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A family leaves their lush river valley in Brazil for a new and unexpected life a continent away. The parents seek a lifesaving treatment for their son, Artur. What they find at the University of Pittsburgh goes far beyond medical cures.

Sweet Odyssey


Cara J. Hayden


 
  The Santos family: Idario, Artur, Soraya, and Vinicius (contributed photo)
 

At noon in a Brazilian river valley, hot January sunshine bakes ripening mangoes, grapes, and bananas as a baby is cupped from his mother’s womb. His father names him Artur, hoping he’ll be strong like a bear and generous as a prince, and his mother coos a lullaby in Portuguese. From the hospital window, the family gazes over the clay terra-cotta roofs of Petrolina, a city made fertile by the São Francisco River.

Parents Idario and Soraya Santos are eager to welcome Artur to their three-bedroom home nestled amid fruit-laden orchards. But, by midnight, their raven-haired infant is so colicky he’s convulsing, wrenching his fists toward his chest. He refuses to breastfeed, and his diapers smell strangely sweet. Later that week, his small heart stops momentarily, and soon he lapses into a month-long coma while physicians ponder what’s wrong.

What’s wrong will take the family on an odyssey to the one place in the world where a cure for Artur may be possible: the University of Pittsburgh and its affiliated medical center UPMC. Here, they discover that hope comes in many forms and from unexpected places.
Idario and Soraya anxiously wait for a doctor to knock on the door of a private waiting room in Children’s Hospital of Pittsburgh. Stiffly propped side by side on a couch, they stare blankly at a television. In Portuguese, the mother Soraya silently repeats Catholic prayers, hoping the operation in progress will allow her family to regain a normal life.

Their world had changed dramatically, irrevocably, with the birth of Artur.

Shortly after he became so ill, reeking of sweetness, his baffled doctors flew him 500 miles to a larger hospital in the Brazilian city of Fortaleza. There, specialists diagnosed him with Maple Syrup Urine Disease (MSUD), a rare genetic disorder that causes his urine and earwax to smell like sap oozing from Pennsylvania maple trees. Because of the disease, his body lacked enzymes that break down certain amino acids, the building blocks of proteins. Some of these amino acids escape from the body, creating a sugary smell. But most aren’t discharged; instead, they accumulate dangerously, acting like poison. That’s why Artur struggled to survive as a newborn. Even a small amount of protein buildup can cause brain swelling, mental retardation, paralysis, or even death.

After the diagnosis, doctors prescribed a low-protein diet and a special nutrition formula. The regimen helped, but Artur wasn’t learning to walk or speak like a normal toddler. He still went into metabolic shock several times each year. His parents constantly feared the episodes when his body would fall limp, his eyes would wobble and wander, and they’d have to rush him to the hospital—again. To pay for the expensive formula, they sold their cars, most of their mango and grape vineyards, and the three-bedroom house where Artur never lived.
Every night, the parents searched the Internet for help. Three years after the boy’s diagnosis, they found some hope in a city of rivers and, yes, maple trees.

Suddenly, a knock sounds. The door opens as a doctor in blue scrubs and a dangling surgical mask steps into the room. With a smile, George Mazariegos reports success: Artur has a new liver. With watery eyes, Idario and Soraya embrace on the small couch. Seating himself in an adjacent chair, Mazariegos gently explains how, after connecting the new liver, he immediately took a blood sample to test Artur’s amino acid levels. The results-chart, which previously looked like a jagged mountain range, now is a steady rolling plain. It means Artur won’t be going into metabolic shock anymore. The new liver is breaking down those renegade amino acids.

Mazariegos, an associate professor of surgery in Pitt’s School of Medicine, speaks in both English and Spanish (his first language growing up in Chicago with Mexican parents) to make sure the parents understand him. Soraya knows some Spanish, but only a few words in English. Idario is semi-fluent in English, a language he only began studying a year ago when he learned about the MSUD liver transplant program in Pittsburgh.

The program, the first of its kind worldwide, started in 2003 through a collaboration between Mazariegos’ team and the Clinic for Special Children in Strasburg, Pa., which primarily serves Old Order Amish and Mennonite populations with high rates of genetic disorders like MSUD. A physician there, D. Holmes Morton, serendipitously discovered the cure when one of his MSUD patients received a liver transplant in 1997 for another medical condition. Surprisingly, the 8-year-old girl’s protein levels stabilized, and she began eating anything without going into metabolic shock. He realized that although MSUD isn’t a liver problem, the girl’s new liver was able to compensate for the lack of enzymes in the rest of her body.

