"We took our first vacation because of the Aerodigestive Program. We went to San Diego, and she rode roller coasters, swam in the ocean, and saw Legoland.”
—Kim Sharpe, Maddox's mom
Kim Sharpe used to drive her daughter Maddox down to Denver about once every three weeks — a 540 mile round trip. There was no other option.
Maddox was born with tracheoesophageal fistula (TEF) and esophageal atresia (EA). The fistula and atresia were repaired in the first week of her life, but TEF and EA have problems that continue throughout childhood. At 2 and 3 years old, Maddox’s issues with breathing and swallowing were getting unmanageable.
“We don’t have any pediatricians in Casper, Wyoming, and we have no pediatric specialists,” Kim says. “There’s no one here who can manage her needs. So we were literally driving to Denver in the middle of the night with Maddox having something stuck in the back of her throat, just to get it surgically removed.”
Discovering the Sie Center for Down Syndrome
Those emergency trips wreaked havoc on the Sharpes’ family life. But Maddox's was a complicated case. In addition to the TEF/EA, Maddie has Down syndrome, and the bevy of specialists she was seeing at an adult hospital in Denver took the frequent emergencies as a foregone conclusion: That was just the way it was.
Kim accepted it, too. But she also read a lot, and she stayed informed. She read another Casper mom’s blog about her own son’s Down syndrome, and the blog mentioned the Sie Center for Down Syndrome at Children’s Hospital Colorado.
“I didn’t even know there was a clinic for Down syndrome at Children’s Colorado,” Kim says. “I was like, ‘Wait a minute. We need to be there.’”
Kim got Maddox in just weeks later to see Francis Hickey, MD, medical director of the Sie Center for Down Syndrome. When Kim told him about the midnight trips, Dr. Hickey referred her to Children’s Colorado’s Aerodigestive Program, which coordinates otolaryngologists, gastroenterologists, pulmonologists and others toward comprehensive care for children with complex issues like Maddox’s.
Working with the Aerodigestive Program
“In patients with a history of TEF and EA,” says Jeremy D. Prager, MD, pediatric otolaryngologist and director of the Aerodigestive Program, “the airway and esophagus remain abnormal in shape and function after repair. Patients have tracheomalacia, or floppiness of the airway, which impedes clearance of secretions and can cause significant breathing difficulties. In addition, abnormal esophageal movement leads to ongoing swallowing issues.”
Compounding that difficulty was Maddox’s Down syndrome, which adds increased susceptibility to infection, decreased muscle tone, compromised airway anatomy and enlarged adenoids and tonsils to an already unstable mix. Being unable to clear mucus from her airway, Maddox frequently had pneumonia, and the obstruction caused severe sleep apnea — in addition to the frequent throat obstructions.
“Before,” says Kim, “Maddox probably missed at least a week every month of school. Getting sick, getting infections, not being able to function because she’d sleep so much. She would sleep literally 15 hours a day.”
Addressing those myriad, interrelated issues required a truly multidisciplinary approach. As one of the Aerodigestive Program’s first patients, Maddox commanded the resources of nutritionists, pulmonologists, otolaryngologists, feeding specialists, occupational therapists and many others to develop a regular, preventative treatment routine Maddox can complete at home.
The impact of a multidisciplinary approach
Now 7 years old, she has regular sessions in a vibrating vest (much like the ones cystic fibrosis patients use) to loosen mucus in her chest, and hypertonic saline to draw it out. She gets nasal rinses, inhaled steroids and a C-PAP machine to keep her airway open at night.
She sleeps better, and she has more energy — lots of energy, actually. She hikes, swims, climbs, dances, rides horses and runs track. She participates in Special Olympics.
“We just did a four-mile hike on a mountain together,” says Kim. “We took our first vacation last year because of the Aerodigestive Program. We went to San Diego, and she rode roller coasters, swam in the ocean and saw Legoland. Before, we couldn’t leave.”
These days, the Sharpes feel confident living — and letting Maddox live — life to the fullest. No more emergencies. No more midnight runs.
“We’re down in Denver only about once every six months now,” Kim says. “That’s huge.”'
Meet Dr. Jeremy Prager