By MORGAN MANNS
Roughly 30,000 people in the United States — 70,000 worldwide — are diagnosed with cystic fibrosis, according to the Cystic Fibrosis Foundation. About 1,000 new cases of cystic fibrosis are diagnosed each year and an additional 10 million people are symptomless carriers of the defective CF gene.
May is Cystic Fibrosis Awareness month and one local family touched by the disease shares their story to increase residents knowledge about the disease.
“This disease affects everything, mentally, physically and emotionally,” said Kara Doerfer, Fostoria, who was diagnosed with CF at birth. She has been attending Nationwide Children’s Hospital in Columbus all 30 years of her life.
CF is a genetic disease that primarily affects the lungs and digestive system, according to Kara. The inherited gene causes mucus to build up in the lungs and other vital organs in the body.
The genetics of the disease causes CF patients to create more mucus than that of the average individual. The thickened mucus damages the organs and traps bacteria, which makes CF patients more prone to contract infections.
“It’s quite a fight for her,” said Arthur Doerfer, Kara’s husband. “It’s a fight for us as a family.”
Kara is unable to work and said she needs an insurmountable amount of effort to move around and do everyday activities.
She takes approximately 54 pills a day, takes daily aerosol treatments and uses an inflatable vest that vibrates the chest at a high frequency to help loosen and thin the built-up mucus. She must use the vest three times a day for 30 minutes. Every 10 minutes during use, she must conduct huff coughs, which is when she coughs really hard to bring up the loosened mucus.
Although she undergoes these treatments, Kara said she still experiences severe coughing fits, which sometimes cause her to pull muscles, crack ribs and even cough up blood.
“It’s a lot you have to do and some days it’s really hard,” she said. “I’ve had many close calls of basically dying.”
Arthur recalled a night Kara was violently coughing up blood and almost lost her life.
“I woke up because I heard a noise; I didn’t know what it was,” he said. “She had started coughing and blood was running out. “¦ She almost drowned in her own blood.
I propped her up and cleaned out her mouth and got her back to breathing. I caught her just in time.”
“I was asleep,” Kara said. “I vaguely remember (Arthur) coming in and turning on the light and I remember him saying my name. “¦ The next thing I know, the EMTs were there.”
Although her parents did not have CF, they each had a defective CF gene. Kara’s older brother, Elvis King, was diagnosed with CF when he was young. He received a lung transplant and passed away two years later, at the age of 24.
“It’s scary,” Kara said. “He still ended up passing away after receiving the transplant.
“I have to make sure that I continue taking care of myself. If I don’t, then I’m not going to be here much longer.”
The doctors recently gave Kara a 30-40 percent chance at living another five years, at most. She said this is why she is considering placing herself on the transplant list for new lungs, although no concrete decisions have been made. Statistically, the transplant will increase her chances for survival by another 30 percent.
“To look at her, you wouldn’t know there is anything wrong with her,” Arthur said. “They don’t know what’s on the inside.”
Arthur said there are times when Kara gets a coughing spell and, because the disease has the greatest impact internally, strangers will assume she has germs from a cold or smokes too much.
“When we’re out in public, she doesn’t have anything on her that says ‘I have a disease,'” he said. “When she starts coughing, people say, ‘Why don’t you light another one?’ And she’s never smoked a cigarette in her life. People are downright rude with her. They don’t realize what CF is.”
Other symptoms include poor growth, poor weight gain, very salty skin and infertility.
Men with CF are 99 percent sterile and women have a difficult time conceiving because of the mucus buildup in the reproductive organs, Kara said.
Kara and Arthur have a 9-year-old son, Draven. He was tested for CF as a baby and the doctors determined he is CF, according to Kara.
“I had a great pregnancy,” she said. “Everything went smoothly and I didn’t have any major complications. He’s my miracle baby.”
According to Kara, Draven knows that she is ill and reacts well to her coughing spells; however, he isn’t completely aware of her condition.
“It makes it hard because he’s so young,” she said. “He understands but at the same time, he doesn’t. How do you tell your kid that there’s a chance mommy could die? He’s my life and I’m hoping I get this transplant because I need to be here for him.
“It makes it rough when you do have a family and you have this disease. Anyone could die at any time but I feel as if I have an expiration date on me because with this disease, you don’t know when it’s going to take your life.”
According to the American Lung Association, 3,708 people in the United States died from CF between 1999 and 2006.
The average life expectancy for individuals with CF in 2007 was calculated at 37.4 years, up from 25 years in 1985. In the 1950s, few people with CF lived to go to elementary school.
“We want people to know that this disease isn’t something to take lightly,” Kara said. “So many people are dying from this disease. We need all the support and awareness we can get to hopefully one day find a cure and have CF stand for ‘Cure Found.'”