For generations, life with cystic fibrosis has meant hours of treatments each day. Families scheduled their lives around inhaled medications, mucus-thinning therapies, antibiotics, and airway clearance routines that filled mornings and evenings alike. It was exhausting, relentless, and necessary. So when a new multi-site study from researchers at CU Anschutz revealed that many people with cystic fibrosis could begin to step back from this burden after starting the triple-drug therapy elexacaftor, tezacaftor, ivacaftor, known as ETI, the moment felt almost surreal. It hinted at a quiet shift after decades of noise.
“This is incredibly meaningful for individuals and families living with CF,” said lead author Scott Sagel, MD, Ph.D. The words carried the weight of years of fear and fatigue, but also something brighter. Hope.
The Drug That Works From the Inside Out
ETI is different from traditional therapies. Instead of managing symptoms at the surface, it helps the malfunctioning protein responsible for cystic fibrosis work more effectively. In other words, it strengthens the lungs from the inside rather than constantly fighting what goes wrong on the outside. That change in strategy sparked a question. If ETI improves lung function so deeply, could people finally reduce some of the daily treatments that once felt non-negotiable.
Researchers were eager to find out. They tracked more than 600 children and adults across multiple CF centers in the United States. Children aged 6 to 11 were followed for up to three years. Adolescents and adults were observed for as long as four and a half. The study, published in the Journal of Cystic Fibrosis, became part of PROMISE, one of the largest long-term efforts to understand how ETI performs in real-world care.
A Slow Unwinding of Burden
As the months turned into years, something unexpected began to unfold. Across all age groups, use of long-standing respiratory therapies steadily declined. Hypertonic saline, dornase alfa, inhaled antibiotics, and oral azithromycin were once pillars of CF care. Yet as people remained on ETI, teens and adults cut their use of these treatments by nearly half. Younger children mirrored the trend.
It wasn’t a sudden leap. It was a slow unwinding of burden.
Most striking of all, stepping back from multiple therapies did not appear to harm health. Lung function held steady. Respiratory symptoms did not surge. This pattern held true regardless of age, sex, or baseline lung strength. Those who reduced treatments tended to start ETI with higher lung function and were less likely to be infected with Pseudomonas aeruginosa, a common CF-related infection. But the overall picture remained clear. Many people were thriving with less.
The Emotional Weight of Time Gained Back
“For decades, people with CF have spent hours every day managing their disease,” Sagel said. “Our findings show that many have stepped back from some of those time-consuming therapies thanks to ETI.”
For any family touched by cystic fibrosis, the emotion behind this statement is immediate. Time is not an abstract idea in CF care. It is a daily negotiation. Treatments crowd childhood, adolescence, adulthood. They interrupt school mornings, dinners, vacations, even sleep. Reducing them is not merely convenient. It is life-changing.
“For many families, the daily time commitment required for CF care can be overwhelming,” Sagel said. “Seeing people maintain good health while doing fewer respiratory therapies is incredibly encouraging. A reduction in treatment burden can have a profound impact on the quality of life.”
A Future That Requires Thoughtful Decisions
But even with such promising results, Sagel emphasized caution. Every person’s body, history, and health trajectory is different. “The opportunity to simplify daily treatment is exciting but decisions to stop or continue therapies should be made through shared decision-making with a clinician who understands each person’s overall health,” he said.
The study could not capture the reasons why individuals chose to stop certain therapies, making communication with clinicians essential. The findings open the door, but they do not tell anyone to walk through it alone.
Next steps for researchers include exploring whether some treatments could still be helpful when used only during respiratory illnesses rather than daily. There is also a need to understand whether mechanical airway clearance remains necessary for people who no longer rely on inhaled mucus-thinning therapies. As Sagel put it, “The goal is to help people with CF and their care teams maintain strong long-term health while easing daily treatment demands.”
PROMISE, the project behind the work, continues to follow people across numerous CF centers, including teams from Seattle Children’s Research Institute, the University of Washington, the University of Alabama at Birmingham, the Children’s Hospital of Philadelphia, Washington University in St. Louis, and The Hospital for Sick Children in Toronto.
Why This Research Matters
This study signals a profound shift in the lived experience of cystic fibrosis. For the first time, many people are not only feeling better because of a therapy. They are reclaiming hours of their daily lives. That change carries emotional, practical, and lifelong meaning. It represents a bridge between medical progress and human possibility.
If ETI allows people with cystic fibrosis to breathe more easily while doing less every day, then the future of CF care may look very different from its past. Not lighter in responsibility, but lighter in weight. A future defined not by endless treatments but by the freedom to choose how to spend time once spent fighting for each breath.
And that, in Sagel’s words, is “incredibly meaningful.”
More information: Scott D. Sagel et al, Elexacaftor/tezacaftor/ivacaftor is associated with long-term reduction in use of chronic respiratory therapies in cystic fibrosis, Journal of Cystic Fibrosis (2025). DOI: 10.1016/j.jcf.2025.11.016
