CCD: Normal is Overrated

At 3 months, Jared Earlenbaugh's motor skills appeared to be typical, although problems would become apparent by age 2. At 16, he required a trach, feeding tube and power chair. His dachshund, Sierra, doesn't seem to mind.
Article Highlights:
  • Jared Earlenbaugh started showing signs of CCD at age 2.
  • Now a junior in high school, Jared relies on a feeding tube and portable ventilator.
  • He keeps up with his schoolwork, plays video games and, for the most part, takes things in stride.
by Margaret Wahl on March 31, 2010 - 4:10pm

QUEST Vol. 17, No. 2

When Jared Earlenbaugh was 2 years old, he “wasn’t running around driving his parents nuts” the way his mother, Alexia Zuege, had expected. However, he was her first child, born when she was only 19, and she didn’t think too much about it.

But as other children mastered walking, running and other motor skills, Jared developed a side-to-side waddle, never ran, could only get up from the floor by holding onto the furniture, and fell down a lot.

He had his first muscle biopsy at age 3, which “came back undiagnosable,” Zuege says, and then another one at age 5, which doctors interpreted as showing a muscle disease called centronuclear myopathy, which turned out to be the wrong diagnosis.

Jared, meanwhile, was walking “with his butt sticking out and his chest forward,” began developing a spinal curvature, and was frequently ill with respiratory infections. During one year, he had pneumonia six times.

In 1999, Zuege moved to Texas and took 6-year-old Jared to see pediatric neurologist Susan Iannaccone in Dallas. Iannaccone, who now directs the MDA Clinic at Children’s Medical Center of Dallas, admitted Jared to the hospital to fight another bout of pneumonia and recommended another muscle biopsy.

In the spring, the muscle biopsy was done, and “that’s when they diagnosed him with central core disease,” Zuege recalls, adding that Iannaccone said Jared’s CCD was among the worst cases she had ever seen.

Jared’s swallowing muscles were so weakened that he was frequently inhaling food and liquid into his lungs, a major risk factor for respiratory infections. “In March 2000 they did a g-button [type of feeding tube] placement,” his mother recalls, ending his days of oral eating and drinking.

Then, in 2001, at age 7, Jared underwent surgery to remove the middle and lower lobes of his right lung, which had been scarred beyond repair from the many pneumonia episodes. And, the following year, he underwent a tracheostomy and began using a portable ventilator.

“Ever since the trach, he has not been hospitalized for pneumonia,” Zuege says. Jared also uses a CoughAssist, a device that helps pull mucus out of the respiratory tract. Zuege calls it a “miracle worker.”

“It gets the stuff right up out of his chest,” she says, “It saved his life a few times. We take it everywhere. If it wasn’t for getting out plugs and secretions, he would probably be sick a lot.”

As for Jared, who didn’t start school full time until fifth grade because of constant illnesses, he’s now a junior in high school and doing well. “He’s smart as a whip,” his mother says. “But he’s behind in school because of being out with so much illness.”

He has a modified school day, lasting from 10 a.m. to 2:30 p.m., part of which he spends in regular classes and part of which he spends in resource classes to help him catch up.

Although his mother would like him to think about his education after high school, Jared prefers playing video games to thinking about college admissions. “I just want to stay home and play games,” he says, “but my mom wants me to have a future, so I guess that’s out of the question.”

The hardest part about his CCD, he says, is not being able to eat or drink by mouth, although he’s allowed what’s known as “recreational tasting.” Zuege describes this as offering “a bite smaller than what you would give an infant, because you don’t want to overstimulate the salivary glands and cause more secretion.” Jared says his feeding tube formula, Jevity, “keeps me from withering away,” but it’s no substitute for his mother’s “awesome spaghetti.”

Zuege says, “For the most part, he’s a happy-go-lucky kid, although he has much to deal with.” She offers a recent statement made by Jared — “Being normal is overrated” — as a good example of his attitude. “He takes things in stride for the most part,” she says. “He’s been through a lot and never really complained.”

Jared says he depends on other people “to get me dressed and get my treatments,” but that he does a lot for himself. His advice to other kids in his situation is: “Love yourself, and don’t ever stop loving yourself.”

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