BET TER LIVING
Andy Lipman first researched cystic fibrosis (CF) when he was 9 years old, wanting to find out more about the disease he’d been diagnosed with at birth. He discovered that people with CF, a progressive, genetic disease that causes thickened mucus to form in the lungs and other organs, rarely lived to age 25. “I was shocked,” he says. “My parents were always telling me, you’ll be fine. But here was the encyclopedia, telling me something else. Which was true? I got really upset.”
Andy Lipman (on his mother’s lap) was diagnosed with CF when he was born. His older sister died of CF as an infant. Left: Andy Lipman, 46, who has CF, uses a percussive vest daily to ease his breathing. He has outlived every prediction due to new drugs and therapies.
Lipman’s sister had died of CF before he was born, living only 16 days. “Growing up, I didn’t talk about my CF or let anyone in,” he says. “My parents told my teachers. I was embarrassed and afraid—I didn’t know how long I was going to live.” His parents encouraged him to live a normal life and play sports, which “really helped me physically.” He went to a pediatric CF clinic for his care all the way through college. “There were no adult clinics back then,” he says. “Adult CF was an oxymoron. People just didn’t live that long.” Lipman, now 46, has benefitted from advances in treatment and medications that have extended the median life expectancy of people with CF to 42 to 47 years, with some patients living far beyond that. His doctor, Emory pulmonologist Randy Hunt, says CF has become a chronic—if still fatal—illness. “It’s still a tough disease to be born with,” Hunt says. “When you’re very young, your parents can make you do all the right things. As you get older and go to school, you have to take handfuls of pills every time you eat, wear a mask at times, and use a vest for high-frequency chest wall oscillation…things that detract from your social experiences. Early adulthood can be a really difficult time. Deaths for CF patients still peak in their 20s and 30s.” Indeed, in college, Lipman experienced depression and anxiety. He had to carry his medications everywhere. The percussion vest he used each day to break up the mucus in his lungs was big and loud. “It was hard to feel normal, like I fit in,” he says. “I stopped doing treatments or taking my medications. I was skinny and felt ugly.” He stopped going to classes, and seldom left his room on weekends. “My lung function went down pretty severely. Things were falling apart,” he says. “But then I got mad at myself and turned it around.” Lipman took a weightlifting class, got stronger, and went from 100 pounds to 165 pounds. He did his treatments every day. “Living life requires more than just breathing. It requires loving every minute we have on this earth,” Lipman says. New medications and therapies are help-
ing CF patients lead longer, healthier lives—a feat that still requires a lot of care and self-discipline. There is still no cure for CF, but there are drugs to help thin and clear the thick mucus from the airways, enzymes to help digest food, and antibiotics to prevent and treat infections. Recently, medications tuned to the genetic mutations that cause the disease have helped people with CF experience fewer flare-ups and hospitalizations and improved lung function. Inhaling hypertonic saline as well as other medications through a nebulizer helps with breathing problems. “Most patients use the nebulizer two times a day for at least an hour, and sometimes up to four times,” Hunt says. “People think of CF as a lung disease, but really it is a systemic illness, impacting a patient’s bones, reproductive system, GI tract, pancreas, and other internal organs. Many patients need to eat 3,000 calories a day just to maintain their weight, and they have to take one to 10 pills every time they eat a meal,” says Hunt, an assistant professor of medicine at Emory
Winter 2020
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