Cystic circumstances

Two brothers — one disorder.

Twenty-one-month old Simon and 5-month-old Abel Chenoweth both had a 3.75% chance of inheriting cystic fibrosis. Their regulatory infant tests showed one gene — a delta F508 mutation — usually meaning the child is only a carrier of cystic fibrosis.

There was about a 1 in 27 chance Simon and Abel would have the disorder as indicated by their initial test results. After sweat tests indicated they both had extremely salty skin, though, there was no denying their diagnoses.

Although born a year apart, both brothers beat the odds together and were diagnosed with cystic fibrosis (CF) a few weeks after birth.

Despite what internet search engines might show about living with cystic fibrosis, though, the disease hasn’t stopped them from leading healthy lives.



Diagnosis

Krista and her husband Jake took their son to Fort Wayne’s Lutheran Hospital on March 11, 2019, for Simon to do a sweat test. Neither Krista or Jake, nor any close family members, have cystic fibrosis, so Krista assumed it was a mistake.

Krista had been struggling with some postpartum depression after giving birth to her first son. She recalled that day as being one of her first “really, really good” days.

It was her 25th birthday.

The medical worker administering the test reassured Krista they always get negative results. Usually with newborns, Krista explained, doctors find both cystic fibrosis genes in the first test. Doctors only found one gene mutation in Simon’s newborn screening.

After Simon’s sweat test, the Chenoweth family stopped for a meal and came home to birthday presents for Krista on the porch. They waited the rest of the day to hear back from the hospital, with Krista calling regularly to hear if they had the results in yet.

About 4:59 p.m., right before the office closed, Krista received a call. She could tell by the nervous-sounding voice on the other end of the line that something wasn’t right. Anything less than 29 millimoles of chloride per liter is a good sign in sweat test results, according to the Cystic Fibrosis Foundation website. Between 30 and 59 millimoles per liter means more testing is needed. Anything 60 or higher is a likely diagnosis.

Simon’s results were around 101 millimoles per liter.

“I was like, what the hell are you talking about? No, he doesn’t (have it),” Krista recalled while tears welled in her eyes. “I was in total denial.”

Though it’s a story she’s shared many times, she seemed to slip into reliving that moment from years before.

“I was in the living room, and Jake took Simon…”

In real time, Abel coughed.

Krista paused.

“Goodness,” she cooed, smiling and holding him close to her chest.

Krista continued then, explaining that after she heard the news, she started yelling at the sky.

“I was like, ‘God, no, not my baby,’” she said. “‘Just take it away; take it back from him.’”

Her thoughts circled to the Google results for cystic fibrosis. The search engine stated the life expectancy was 37 years old. Would her son be able to get married? She wondered.

Krista’s mother, Teresa Taylor, came over to pray for them, and Jake left to pick up some facial tissues.

“Of course in the beginning, it was pretty devastating,” Taylor said. “We didn’t know much about it, and what we did know about it wasn’t good.”

When Jake returned from the store, he said something that’s always stuck with Krista.

“Simon’s going to take CF by the horns,” he told her. That’s when she realized the whole time she had been reacting to the news, Simon had been napping or smiling through it.



Devotion

Family and friends began regularly praying for the Chenoweths. Krista’s father, Burl Taylor, and Jake kept saying Simon would be fine. Still, the family didn’t have much reassuring information to read, hear or watch at the time.

Teresa Taylor, who works as a nurse at State of the Heart Care nonprofit medical center, said she hasn’t seen much of cystic fibrosis.

“After researching some things on days that I felt like I could handle it, I couldn’t handle it very well,” she said.

Common symptoms for patients with cystic fibrosis include persistent coughing, frequent lung infections, wheezing or shortness of breath, poor growth or weight gain and frequent greasy, bulky stools or difficulty with bowel movements.

Those who have cystic fibrosis like Simon and Abel are also in the high-risk category for illnesses like COVID-19.

Neither Krista or Jake slept well through the first few nights knowing about their son’s ailment.

The days became easier, though, as they found hope in Simon’s regular doctor visits.

His doctors wanted him to gain at least eight ounces a week, and he gained a pound each week.

Krista wasn’t surprised when Abel, their second son, also tested positive for the same mutation about a year later. But she wasn’t happy.

