Stephanie Hansen was just 3 years old when her parents were given the reason for her persistent cough and digestive problems. She had cystic fibrosis (CF). And according to her doctor at the time, the long-term prospects weren’t good.
“When I was diagnosed, my parents were told that I wouldn’t graduate high school,” Hansen, now 28, says. “Then, when I graduated, the life expectancy for people with CF had gone up to 30 or 35 years old. Now the number is in the 40s. I’ve got a lot of hope that I’m going to be around into my 50s and 60s. Maybe longer.”
Hanson receives care at University Health System’s Cystic Fibrosis Center, a partnership with UT Health San Antonio. She credits the care she’s received for her new fondness for exercise and sense of optimism about survival.
Cystic fibrosis is a disease that affects nearly 30,000 Americans. It is a genetic condition that results in thickened secretions in the respiratory and gastrointestinal tracts. Patients with CF have problems with recurrent lung infections that result in progressive destruction of the lungs, as well as difficulties gaining and maintaining weight. They are also at an increased risk of developing diabetes.
The University of Texas Health Science Center San Antonio Cystic Fibrosis team began caring for patients at University Health System in 2013. The pediatric program was accredited by the Cystic Fibrosis Foundation in 2014 and the adult program in 2017. Today the center cares for nearly 200 individuals with cystic fibrosis from all over San Antonio and South Texas.
Dr. Donna Willey-Courand, director of the Cystic Fibrosis Center and chief of pediatric pulmonology at UT Health San Antonio credits improvements in care, patient education and research for patients living longer, more normal lives. Drugs targeting some of the most common genetic mutations that cause the disease are now available.
And for that reason, the center has evolved from a largely pediatric program to a comprehensive center that offers lifelong care. “Now all these kids with this chronic disease are surviving into adulthood, and we have the only adult cystic fibrosis program in South and West Texas,” said Dr. Willey-Courand.
Children with the disease transition at around age 18 to the adult program focused on the different challenges that older CF patients face.
Care is delivered by a specialized team of pulmonologists, an endocrinologist, respiratory therapists, a dietician, a social worker, nurses, a psychologist, and physical therapists all trained to manage the physical and psychosocial manifestations of the disease. The team provides care to people with CF throughout their lives – from the diagnostic testing performed on infants suspected of having cystic fibrosis based on newborn screening, through childhood and adulthood – including caring for adults with advanced lung disease in conjunction with the University Health System Lung Transplant Program.
It is the only center in San Antonio actively engaged in clinical research to develop new therapies to treat CF.
A new expanded Cystic Fibrosis Center opened at University Hospital in early 2019. It features eight exam rooms, an infusion room, a pulmonary function testing lab, and workspace for the nurses, social workers, respiratory therapists, psychologists and others who make up the CF team.
It will also have dedicated space for clinical trials, with comfortable areas for enrolled patients to receive study protocols and education.
“This enhanced space will enable us to fulfill our mission of delivering state-of-the-art care, providing education to the next generation of CF clinicians and researchers, and conducting research towards finding a cure for cystic fibrosis,” said Dr. Willey-Courand.