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School of Medicine
The Healing Fields
In Cambodia, where children with heart ailments face bleak odds, Luca Vricella and his Johns Hopkins team of volunteers are working to help create a lasting solution, one surgery at a time.
Photography by Nicolas Axelrod
Luca Vricella and Cambodian nurse Sam Rin examine a 1-year old patient.
1 per 3.5 million people: cardiac surgeons in the United States and Europe
1 per 25 million people: cardiac surgeons in Asia
SOURCE: 2013 study published in the Cardiovascular Journal of Africa
It’s a sweltering May afternoon in Siem Reap, Cambodia, but the boy in the hospital bed is tucked under a thick blanket. His dark doe eyes wander around the room anxiously, an uneasy, barely there smile flickering at the corner of his mouth.
He is tall but just shy of 40 pounds, notably underweight for an 11-year-old. A group of clinicians in green and blue scrubs congregate around the child. They go through his chart and check the vitals displayed on a monitor above the bed. The boy’s oxygen levels are dismal, hovering between 60 and 70 percent, only occasionally breaking into the 80s. Suddenly, the group’s attention is drawn to his fingers — long and slender, with awkwardly swollen tips and purplish, bulbous nails.
“Clubbing,” murmurs Johns Hopkins pediatric heart surgeon Luca Vricella. He takes the child’s hand and examines it closely, marveling at a feature so rare that most American clinicians nowadays see it only in textbooks. “You don’t see clubbing like this in the U.S. anymore,” says Vricella, who is leading a team of Johns Hopkins clinicians on a weeklong surgical mission in Cambodia — the group’s third trip there since April 2013.
The hallmark of long-standing heart and lung disease, clubbed fingers were a common finding in the United States until the 1950s, when corrective heart surgery became widely available. Today they are almost never seen, since most congenital heart defects are diagnosed and treated within a year of birth.
Sok Nang Meas, the 11-year-old Cambodian boy, has uncorrected tetralogy of Fallot, or “tet” in cardiology speak, a constellation of four structural anomalies, two of which — a hole between the lower chambers of the heart and an abnormally narrow artery between the heart and lungs — prevent the blood from getting oxygenated properly, giving the skin a signature bluish hue. Prominent finger and toe clubbing stemming from long-standing oxygen deprivation is another telltale feature. Unrepaired tets invariably progress to heart failure. The survival odds for those who make it past the first year of life are bleak. Seventy percent of untreated children die by age 10.
The fact that Meas has made it so far is a testament to his resilience, his body’s ability to find workarounds and cope with disease, Vricella explains. Still, the boy’s outlook is rather grim — without corrective surgery, 95 percent of tet patients die by age 40.
For years, Meas had progressively worsening fatigue and shortness of breath brought on by simple daily activities, such as walking, says his grandfather, Nop Nang, who has been keeping a constant vigil at his grandson’s bedside since the day he was admitted. Three years ago, a group of physicians from Phnom Penh arrived at Meas’ village to perform free exams. They told Nang his grandson had a serious heart defect but advised against surgery, stating his chances for making it through the procedure were 30 percent, and he’d be dead by age 30 anyway, Nang recalls. Shortly after, he heard from a neighbor that a children’s hospital in Siem Reap run by a foreign doctor offered free care, but the 300 miles separating their village from the city loomed like an impassible divide. A local church helped collect money for the trip, and in May, the grandfather and grandson arrived here at Kantha Bopha III Children’s Hospital. The hospital has provided free care to children since 1999 as one of five pediatric hospitals run and operated—almost entirely through philanthropy — by Swiss pediatrician and cellist Beat Richner. Collectively, the hospitals care for 85 percent of hospitalized Cambodian children.
“Our hospitals are islands of peace and justice,” Richner says. “Eighty percent of Cambodian families live on less than a dollar day. Other hospitals require upfront payments. Kantha Bopha hospitals are free.”
