The Best American Travel Writing 2014 (5 page)

In one room, we found two aged sisters, 79 and 80, delicate women wearing headscarves, pajamas, and thick woolen socks. The elder, tiny and emaciated, lay with her eyes closed, resembling a dead pharaoh. The younger was watching a television broadcast of Romanian folk dancing. She said that they had lived all their lives on a farm nearby, and that their father had died of
BEN
. She added that they knew what aristolochia was but had never taken it as medicine. The pigs, she said, wouldn't touch it, but goats ate it sometimes, and then the cheese came out bitter.

Outside, the sun shone through a white haze. Tatu was on his cell phone trying to set up a house visit, but everyone he could think of was dead.

 

When scientists started investigating
BEN
, they thought that it might be a gradual, cumulative form of lead poisoning. Researchers working in a Serbian endemic village found high levels of lead in patients' blood and hair, as well as in the local flour: the miller had been using a lead-based grout to repair his millstone. The government duly dismantled 36 water mills in Serbia. Further investigation, however, revealed that
BEN
patients in other villages didn't have high levels of lead in their blood, and many had never used water mills.

My father compares
BEN
research to the story of the blind men and the elephant: everyone noticed something different and built a theory around it, and nobody saw the whole picture. Data from one village, or the expertise of one specialist, or the aftereffects of one environmental trauma, would indicate a solution, only to crumble in light of other data. Virologists, studying a village where all the
BEN
patients had hidden in an oak forest during the Second World War, attributed the disease to a virus native to oak forests. A Serbian geochemist, citing the low selenium content of soil in Serbia, suggested that
BEN
was triggered by selenium deficiency. In five villages in Kosovo, Muslims, who made up half the population, were found to be 25 times less likely than Christians to get
BEN
. Virologists argued that the disease was a virus transmitted by pigs, and that Muslims were spared by their avoidance of pig husbandry. Geneticists, believing
BEN
to be hereditary, saw the same data and ascribed the lower incidence among Muslims to their ethnic makeup. Confusingly, the Muslims in Bulgaria, known as “white gypsies,” often did get
BEN
, though actual Gypsies did not, and was that because of their genes or because they didn't work on farms? A Bulgarian researcher claimed that he had identified a chromosomal marker, but nobody else could find it.

In the 1950s, the Bulgarian village of Karash was hit particularly hard by the disease. The communist government, having decided that the problem lay in the village itself, shut Karash down and relocated the population to Sofia. Twenty years later, some of the Karash exiles began to develop
BEN
, but those who had moved as children never got the disease—only those who had lived in Karash for 15 years or more.
BEN
, it seemed, was a super-slow time bomb. Fifteen years of exposure would set the clock ticking.

A shift in
BEN
studies came in the 1970s. A charismatic Danish veterinarian named Palle Krogh had been studying a strange outbreak of kidney disease affecting pigs in Danish slaughterhouses. There were no obvious differences between the lives of the sick pigs and those of the healthy ones. The only clue was that the disease seemed to worsen after rainy summers. Krogh eventually determined that the pigs' grain had been contaminated by a fungal toxin called ochratoxin A, which produced effects not unlike
BEN
: the kidney damage was similar, and both were aggravated by wet weather. Sure enough, when tests were run in Yugoslavia, ochratoxin A was found in the blood and the urine of
BEN
patients, as well as in their grain supply.

Continued research, however, revealed that ochratoxin A was far more common than initially suspected: it appears in grains, coffee beans, wine, and other stored substances all over the world. Some of the highest levels of contamination have been recorded in countries with no known
BEN
-like disease. The story of ochratoxin illustrates the fundamental challenge in epidemiology: proof of exposure isn't the same thing as proof of causation. Every day, we're exposed to countless potential pathogens and toxins, most of which don't make us sick. Identifying the “right” toxin is particularly difficult when the disease affects the kidney, an organ whose main function is to clear the blood of toxins.

