scar tissue builds up in the lungs. It can take months or even years for symptoms—breathlessness and a dry, hacking cough—to develop. There is no known cause, though there may be a genetic component. The median survival time is 3.8 years.

Morthanos first noticed he was short of breath while tying his shoes or drying off after a shower. Concerned about his breathing, he went to his primary physician, who diagnosed him with sinusitis, beginning a series of visits to specialists who found nothing wrong.

 “I continued to go downhill,” he says, adding it was becoming difficult to perform simple tasks. “I wasn’t able to walk 20 feet without stopping.” A biopsy confirmed he was mildly fibrotic.

 An urgent turn

Morthanos’ cardiologist performed a nuclear stress test, which showed he had a strong heart. But he was still having difficulty breathing. His doctor sent him to Yale Medical Center, where he was diagnosed with IPF and placed on oxygen therapy, which is the standard course of treatment along with pulmonary rehabilitation.

A small percentage of patients receive a lung transplant. John was one of the lucky ones; he received a single lung transplant in 2012. “It feels like 1984,” he glows. Morthanos adds that he can walk up stairs without being short of breath now, and his wife’s support has been indispensable. “I couldn’t have done it without her,” he says. “Fighting IPF is a full-time job.”

 A new life

Today, Morthanos serves as an ambassador for the Pulmonary Fibrosis Foundation and encourages individuals who are having breathing problems to see their doctor. “If I had done that, my journey wouldn’t have been as long or painful,” he says. “If your doctor says you’re fat and allergic to cats, find another doctor.”

Recently, two drugs have been approved that slow the progression of IPF. There are new drugs in clinical trials and more are in the pipeline. “The future is very bright,” sums Dr. David Lederer, senior medical advisor for the Pulmonary Fibrosis Foundation. He looks forward to finding not only a cure, but a way to prevent this disease and “wipe it off the face of the earth.”