Tracheomalacia: Sarah's Story
Pediatric Care in Florida
It was the size of her that rocked her daddy’s world …
Baby Sarah had entered the world early — at just 27 weeks — weighing less than a pound.
“I was just shocked at how tiny she was,” says her father, Darlin. “If I could have held her, she would have just about fit into the palm of my hand.”
But Sarah’s parents could not hold her. She was far too fragile, fighting for breath, fighting for life. There were tubes everywhere.
In her first weeks and months, the infant struggled with many of the complications that can come with prematurity, including chronic lung disease. Doctors at the Tampa hospital where she was being treated suspected another issue as well … something called tracheomalacia — a condition where the trachea narrows or completely collapses in one or more places — making breathing extremely difficult or outright impossible.
In baby Sarah’s case, her airway collapse was so severe that, even with mechanical ventilation, she had to be perfectly positioned and often heavily sedated to avoid dangerous problems.
“I have faith in God and in science,” says her mom, Marie. “And while I was hopeful, I still worried that this wouldn’t finish well.”
Coming to Terms
As Sarah continued to overcome tough odds, her parents had to come to terms with a difficult reality. To sustain their little girl’s life, she would need continued help breathing. That meant a breathing tube and breathing machine at home. Maybe for years, maybe forever.
While a tracheostomy was a common medical solution for this type of issue, it was far from the ideal outcome these parents had pictured for Sarah. Their hopes that their daughter could be freed from the breathing tube had been dashed. With limited options, Marie and Darlin moved forward with a tracheostomy for their baby.
Even the tracheostomy proved to come with complications, partly due to Sarah’s tiny size. But just days before they were scheduled for a second surgery — her parents got some encouraging news — news that would change everything.
A Different Path
Marie and Darlin learned about a highly skilled pediatric surgeon who had just joined the surgical team at Johns Hopkins All Children’s Hospital, bringing with him a wealth of experience and knowledge regarding airway and esophageal issues.
C. Jason Smithers, M.D., had come from Boston Children’s Hospital, where he spent more than a decade helping to establish and develop the hospital’s Esophageal and Airway Treatment Center. He served on a team that pioneered and perfected procedures for a range of rare and complex issues related to the airway and esophagus — and specialized in new approaches to solving problems. Now he brings that expertise to establish a similar Esophageal and Airway Treatment program at Johns Hopkins All Children’s.
Smithers evaluated Sarah and met with her parents. He told them he believed he could help their baby with a surgical procedure called a tracheopexy — a specialized approach to opening up areas where the trachea has collapsed.
Not many hospitals offered the surgery, and few surgeons have knowledge of it, but Smithers had performed hundreds of these procedures over the past seven years. It would not be a quick surgery — or a simple one, but it might just free their child from life on a breathing machine.
“A tracheostomy would have been a reasonable option, but it would not have provided Sarah with the best possible outcome,” Smithers says. “This was something we believed we could surgically fix.”
Sarah’s parents were elated by this new option for their baby. Could this be the answer?
They needed to find out.
Sarah was checked in to Johns Hopkins All Children’s and on surgery day, the wait began. It was a long wait. Seven hours of surgery … much of that time spent evaluating the infant’s particular airway issues and then moving critical structures out of the way to be able to make the repair.
“You must take great care not to injure anything,” Smithers says. “You have to move the aorta, move the esophagus, move the trachea. There are critical nerves in there. So much of the process is about taking all the time needed to completely identify and understand the problem.”
Smithers and his team determined it was Sarah’s lower trachea that was collapsing, along with the first branches of her left and right bronchi, going into her lungs.
Now, the fix. It was to be, in large part, a high-end sewing job. The surgeon carefully sutured collapsed portions of the back of the trachea to the front of the spine, effectively opening those parts of the trachea back up, and preventing them from future collapse. He did the same thing with portions of Sarah’s bronchi.
“When you have your baby in a seven-hour surgery, it’s very scary,” Darlin says. “But the team was so professional. About every hour, someone would come out and let us know … ‘Here’s where we are. Here’s how it’s going. We’re going to keep moving to help Sarah.’”
Sarah’s surgery went smoothly. What happened next was exciting. She began to recover and improve almost immediately. She surprised medical staff by coming off the breathing support sooner than expected.
“I felt so happy!” Marie says. “Her situation had been so very serious.”
One month and two days after Sarah had come to the hospital for surgery, her parents were able to take her home. Without a breathing tube and without a breathing machine.
“Helping these babies, this is truly one of the joys of the job,” Smithers says. “Sarah has every chance of just growing up like a normal kid.”
Smithers says conditions like Sarah’s often get overlooked or misdiagnosed, and even when evaluated correctly, few surgeons have the training or expertise to fix them.
“So, part of the mission is to help educate people — including physicians — on what is the best possible outcome for these children and to redefine the standard of care,” Smithers says.
Sarah scoots across the floor in a fast crawl. With a giggle, she heads for her favorite toy — her “cell phone” — which she picks up and renders her best version of “Hi, Dada!”
Tipping the scales at 22 pounds, she is a happy and thriving 16-month-old. Due to her prematurity, she will need some other therapies and some early intervention in these first few years, but the most critical problem — the one that almost took her life — has been resolved.
“This is the very best gift we could possibly receive for our daughter, the best thing that could possibly happen for her,” Darlin says. “Because breathing … well, it’s everything.”