Adult Cystic Fibrosis Patient Thriving with the Help of UH Program

Nick Kelly spends a third of his year at University Hospitals – when he isn’t boating, ziplining through the jungle or designing fashions.

The Cleveland man was diagnosed as an infant with cystic fibrosis (CF), at a time when children with this incurable genetic disorder were not expected to survive into adulthood. He leapt past every life expectancy mark since his parents found the experts at UH Rainbow Babies & Children’s Hospital, which has been at the national forefront of cystic fibrosis care since 1957. Featuring comprehensive clinical care and opportunities for people with CF to participate in numerous clinical trials, it has drawn patients from across the country.

And now, thanks to advancements in treatment of CF, more children are becoming adults. In fact, approximately 60 percent of people living with CF in the United States in 2023 were at least 18 years old.

“I’ve been coming to UH my whole life,” says Nick, 38, who has been an author, lecturer, professional dancer, DJ and clothing designer. He wrestled and played baseball competitively in school and went to college.

“For me, cystic fibrosis was just a part of my life,” he says. “My parents never allowed me to see myself as different or sick, and that attitude resonated throughout my life.”

The Most Advanced Care for CF Patients

CF is caused by dysfunction or absence of a cellular protein called CFTR that balances the salt and water content of secretions, explains Alex Gifford, MD, FCCP, Director of UH’s Adult CF Program and CF Therapeutics Development Center at UH Rainbow. Lack of CFTR activity creates sticky mucus in the lungs that damages bronchial tubes and allows germs to cause chronic infections. In 85 percent of patients, CFTR dysfunction prevents digestive enzymes from being released into the intestine, leading to malnutrition. Damage to these and other organs is progressive but treatable, Dr. Gifford adds.

UH Rainbow’s LeRoy W. Matthews, MD Cystic Fibrosis Care Center provides the most advanced care available, including a wide range of inpatient and outpatient services, access to cutting-edge clinical trials, and unwavering support from a compassionate multidisciplinary care team. It’s named in honor of the physician who pioneered the country’s first comprehensive CF treatment program in 1957.

“Every innovation our community makes builds upon the last to promote the health and welfare of these patients,” says Dr. Gifford, noting the contributions of generations of physician-scientists.

CF patients have abnormalities in both copies of the CFTR gene, the blueprint that cells use to make CFTR protein. New medications designed to be taken by mouth called CFTR modulators increase the amount and/or function of CFTR protein, targeting the root cause of the disease rather than just the symptoms it produces.

Coordinated Care for Improved Outcomes

CF patients have been admitted to hospital wards for decades, but as science and medicine advanced, CF patients began living longer and well into adulthood. As recently as a decade ago, when highly effective CFTR modulators were not yet available, the 10-bed CF unit at UH Rainbow was routinely full of patients who required treatment for problems ranging from flare-ups of chest infections requiring intravenous antibiotics to severe constipation, pancreatitis and malnutrition. The advancement of modulators has caused hospital admissions to decrease across the country, although for some there is still an unprecedented need.

“Very few other conditions have this much infrastructure built around a patient,” says Ankica Katic, MSN, FNP-C, an adult nurse practitioner and Program Coordinator of the LeRoy W. Matthews Cystic Fibrosis Center who coordinates care for adult CF patients along with respiratory therapists, pharmacists, dietitians and social workers. “Each team member plays an integral role in the care of CF patients. We are the true definition of multidisciplinary care.”

Patients like Nick now go to Lakeside 55 to receive care among other adults. Nursing Director Arneta Montgomery, BSN, RN, CMSRN, notes the strength of the collaborative partnership between Dr. Gifford’s team and Lakeside 55, combining clinical expertise, quality, and patient perspective to coordinate the optimal healing environment for adult patients.

‘No Expiration Date’

Nick knows he needs to get to the CF clinic or emergency department when he’s extremely fatigued and can’t do his usual activities without being short of breath. During antibiotic infusions that can last 12-14 hours of each day in the hospital, Nick writes chapters for his children’s book series, produces inspirational talks and works on a variety of other hobbies.

“I’ve been blessed to find multiple passions in life, and patient enough to perfect them,” says Nick, who has blown past every milestone mark and stopped paying attention when he lived past his teens. “I have no expiration date.”