After observing the girl’s success, and with encouragement from desperate parents in the international MSUD Family Support Group, Morton and his clinic partner, Kevin Strauss, began searching for a liver transplantation program that could develop a protocol specifically for patients with MSUD. Their criteria were that transplants “had to be done by people who do it all the time and by people who are the best at doing them.” The search ended at the Thomas E. Starzl Transplantation Institute at the University of Pittsburgh and UPMC. The institute’s pediatric program, which Mazariegos directs, has performed more than 1,100 liver transplants in 25 years, all while maintaining a 93 percent survival rate. The team performed the first MSUD liver transplant in May 2004, and now, in August 2005, Artur is the 14th patient and the first Brazilian to undergo the surgery.

Mazariegos, who waived his professional fee for the surgery because of the Santos’ financial situation, exits the waiting room, promising another update soon. Idario and Soraya slouch in relief, feeling blessed for the family, friends, and strangers who helped them move to Pittsburgh for the transplant. They first met Strauss at a 2003 MSUD symposium in Brazil. In addition to telling them about the cure, he solicited two U.S. nonprofits—The Stamps Foundation and First Hand Foundation—to pay the transplant medical bill.

Soraya dials home to report the good news. Idario thinks of the vast orchards of Petrolina and how the gifts of the trees are no longer forbidden fruit for his son.

Later that fall, a stranger slips into a room at The Children’s Institute in Pittsburgh and kisses Idario on each cheek in Brazil’s traditional manner. Idario’s smile is warm and welcoming considering Artur’s condition, which surprises his visitor, Ana Paula Carvalho (FAS ’98). The thin boy’s unmoving arms and legs are cramped against his abdomen, and his skin is unsettlingly pale. He’s recovering from unexpected complications of the liver transplant.

Carvalho, Pitt’s Portuguese language coordinator and instructor in the Department of Hispanic Languages and Literatures, is a native of Fortaleza, the city where Artur was flown for his MSUD diagnosis. Through a small-world coincidence, one of Carvalho’s Brazilian friends knew about the Santos’ difficult situation. When the friend heard that the family was in Pittsburgh, she called and encouraged Carvalho to visit.

In Portuguese, Idario explains Artur’s situation and the family’s troubles in their new country. As Carvalho listens, watching Artur’s tired, blank eyes, she thinks about the many resources at Pitt and how her students can help. It doesn’t take long to come up with a plan.

During the following spring semester, in a cluttered basement playroom that smells like fabric softener, Soraya and Pitt freshman Emmy Mandel sink into a worn couch backed by an ocean mural. Mandel, majoring in Spanish and political science and minoring in Portuguese, is part of a rotating fleet of 20 Portuguese language students from Pitt who are tutoring Soraya six afternoons a week. They meet at the Santos’ home at Pittsburgh’s Ronald McDonald House, a low-cost residence for families with children receiving medical treatment at local hospitals.

“Hello, good to meet you,” Soraya reads hesitantly from her homework.
“I am teacher.”

“You need an ‘a’ between there,” Mandel says, pointing to the English workbook donated by Pitt’s linguistics department. Soraya’s brown, chin-length hair falls across her cheek as she laughs at her mistake, then fixes the sentence.

“I am a teacher,” she states correctly, poking the air with her pencil. Suddenly, the elevator dings and Artur zooms into the basement on his red Radio Flyer tricycle, momentarily interrupting the lesson. He ignores his mother’s calls and pedals toward the droning washer and dryer in the adjacent room, followed by his laundry-basket-toting grandmother. Artur is healthy enough to live with his family again, but he regularly attends physical and occupational therapy sessions.

While Avó (Portuguese for Grandma) tests the wetness of the clothes in the dryer, 4-year-old Artur spreads his hand like a starfish against the smooth, white metal of the jittering washer. Avó rolls her eyes and shakes her head in amusement as Artur, only half the height of the machine, babbles in Portuguese about how the agitator spins. Since his recovery, he has been explaining how vacuums suck dirt and dishwashers shine plates. Family, friends, therapists, and doctors hope it means he’ll become a successful engineer. Mostly, they hope he’ll be able to attend kindergarten soon.

Upstairs in the Santos’ apartment, freshman Elizabeth Williamson—a Spanish major and Portuguese minor—is stretched across the floor, connecting Legos with Artur’s 5-year-old brother, Vinicius. The Pitt students visit the family in pairs so one can tutor while the other babysits, allowing Soraya to concentrate on her lesson. Next week, Williamson will teach and Mandel will play. The daily playtime is a treat for Vinicius, who sometimes feels neglected in the shadow of his miracle brother.

In the kitchen, Idario is scooping ground beans into a coffeemaker as he prepares to serve java at the end of the tutoring session. The family and students often chat in Portuguese over coffee so the students can practice their new language and learn about the Santos’ culture. Like many of the tutors, Williamson and Mandel are members of Pitt’s Brazil Nuts Portuguese Club, which explores Brazilian culture through Portuguese coffeehouse discussions, soap operas, movies, and samba dancing. When Carvalho—the club’s faculty advisor—told the students about the Santoses, they immediately coordinated a volunteer effort to help the family adjust to life in the United States.

