Breath of Hope After Devastating Diagnosis

It started as a nagging dry cough. When it became so bad that she felt like she was choking, Delfina Stefhon went to the emergency room (ER). She later learned that she has pulmonary fibrosis. “I was really devastated by the diagnosis,” she said.

Pulmonary fibrosis is a chronic and progressive disease in which scar tissue develops in the lungs. They become stiff, making it difficult to breath. The disease also reduces the body’s ability to get oxygen from the airspaces in the lungs into the bloodstream. As the condition worsens, patients become more short of breath.

There is no cure. Stefhon’s doctors in New Mexico said her life expectancy was about five years. “I didn’t have a lot of hope,” said the 79-year-old great-grandmother. Her doctors told her they couldn’t do much except put her on steroids.

That wasn’t good enough for Stefhon’s son. He did some online research and told her, “We need to try National Jewish Health in Denver. They’re supposed to be the best respiratory hospital in the nation.”

 

Renewed Hope, Deeper Diagnosis

Stefhon called and made an appointment. Then she and her son made the trip to Denver. There they met Stephen Frankel, MD, a pulmonologist at National Jewish Health who also serves as the chief medical officer.

“One doctor treats you like you’re dying. Another treats you like you’re alive. Dr. Frankel did that for me,” Stefhon said. “The first thing I felt at National Jewish Health was hope. They made me feel like there’s more to this. Try to keep on living.”

A series of tests proved there was, in fact, more to Stefhon’s case. She has a rare form of pulmonary fibrosis called pleuroparenchymal fibroelastosis (PPFE). In PPFE, scarring in the lungs is made up of different cells and proteins than those typically seen in other forms of pulmonary fibrosis. The disease primarily attacks the upper lobes, the delicate lining around the lungs, and the lung tissue immediately adjacent to the lining.

As luck would have it, Stefhon was being treated by a PPFE pioneer. Dr. Frankel and his National Jewish Health colleagues in the Interstitial Lung Disease Program first described PPFE as a novel and distinct subtype of pulmonary fibrosis in a 2004 manuscript in the CHEST® medical journal.

To treat Stefhon's PPFE, Dr. Frankel prescribed medication to slow its progression.

 

Complex Surgery for Rare Form of Disease

Stefhon did well until two years later when, in May 2017, she had a collapsed lung, a known and serious complication of PPFE. ER physicians in Roswell, N.M., placed a chest tube to help her breathe. An abnormal passageway between the large airways in her lungs and the space between the membranes that line her lungs complicated her situation.

To address this complication, Dr. Frankel recommended highly specialized thoracic surgery. Stefhon couldn’t find a surgeon in New Mexico or Texas who could perform the complex operation. She and her fiance, Ralph Green, traveled to Denver, this time to Saint Joseph Hospital. There thoracic surgeon Robert Meguid, MD, removed multiple large air sacs and resolved the air leak in Stefhon’s right lung. The surgery was a success.

“Everything went perfectly. It was the best experience. Dr. Meguid is one of the sharpest tools in the shed," Stefhon said. "Saint Joseph Hospital has such great service, great staff and great food. They are just wonderful people. They treated me like royalty.”

While recovering, she watched her granddaughter get married through a live video feed on Facebook. “I got to see everything. She looked so beautiful,” Stefhon said. “I didn’t miss out on anything.”

 

Active and Thriving

Since returning home last summer, Stefhon is able to spend time with Green, her five children, 14 grandchildren and 15 great-grandchildren. “Before, I couldn’t do anything. I didn’t have enough energy because I couldn’t breathe,” she said. “Now I’m active. I can speak. Before, I’d get tired just talking.”

Stefhon continues to visit National Jewish Health for check-ups. She is grateful that her son urged her to make that first trip to Denver. “Without my doctors at National Jewish Health and Saint Joseph Hospital, I really don’t think I would have lasted this long. Dr. Frankel, Dr. Meguid and the wonderful people at Saint Joseph Hospital saved my life,” she said. “I’m happy to be alive and enjoying life.”