Featured News - Current News - Archived News - News Categories

Cystic fibrosis research at UB has been transforming patients' lives for decades


Fri, May 31st 2024 09:10 am

During National Cystic Fibrosis Awareness Month, UB highlights its commitment to studying and keeping up with the dramatic advances in treating CF

By the University at Buffalo

During the 1950s, a child with cystic fibrosis rarely lived long enough to attend elementary school. Today, many people with CF are achieving their dreams of pursuing careers, getting married, having children, and living into retirement.

Over the past three decades, critical aspects of that dramatic success story have unfolded in the laboratories and clinics of faculty members in the Jacobs School of Medicine and Biomedical Sciences at the University at Buffalo. Those efforts have now been documented in a three-part series posted during May, National Cystic Fibrosis Awareness month, on the Jacobs School’s website.

Through all the stories a single theme emerges: How dramatically the research breakthroughs have led to real, tangible improvements in patients’ lives. Patients describe how they had grown up thinking their lives would be prematurely cut short and now find themselves able to live fully into adulthood.

The series, titled “UB contributing to dramatic results in CF care, research,” describes the changes that have transformed CF care and what UB’s role in them has been, starting with the development of the cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies. These therapies precisely correct the malfunctioning protein that is made by the CFTR gene.

The first patient in the U.S. in a multisite clinical trial of CFTR modulators was enrolled in Buffalo in 2009. The first of these drugs was approved for adults with CF in 2012, and this February it was approved for use in infants as young as 1 month old. If diagnosis is made that early, UB researchers say, this drug can prevent most CF symptoms from occurring, an almost unimaginable improvement in the patient’s quality of life compared to those born just 10 or 20 years earlier.

The story titled “Borowitz oversaw key CF research breakthroughs” describes the legacy of pioneering UB researcher Drucy S. Borowitz, M.D., clinical professor emeritus of pediatrics, who was director of the Cystic Fibrosis Center at John R. Oishei Children’s Hospital in Buffalo for more than 25 years.

During that time, Borowitz helped bring multiple clinical trials for drugs to Buffalo, resulting in many of the improvements from which CF patients worldwide are now benefitting. For many years, she also was the primary physician for families experiencing CF in or near Western New York.

One of those families is the Argentieri family of Hornell, New York, whose two daughters, now adults, have lived with the disease. In the story about their experience, titled “Love, tenacity guide family journey through disease,” their mother, Colleen Argentieri, described that their daily routine as kids consisted of removing their feeding tubes every morning (they needed night feeds to keep their weight up) and hooking themselves up to the chest vest to do chest physical therapy exercises. They took medications to clear their airways of mucus, and antibiotics through a nebulizer. Whenever they ate, they had to take special enzymes. At night, they were hooked back up to their feeding tubes.

Both Carley and Alexandra Argentieri now work full time and are married. Both have had serious, life-threatening struggles as a result of CF. But through it all, they say that in addition to their family’s strength and support, the care they received from the physicians at UB, Oishei and Buffalo General Medical Center, especially Dr. Borowitz and Carla A. Frederick, M.D., associate professor of medicine and adult pulmonologist at the CF Center of Western New York, has been their lifeline. Adults with CF are cared for at Buffalo General Medical Center.

“Honestly, we owe everything to Dr. Frederick and that team in Buffalo, including Dr. Borowitz, who was my doctor my whole childhood,” Carley said. “They are family to us. With everything Alexandra went through and Dr. Frederick fighting for her, there are not enough words to describe how grateful I am.”

Hometown News

View All News