Richner first came to Cambodia as a Red Cross physician in the 1970s but left in 1975 following the Khmer Rouge military coup. The regime’s short but brutal reign claimed millions of lives and destroyed Cambodia’s health care. In 1992, after the re-establishment of the monarchy, Richner returned to help rebuild Cambodia’s pediatric health infrastructure. He never left.
Meas embodies the dark legacy of the Khmer Rouge’s policies. He is one of countless Cambodian children living with uncorrected congenital heart disease. Cardiac surgery in Cambodia is limited, and pediatric cardiac surgery borders on nonexistent. The Heart Center at Calmette Hospital in Phnom Penh may be the only fully operational heart surgery hospital in a country of 15 million, but as a fee-for-service clinic, it remains out of reach for most Cambodians. Children die of easily correctable malformations, such as ventricular septal defects, which when treated promptly often give a child normal or near-normal life span, Vricella says. Many remain undiagnosed until they have developed serious heart failure and other organ damage, rendering them surgically challenging, even inoperable.
Richner is on his way to change this reality, and the Johns Hopkins team led by Vricella is central to this effort. Two years ago, Vricella heard about Richner’s work through a Johns Hopkins colleague, the Swiss-born radiologist Ulrich Willi. Intrigued, Vricella contacted Richner. The timing was fortuitous — Richner was just starting to build a pediatric cardiac care program from scratch. He had to. Uncorrected congenital heart disease was the condition with the highest mortality rate at Kantha Bopha hospitals, claiming one-fifth of children’s lives in intensive care units.
A flurry of emails was quickly followed by an exploratory trip. As soon as he set foot inside Kantha Bopha, Vricella knew he was in for the long haul. The cardiac intensive care unit was packed with children, many of them in heart failure, blue and seriously hypoxic, Vricella says.
“So many children with heart disease, more than one would ever want to see in a lifetime,” he recalls.
For a split second, Vricella wondered whether he could make a difference with an episodic trip here and there, but then he saw potential. With the hospital’s well-oiled infrastructure, dedicated cardiac intensive care unit and operating room, and talented — albeit somewhat novice — cardiac staff, he had the opportunity to make a serious impact. Granted, the program was in its nascent stages — the team was doing simpler cases in older kids—but the thirst for knowledge among them was palpable, Vricella says.
Richner had already successfully reduced deaths from infectious diseases, such as dengue fever and tuberculosis, which are endemic to Cambodia. Chronic conditions like heart disease were emerging as the next frontier in his public health efforts.
“Helping build a cardiac surgery program was a chance to effectively lower childhood mortality,” Vricella says. “It felt right.”
By the time he boarded the plane back to the U.S., Vricella had a plan and a crew in mind — a squad of top-notch Johns Hopkins clinicians he’d previously led on cardiac missions to Cuba, China, Panama and Italy, a like-minded group who shared his sense of a humanitarian obligation to do something for the developing countries by giving time, knowledge, skills and surgical supplies, some donated by companies, many left over from Johns Hopkins operating rooms. The team members use vacation time for their trips, while travel costs are covered by On My Way, a foundation co-founded by Vricella himself dedicated to providing pediatric cardiac care to children worldwide.
Pediatric cardiac critical care specialist Kristen Nelson, who’s been part of the team since its first mission, says the decision to come to Cambodia was a no-brainer not only because of the team’s self-propagating passion and the opportunity to change lives, but also because of the stimulating nature of the work.
“Repairing a child’s heart poses unique physiologic and postoperative challenges compared with other surgeries,” Nelson says. “In addition, many of these kids already have compromised physiology and depressed organ function.”
Pediatric cardiologist and echocardiography expert William Ravekes says the trips provide an opportunity to study the natural history of untreated heart disease. Beyond that, he says, the missions are a chance to spend time with patients and practice pure medicine. “We meet a lot of amazing people and families and have a chance to make a lot of impact in a very short time,” Ravekes says.