Oddly, or perhaps not so oddly, Palle Krogh himself died of kidney cancer, in 1990. Krogh's tumor was later dissected and analyzed by a group of his colleagues, including Tatu. Tatu suspected that the cancer had been caused by ochratoxin. My father pointed out that Krogh had been a chain smoker—smoking is a leading cause of renal carcinoma—and Tatu conceded that he could remember Krogh waving a cigarette in excitement at his latest findings. Nonetheless, it was difficult not to be struck by the death from kidney cancer of a scientist who had devoted many years to the study of nephrotoxic and possibly carcinogenic mold.

 

Two days later, in a high-ceilinged coffeehouse in Zagreb, my father and I met with Bojan Jelaković, a mild-mannered Croatian nephrologist who has worked closely with Grollman on the aristolochic-acid theory. He took us on a tour of an endemic region two hours southeast of Zagreb. We passed the towering grain silos of an industrial-looking mill. According to Jelaković and Grollman, such mills are eradicating
BEN
, because they combine wheat from so many different farms that the aristolochic acid is diluted. Patients we spoke to at a local dialysis clinic confirmed that they had once ground their own flour from their own wheat, but had switched to bigger communal mills after the war.

In the village of Kaniža, Jelaković showed us two churches standing face-to-face: one Croatian Catholic, with a white pointed steeple, and the other Ukrainian Orthodox, with an onion dome. These churches have come to stand for another famous “natural experiment” from the annals of
BEN
. Around 1905, a community of Ukrainian immigrants settled in Kaniža, having been offered free farmland by the Austro-Hungarian emperor. Although the Ukrainians kept their religion and did not intermarry with the Croats, they ended up getting sick just as frequently as the native villagers. The case proved that the disease isn't inherited in the classical Mendelian sense.

In a nearby village, Jelaković showed us a so-called black house—left empty and gone to ruin after its occupants were stricken with
BEN
. He told us that a household of around a dozen people had died here in the 1970s. Next door, a boy and a girl watched us through the front windows, then pulled the curtains closed.

Like most
BEN
regions, this one is prone to flooding, and we had parked our cars by a monument to a 19th-century deluge alongside the Sava River. Across the river was Bosnia. There had been a bridge here before the war. “A wooden bridge,” Jelaković said. “A very beautiful one.”

We parted with Jelaković and drove across the river into Bosnia. At a hospital in the city of Odžak, we were given the use of an ambulette and a driver, so that we could visit
BEN
patients in nearby villages. We sat in a sunny garden with an elderly husband and wife, both of whom had
BEN
. They had heard of aristolochia by one of its local names (wolf's paw), but they didn't know what it looked like. We showed them photographs, and they said they might have seen it around but they didn't pay much attention to weeds. When asked what they thought caused the disease, they immediately said water: more people got sick in the parts of the village that were prone to flooding, and fewer people were getting sick now, because of improvements to the water system. They noted that the disease didn't affect anyone who lived in the hills, with the exception of women who had grown up in the lowlands and had moved to the hills only after getting married. We heard something similar in another village, where a man with
BEN
, who had lost his father, his aunt, and three siblings to the disease, told us about a neighbor who had no
BEN
in his family. “You see, he comes from the other side of the street,” the man said. It emerged that every house on one side of the street had one or several cases of
BEN
, but the other side, which was on slightly higher ground, was almost completely free of the disease. Annie Pfohl-Leszkowicz, a proponent of the ochratoxin theory, has cited similar patterns as evidence that
BEN
is caused by a fungal toxin: the healthy side of the street, she proposes, gets direct sunlight, which discourages the growth of mold.

In Odžak, my father and I met with Enisa Mesić, a nephrologist from nearby Tuzla. A magnetic presence, with a large head, copious dark hair, a deep voice, and piercing gray eyes, Mesić told us about the bureaucratic obstacles faced by
BEN
researchers. After the war, political power was decentralized in order to preserve equilibrium among different ethnic groups. As a result, Bosnia-Herzegovina now has no fewer than 13 ministries of health: one at the federal level, one for each of its 10 cantons, and one each for the self-governing Brčko District and the Republika Srpska. The two major centers for
BEN
research, in Tuzla and Sarajevo, are in cantons outside the endemic region, which means that before researchers can actually study any patients, they must submit requests to two different ministries.

