A year ago this summer, Kelly and B.J. Klotz looked forward to welcoming their first child into the world.
No reason to fear, they thought. Everything seemed on track.
“The ultrasounds looked normal, everything was normal throughout the pregnancy,” Klotz said. “Everything was pretty status quo.”
But on the morning of Aug. 3, 2017, as she went to work in the advising registration office at Aquinas College, a turn for the worse spun so wildly she could barely keep up.
“I felt really nauseous,” she said. “I didn’t feel well at all. I had a doctor appointment scheduled for the next day so I was just going to wait.”
But symptoms persisted.
“About 10 a.m., I finally said, ‘OK, I’ll call, because I’m still feeling really nauseous,” Klotz said. “The nurse asked when I last felt the baby kick.”
Klotz realized it had been a couple of days, but it didn’t seem out of the norm. It had been a pattern throughout her pregnancy.
“The nurse said, ‘No, you need to go to ER and I’ll tell them you’re on your way,’” Klotz recalled. “When I got to ER, they hooked her up to a heart rate monitor and wheeled me down for an ultrasound. My husband was on his way.”
Shortly after Klotz had been wheeled back to her room, Jessica Lalley, MD, a Spectrum Health Medical Group obstetrician, revealed the ultrasound results.
“She said she’s not safe anymore, we have to deliver her today,” Klotz said.
At 4 p.m., little Kamryn Ann Klotz entered this world via emergency C-section, six weeks before her scheduled due date.
“My husband announced it was a girl,” Klotz said. “They put a CPAP mask on her face. Her abdomen was really distended. We didn’t have time to hold her because they rushed her to the NICU. They took her right away for testing.”
Staff called Klotz’s room with updates.
“The surgeon found a problem,” Klotz said. “She had a severe bowel blockage in her small intestine and needed surgery. He said they had to remove a portion of her small intestine and reconnect the pieces we have left.”
Then came the unexpected question.
“He asked if we had any family history of cystic fibrosis,” Klotz said. “I said, ‘No, I’ve never heard of it really. I know nothing about it.’ He said sometimes this can be an indication. Babies with CF have extra mucus in their bodies. Newborn meconium is so sticky they can’t have a bowel movement. Everything got backed up, causing her intestines to twist.”
Baby Kamryn underwent a successful bowel surgery, but then came more concerning news.
“Exactly one week later, I grabbed lunch and came back to the NICU room,” Klotz said. “The doctors were surrounding her. They found a perforation in her intestine.”
They performed surgery, but upon examining, found Kamryn’s body healed the small hole on its own.
Still, her life hung in the balance.
“It was touch and go for so long,” Klotz said. “We had to wait for her system to learn how to have a bowel movement. After the third or fourth barium enema, they used a medicine called Mucomyst. That did the trick.”
Words of encouragement
The newborn screening confirmed cystic fibrosis, but Dr. Lalley reaffirmed how fortunate the family was.
“She said, ‘You have someone watching over you,’” Klotz said. “If we had waited one more day, this would be a different story. We’re lucky it happened how it did. Everything happens for a reason.”
Klotz researched and read everything she could about cystic fibrosis. But the best news of all came from John Schuen, MD, a Spectrum Health Helen DeVos Children’s Hospital pediatric pulmonologist.
“I saw all the worst cases,” Klotz said. “They sent pulmonology over and they were amazingly wonderful—Dr. John Schuen and his PA, Anne Thomas. They sat with us for two hours explaining how far things have come, that CF is not what it used to be. Dr. Schuen explained that we are raising an adult. They were very encouraging. They said, ‘She’s going to play sports, she’s going to go to college.’”
Kelly and B.J. learned she carried the most common gene for CF and he, the second most common.
On her actual due date, Sept. 12, Kamryn went home. And finally, after weeks of stress and fret, life could begin, even though it’s not the life they initially imagined with their first born.
In some ways, it’s better.
Their little girl is a bundle of joy. And energy.
Managing the disease
“She’s extremely energetic and very feisty,” Klotz said. “She’s very active and she climbs on everything.”
Due to her disease, Kamryn dines on high-fat, high-calorie foods. Children with cystic fibrosis tend to have trouble gaining weight, but fortunately, that’s not the case here.
“Her weight gain is phenomenal,” Klotz said. “She loves high-fat yogurt with heavy cream, eggs with lots of butter, avocados, French toast sticks…”
Twice a day for 30 minutes, Kamryn dons a “shaky vest” as the family calls it. The vibrating vest helps loosen mucus inside the little girl’s body.
While the vest vibrates, the brown-eyed girl intently watches cartoons on Klotz’s cell phone, swaying to “Wheels on the Bus” and Daniel Tiger tunes.
Twice-a-day nebulizer treatments open her airways.
Kamryn also takes enzyme pills before every meal to help her digest fat and gain weight.
She visits the Helen DeVos Children’s Hospital Cystic Fibrosis Care Center once a month.
“It’s a lot of managing and a lot of maintenance, but it’s doable,” Klotz said. “She goes through a lot to stay healthy, but she’s very good natured about it. She even takes her liquid iron supplements like a champ. She’s never given us any issues about medications or treatments.”
Instead, the little girl applauds life, and all it has to offer.
“She loves to clap,” Klotz said. “And she loves to smile. She’s a joy. We’re blessed to have her.”
As if following Dory’s advice to “just keep swimming,” Kamryn is enrolled in swim lessons at the YMCA.
“She’s the loudest one in the class,” Klotz said, laughing. “She claps, squeals and splashes.”
And if Kamryn follows recent statistical averages, there will be many more claps, squeals and splashes in her future.
“Dr. Schuen said the current lifespan is over 47 years,” Klotz said. “He said 20 years ago, it wasn’t even 20. The numbers have gone up roughly a year every year. Two years ago, it was 40. We were told if there’s ever a time to have CF, it’s right now because of all the advancements and the great potential of these modern babies. They just have really great potential.”
Dr. Schuen said Kamryn’s future is bright.
“We’ve taken a disease that was fatal during childhood and now most pediatric pulmonologists agree that CF is a disease that’s not fatal during childhood,” Dr. Schuen said. “With the median life span of 47.7 years, even that is underestimating the future for someone like Kamryn who’s just starting out in life.”
The future could become even brighter than it gleams right now.
“They are on the verge of breakthroughs with medications and treatments,” she said. “They’re very hopeful she can live a normal life. We’ve been blessed to have encouraging words. But, you have to be careful with germs because the mucus in her lungs can hang onto bacteria. We do have to watch.”
That means hot tubs and ponds are out for Kamryn. But sports will be very much part of the playing field.
“We will treat her as normal,” Klotz said. “We’re staying on top of treatments, doing the best we can and taking it one day at a time.”
Although the initial fear and diagnosis tumbled their world, the Klotz family carries on.
“You go through all the emotions—angry, sad, then you kind of stand up and put one foot in front of the other and say, ‘OK, we got this.’ You don’t really know how strong you are until you have to be. We’re having fun with her.”
They swim together, take walks together and visit family and friends together.
“Kamryn really has an army behind her,” Klotz said. “Family members have nebulizers at their houses. They know how to do her treatments. It’s a team effort. We’re not going to let this define her. She’s a kid who happens to have CF.”