In addition to Nelson and Ravekes, Vricella’s team includes pediatric anesthesiologist Chinwe “Chi” Unegbu, critical care fellow Melanie Cooper Flaigle, cardiac surgery fellow Nhue Do, operating room nurse Becky Kayes, perfusionist Jim Biewer, pediatric intensive care nurses Mike Sloan and Michelle Kane, and interventional cardiologist Carlos Mas — a longtime colleague and collaborator of Vricella’s from Costa Rica. And while the composition of the team has varied from trip to trip, their dawn-till-dusk and dusk-till-dawn schedules have remained invariably punishing.
In the span of five days last May, the team performed 11 complex heart surgeries, 14 cardiac catheterizations and close to 100 echocardiograms. Sleep deprivation is a constant. Over the first three days, Nelson would pull three 19-hour shifts in a row, grabbing shut-eye for two to three hours at a time and fueling herself with Coke until the midweek arrival of Cooper Flaigle.
For a crew of seasoned clinicians, exhaustion is part of the routine, a mere nuisance. The real challenge is to plan and map out all surgeries ahead of time yet remain nimble if and when needed — a lesson they learned early on. One day, during the group’s first mission, Nelson grabbed Vricella. “Luca, you have to see this!”
On a stretcher was a small baby, no heavier than 8 pounds, in full-blown heart failure, Vricella recalls. The child had a large hole between the lower chambers of the heart — a ventricular septal defect. The infant was not on the operating schedule. He was about to go home and probably die, Vricella says. They operated on him.
“He came off bypass like a rocket, immediately looking better and fully corrected,” Vricella says. The baby’s mother spoke no English. She walked up to Vricella and clasped her hands together in front of her face, gently bowing her head. “It was worth a million words,” Vricella says.
Children who need treatment greatly outnumber the days and hours allotted for the mission. Choosing who to operate on and who to turn away is the hardest part of the trip, they all agree.
At the end of day one, the team gathers to debrief. Ravekes has echoed more than 20 children in a single afternoon. He rattles off his findings. The team has already mapped out their surgical cases for the week, but Ravekes’ report throws the plans into question. Some of the children he saw that afternoon need surgery — soon. Others can wait a few months, possibly until the team’s next trip in November, but it’s an odds game. Silence descends upon the group.
After a few pregnant moments, Vricella says what everyone is thinking: “The truth is we need four missions a year.”
Then he gets up to check on a patient.
Twelve-year-old Eang Kun Nea is one of the children who cannot wait. Admitted to Kantha Bopha III in 2013, she is in serious heart failure due to tet, an abnormally formed lung artery, and three out of four heart valves severely damaged by the combined effects of acquired rheumatic heart disease and her underlying heart malformations. A private clinic in Phnom Penh diagnosed her tet and put her on vitamins and iron supplements. Over the last 10 years, Nea’s heart function has been on a steady decline. No other hospital in Cambodia would touch the child, her mother says.
Nea is a high-risk patient for surgery, but doing nothing is the far graver threat, Vricella says, so on day three of the trip, he and Do go in. They manage to correct her tricuspid and aortic valves, but her mitral valve is unsalvageable; she needs a new one. Vricella replaces it with a mechanical one — the hospital’s only artificial mitral valve. Then they hit another snag. Nea’s heart wouldn’t restart for a good 15 minutes. She bleeds profusely. The intraoperative complications are a direct result of her long-standing heart disease, Vricella says. Over the years, her heart muscle has become progressively weaker and has lost its ability to pump efficiently, causing blood to pool into her liver. The swollen, malfunctioning liver is unable to produce enough clotting proteins, rendering her prone to excessive bleeding. At last, Nea’s heart begins to beat — hesitantly at first, then full force. With her heart fully repaired, Nea’s cardiac function should improve noticeably, Vricella says.
The postoperative care of patients like Nea can be just as challenging as surgery itself. In addition to advanced heart disease and secondary organ damage, many children in Cambodia have nutritional deficiencies and growth retardation, which further exacerbate any pre-existing pathology. For example, low calcium levels affect the heart’s ability to contract properly, worsening the function of an already suboptimally pumping heart muscle, says Cooper Flaigle. In addition, children with chronic heart disease have abnormally high levels of red blood cells—the body’s way of coping with chronic oxygen deprivation by cranking up production of hemoglobin that gobbles up any and all available oxygen. Yet too much hemoglobin makes the blood thicker, fueling the risk of dangerous blood clots. “All of these variables make these children very challenging to care for postoperatively,” says Cooper Flaigle.
