ST.LOUIS _ Cystic fibrosis has received international attention recently through the story of Dalton and Katie Prager, a young married couple fighting to see each other one last time.
Katie Prager died Thursday, five days after her husband. Both had cystic fibrosis and complications from lung transplants.
"We were given a great gift, we knew her time was short and she was able to do a few things that she wanted, and I am grateful for that," Katie Prager's mother Debbie Donovan wrote Thursday on the couple's Facebook page. "The days to follow will not be easy but I find comfort in knowing that my girl lived, she really lived."
Katie Prager, 26, was in hospice care at her home in Kentucky.
Dalton Prager, 25, died Saturday at Barnes-Jewish Hospital. He had returned to the St. Louis area for treatment to be closer to family.
The couple longed to see each other again before they died, but neither was healthy enough to travel.
Their story is tragic, but progress is being made in treatments.
Today there are more adults than children living with cystic fibrosis, a progressive lung disease that used to kill children before they reached adolescence.
Major advances in treating the disease started with the discovery of the cystic fibrosis gene in 1989. While the median age of survival is still just 40, new drug therapies and research bring optimism that babies diagnosed with cystic fibrosis today could have close to normal lifespans.
"We tell parents of newborns that our outlook is very positive. I fully expect them to play sports, graduate high school, go to college, with no limitations," said Dr. Blakeslee Noyes, director of the cystic fibrosis center at Cardinal Glennon Children's Hospital.
Katie and Dalton received lung transplants within the last two years and developed complications including lymphoma.
They had aggressive forms of the disease in part because they shared a dangerous drug-resistant bacteria.
Children and adults with cystic fibrosis are discouraged from having close contact with each other because of the risk of infections. Cystic fibrosis summer camps and group activities no longer exist. The Pragers knew the risks but decided having time together was worth the chance of dying younger.
About one in 3,000 to 3,500 babies are born with cystic fibrosis. Most commonly occurring in Caucasians, the disease is inherited when both parents are carriers of a defective gene. Each of their children has a one in four chance of developing cystic fibrosis, while other children may become carriers.
Universal newborn screening for cystic fibrosis, now required in all states, has helped families get an early diagnosis and line up care before symptoms start.
The disease damages the body's ability to regulate sodium and causes thick mucus to build up in the lungs, airways and digestive tract, especially the pancreas. The mucus makes it difficult to breathe and absorb nutrients from food.
While there is no cure for cystic fibrosis, some medications and treatments can slow its progression. The disease can vary in its severity, but the average treatment regimen is intense.
"People are no longer dying from cystic fibrosis, if we do our job right, but living with cystic fibrosis," said Dr. Peter Michelson of Washington University, director of the cystic fibrosis center at St. Louis Children's Hospital. "Just like any chronic illness, it's going to require close attention and consistent management."
Luke Price, 10, of Sunset Hills, takes 32 pills a day, inhales three other medications and wears a vibrating vest for two hours a day to help loosen the mucus in his airways. He's also in a clinical trial of a new drug, Orkambi, that targets proteins on the defective gene with a goal of improving how the body clears mucus.
It's too early to tell how successful the drug will work across the disease population, but Luke has seen improvements in his lung function and his weight.
"For the first time in his life he really doesn't look sickly," said Luke's dad, Mike Price.
Price said he feels optimistic about Luke's future but still worries that the drugs could stop working or cause serious side effects.
"I see how well he's doing now, but I also understand that five years ago Katie (Prager) looked as healthy as Luke does today," he said. "Every time he displays a change in condition like a heavier cough or he gets sick suddenly we always wonder, 'Is this the time that it's going to change for him?' We tend to really, really enjoy days that are just normal days to everybody else."
Lung transplants are a last-resort treatment for cystic fibrosis patients and are being performed less frequently since the improvements in drug therapies. Price hopes the transplants, which the Pragers never recovered from, will become unnecessary in the future.
"No matter how many kids start doing better we're not going to relax or slow down, we're going to keep working until we don't have to have another story like Katie and Dalton," he said.
