The Wait of the World
11 May 2018
It's hard not to stare; it's the first thing you notice. A mass the size of a pineapple hanging from the right side of his face, pulling and stretching his eye and mouth under its weight. Harder to see though are the years of relentless torment he's had to endure, and the lonely boy lost behind the mask. That was the boy discovered by the Continuing Promise 2018 team. 17-year old, Axel Medina Roca of Guatemala was born with a rare disorder known as neurofibromatosis (NF), a condition that causes normal nerves to grow into disorganized tumors. NF is a challenging disorder even for those with access to healthcare, but without money or opportunity, Axel's mass has been left to grow untreated his whole life.
"I've taken him to several hospitals over the years, but none had the ability to help us," said Edgar Medina, Axel's father. "About four years ago we took him to a pediatric hospital. They told us they could remove the mass, and we were hopeful. Ultimately, they weren't prepared for what they found and did nothing. They opened the mass and closed it, leaving him only with a small scar to show for it."
Edgar's face was tired, but pleasant, the face of a man who had done his best to help his son. He reserved eye contact only for the high points of each conversation, and even then, briefly. The rest of the time he studied the floor before him and indicated his understanding through exaggerated nods. He explained that over the years priests and missionaries would visit Guatemala and take interest in Axel's situation and promise to secure him a visa to get surgery in the States. However, in each case they would leave, and nothing would materialize.
"My son has always been a very sad boy," said Edgar, shaking his head. "The only times I've ever seen him happy is when there was some hope that something could be done about his condition. But each time he was told it wouldn't happen or it was impossible, he would fall farther into depression and despair. He would stop talking for weeks at a time."
Father Anton Grech of the GuateMalta Foundation, a charitable organization that operates in Guatemala and Malta, heard about Continuing Promise 2018's visit to Puerto Barrios and immediately thought of Axel. The Medina Roca Family were thrilled by the news, but quickly realized the 7-hour trip and hotel stay required to see the doctors were much more than they could afford. The good people of their church also recognized this dilemma and quickly took up donations to buy them bus tickets. They also found a parishioner in Puerto Barrios who agreed to take the family in for the duration of their stay.
Meanwhile, aboard the Military Sealift Command's expeditionary fast transport vessel USNS Spearhead (T-EPF 1) en route to Guatemala, the doctors of Continuing Promise were first learning about Axel. Lt. Cmdr. Jason Souza, a plastic surgeon stationed at Walter Reed National Military Medical Center was given a photo of Axel and studied it during the trip.
"When we were on the ship, I was already thinking about his case," said Souza. "It was pretty obvious from the photo that it was a plexiform neurofibroma and I was optimistic that we could help him. So when we got to Guatemala, we set out to find him."
It didn't take long for Axel and his family to find themselves sitting outside the Expeditionary Medical Unit, a series of tents used by the Continuing Promise team as a mobile military surgical site. It was here that Dr. Souza had his first encounter with the boy who had unknowingly waited a lifetime to meet him. Dr. Souza was friendly and reassuring. He examined Axel thoroughly and with the quiet confidence of a man who truly appreciates the importance of his profession.
"I knew immediately that the procedure would be challenging," said Souza. "The problem is, this thing originates in normal nerve, but turns that nerve totally abnormal. Somewhere in that mass were connections that were still viable, allowing him to move his eye and mouth. This prevents you from being able to excise the mass entirely if your hope is to preserve any facial function."
Axel sat on the examination table with his hands in his lap, listening intently to the doctors speak a language he didn't understand, studying their body language for any indicators of meaning. His eyes were glassy and he briefly turned his head to wipe them. When a Corpsman asked if he was scared, his translator relayed his response, "No, he's just happy."
"He was so happy because Dr. Souza seemed so optimistic, and although he's heard it before, he was confident it would happen this time," said Edgar. "His mother and I, on the other hand, having endured so many disappointments, were telling him not to get his hopes up. We just didn't want him to be any further broken."
Despite the challenge, Dr. Souza decided he would perform the surgery, as long as the local hospital and surgeons were interested in supporting it.
"In many ways, the most challenging parts of this case were the coordination and making sure that this was a case local doctors wanted to be a part of," explained Souza. "Once we knew they were interested and committed to his post operative treatment, we decided we were in."
In order to limit the costs to the hospital, the Continuing Promise team did all the preoperative work ups, all the labs and the imaging. They decided to perform the case at the local hospital as opposed to the EMU in order to maximize their recovery capabilities and use their ICU if needed.
