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 pediatric otolaryngologist Jeremy D. Prager, MD, “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.”
"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
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.
Tracheapexy: a new procedure
Among the first orders of business were to clear mucus and open the airway. The team accomplished that with an antibiotic regimen, hypertonic saline, a C-PAP machine for nighttime and a couple of daily treatments in a vibrating vest, much like the ones cystic fibrosis patients use. “We call it her shaka-shaka,” says Kim.
That helped — a lot. The treatment effectively ended Maddie’s midnight runs, and it opened a whole world of possibility. Maddie started swimming, dancing, climbing, riding horses. She ran track and participated in the Special Olympics. The Sharpes went on their first family vacation, to San Diego.
It was wonderful, but it was limiting, too. The Sharpes are an active family, and the burden of treatment was steep.
“It’s hard to take a C-PAP machine camping,” Kim jokes. “Try finding a yurt with a plug-in. That’s the real deal.”
“She was on a lot of meds and still having quite a bit of trouble,” says pediatric pulmonologist Emily DeBoer, MD, Medical Director of the Aerodigestive Program. “And her tracheomalacia was still really limiting her in a lot of the activities she wanted to do. All that made her a good candidate for the tracheopexy.”
A relatively new and state-of-the-art surgery, the tracheopexy involves attaching the back of the trachea with sutures to ligaments of the spine, essentially holding the trachea open. It’s performed at just a few centers around the world.
It’s not without risk. Stig Somme, MD, one of a handful of surgeons who performs the procedure, estimates it’s effective about 80% of the time for babies and kids with tracheomalacia — but that still leaves 20% of the time it’s not. Maddie would be one of the first kids to get it at Children’s Colorado.
The Sharpes decided to go for it.
A lot… of freedom
That was nearly five years ago now. Maddie is 11, nearly 12, and she has been living life to the fullest — even during the coronavirus pandemic.
“We spend our life in the mountains,” Kim jokes. “We social distance by choice, always.”
She was able to get off the C-PAP and the antibiotics. She still uses the shaka-shaka once in a while, but it’s only when she’s sick, which isn’t all that often. It used to be nearly all the time.
And she still does all the things she started doing back before the tracheopexy — running, horseback riding, hiking, track — but now even more. Much more. Maybe too much.
“She is full go, all the time. She does not get tired,” Kim says. “It’s a lot.”