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(ST. LOUIS, MO) – May 17, 2004 – Dr. Thomas Ferkol has a mission in life: To push the life expectancy of children with cystic fibrosis (CF) "off the map."
"I tell the parents of my new patients that I’m going to dance at their children’s weddings," says Dr. Ferkol, who has devoted his career to research and treatment of CF, the most common genetic disease in America and a major cause of death among children. Dr. Ferkol, Cystic Fibrosis Care Center Director at St. Louis Children’s Hospital and Assistant Professor of Pediatrics at Washington University School of Medicine, has specialized in treating CF patients for more than a decade. Although he hasn’t danced at any weddings yet, his dancing shoes are ready. "Some of my patients are now adolescents and young adults who are in serious relationships," he says.
One of Dr. Ferkol’s success stories is patient Caryn Wixom. Caryn currently is a freshman in high school, an excellent student and, at 15 years old, an accomplished athlete. She swims, plays soccer and runs. In fact, she went to the state’s high school competition in cross-country this year. She also has CF.
Caryn was diagnosed with CF when she was a baby. "My mother took me to the doctor because I was wheezing and sweated a lot," Caryn says. During the visit, her doctor discovered that she had not gained weight in two months.
"Caryn is a phenomenal success story for cystic fibrosis," says Dr. Ferkol. "She shows what you can achieve if you battle the disease and don’t let it control you."
Her success is a result of the aggressive therapy for CF that is advocated by the Cystic Fibrosis Center. Her treatment includes daily medications and breathing treatments. "I have airway clearance twice a day. Until five years ago, my parents had to pound my chest every day to clear my lungs. Now, I put on a little vest, which vibrates and clears my lung," Caryn says.
"In the 1950s, the life expectancy for CF was about five years old," Dr. Ferkol says. "By the 1970s, it had increased to 16 to 18 years of age. Today, the average life expectancy is about 34 years." Dr. Ferkol believes it can be higher – even now, before hoped-for breakthroughs in treatment. Dr. Ferkol has three key messages to educate parents, physicians and the public about cystic fibrosis:
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"Don’t just stand there, do something." Aggressive treatment can make a difference in CF. "Battle the disease and you can win."
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Not everyone who has CF is Caucasian. While it is more common among people of northern European descent, it also occurs in African Americans, Hispanics and Asians. Dr. Ferkol wants to get the word out to pediatricians and other physicians who treat children with lung problems to consider CF as a diagnosis in all children – because the earlier you treat CF, the better.
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Children with CF need Center-based care. "One of the most important things that happened over the past half century is the advent of multi-disciplinary care. I believe the most important impact on survival has been the coordinated, aggressive treatment by physicians, nutritionists, physical therapists, respiratory therapists, and others who specialize in this disease."
"Cystic fibrosis can sneak up on you," Dr. Ferkol says. "You may not realize that pulmonary function has begun to decline, and it can reach a point where you can’t regain lost lung function. But sometimes when patients are doing very well, there is a natural tendency to relax the therapies prescribed for CF and not be as aggressive as recommended. Even in kids who seem healthy, the effects on the pulmonary and nutritional deficits are still occurring."
According to Dr. Ferkol, pulmonary complications are the primary reason for shortened life spans and cause 95 percent of all deaths from CF. "We want to intervene when children with CF are very young to maintain normal nutrition and lung function for as long as possible. I have every expectation that, if every child received the best therapy, we could get an average life expectancy well into adulthood."
Dr. Ferkol’s patient Caryn looks forward to her future. "I want to be a veterinarian or an interior designer. I’ve had dogs and guinea pigs all my life; I love animals. And I also love to decorate. I did my room, and redecorated the living room and dining room of our house. Everyone in my family likes what I did."
"I wish I didn’t have CF, but it gives me something to think about in life. I try to be happy every day," she says. "I’ve been playing soccer since I was in kindergarten. I started cross-country competition in the fall, partly to compete with my older brother. I was always much faster than he was, but then he got faster than me and I decided to take him on.
"I feel challenged that I have CF and want to tell my story, because I know there are others who don’t push themselves so hard. I want to tell them that it’s healthy to push yourself. I know if I do, I’ll live longer."
Caryn’s mother, Yvonne Hill, first learned Caryn had CF when her daughter was nine months old. "The first thing I did was go to the medical library to learn more about the disease. I found a study that said, generally speaking, boys with CF had a longer, higher quality of life. The only thing they could figure out that made a difference was that boys played football, girls played piano. I thought there was more motivation in competitive sports.
"We had always encouraged Caryn to be physically active. She started swimming at the YMCA when she was six months old. We continued to encourage her when she got into competitive sports.
"We gave her medications, and did what the doctors recommended. But we also encouraged her in athletics; we didn’t try to hold her back because she had CF."
According to Yvonne, Dr. Ferkol is right: you can take control of the disease, and Caryn is an example of what you can accomplish.
St. Louis Children's Hospital has provided specialized care for children for more than 120 years. Children's Hospital is affiliated with Washington University School of Medicine, ranked the second best medical school in the country by US News & World Report. In 2003, Child magazine ranked St. Louis Children's Hospital sixth on its list of the 10 Best children's hospitals in the country, and fourth in neonatology/neonatal intensive care unit (NICU). St. Louis Children's Hospital also is a member of BJC HealthCare.