He was skeptical. She was excited. He had been accepted into a new double-blind drug trial, which meant he had a 50/50 chance of getting either a placebo or the real medicine being studied.
“I have grown up hearing about wonder drugs my entire life,” Tom Kerekes said. “Either they didn’t apply for me or they didn’t work for me.”
Maybe this time, Natalie Adams thought. They were in their mid-20s. Kerekes had a terminal disease, his suffering was growing, and it wasn’t clear how much longer he would live.
With his “big smile and brightest eyes,” she had fallen in love with him a few years earlier during their freshman year at the University of Georgia. They were now engaged, though he fretted about being a burden to her. Both wanted kids, but she figured she’d need a really good job. Because odds were she’d end up as a single mom.
They drove to a nearby Chick-fil-A — he needed to take food with the new drug combination he’d just been handed. She snapped a photo as he popped two pills in his mouth, like a kid eating a handful of M&Ms.
That evening, in October of 2018, the East Atlanta couple went to a movie theater. Kerekes started coughing and had to walk out. People with cystic fibrosis tend to do that a lot. But this was different. Deep, violent, really scary coughing.
The coughing fits lasted for days. Then a remarkable, wonderful thing happened: Their lives changed essentially overnight in almost unimaginable ways.
Credit: Natalie Adams
Credit: Natalie Adams
Not so long ago, cystic fibrosis was known as a child killer. Many with the genetic disease, which gums up organs with mucus and makes it hard or impossible to breathe, didn’t live long enough to become teenagers. More recently, many made it into young adulthood, but not much longer.
Then, three years ago this month, the Food and Drug Administration approved the new drug combination that Kerekes had been among the first to try in a clinical trial. For many of the nearly 40,000 Americans with CF, the medicine has been transformative.
They can breathe better and live more normally. And imagine living deep into middle age or even older, something many had never contemplated or planned for.
There have been breakthroughs in battling other diseases, of course, such as AIDS, polio, juvenile diabetes, hemophilia and a range of cancers. In the case of cystic fibrosis, the breadth and speed of the impact has stunned not only patients but their doctors.
Credit: Natrice Miller / Natrice.Miller@ajc.com
Credit: Natrice Miller / Natrice.Miller@ajc.com
Going for a run
One of Jenna Hamlin’s earliest childhood memories: Her dad pounding on her chest and back to loosen mucus that could clog her lungs, trap bacteria and spark deadly infections.
Her lungs and energy were still robust. As a teen she competed in club soccer, a midfielder who ran and ran and ran.
But even at a young age she knew the disease was always lurking. In middle school, she never pictured a future for herself beyond the next five years. Looking out further didn’t seem to make any sense.
By her early 30s, antibiotics no longer really knocked down her infections. No matter how many treatments she endured, no matter how well she tried to take care of herself, her lungs worsened. She was always fatigued, spending less time with friends. She’d wake up in pain morning after morning.
“I felt a loss of control for so long,” she said.
Doctors had her evaluated for getting on a lung transplant waiting list. She wasn’t quite bad enough, but close.
She moved to Decatur for a job as a bioinformatician at the Centers for Disease Control and Prevention, partly because she wanted to be near Emory University, where she assumed she’d need to be in the running for a lung transplant within the year.
Then the new drug combination, Trikafta, was approved. The day after starting the pills she called her mom to say she was feeling better, good enough to go shopping.
Within days, Hamlin felt so good she went for a run, something she hadn’t been able to do for more than a year. She bounded a mile down Clairemont Avenue.
She laughed — literally laughed — the whole way.
“It was just pure joy. I was passing people who probably thought, ‘Who is this weirdo?’”
Credit: Christina Matacotta
Credit: Christina Matacotta
A world of possibilities
The medicine isn’t a cure and not everyone sees meaningful improvement. Long-term organ damage remains. The drug combination has yet to be approved for very young children. And Trikafta carries a steep price: about $311,000 for just one year of treatment. Most is usually covered by insurance.
Some endure vestiges of their old time-consuming, equipment-heavy treatment regimes, regularly using a misting machine as well as a special vibrating vest to help clear lungs, and taking about 40 assorted pills a day in part to help with digestion.
But others have eased back on some of the treatments. Hospitalizations have plummeted and the number of people with CF awaiting lung transplants has been slashed. About 90% of people with CF are expected to benefit from it.
“People’s lives have been changed and it happened for some people in a matter of hours after they began therapy,” said Whitney Brown, senior director of clinical affairs for the Cystic Fibrosis Foundation.
Eventually for Hamlin, the proportion of air she could expel from her lungs in one second rose from about 35% to 59%. That’s still low. She can get winded carrying things or walking up stairs.
But she rides her bicycle 50 to 60 miles a week, runs and does yoga. Doctors stopped talking about a transplant for the now 38-year-old.
“I still wake up and am amazed at how I feel,” she said. “You don’t know how much pain you are in until it goes away.”
Credit: Christina Matacotta
Credit: Christina Matacotta
Once unfathomable options now seem possible. She thinks about living in Europe and working remotely. A friend wants to take a walking tour in Scotland or Spain.
“I said, ‘Yep, sign me up.’ ”
She began contributing to her 401(k) at work. If she pulls money out of the retirement plan before she is 59½ she could face stiff penalties. Hamlin thinks there’s a decent chance she won’t face that dilemma.
Before, she had accepted the idea of death at a young age. Lately, though, she’s felt a kind of anxiety that’s new to her. She thinks it is how many people without a fatal disease must view mortality.
“Now, I want to do more in life. What if I can’t?”