The Hospital de Puerto Barrios was small, but busy. Throngs of people waited outside, sitting on curbs or lying wherever they could find a swatch of shade. The structure itself was blue, and if not for the great soft clouds, almost indistinguishable from the sky behind it. Axel was led into the O.R. waiting room barefoot, wearing a thin, faded gown where he was met by Dr. Souza and the local surgeon who would perform the procedure alongside him.
Dr. Jonathan Maldonado, a boyishly handsome 27 year-old wunderkind surgeon who began medical school at the age of 15, entered the room and spoke a few words to Axel, extending a thumbs up. He's regarded as something of a celebrity at the hospital, stopping to chat with adoring hospital staff every few yards.
The operating room was a hive of activity. Corpsmen and Guatemalan nurses wheeled squeaky carts full of glistening tools into the space. Doctors flipped switches and pushed buttons on colorful displays. By the time Axel entered the room, the team was ready.
Working as a team, the U.S. Navy and Guatemalan anesthesiologists got to work. Since the mass presented a challenging airway, they had planned to perform a fiberoptic intubation, providing an opportunity to demonstrate a technique not otherwise available in Guatemala. The intubation was uncomplicated and the surgeons scrubbed in to get to work.
"The surgery was every bit as challenging as we anticipated," said Souza. "The depth and extent of the mass meant that we experienced bleeding that was expected, but required all team members to be working cohesively. This is a challenge in an unfamiliar environment without a common language. This is where we really needed to lean on Hospital Corpsman 3rd Class Mark Jiminez, our scrub tech and interpreter, he kept us all on the same page.
The excision proved to be slow going, meticulous work. Hours into the procedure, Axel needed blood. Having prepared for this, the team administered the blood his parents had donated a day before.
Outside, Axel's parents paced the hallways, waiting patiently for word. They would snap out of their thoughtful silence each time a member of the medical team emerged from the O.R. with an update.
Once the mass was removed, the process of reconstructing his face began. Dr. Souza painstakingly snipped and sewed and stapled flaps of skin in place until he was satisfied, checking and double-checking his work.
After eight grueling hours, the surgery was complete. Axel had a new face - his old face, the one not yet deformed by the mass. Dr. Souza snuck a few final looks at his work before snapping off his blood-stained gloves. The medical team began cleaning the room and accounting for their tools. There were fist pumps and handshakes.
"This was a combined effort," said Souza. "Dr. Maldonado did as much of that surgery as I did and that's exactly how it should go. Our entire mindset from the beginning was to expand the capabilities that already exist, rather than replace them. I think Axel's case is a great reflection of that because it was only through the coordination with the Guatemalan providers and the hospital that we were able to accomplish this surgery."
Dr. Souza walked into the breezeway and pulled down his mask. Through an interpreter he described for Axel's parents the details of the procedure. Teary, Elsa threw her arms around him as Edgar nodded, smiling. They were shown the photo of their son's new face and stood in silence as they took it in.
"It's the only thing I've wanted for my child," said Edgar. "I have no money to pay for surgery or for a better life. I will be eternally grateful and will always remember what you did for our family and how you gave my son happiness. It's something that I can never repay. What we want most for him now is to catch up on all that he's missed out on and finally be able to enjoy his life."
Edgar and Elsa were led into the small ICU where Axel was recovering. His face was wrapped in gauze and he was still asleep. They shuffled to his bedside wearing yellow protective gowns. The medical staff casually backed away to give them a moment. They leaned over their son and whispered words of love and encouragement.
"I've found from doing this that there's a great support structure around him," said Souza. "One of the things that predicts whether or not a patient does well is what's called self-efficacy, basically what a patient thinks he or she can do. A lot of that is based on their support structure. In the absence of very involved parents and involved physicians and the support of this NGO, we may very well have chosen not to do this case. That's how critical it is and Axel absolutely has that in his favor."
As planned, Dr. Maldonado and the host nation team will continue Axel's postoperative treatment after the departure of the Continuing Promise 2018 team. Given the tumor type and size of the mass, Dr. Souza explained it is likely that more surgeries will be needed. However, all involved have expressed a commitment to do what is needed to finish what has been started.
U.S. Naval Forces Southern Command/U.S. 4th Fleet has deployed a force to execute Continuing Promise to conduct civil-military operations including humanitarian assistance, training engagements, and medical, dental, and veterinary support in an effort to show U.S. support and commitment to Central and South America