Keller Hoch appears to be a very happy and healthy young boy, but there’s a life-threatening bacteria living inside him. Diagnosed with cystic fibrosis, his family asks the community to join them in their fight
Six-year-old Laney Hoch got her baby brother, Keller, about three-and-a-half years ago. Back then, her parents, Jenna and Corey Hoch, were reveling in the joy of their perfect nuclear family on a daily basis.
Unfortunately, that didn’t last long.
“Keller seemed constantly hungry pretty much from the beginning, but we’d assumed at the time that it was just a peculiarity of his digestive system and growth pattern,” said Jenna, who, with Corey, owns
Companion Homecare of the Eastern Shore in Salisbury. “He was also having eight to ten bowel movements a day, but the problem was they weren’t healthy ones. We tried dairy-free diets, gluten-free diets; you name it. By the time Keller was six months old, and there was still no improvement, we knew something was wrong.”
After taking all the appropriate steps to determine what was going on with Keller, Jenna and Corey ultimately wound up at Johns Hopkins in Baltimore, hoping they would finally get answers to the questions that had been plaguing their family since his birth.
Easier said than done.
“They took all the requisite tests,” said Jenna. “They took blood samples, stool samples and still nothing. It wasn’t until they’d performed a rather unpleasant two-hour sweat test six months later that we finally learned the truth.”
So, at age two, after what felt like an eternity of worry, uncertainty and frustration, Keller was diagnosed with cystic fibrosis. Keller’s type is called DDF508, the most common kind on the CF spectrum of genetic mutations. CF is a life-threatening disorder that is characterized by pathologically thick secretions of mucous that can adversely affect the lungs, pancreas, liver, kidneys and intestines. As the discharging mucous coagulates, it tends to not only clog ducts that are essential to proper organ function but also serves as a kind of incubator for the accumulation of potentially deadly bacteria. The result is a median lifespan in the mid-20s, and there is no cure.
“When we first got the diagnosis, our world kind of came crashing down around us,” Jenna admitted. “It was like getting the news of a loved one’s death. We were scared… more like devastated, to be honest. We envisioned many years of a challenged and complicated life for Keller, and that broke our hearts.
“But one thing I want to really emphasize to parents,” added Jenna, “is that they need to be there with the doctor when he or she analyzes their children’s test results, and they must know what questions to ask.
Too often, the markers for cystic fibrosis are missed on the initial screenings. In our case, it wasn’t until they did a sweat test, in which the salinity of the child’s perspiration is measured, that the results were conclusive and an accurate diagnosis was made.”
Still, there was silver lining within the dark cloud of Keller’s diagnosis. Though his mucous secretions did have a viscosity consistent with CF, they were not compromising his lungs or respiration in any serious way. This is especially important because normal, unimpeded lung function is essential to a child’s proper growth and development.
Today, Keller is a strong, chatty, three-and-a-half-year-old who is in the 95th percentile for height and 97th percentile for weight. With the aid of externally administered pancreatic enzymes and a special vest he wears 90 minutes a day, to break up mucous secretions, Keller is enjoying a normal, happy life for a boy his age.
But if things are looking good for the Hochs now, the future looks even brighter. In July, the FDA is scheduled to complete its priority review of a new treatment proposed by Vertex Pharmaceuticals, in which its drugs lumacaftor and ivacaftor are used in conjunction to address Keller’s type of CF at the cellular level. If approved, the unprecedented protocol is expected to increase by decades the life expectancy of CF patients, offering new hope to thousands worldwide.
Editor’s note: The Hoch family invite you to become part of Team Keller on Sunday, June 14, at Salisbury’s Green Hill Country Club, for the Great Strides walk for cystic fibrosis. Last year, more than 300 people turned out for the family-fun event, which includes a walk around the golf course, a pig roast, Sunshine the Clown, live music, DJs and swimming. Check-in is at 11:00 a.m.
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