Idario, wearing a Phipps Conservatory baseball cap and yellow Brasil bracelet, arranges ceramic mugs, a sugar jar, and powdered creamer on a tray. He purchased some of the food stashed in the surrounding white cupboards with grocery-store gift cards solicited by the Brazil Nuts. A stack of diapers donated by Pittsburgh’s Catholic Charities lies in the bedroom near where Vinicius and Williamson are building with the hippo-toothed Lego blocks. Krista Contino Krahn, a second-year Pitt law student and Brazil Nuts member, called the charity to request the diaper supply. The family has also received about $1,000 in donations from both Americans and Brazilians through a Web site, www.helpartur.com, which Krahn designed and maintains. The bilingual site includes photographs, MSUD information, and updates about Artur’s progress. Recently, the Brazil Nuts donated funds raised at their annual Brazilian Festival in the William Pitt Union.

In the basement, Artur pedals into the playroom, red handle-streamers swishing. Avó, who is visiting from Brazil for a few weeks, hoists Artur from the triangle-seat and slides him into a Flintstones-style car. (Artur is still learning to walk, and when he’s not riding his tricycle or driving kid cars, he teeters around holding an adult finger for balance.) Avó adjusts the toddler’s striped shirt, pulling it over his scarred and liver-swollen belly—his body is still too small for the organ—and he begins pumping his feet, maneuvering the plastic vehicle between the coffee table and video game center. Exhausted, Avó sits on a kid-size stool and watches her energetic grandson as she waits for the next load of clothes.

On the couch, Soraya and Mandel are focused on the vocabulary lesson.

Soraya struggles to say “unemployed” in her Brazilian accent, and Mandel echoes in her American accent. “Unemployed, unemployed, unemployed,” Soraya and Mandel repeat over and over, until Soraya masters the pronunciation.

This mantra is a struggle for Idario, too. Now that he doesn’t have to search for a cure anymore, he surfs the Internet every night in hopes of finding a job.

A former businessman and irrigation system engineer in Brazil, he’s been looking for employment for several months. Although he appreciates the financial support from charities and the Brazil Nuts, the 37-year-old wants to establish U.S. residency and become the breadwinner for his family again.

Obtaining legal permission has been difficult because he needs to find a job with a company that’s willing to process a work visa. Federal law requires a business hiring a foreigner to prove that the candidate is a qualified professional. To encourage a company to employ Idario, Pitt adjunct immigration law professor Lawrence Lebowitz (LAW ’87) has agreed to assist—pro bono—with the complicated visa paperwork. Last fall, Brazil Nuts President Suzanna Publicker, a senior with dual majors in Spanish and political science, arranged a meeting with Senator Arlen Specter’s assistants, gaining visa support from the senator’s office, too. Pitt’s Hispanic Law Society is also helping the Santoses with legal-related issues. Krahn, the society’s secretary, is searching for a method to solicit tax-deductible donations so donors are more likely to give to the family and more likely to give larger gifts. Once that’s solved, the society plans to host a fundraising dinner to benefit Artur and his family.

In the playroom, Artur is busying himself at a kiddie kitchen, talking in sing-speak while sitting on a toy bus. The oven is full of plastic foods, and he inspects a banana that’s perfectly yellow, like the real bananas his mother used to genetically engineer in a tropical Brazilian lab. He clasps a waffle and then examines a clump of purple grapes with his bright, black eyes framed by delicate lashes.

“It’s funny,” he exclaims in Portuguese. “I can eat everything now! I changed my battery!”

Artur calls his new organ a battery, an explanation devised by his parents, which makes perfect sense to a boy with a keen interest in household appliances and electronics. The idea of connecting plastic food, his disease, and the transplant cure is a thought he couldn’t have formulated a year ago, when he didn’t even have the ability to decide which toy to play with. His neurological improvement and the rapid cognitive development of other MSUD liver transplant recipients have been unexpected and pleasing outcomes of the surgeries. Doctors Mazariegos, Strauss, and Morton reported this success in their internationally groundbreaking MSUD liver transplant study published in the March issue of the American Journal of Transplantation. Pitt doctors have transplanted four more patients since Artur, and others are on the waiting list.

At the partner clinic in the farmlands of eastern Pennsylvania, the Amish call youngsters with MSUD “special,” because they say these children teach family, friends, and strangers how to be good people. The Brazil Nuts, Mazariegos, and many others from Pitt who’ve devoted themselves to the family agree—they’ve learned humbling life lessons from the youngest Santos, the “special” one.

Sitting on his toy bus, Artur holds a plastic grape bunch in his hands as a playtime distraction. He curls his fingers over the humps of the grapes—a reminder of the juicy fruit in the vineyards of his distant homeland. In his new landscape, a bountiful lifetime of lessons is just beginning.


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