Child born without trachea goes home for the first time

By David Wenner

dwenner@pennlive.com

Leanne Martin knew something was drastically wrong. Her baby born seconds ago wasn’t crying.

Still, she and her husband, Robert, could have never imagined their daughter would spend her first 13 months of life at Penn State Children’s Hospital in Dauphin County. Or that it would be months before their baby would be allowed to move and they would be able to pick her up.

Or that on Wednesday, their daughter, Ramiah, would become what her doctors believe is only the second child born without a windpipe to survive and leave a hospital in the United States.

“We put all our faith in God and sure enough here it is today and we’re going home,” Robert Martin said.

According to her doctors, it required all the thinking and skill that could be mustered among a multitude of doctors and staffers at the children’s hospital along with ideas drawn from several other hospitals around the country.

Ramiah was born in Lancaster County and transferred to Penn State Children’s Hospital. She had trachael agenesis, which caused her to be born without a trachea. Her doctors say about three to six children per year are born with it in the United States, and almost all of them die soon after birth. The trachea, or windpipe, carries air from the throat to the lungs.

Early on, the Martins had to decide whether to continue the intense medical treatments needed to keep Ramiah breathing and alive. Robert Martin, who is deeply religious, said it didn’t take long. “I wanted to do everything I could to give my daughter a chance,” he said.

Yet it would be several more months until doctors at the children’s hospital felt they had come up with a plan that might enable her to someday go home.

After consulting with other hospitals around the country, they settled on reconstructing her airway using her esophagus, which is the tube that connects the throat to the stomach. While they built on work of others, the Penn State Children’s Hospital doctors believe they are the first to use a 3-D printer to create a splint which they used to support the reconstructed airway.

Describing the challenge they faced, Dr. Anthony Tsai, one of the surgeons, said “We knew we had one chance at taking care of this problem.”

In researching Ramiah’s condition, they found write ups of only 11 cases worldwide, Tsai said. During the time they were contemplating how to help Ramiah, they learned of three other children in the United States who had recently been born with the condition. None lived, Tsai said.

Dr. Robert Cilley, another surgeon involved in Ramiah’s care, stressed that it took doctors and staff across multiple departments to figure out how to care for Ramiah, and tremendous daily effort on the parts of many to keep her alive until, after several months, they rebuilt her airway. The Penn State Center for Medical Innovation was deeply involved in the effort, the doctors said.

Doctors said Ramiah’s long-term prognosis is “uncertain,” for reasons including the fact so few children with trachael agenesis have lived. One child who survived and who lives in Wisconsin is about four, they said. Ramiah, who also has a heart condition, will need additional surgeries. Regarding her rebuilt airway, the doctors said it’s possible the splint they used will be absorbed and other tissue will take over support of her airway. Or they might have to eventually give her a new splint to accommodate growth.

Her parents took her home Wednesday with a ventilator hose attached to her throat to help her breath. Shortly before that, she seemed to bubble with happiness as she fidgeted in her stroller, played with its straps and continually glanced about the room where her parents and doctors met with the media.

Her parents said they expect she will soon be on the floor playing with her siblings — she has three sisters — and learning to crawl and walk.

The Penn State doctors said they believe the method they devised for Ramiah is an advancement that can be used by other hospitals.

Given his first chance to speak during the event, Robert Martin turned to face the doctors. “God has definitely blessed your hands and minds. He works through people,” he said.

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