Because of the war, nearly every patient's file is missing at least four years. There is no central
BEN
database, and establishing one would require the cooperation of all 13 ministers of health. The municipalities used to have local databases, but these were discontinued after the war. When asked what had happened to the databases, Mesić ticked off their fates: “In Bijeljina, the archive probably still exists, but it's difficult to access. In Å amac, it probably doesn't exist anymore. In Odžak, it was destroyed in 1992, together with the hospital.”

 

Early in the 20th century, after the fall of the Ottoman Empire and the ensuing chaos in the Balkans, a new verb entered the English language: “Balkanize,” defined by the OED as “to divide (a region) into a number of smaller and often mutually hostile units, as was done in the Balkan Peninsula in the late nineteenth and early twentieth centuries.” Most European languages have an equivalent: the French
balkaniser,
the Italian
balkanizzare,
the German
balkanisieren,
and the Russian
balkanizirovat'
—attesting to the special relationship between the Balkan Peninsula and the human tendency toward division and faction. It's an apt word to describe the study of Balkan nephropathy, and its fragmentation along geopolitical, ethnic, religious, linguistic, and even disciplinary lines. Researching the disease requires expertise in a wide range of fields—nephrology, epidemiology, genetics, oncology, microbiology, hydrogeology, botany, toxicology, biochemistry—each of which can be as hermetic and insular as a tiny country, with its own language, customs, and sovereignty.

The basic philosophical question surrounding
BEN
is whether it's a big problem or a little one. For Arthur Grollman,
BEN
is part of a worldwide crisis of aristolochic-acid nephropathy: a story in which the true culprits are government agencies that fail to regulate herbal medications. When I met him later, at his apartment in Manhattan, he assured me that
BEN
was a closed case, and that a greater source of concern was the public health risk caused by the use of a variety of aristolochia in Chinese herbal medicine. He described his recent collaborations with researchers in Taiwan, where aristolochia is a commonly prescribed remedy, and where the reported incidence of upper-urinary-tract cancers is the highest in the world. Whereas a hundred women got sick in Belgium, Grollman says, millions of people may be at risk in Taiwan and China.

Researchers like Tatu, on the other hand, think that
BEN
is unique: although its causes may occur individually all over the world, their combined effect is specific to the endemic regions. For my father, too, the disease is defined by a set of particular locations. He thinks it's significant that patients speak of doomed houses—that they feel it's the places and not the people that are sick. He often quotes a remark by an old colleague, now deceased: “I could live in this town for twenty years, and I'd know which house to live in, to not get sick.”

On our last afternoon in Bosnia, the driver drove my father and me around the countryside to look for aristolochia. We stopped at a swamp overgrown with creeping tendrils, trembling fronds, and strange, earlike formations. We did not find aristolochia. We stopped by a cornfield, and walked along the perimeter and down one of the rows. A sudden commotion broke out among the cornstalks, a violent rustling and shaking, as if from the thrashing of some hidden beast. A moment later, the source of the disturbance revealed itself: a glossy, compact pheasant, running through the corn.

We got back into the van. The sun hung low over the late-summer fields. The cornstalks seemed to be standing around chaotically, like skinny, crazy people, their arms flung in all directions. As we drove past, there was one magical moment when they arranged themselves into rows and it was possible to see clearly all the way to the end, before they dissolved back into disorder.

FROM
Virginia Quarterly Review

 

I
F THERE WAS ONE THING
Sandra knew well, it was hair. She knew hair from root to split end. In beauty school, she had learned the shape of the human head and how the best thing to do when trimming its hair was to section the skull into eighths. Her long nails shone red as she held her soft hands in front of her to demonstrate on an imaginary client. Her gold rings glinted. When she tired of haircutting techniques, she waved her hands quickly and her fingers sparked through the thick night like fireworks.