For her entire life, Jenny Livingston has lived with the devastating effects of cystic fibrosis, which used to send her to the hospital three to four times each year. However, the dedicated providers at the Cystic Fibrosis Center have never given up on her.
“They have seen me through some incredibly difficult times,” the 36-year-old Manti resident said. “They care deeply about me as a person.”
Since the Cystic Fibrosis Center opened 60 years ago, the outlook has changed dramatically for patients like Livingston. Once expected to survive only a few years at most, most are now living into middle age and beyond.
Livingston, who has a 15-year-old daughter, is in graduate school studying social work at Utah State 㽶Ƶ.
“There’s been times where, not seriously, but sometimes we talk about if we would ever leave the state,” she said.
One of the main reasons I wouldn’t want to leave Utah is this center.
People with cystic fibrosis typically experience respiratory and digestive problems, because the genetic condition causes mucus in their body to be sticky and thick. The thick mucus leads to infection and blockages. Nearly 40,000 Americans live with the disease. That group is increasingly diverse. As of 2023, more than 17 percent identified as non-white.
In Utah, patients like Livingston often work with Teresa Kreger RRT, a respiratory therapist who has been at the center for 30 years.
“When you come to work at our Cystic Fibrosis Center, you either decide this is not for me or you stay for life,” Kreger said.
Kreger first cared for patients with cystic fibrosis at the 㽶Ƶ of Utah Hospital, providing them with three treatments daily and staying at the patient’s bedside for 30 to 45 minutes each time.
“You really got to bond with them,” she recalled.
When Kreger joined the center a few years later, it had 70 adult patients with cystic fibrosis with a median life expectancy of about 27 years. Many young adults never made it into their 20s.
But, during the 1990s, new medications started to become available. One thinned the secretions in patients’ lungs. Another, an inhaled antibiotic, helped decrease bacterial infections.
These drugs helped dramatically improve patients’ quality of life and lung function, resulting in many living years beyond what had been previously expected.
Patients with cystic fibrosis now have a median life expectancy of 61. With patients surviving longer, the patient population has boomed. Today the center serves almost 400 people and is in the process of hiring a third respiratory therapist.
In many ways, it’s been leading the way all along.
A History of Cystic Fibrosis Care
More than 20 years ago, the center was the first to bring quality improvement to cystic fibrosis care.
Bruce Marshall, MD, led the initiative as more children with cystic fibrosis survived into adulthood. Dr. Marshall became the first director of the newly-created , which was established independently of the pediatric center to address the distinct nature of the disease in young adults.
The goal was to determine which medical practices worked best and to apply them consistently—while decreasing unnecessary tests.
“We’ve seen sea changes, really,” said Theodore Liou, MD, Director of the Adult Cystic Fibrosis Center since 2001. “Quality improvement has been a huge part of it.”
After Dr. Marshall moved to the Cystic Fibrosis Foundation, quality improvement spread nationwide.
The 㽶Ƶ of Utah Cystic Fibrosis Center was also one of the first in the nation to have full-time pharmacy support integrated in the delivery of care directly to patients, due to the efforts of . The center is also the first to have a full-time pharmacy technician to help patients navigate insurance and other challenges in obtaining life-preserving medications.
Thanks to a groundbreaking prediction model developed by Dr. Liou, providers can now gauge whether a patient will survive for at least five more years. This information can alter the course of someone’s treatment—and their life.
Fifteen to 20 years ago, patients weren’t thinking about getting married or going to college. Then when Dr. Liou came up with this prediction model saying you have a 97 percent chance of living 5 more years … it changed the outlook of a lot of patients.
Dr. Liou recalled a patient with cystic fibrosis who was shocked to learn she was not as sick as she assumed.
“I gave her a note saying she had ‘mild disease.’ And then the tears start rolling down her cheeks and she said, ‘Does this mean I’m not going to die?’” he remembered. “Her mother was in the corner sobbing.”
Though she later moved away, Dr. Liou remains in touch with his former patient — more than two decades later.
The Cystic Fibrosis Center now has multiple patients in their 70s.
“I used to say, ‘My job is to turn you into a little old lady or a little old man, and I need your help to do that,’” he said. “I think a lot of the patients early on didn’t believe me, but there are patients who are getting there.”
Steeped in a culture of research, the center has participated in dozens of clinical studies. Livingston has been involved in many.
“It feels like a way to give back,” she said. “I have lost more friends than I can count to cystic fibrosis. It’s devastating to feel like there’s nothing you can do.”
Thanks to new medications, Livingston no longer goes to the hospital three to four times each year. In fact, since starting a new drug called Trikafta in 2019, she hasn’t been hospitalized at all.
But it’s not just the drug, it’s the people at the Cystic Fibrosis Center that help her stay positive.
“They’re so invested in my success,” she said. “They’re always going to do what they truly believe is best.”