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Wear The Gown: Managing the rare disease of cystic fibrosis

Only 30,000 people in the U.S. have the disease.

SAN ANTONIO — There are 70,000 people in the world with cystic fibrosis. 30,000 of them are here in the U.S. In this Wear The Gown, we take you into the only "CF" clinic in San Antonio that takes care of patients from birth, all the way into adulthood. 

"Cystic fibrosis is a genetic disease that causes a defect in a protein that is responsible for helping the mucus in your airways and in your G.I. tract be thin and easy to clear," said Dr. Holly Keyt, the Director of the Adult Cystic Fibrosis Program at University Hospital within the University Health System. She said those with CF are living longer thanks to medical advancements. "When I started as program director in 2015, people were living into their mid-30s. That was the life expectancy, but now it's the late to mid-40s; people are living longer than they ever have before."

If the protein causing CF, called the CFTR protein, doesn't work, you can get thick, sticky mucus that clogs up your airways leading to infection and downstream complications like pneumonia or bronchiectasis. "It's important to get to care right away and get established with a good doctor that you really trust, and can work with you throughout childhood, adolescence, and then transition into our center as adults," Dr. Keyt said.

Dr. Keyt said managing CF is not easy. She added, "People who have cystic fibrosis, they have to do these multiple therapies every day and they take sometimes hours to set up the equipment to do the treatments."

When patients come in for a clinic appointment, they take a pulmonary function test, which measures lung capacity. Eyewitness News reporter Jeremy Baker tried one for himself, but even for someone like him with healthy lungs, the test was difficult. But for a CF patient, it's even harder but necessary.

For more information about family health, call 210-358-3045. You can also find the rest of our Wear The Gown stories, just go to WearTheGown.com.