‘There really was no future’
Natalie Adams and Tom Kerekes remember their near-instant connection when they met at a UGA frat party in 2013. She had on a goofy shirt he loved and was down-to-earth and easy to talk to. He had that wonderful smile and was exciting to talk to.
It hadn’t registered with her how remarkably thin he was: 135 pounds on his 5′11 frame. What she remembers was having butterflies.
Credit: Natrice Miller / Natrice.Miller@ajc.com
Credit: Natrice Miller / Natrice.Miller@ajc.com
Deep in the conversation, he casually mentioned he had cystic fibrosis. She googled it the next day. Life expectancy: 33 years.
Days later, in class, she was assigned by coincidence to review a project by a photographer documenting a best friend’s final week of life before dying of CF.
Adams was 19. She asked herself, was this really a path she wanted to go down with Kerekes?
“But his soul is so big and so pure and so good.”
A few days later they went on a date: takeout from Shake Shack. They ate in the parking lot. CF stayed in the background. At first.
That summer, he landed in the hospital. She thought he might die there. He was coughing up blood. As a kid he had scoffed at the idea that CF would cut his life short. By college, he was ignoring warning signs.
“I just wanted to be a normal college kid, and it damn near killed me,” recalled Kerekes.
His life quickly narrowed. A nasty bacteria was embedded in his lungs, requiring cycle after cycle of increasingly powerful intravenous antibiotics. He rushed to the hospital multiple times a year. Year after year. His lung function was sinking. His grades plummeted. Exhausted, he dropped out of UGA two credits shy of a degree.
“I was just living day to day instead of planning for the future,” he said. “There really was no future. It was just existence.”
Credit: Natrice Miller / Natrice.Miller@ajc.com
Credit: Natrice Miller / Natrice.Miller@ajc.com
Then came that moment at a Chick-fil-A and his first dose of Trikafta.
Within days, his lung function improved to what it was when he was 18. Soon, he was gaining needed weight. His fatigue eased.
“I’m still gobsmacked to this day,” said Kerekes, who is now 28 and living in East Atlanta. “I literally got my life back. It still to this day doesn’t feel real.”
‘You bet on yourself’
People often ask kids what they want to do when they grow up. The question bothered Andy Lipman.
He’d say, a doctor or a cowboy. But, “in the back of my mind I thought, ‘Am I going to grow up?’”
When he was an elementary schooler he had read about cystic fibrosis in an encyclopedia and learned he probably wouldn’t live beyond 25. His parents hadn’t wanted to scare him.
He didn’t have much motivation after realizing he had a terminal disease. He only went to college because that’s what people around him did. He assumed he wouldn’t marry or have kids or a career or, someday, gray hair.
By his sophomore year he grew intensely depressed and anxious. He stopped crucial treatments. He stopped going to class or eating much. He was sick and coughing.
But he did play a pickup game of basketball. Another player knocked him down, then grabbed one of Lipman’s scrawny arms to help him up and jokingly asked if he was entering a weightlifting contest.
“I was really angry at myself,” Lipman said.
He decided he was going to live like even a short life mattered. He started lifting weights, took better care of himself, got more involved in college and sought mental health treatment. Even before Trikafta, he was living longer than dozens of people he knew who had CF.
Credit: Alyssa Pointer
Credit: Alyssa Pointer
Lipman’s anxiety over health issues hasn’t evaporated. But he turned 49 last month. He and his wife, Andrea, just celebrated their 20th anniversary. He has two teenage kids. And gray hair. A former purchasing manager, he is now a motivational speaker and author in Sandy Springs.
He praises his doctors and the care he’s received. He credits daily exercise and treatments and just dumb luck. And, more recently, the new medicine.
Since starting Trikafta he can run more without coughing. Now, he almost never wakes up in the middle of the night feeling like he’s choking on mucus.
“I don’t necessarily know what is going to happen. None of us do,” he said. But you hope. “And you bet on yourself.”
A calendar stuffed with life events
A month after Tom Kerekes started in the clinical trial for Trikafta, a close friend’s daughter died of complications of CF after receiving a double lung transplant. She was 33, the same age that Natalie remembered seeing when she first looked up how long people with the disease might live.
Credit: Natrice Miller / Natrice.Miller@ajc.com
Credit: Natrice Miller / Natrice.Miller@ajc.com
Kerekes said he feels survivor’s guilt for living when others haven’t.
He hasn’t been in the hospital since starting the new medicine. He still has CF, but he no longer really feels sick. He can’t believe how much has changed.
He returned to school and finished his degree. He married Natalie. He pivoted to a new career in pharmacy software. He bought a house with a 30-year mortgage. Got a dog. Started contributing to retirement investments. And came to believe he might live much longer. He’s thinking about going back to school for more training. “None of it would have happened without” the new drug combination, he said.
While there is not yet a true cure for CF, he thinks about what is now ahead for many children who have the disease.
“They will never have to go through the dark ages.”
He and Natalie said they take pleasure in normal life. The monotony of daily errands. The minor disputes that couples have about regular life stuff, like what temperature to set the thermostat at.
He now has a calendar stuffed with plans. There’s a friend’s wedding coming up, concerts, a bachelor party, a trip to Europe next year. Then, with a reporter on the phone, the thought came to him to add one more. He’s been meaning to get back into exercise. “I’m going to pick a half marathon.”
It’s on his calendar now for June.
“Life is too short,” he said, his voice breaking. “It is not something I am going to squander.”
Credit: Natrice Miller / Natrice.Miller@ajc.com
Credit: Natrice Miller / Natrice.Miller@ajc.com
About the Author