The cardiac recovery room, known as the “heart corner,” is an eight-bed intensive care unit situated immediately next to the hospital’s two cardiac operating rooms. It is a small space, almost astringent in its austerity, but equipped with modern cardiac monitors, infusion pumps, ventilators, and mobile X-ray and ultrasound machines. Still, there are shortages. Nurses Sloan and Kane have just finished their medication inventory. There are no good drugs to control pulmonary hypertension, Sloan says, nor many choices of vasopressors, medications that selectively constrict the vessels to maintain blood pressure.
However, challenges like these, says Sloan, are a learning experience for the entire clinical team, forcing them to question every decision, pushing them to think creatively.
Biewer, for example, is intrigued by the blood-conserving potential of the French perfusion system used by his Cambodian colleague. It runs with much less blood than U.S. perfusion machines. “I am going to look into this and see what we can learn,” he says. “We want to know what works and how it works. If you can use less blood, why not do it? It’s all about blood conservation and minimizing patient exposure to donor blood.”
The idea of building a pediatric cardiac surgery program at Kantha Bopha III seemed radical at first. There were no cardiac surgeons in Siem Reap and barely any in all of Cambodia, so Richner decided to home-grow his own.
“There was so much congenital heart disease, and Richner needed someone to start operating on these kids,” Vricella says. “He basically asked, ‘Who wants to do heart surgery?’ Whoever volunteered got picked.”
Ponn Ladin didn’t exactly volunteer, but he was made an offer he didn’t quite know how to refuse. Ladin, a solid man with broad face and bright, cheerful eyes, had been doing orthopaedic surgery for nearly a decade when Richner asked him if he’d consider retraining as a pediatric cardiac surgeon. Ladin found the offer troubling. The differences between orthopaedics and heart surgery were too daunting, he thought. The consequences of a less-than-perfect technique or miscalculation are serious in orthopaedic surgery. In cardiac surgery, they are often fatal.
“At first I was anxious, because this was a whole new field,” Ladin recalls during a brief break between two cases he’s working on alongside Vricella. “It’s really life and death in cardiac surgery, and pediatric hearts require a softer, gentler hand.”
He accepted with trepidation. He went to a four-month boot camp at the Phnom Penh Heart Center, followed by a monthlong course in Saigon, and he received training from French and Swiss visiting surgeons. Ladin started out cautiously with simpler repairs in older children, gradually moving onto more challenging ones in younger kids. In November 2011, the Kantha Bopha team performed their inaugural case alongside a French cardiac team led by Gerard Babatasi, who also was instrumental in launching the Kantha Bopha program.
In 2013, Ladin began working with Vricella. The two have since performed more than 40 cases together.
Over the last few years, Ladin has blossomed as a cardiac surgeon, Vricella says. “To even think that he was doing orthopaedic surgery three years ago … ” Vricella shakes his head.
“One of the best parts is seeing the local team grow,” Vricella says. “You see that what we left the last time we were here is still there, and they’ve built upon what you’ve taught them. We are now starting to see the results of having been here several times. They’re doing more and increasingly complex cases on their own between missions.”
This rapid achievement, Vricella says, bodes well for a time when the Cambodian surgeons would be flying solo on even the most intricate of surgeries and the Johns Hopkins team presence would be obsolete. That, after all, was the long-game plan all along, Vricella says.
Initially, the Johns Hopkins mission was tightly focused on training surgeons and operating room personnel, but during the last two trips, the task evolved beyond that. All members of the Johns Hopkins team are now teaching their local counterparts. The most acute need is training physicians and nurses in the intricacies of cardiac critical care, Nelson says. There’s a dearth of intensivists in Cambodia, and pediatric cardiac intensivists simply do not exist. Yet with the growing ability to perform heart surgeries, there is a growing parallel need to train clinicians capable of caring for these children postoperatively, Nelson says.
