It was a hot Friday afternoon in a remote village in Belize, and the UT group there on a medical mission was ready to call it a day. But they knocked on one more door. That fateful visit could turn out to be a lifesaver for Atiliano Jones Jr., a 15-year-old with a tumor the size of a cantaloupe growing inside his head.
“Atiliano was so embarrassed for us to see his medical condition,” says Amy Cranfield, a senior nursing student at UT Knoxville. “His parents came in and gathered around the kitchen table and held hands. The family began to pray fervently in Spanish.”
Atiliano suffers from juvenile nasopharyngeal angiofibroma (JNA), a benign yet aggressive vascular head and neck tumor. JNA is a rare condition that almost always affects adolescent boys. UT Knoxville College of Nursing clinical instructor Mary Sowell realized the danger the boy faced.
“The tumor is around his nasal area, inside his head. It’s full of blood vessels. It was pressing on his auditory nerve, so he couldn’t hear out of his right ear. He’d lost his sense of smell,” she says. “You could see the tumor growing down his nose. You could also look up in the roof of his mouth and see it.” Atiliano was having trouble talking and breathing. “He was getting terrible nosebleeds, and it was bleeding down the back of his throat into his stomach, which was making him vomit,” Sowell says.
The group left knowing they had to help save Atiliano’s life. “We wouldn’t be good nurses if we didn’t try to do something,” Sowell says. “I think it was a turning point in the students’ lives because it showed them why they’re in nursing.” Sowell, who’s also an oncology nurse at Fort Sanders Regional Medical Center in Knoxville, lead the 2007 spring break trip to Belize with another member of the nursing faculty, Karen Lasater. They took 10 undergraduate nursing students for a combination service-learning experience.
Knoxville Reaches Out
As soon as Sowell returned to Knoxville, she and her husband, Jonathan Sowell, an ophthalmologist at UT Medical Center, began planning to bring Atiliano to the U.S. The College of Nursing paid for the trip for Atiliano and his mother, Nadean, and UT Medical Center pledged to provide the needed medical care without charge. Atiliano and Nadean arrived in Knoxville in mid-May, and the Sowells took them into their home. Everyone expected treatment to be swift.
Dr. Eric Carlson, an oral, head, and neck surgeon at UT Medical Center, ordered an MRI and other medical tests, which showed that Atiliano’s tumor was the size of a melon. “It was a phenomenal tumor,” Carlson says. “Due to its size and its obstruction of Atiliano’s airway, I feared that the tumor would take his life if we didn’t provide medical treatment.”
The only hope, Sowell says, was to find a way to shrink the tumor: “Surgery was out of the question because it would cause gross disfigurement and prevent a good quality of life.” Sowell’s husband and her brother, Dr. Charlie Barnett, an internist, began contacting doctors in Memphis and Nashville to no avail.
The News Sentinel, Knoxville’s daily newspaper, ran a full-page feature about Atiliano’s plight, and offers for help began pouring in. Sowell learned that Boston Children’s Hospital was a leader in treating Atiliano’s condition, and she appealed to doctors there for help. The case was taken under consideration, and Sowell submitted medical files for review. In July, Boston Children’s Hospital agreed to take the case.
Stops and Starts
Reality quickly tempered Sowell’s excitement when she learned the hospital would have to charge for Atiliano’s care. The cost of his treatment was estimated at $200,000. Meanwhile, with the tumor getting larger, Atiliano was having such difficultly breathing and eating that he was nearly bedridden.
“He was going to be dead within a month,” Carlson says.
In mid-July, Carlson performed a tracheotomy, inserting a tube surgically into Atiliano’s trachea to help him breathe. Atiliano also got a feeding tube so he could get nutrition without having to swallow food. “Providing for a surgical airway and nutrition gave us time to plan therapy,” Carlson says. Through hospital discounts and donations, Boston Children’s Hospital managed to reduce the charges for Atiliano’s care to $50,000. Sowell and her extended family were just about to say yes–knowing they might have to foot the bill themselves–when another option arose.
World-renowned MD Helps
While picking up a prescription for Atiliano at a nearby pharmacy, Sowell ran into a local couple, both doctors, who had read the recent newspaper story about Atiliano’s struggle. They told Sowell their neighbor was related to Dr. Judah Folkman, the retired chief of surgery at Boston Children’s Hospital. Business Week magazine profiled Folkman in June 2005, calling him “one of the world’s most renowned cancer researchers.”
“He’s often called ‘the father of anti-angiogenesis,’ because he was one of the first to theorize, some 40 years ago, that cancer could be stopped if a tumor could be deprived of new blood-vessel growth, or angiogenensis, thus starving the tumor to death,” the magazine reported.
Carlson knew of Folkman’s work and began talking with him about the case. “We spoke several times and developed a protocol for an experimental treatment using low-dose interferon, which I was able to get approved by UT Medical Center,” Carlson said. Carlson also coordinated with Dr. Ray Pais, a pediatric hematologist and oncologist at East Tennessee Children’s Hospital, to help with Atiliano’s care.
In mid-August, Atiliano began receiving daily injections of low-dose interferon. “Interferon alpha is a normal protein in the blood that inhibits abnormal blood-vessel growth,” Folkman explained. “Atiliano is receiving low-dose interferon, similar to that used in babies. It is about one-sixteenth of the conventional dose.” The treatment will last 6 to 12 months, and doctors hope the drug therapy reduces the blood supply to the tumor.
Sowell and Barnett are buying the interferon at about $40 per day, which could amount to about $15,000 for a year’s treatment. Sowell gives Atiliano the daily injections. Because the dose is so low, Atiliano should experience few side effects from the treatment other than temporary flu-like symptoms.
Road to Recovery
Within 5 weeks of starting the interferon injections, Sowell says she could see Atiliano getting better. Atiliano is starting to breathe through his nose again. He has recovered some hearing in his right ear. The nosebleeds have decreased in frequency and intensity. And Sowell says she once again can see an opening in the back of Atiliano’s throat–evidence the tumor is shrinking. Carlson says he plans to do another MRI soon and may then be able to remove the tracheotomy.
As for the boy’s prognosis? “I think it’s still too early to tell, but we are cautiously optimistic. We’re going to hope for the best,” Carlson says.
Meanwhile, Atiliano and his mother have moved into an apartment at Fellowship House, near Fort Sanders Regional Medical Center in Knoxville. They stay there free, with much of their food provided. The Sowells have lent them a car. Having gotten some relief from the effects of the tumor, Atiliano is up and about again. The Dream Connection, a charity that helps children with life-threatening illnesses, gave him a drum set and is providing weekly drum lessons.
Nadean Jones says she was “scared to death” that she would lose Atiliano but now has hope she’ll see her son grow up and lead a normal life.