Steve L. says he “never had an inkling” he would develop a serious lung problem. In good shape his entire life, he ran cross country in high school and coached youth ice hockey as an adult. Even his job as a custom cabinet maker kept him active and on his feet all day.
That’s why he was shocked when he found himself gasping for breath on his way into a Philadelphia Eagles game.
“We took the stairs to our seats, and suddenly I was doubled over and couldn’t breathe,” he says. “My kids were freaking out…they thought I was having a heart attack.”
Instead of a heart attack, Steve was experiencing the first sign that he had a serious genetic disorder called Alpha-1 Antitrypsin Deficiency (AAT).
A surprising diagnosis
A series of appointments with various doctors followed that Eagles game, but Steve struggled to find a diagnosis.
“My cardiologist said I had the heart of an 18-year-old, but it wasn’t until I visited a local pulmonologist that I was diagnosed with AAT. I was stunned.”
AAT is a genetic disorder that occurs when there is a severe lack of a protein in the blood called Alpha-1 antitrypsin. The main function of the protein is to protect the lungs from inflammation.
As the disease progresses, patients become increasingly out of breath. There are medications to slow AAT’s progression, but there is no cure. Long term, many patients require a lung transplant to survive.
Although he hadn’t yet reached that point, Steve’s pulmonologist recommended he get a second opinion. Steve chose the Temple Lung Center.
More options for a better quality of life
At Temple, Steve met with pulmonologist and AAT specialist Matthew Gordon, MD. Dr. Gordon determined that Steve didn’t need a lung transplant yet. Instead, he recommended a procedure called Bronchoscopic Lung Volume Reduction, or BLVR for short.
During BLVR, tiny valves are implanted into a patient’s airways to block off diseased parts of the lung. The valves allow healthier regions of the lungs to expand and function more efficiently. The result can be easier breathing, improved exercise tolerance, and a better quality of life.
By this time, Steve was increasingly having trouble breathing. He had to retire from the job he loved, couldn’t walk more than 25 feet without getting winded, and was using supplemental oxygen. He decided to proceed with BLVR.
A game-changing procedure
In 2019, Temple Lung Center Director Gerard J. Criner, MD implanted two valves in the middle lobe of Steve’s right lung. The valves allowed the lobe to “deflate,” thus allowing healthier lobes around it to expand and work more efficiently.
According to Steve, “it’s made all the difference in the world.”