Sloan, for instance, has put together an improvised checklist to help Cambodian nurses implement structured postop care. Typing frantically on a laptop in the intensive care unit, he is drawing a grid that lists the normal parameters for blood chemistries, continuous infusions and vital signs. “My goal is to have some sort of a tool whipped together,” says Sloan, who says he enjoys teaching just as much as patient care. “There’s a ripple effect in teaching. You end up helping more children, in a way.”
Cambodian nurses absorb his instructions voraciously. It is uncommon for physicians in Cambodia to teach nurses, Sloan says, so training nurses to take a more active role in patient care has been a cultural shift.
“They’ve made significant progress since the last time,” Sloan says. “They’re notably more comfortable mixing medicines, but also understanding the rationale behind doing so. You can see they take pride in that.”
Two days after being admitted, Meas will undergo the heart repair he should have had a decade ago.
In the United States, pediatric patients are escorted into the operating room by a parent who doesn’t leave their side until the child is under sedation. Operating room escorts are not allowed in Cambodia. Meas’ parting with his grandfather is low-key — a few whispered exchanges. No hugs, no kisses, no tears. As Meas is wheeled into to the operating room, a nurse, a surgery technician and an anesthesiology assistant descend upon him. As the surgical tech begins to insert an IV into his forearm, Meas is the epitome of coolness, lying on the operating table with eyes firmly fixated on the surgical light above his head, his face expressionless. But as a nurse starts rubbing iodine up and down his torso, Meas’ body begins to shake violently. His eyes never let go of the light above the table. The anesthesia assistant injects midazolam, a sedative, into the IV. Meas’ shaking subsides. A single tear trickles down the side of his face. Then, without making a sound, he is out. Stoicism is a cardinal feature of Cambodian character, evident even among children. “One of the best things I see here is these children’s incredible tolerance, incredible resilience,” says Cooper Flaigle. “These kids are so stoic. It’s cultural. It’s parental. Their whole lives they’ve coped with hardship.”
While Meas’ heart is being mended, his grandfather — a quiet man with melancholic eyes — sits on a bench outside the cardiac care unit. He nods respectfully to the doctors and nurses zooming by, eagerly searching their faces for a clue but never quite summoning the courage to ask for an update. For hours, he doesn’t leave the bench. Lunchtime comes and goes. He doesn’t eat. He waits. He says he is not afraid because he has full confidence in the surgeons. “This surgery,” he says, “it’s my grandson’s only chance.”
Nearly five hours after his operation started, Meas is being wheeled out of the operating room and into the recovery room. His fingers are visibly pinker and getting more so by the minute, his oxygen levels now close to 100 percent.
A little later that afternoon, Vricella walks into the intensive care unit to check on Meas. Taking the boy’s hand into the palm of his own, he notes the child’s rapidly vanishing cyanosis, or bluish coloring. “This week, we’ve performed three tetralogies of Fallot,” Vricella says. “I find it remarkable that we’re able to bring our team to Cambodia and correct the very disease whose treatment was pioneered at Hopkins 70 years ago.”
Postscript: In late November, the Johns Hopkins team returned home to Baltimore from its fourth mission in Siem Reap, where it had performed 10 open-heart surgeries, half of which Ladin did solo. A Pennsylvania State University medical student, Chris Kalmar, joined the group and launched a database cataloging the Cambodian team’s efforts dating back to the beginning of the program in 2011.“This will be a very rich database with a lot of granular detail,” Vricella says. “These data can help generate valuable insights and inform future efforts.”
90% of children with heart disease in low- and middle-income countries have no access to cardiac services due to poverty and lack of specialized care.
SOURCE: 2014 report in the World Journal for Pediatric and Congenital Heart Surgery
Children who need treatment greatly outnumber the days and hours allotted for the mission. Choosing who to operate on and who to turn away is the hardest part of the trip.