When Lisa Ranzino of Sewell, NJ, took her 11-year-old daughter, Brianna, to the family doctor in 2007, she was worried about Brianna's discomfort during frequent coughing fits. Less than two years later, Lisa feared that Brianna may lose her life.
Initial speculation about the cause of Brianna's coughing bouts ranged from bronchitis to sports-induced asthma. It wasn't until a CAT scan was taken in March of 2007 that the root cause was finally discovered – a large tumor crushing her trachea.
Brianna's parents were initially relieved when they were told that the tumor was benign, but that relief was short-lived. The discovery of the tumor was followed by a series of surgeries at hospitals throughout the eastern U.S. – to try to remove the persistent tumor and to address subsequent complications with Brianna's trachea and esophagus. "This tumor that wasn't supposed to be such a big deal was becoming a big deal," said Lisa.
Throughout Brianna's ordeal, her life was far from that of a typical young girl. Her medical condition prevented her from attending school from seventh to ninth grade, so she was tutored at home. Physical exertion amplified her coughing fits and closed off her airway even further, so she stopped playing sports or even walking long distances.
But, of all the adverse effects of the tumor, the inability to eat was perhaps the most difficult issue for Brianna, and her mother, to handle. For two-and-a-half years, Brianna was tube fed, and, thus, denied a pleasure that most people take for granted – tasting food. And despite being tube fed, she continued to lose weight. At one point, Brianna weighed only 67 pounds.
Soon after her second surgery, it was discovered that Brianna had developed a tracheoesophageal fistula, a hole connecting her esophagus to her trachea. The condition aggravated all of Brianna's symptoms, and she was ordered not to swallow anything, not even her own saliva.
When surgeons went in to try to repair the fistula in 2009, they found that the tumor had regrown. After Brianna woke up from anesthesia, she knew that something was wrong. Through tears she asked, "Why didn't they fix me?"
The doctors said that there was nothing more they could do, that the tumor would soon overtake Brianna. Her family was told that she only had a year left to live.
Rather than give up, Brianna's parents and family doctor started an intense search for someone, anyone, who could help. Finally, after reaching out to medical experts in Chicago, Washington, D.C., Maryland, and France, the Ranzinos ended up in Boston at Brigham and Women's Hospital.
At Brigham and Women's Hospital, Charles Vacanti, MD, had developed a novel procedure in which he was able to use stem cells to successfully grow functional tracheas in sheep muscle tissue. The technique had not been attempted with a human yet, but Brianna was willing to be the pioneer. This unique procedure not only gave Brianna hope, but also inspired her to give herself a new nickname, "Sci-Fi."
Vacanti's team began the process by taking cartilage cells from one of Brianna's ribs and growing them in an incubator. The cells were combined with polymer fibers to help spur growth and wrapped around a tube shaped like a trachea. After the cells successfully grew for several months, the engineered trachea was wrapped in fatty tissue and attached to Brianna's abdomen. The team then waited for the new trachea to draw nourishment from Brianna's body and grow. If the trachea grew sufficiently, it would be detached from her abdomen to replace her damaged trachea.
About two months later, on March 18, 2010, a surgical team led by Dr. Sugarbaker and Raphael Bueno, MD, went in to check on the progress of the tissue-engineered trachea. The team determined that the trachea had not grown enough to effectively replace Brianna's damaged trachea, and rather than wait for further growth, they proceeded with an aggressive backup plan.
Brianna's medical history suggested that the tumor had progressed too far to attempt to remove it and repair what was left of the trachea and esophagus. The team, however, went in for a closer look, whereupon they discovered that the tumor was indeed small enough to proceed with their alternative plan. Over the course of 14 hours, surgeons removed the entire tumor, damaged portions of her trachea, and nearly her entire esophagus; stitched the healthy portions of her trachea together; and created a short esophagus from a portion of her stomach.
Although, in the end, Brianna didn't need the tissue-engineered trachea, Dr. Vacanti noted that she had played a key role in helping build knowledge about a medical technique that could save the lives of many patients in the future.
Life has been a lot different over the past year for Brianna, now 16 years old. She's back in school and making plenty of friends. The feeding tube is gone, and, despite her slim figure, she eats plenty. She breathes easier, and she coughs much less. All these improvements are a tribute to the Ranzinos' determination to find a hospital that had a solution.
"We are eternally grateful," said Lisa. "I don't know how we found this place, but we did."
"I was going to either die sitting here or die trying," added Brianna. "I'd rather die trying."
Or, as it turns out, survive trying.
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