“For a little while, I felt like God had dropped me and left,” she wrote in a blog post. “I felt alone, and even punished.”

But today, both boys have clear lungs and seem healthy as can be. Every day when Krista looks at Simon, she’s reminded Abel will thrive, too.

“We have not seen anything,” Teresa Taylor said. “They are absolutely no different than the rest of the grandkids.”

Both Simon and Abel have been peaceful and healthy babies, although Simon started whirling around like a tornado when he hit 1 year old, Krista recalled.

“I mean, (he’s) a happy little tornado,” she said.

There are 220 recorded cases in the United States of the specific gene mutation named 2184insA, which contributes to cystic fibrosis. Two of those cases are Simon and Abel. About 80% of the 220 affected need to take enzymes in order to gain weight. Simon is in the 20% that doesn’t. So far, it seems Abel is in the same place.

Krista thinks there are so few cases for the specific mutation because it appears symptomless. When they visited the hospital for Simon’s sweat test, Krista and Jake learned about a 70-year-old who had just been diagnosed with cystic fibrosis. She compares this to the internet search results that show people affected by cystic fibrosis every day.

“People can go their whole lives without knowing that they have it,” she said.



Daily life

The Chenoweths usually get up around 8 a.m. and begin their morning chest physical therapy. The process consists of Krista or Jake patting out the boys’ chests and backs with silicone cups, which helps to loosen any mucus in their lungs.

Neither Simon or Abel have or have ever had mucus in their lungs, but the family does therapy — which they know as “the pitty pats” — daily as a preventative measure. Simon gets a breathing treatment to ensure the mucus in this throat doesn’t sink to his lungs. He also takes a daily nasal spray.

Now that the weather is turning colder, both boys are taking cough medicine. The fall and winter seasons are a lot busier with preventative treatments, Krista explained.

Krista and the boys usually spend their days watching a movie, playing with toys, baking or dancing to music. They enjoy listening to musicians like Mötley Crüe, Poison, Machine Gun Kelly, Blink-182 and others.

“Cystic fibrosis has not taken anything away from us,” Krista said, who started working part-time as an evening bartender and waitress at T’opher’s Place in Portland when the business opened in October.

Simon likes playing with their golden retrievers, 4-year-old Opie and near 14-year-old Reggie. He also enjoys visiting Chenoweth Family Swine with Jake, who raises show pigs at the facility. Besides the few daily treatments the boys need, Jake said he feels like he’s got a “pretty normal Dad life.”



Delivering the news

Something Krista finds intriguing is that the movie “Five Feet Apart,” a love story about two patients with cystic fibrosis, came out the same week Simon was diagnosed.

She recalls at the time she was trying to put the cystic fibrosis diagnosis out of her mind, and she noticed an ad for the movie on Instagram.

“When you have cystic fibrosis, so much is taken from you,” a narrator read over the trailer.

The line hit her like a rock, and the irony shocked her. Still, it gave her some hope because it showed two kids who had grown up despite having cystic fibrosis.

The internet often depicts more horror stories about people with the disease. There are more success stories like Simon and Abel’s, Krista explained, that don’t always appear for the general public to see.

She’s spent the last year advocating for children and adults diagnosed with cystic fibrosis. She shares regular updates on friend and blogger KyliAnn Lemons’ website.

Krista has tentative plans to do a benefit in Jay County for the Cystic Fibrosis Foundation, which she’d like to be an annual event. She and Jake are also hoping to host a benefit for the foundation next summer at Celina Lynx Golf Club in Ohio. COVID-19 made it difficult to do the benefit this year, she explained.

She’s also in the works of selling t-shirts with her sister, Danielle Heinrichs, and Lemons, in partnership with Sister Duo Design Market for the foundation.

“As much as it was hard for her when they were first diagnosed, I think that God sent them to her to get the word out,” said Krista’s friend, Kelsey Fiely. They’ve known each other since middle school or about 15 years. Fiely, who’s expecting a newborn in January, likes to tell Krista that if she’s half the mom Krista is, she’ll be happy.

Krista doesn’t like for others to pity their family or feel bad for them. She celebrates the relationship she has with her sons, and she thanks God for both of her boys’ good health.

“It’s just so crazy how you don’t think you’re ever going to be happy again,” she said. “But you end up being the happiest you’ve ever been — even with the circumstances.”