Kristina Beagle, St. Clair, doesn’t mind so much when her 2-year-old daughter, Danika, wants to blow bubbles in the house. While some moms might cringe at the thought of bubble residue on the floors or walls, Beagle encourages the activity because for Danika, blowing bubbles is like exercise.
Born with cystic fibrosis or CF, an inherited chronic disease that primarily affects the lungs and digestive system, Danika has unusually thick, sticky mucus that can clog her lungs and obstruct her pancreas to stop natural enzymes from helping her body break down and absorb food.
“Blowing is good,” said Beagle, who also has a son, Zechariah, 7, with CF. “So is jumping. Actually, exercise in general is good.”
Anything that breaks up the mucus and helps the person get it out is good, she noted.
Coughing, a main symptom of CF, is a necessary means for “cystics” to get the mucus out, added Gina McDaniel, Gray Summit, whose 14-year-old son, Gavin, has CF.
“They have really sticky mucus in the their lungs, so anything they breathe in, sticks,” said Beagle, noting that makes them far more susceptible to flu and other infections.
“Their (mucus) is three times thicker than ours,” added McDaniel.
These three Franklin County young people — Gavin, Zechariah and Danika — are among the 30,000 Americans (just 70,000 worldwide) who live with CF. It is a rare disease, to be sure, and primarily affects only people of Caucasian descent.
The two families, who met three years ago through their church, Mount Zion Baptist Church in St. Clair, have become close because of their shared experiences with CF.
They support and encourage each other emotionally, but also think of themselves as a team in the fight to cure CF. Last year the two families worked together to raise funds for the Cystic Fibrosis Foundation’s Great Strides Walk in St. Louis.
In just a few short weeks, they raised over $10,000, making their team, “Vest Buddies,” one of the top 10 in the St. Louis area.
Great Strides is the CFF’s largest fund-raising event, with 600 walks being held around the country. CFF is the world leader in the search for a cure for CF.
McDaniel pointed out that just 30 years ago, children born with CF weren’t living past 2.
“So the research money is so important, because otherwise these guys wouldn’t be alive now,” she stressed.
“In the few years since Gavin was diagnosed, life expectancy has gone from like 30 to now like 35 or 36.”
And right now, there is a lot of excitement in CF research because recently “a cure” was found to help cystics with a rare form (just 3 percent) of the disease.
“Cure” means that medications were found to help control the side effects, explained Beagle. It will extend their lives, improve quality of life, require less coughing.
“We heard from a guy who said, ‘I know what it’s like to sleep through the night without coughing now,’ ” said McDaniel.
Now researchers have turned their attention to the most common form of CF, Delta f508, the kind that Gavin has, which has both families very excited for the possibilities.
“We are so close to a cure right now,” Beagle remarked.
The CF Foundation doesn’t receive money from anywhere else except these types of fund-raisers, and 90 cents out of every dollar go to research, said Beagle.
The families’ team name refers to a treatment that their children, like everyone with CF, do every day a couple of times a day to help “shake” the mucus in the their lungs loose. They put on a vest hooked to a machine that, basically, shakes their torso to loosen the mucus in their lungs, explained McDaniel.
Each vest treatment lasts 30 minutes, which seems like it might be an ideal time to watch a TV show or play a video game, except the constant shaking makes it difficult to do much of anything, the kids say.
“It’s not enjoyable,” Gavin remarked, noting he can’t even read while wearing his vest.
Even talking is a challenge because the shaking makes their words difficult to understand.
At same time the kids are receiving their vest treatment, they also receive an inhalant that goes into their lungs to provide a medicine.
And all of this is when the children are feeling well, said McDaniel.
If they are sick at home, the treatments are increased to three to four times a day, she said. If they are so sick that they are in the hospital, the treatments are increased to four to five times a day.
“They hate it. They all hate it,” said McDaniel.
‘He Saved Us’
These days there is a newborn screening test that checks babies for CF, but that wasn’t the case when Gavin or even Zechariah were born. And with no family histories of the disease, no one suspected it.
The diagnosis took both families by surprise. They knew very little about the disease.
Gavin was 11 months old when he was diagnosed with CF. He had been sick constantly, in and out of the doctor’s office, but never with any firm answers.
McDaniel, who with her husband Jamie, has two other children — Jon, 19, and Hannah, 10, who don’t have CF — admits she wasn’t at all familiar with what cystic fibrosis meant, but realized that it was definitely a hardship and would shorten his life.
In the beginning there were a lot of tears, she said, but her perspective changed on the third day after Gavin’s diagnosis, when they saw another child in the hospital with a situation that was far worse than his.
That’s when she and Jamie decided to make some changes.
“Before we found out (Gavin) was sick, we didn’t go to church, we smoked cigarettes, we partied.
“We worked, we paid our bills, we did what we wanted . . . OK, they’re telling us our kid only has so many years, we’re going to live to the fullest. So now we try to vacation as much as we can . . . we ride motorcycles, we camp, we do everything we can to make sure Gavin can have a full, wonderful life with brother and sister.
“We started going to church, we just dramatically changed. We quit smoking . . . for me, that’s what happened. We found the Lord.
“We tell Gavin all the time, he saved us, because who knows where we’d be now, what we’d be doing . . . Gavin is the reason the Lord opened our eyes.”
