Newswise — DALLAS – March 31, 2015 – At 77, John Bunton rode a ski lift up a mountain near Ogden, Utah, and spent an hour hiking around at 11,000 feet, admiring the craggy scenery and breathing in deeply the crisp mountain air. In June, he plans to walk his daughter down the aisle.

Three years earlier, Mr. Bunton was on oxygen 24 hours a day and a simple trip to the grocery store was an undertaking. Travel anywhere above sea level was out of the question. A double lung transplant at UT Southwestern Medical Center gave Mr. Bunton back the active life he’d always led.

“My healthy new lungs are a precious gift. Should my donor’s family ever know who I am, it is important to me that they find that I am taking very good care of my gift and that I do worthwhile things with my life,” Mr. Bunton said.

A building contractor, Mr. Bunton had spent lots of time working outdoors and he had always been heavily involved in community projects, including serving as a construction manager for Habitat for Humanity. When a report from a routine physical at age 67 included a comment about possible early pulmonary fibrosis, he didn’t think much about it. But three years later, when a physician told him he heard crackles in his lungs, he took it seriously.

Idiopathic pulmonary fibrosis is a disease in which scarring of the air sacs of the lungs makes it difficult for oxygen to pass through the air sac walls into the bloodstream. It’s part of a group of restrictive lung diseases that make it difficult for the lungs to fill with air. When another family member has the same or similar lung disease – as is the case for Mr. Bunton ­– it’s considered familial pulmonary fibrosis. About one in 50 pulmonary fibrosis patients have this inherited form of the disease.

UT Southwestern researchers, led by Dr. Christine Garcia, Associate Professor of Internal Medicine and the Eugene McDermott Center for Human Growth and Development, have identified mutations in three genes that are linked to familial pulmonary fibrosis. Mutations in two of the genes affect telomeres, which are specialized structures at the ends of chromosomes, and the third affects a protein expressed only in the fluid that bathes the lung’s epithelial cells.

“Our ultimate goal is to understand what causes the genetic form of this disease so that effective medications can be developed to stem its progression,” said Dr. Garcia, who holds the Kern and Marnie Wildenthal President's Research Council Professorship in Medical Science.

Mr. Bunton knew his disease was progressing, and he began talking to doctors about the possibility of a lung transplant. Pulmonary fibrosis is the cause behind more than half of all lung transplants.

Lung transplants are more challenging long-term for patients than other types of organ transplants because the lungs are exposed to the environment in a way that internal organs are not. Every breath of air brings the possibility of exposure to microorganisms that could cause infection.

But the ability to breathe freely is well worth the care these patients must take to avoid exposure to respiratory infections for the rest of their lives, said Dr. Fernando Torres, Associate Professor of Internal Medicine at UT Southwestern.

“For years, they cannot walk to the mailbox, cannot walk that flight of stairs, without gasping for breath. Suddenly, you can do all the simple things that make up life again,” said Dr. Torres, head of the Pulmonary Hypertension Program.

Mr. Bunton said some medical institutions initially told him he wouldn’t be considered for a transplant because he was over 65. The most common age range for lung-transplant recipients is 50 to 64, but there is a growing trend for lung-transplant recipients to be 65 or older, according to the Organ Procurement and Transplantation Network. In 2013 – the most recent year for which data is available – 549 lung recipients nationwide were 65 or older. Altogether, 1,932 lung transplants were performed nationwide that year.

Dr. Fernando Torres, Medical Director of the Lung Transplant Program at UT Southwestern, said the UT Southwestern program looks at the patient’s physiological age, not chronological age. 

“We don’t consider age an absolute contraindication to transplantation,” said Dr. Matthias Peltz, Assistant Professor of Cardiovascular and Thoracic Surgery and Surgical Director of Cardiac Transplant at UT Southwestern. “If an older patient is an otherwise good candidate, we are comfortable transplanting them. Mr. Bunton fit that profile. His end-stage interstitial lung disease was his major illness. He was otherwise quite fit and highly motivated. In fact, he kept himself in much better condition than many of our younger candidates.”

In the spring of 2013, Mr. Bunton, who lives in Georgetown, Texas, was told to find a temporary place to live in Dallas in preparation for his surgery. “We were told the surgery was going to last six to eight hours, that a heart lung machine could be needed, and that I’d be unconscious and on a ventilator for six or seven days,” Mr. Bunton said.

In 2013, he headed off to surgery. Five hours later, Dr. Peltz greeted Mr. Bunton’s wife, Sue, with the news that the transplant lungs had been a perfect fit, that the heart-lung machine had not been needed, and that the surgery had gone exceptionally well. Four days later, Mr. Bunton was able to walk a few steps. Eleven days after his surgery, he left the hospital. Six weeks after his surgery, he was able to walk a mile.

Today, Mr. Bunton said he is careful to take his anti-rejection medicines twice a day and will cancel a visit to his son’s home if one of the grandchildren is sick. But for the most part, he said he is back to the life he led before his pulmonary fibrosis diagnosis. He travels and does volunteer work, trimming and patching the oak trees in his neighborhood.

Dr. Torres said transformations like these are a regular occurrence.

“As an academic medical center, we bring our advanced research and our experience to the bedside, benefitting patients who come to our institution with few remaining alternatives,” Dr. Torres said. “UT Southwestern is consistently one of the top 10 centers in the nation for lung transplant, having done more than 60 a year for the last three years.”

UT Southwestern’s one-year and three-year survival rates are the highest in North Texas, according to the Scientific Registry of Transplant Recipients.

“At UT Southwestern, with the efforts of our multidisciplinary team, we are comfortable transplanting patients that other programs might not consider and, more important, we achieve good outcomes in these higher-risk patient populations,” Dr. Peltz added.

UT Southwestern will soon join an elite group of about 20 medical centers nationwide that have performed 500 or more lung transplants, according to the Organ Procurement and Transplant Network.

Mr. Bunton is delighted to be part of these statistics. “I feel the same as I did when I was 50, and I was one healthy dude then.” 

About UT Southwestern Medical Center

UT Southwestern, one of the premier academic medical centers in the nation, integrates pioneering biomedical research with exceptional clinical care and education. The institution’s faculty includes many distinguished members, including six who have been awarded Nobel Prizes since 1985. Numbering approximately 2,800, the faculty is responsible for groundbreaking medical advances and is committed to translating science-driven research quickly to new clinical treatments. UT Southwestern physicians provide medical care in 40 specialties to about 92,000 hospitalized patients and oversee approximately 2.1 million outpatient visits a year.