For the Beagles, Zechariah struggled for six months before doctors discovered that he had CF. Kristina and her husband, Daniel, had two other children, Payton, 10, and Caden, 8, who didn’t have CF, so no one suspected that Zechariah would.
Then one day, Beagle called the doctor about a rash that had developed in his diaper area and wouldn’t go away. The nurse told her to bring him in to have it checked.
By the time they made it to the doctor’s office, he also had a red spot on his face, said Beagle.
It didn’t take the doctor long to realize that something serious was going on. He initially believed it was one of three things, said Beagle — a blood disease, leukemia or a tumor. Blood work confirmed the seriousness of the situation. Zechariah’s platelets were at seven, said Beagle, with tears in her eyes as she recalled the emotional time. “At zero, you die.”
Zechariah was rushed to then-St. John’s Mercy Medical Center in Creve Coeur, where he stayed for two weeks as doctors ran tests, still thinking he most likely had a cancer.
When the diagnosis of CF came back, the family was actually relieved because he didn’t have cancer, and they were told that with medication he would be fine. And he has been.
Since his diagnosis, Zechariah hasn’t had any weight problems and been fairly healthy, said Beagle.
Danika had the opposite experience. She was diagnosed at 10 days as soon as the test was back, but her first six months she was in the hospital a couple of times with feeding tubes because she couldn’t gain weight, said Beagle.
At 6 months old, she was still her birth weight — 10 pounds. Her eyes were sunk. Her nutrition was so poor. She couldn’t absorb her food.
But today, she’s finally doing well.
‘They Look Normal’
“We get that so often . . . people look at our kids and ask, ‘What’s wrong with them?’ ” said McDaniel.
Yes, he can often appear healthy.
“His weight is healthy, he’s captain of his basketball team (Cornerstone Baptist Academy through Mount Zion Baptist Church in St. Clair), he outruns other kids on the team, but guess what — he takes over 50 pills a day . . . lung treatments galore. He finished a tournament recently and went into the hospital two days after,” said McDaniel.
“We constantly get, ‘He looks normal’. . . people tend to forget that he needs a cure.”
Beagle said she’s heard CF described as “like drinking through a coffee straw. That’s what they feel like.”
Even with the medicine it’s difficult to breathe, she said.
Gavin was recently in the hospital because his lung function started dropping.
“When lung function drops, that means you’re having some sort of blockage,” said McDaniel.
So Gavin goes in to receive IV antibiotic treatment and when he leaves he realizes he can breathe better.
The reality for cystics is that as they get older, their condition gets worse.
“Gavin’s going through quite a bit right now,” said McDaniel. “In the last two years, we have been in the hospital four times . . . sinus surguries, over 12 PICC IV, his lung has collapsed, he’s had bronchoscopy to go in and clean out the lungs.
“The last two years, we have been diagnosed with aspergillus, ABPA. It’s a fungus growth in his lungs. That has created severe problems for him, to where now we go every two weeks for shots to treat it.
“We had to do heavy doses of steroids to treat it.
“We’ve had to do a bunch of diabetes monitoring. This Christmas, he was diagnosed with liver disease now. So now his liver is starting to fail and his spleen is enlarged. If it gets any bigger, he cannot play basketball anymore.
“He’s having severe joint issues now, to where he can’t stand up straight. He can’t get his arms straight. His elbows hurt really bad. His wrists hurt.”
It’s all related to the CF, said McDaniel.
“The CF is what’s killing his liver. The scar tissue is building up in the liver and destroying his liver.
“It’s a progressive disease.”
‘We Can’t Sit Around and Wait’
That’s what keeps these families motivated to do all they can to raise funds for CF research as quickly as possible.
“Both of us feel like we can’t sit around and wait,” said McDaniel. “What if they get sick, and we didn’t do anything to try to help them?”
“I want my kids to look back and think, my mom raised money and did everything she could,” said Beagle.
Working together to raise funds has made it easier for the families.
“It’s hard to do fund-raising alone,” said Beagle, “especially if one of the kids gets sick . . . we can pick up each others’ slack.”
Not Letting It Slow Them Down
Still, none of these kids is letting their CF slow them down.
There are times when the McDaniels are concerned that Gavin may be pushing himself too hard to stay in a basketball game, but they know he doesn’t want to let CF stop him from living his life to the fullest.
“Every day he’s outside playing basketball, and come night, he’s so miserable and sore with the joint issue that he has right now, he has a hard time getting up the stairs . . . but he doesn’t want to stop. To him, basketball is everything.
“That’s the thing with these kids that we notice. You would think that (CF) would slow them down or stop them, but it’s almost like it gives them the fight to go more.
“With Gavin there have been times when all of us were like, ‘Gavin, just take a break.’ But he won’t.”
He just smiles.
“Like I always tell my coach whenever I get knocked down on the court, I always get up and be like, ‘There ain’t nothing going to stop this man,’ ” said Gavin. “That’s what I do whenever something happens. I just get that in my mind, that mentality.”
“That’s why Gavin is the captain of his team,” said McDaniel. “Because not only is he a great player, but he’s a great inspiration, a great leader for the team.’ ”
His Vest Buddies, Zechariah and Danika, look up to him just as much too.