Mercies in Disguise Page 3

  But when the scientists did the usual tests—those that were used routinely in the late 1950s—injecting brain tissue and serum into fertilized eggs and the brains of newborn and adult mice—they found nothing. Not only were there no viruses, there wasn’t even a clue as to what it might be.

  Soon afterward, a young American doctor, Daniel Carleton Gajdusek, was on his way home to the United States after doing hepatitis research in Melbourne with his mentor, the renowned scientist Frank Macfarlane Burnet. He decided to make a last-minute detour to visit some scientists in nearby Port Moresby. When the researchers there told him about Dr. Zigas and his search for an explanation of kuru, Gajdusek was intrigued. Impulsive by nature, he decided to go see the doctor—and this disease—for himself.

  What Gajdusek did not do was ask official permission for his independent medical expedition, and this was not the way things were supposed to be done. Upon learning of his mentee’s plans, Burnet became furious. He had invited Gajdusek to work with him, he respected Gajdusek’s intelligence, and he was used to what he saw as Gajdusek’s emotional immaturity, but this breach of protocol was simply unacceptable. Gajdusek had to be stopped. He pointed out that arrangements had already been made for an Australian doctor to help Zigas. “Gajdusek is not authorized to undertake work in the kuru area,” Burnet sputtered in a telephone call to a colleague. “Another American invasion,” he continued. “Inform the minister by radio.”

  But Gajdusek was already on his way. He flew to a small airstrip, a dirt runway almost overgrown by tall grass in Kainantu, a small gold-prospecting town about twenty miles from the start of kuru territory. From there, it took a few hours for him to reach Zigas—a harrowing trip by jeep over mountains, through tropical rain forests and thickets of towering bamboo trees and sugarcane taller than Gajdusek himself. This was a region of near-constant rain, of mudslides and suffocating heat. Here, people lived in thatched-roof huts in isolated villages, a Stone Age people with no written language and little knowledge of the outside world. Both men and women wore grass skirts; their diet was made up of taro roots, bananas, and fungi.

  Gajdusek was undaunted. He had no particular zeal for Western luxuries. He had already been to the valleys of the Hindu Kush, the jungles of South America, the coast and inland ranges of New Britain, and the swamps and high valleys of Papua New Guinea and Malaysia. He’d seen people with rabies, choking on saliva because they could not swallow. He’d seen victims of plague, and those shivering and burning with hemorrhagic fever. He’d seen encephalitis patients screaming in pain from terrible headaches. Now he would see kuru.

  When Gajdusek arrived unannounced on March 13, 1957, Zigas was taken aback. He observed the tall, skinny, and bedraggled man before him, wearing threadbare shorts, an unbuttoned plaid shirt over a soiled T-shirt, and worn-out sneakers, his black hair in a ragged crew cut. Zigas thought Gajdusek was “one of those globetrotters, a kind of ‘freelance anthropologist’ looking for fertile hunting ground for whimsical studies.” And he talked nonstop, hitting Zigas with a barrage of monologues and questions. Zigas was astonished when Gajdusek told him he was a doctor, with a degree from Harvard and specialty training in pediatrics and infectious diseases. And yet to Gajdusek’s eyes, it was Zigas who was eccentric. He reminded Gajdusek of the comedian Danny Kaye both in his appearance and his demeanor.

  Despite his misgivings, Zigas was glad to have another Westerner around. He’d arrived a few years ago, to a rudimentary hospital so filthy with dirt and splashes of red betel nut juice, spit on the walls by patients, that he had to spend weeks scrubbing to achieve even a semblance of cleanliness. Native doctors there made meaningless diagnoses of diseases that may have been serious or trivial—like “bad foot” and “bad blood.” Zigas treated people with infections and injuries as best he could, but he felt so isolated, so alone out there in the mountains, that he longed for the company of another Western doctor. Many of his predecessors had become addicted to drugs or alcohol as a way to cope.

  After his initial astonishment, Zigas eased into the role of Gajdusek’s guide in this remote place. The two doctors treated the diseases that were treatable—a skin disease of children called yaws; ulcerating open sores; leprosy—and won wide-eyed respect from the Fore when some of their antibiotics and drugs cured diseases they attributed to sorcery. But kuru was another story. It was too powerful for the doctors’ medicines, the Fore said.

  And they were right.

  Gajdusek saw his first kuru victims the day after he arrived. He and Zigas drove four hours in Zigas’s jeep to pick up two middle-aged women, unable to walk, trembling, unable to speak or feed themselves. In a dispassionate letter to Burnet, Gajdusek described their “fixed and pained faces.”

  He was particularly fascinated by the children with kuru. The first one he saw was a little boy, about seven years old, who’d been carried to Zigas and Gajdusek by frightened villagers. Like the women, the child could not walk and his speech was so labored and slurred as to be almost impossible to understand. He could not feed himself. His limbs were trembling. Only a few months earlier, the villagers told Zigas and Gajdusek through an interpreter, the child had been normal and healthy, running and playing, chattering with his friends.

  Gajdusek wrote to his mentor in the United States, Joseph Smadel, associate director of the National Institutes of Health, hardly able to contain his enthusiasm and his horror: “I looked at those kids and said, ‘Jesus Christ. No goddamn bastard on earth has ever seen a disease like this thing. This is something, Joe. This will change the world.”

  Gajdusek knew of no disease so widespread in a small group, so quickly incapacitating. Kuru, he wrote to Smadel, “is so astonishing an illness that clinical description can only be read with skepticism, and I was highly skeptical until two days ago when I arrived and began to see the cases on every side.”

  New diseases are rare; new diseases as strange as this one were all but unheard of. If Gajdusek could claim this new disease, his name would go down in medical history, maybe even attached forever to the disease. Kuru also seemed as if it might hold the key to understanding brain diseases like Alzheimer’s or Parkinson’s. Gajdusek knew that if he could shed light on those, it would surely mean instant fame.

  He also knew, however, that if he was to take ownership of kuru, he would have to be first to publish a paper identifying it and describing it, even if he could not explain it. He set his sights on the world’s most prestigious medical journal, The New England Journal of Medicine.

  Kuru was such a riddle, though. And his goal was to explain it, not just describe it. But how do you find the cause of a disease that leaves no trace of infection in the brain or in the victim’s blood? Perhaps it was not an infection, but then what was it—a poison? Where to start? He had only the most rudimentary laboratory facilities. And the possibilities in this remote area with plants and insects, snakes and toxins, all so foreign, seemed endless.

  Just the logistics of finding kuru patients—a first step in figuring out the disease—took so much time and effort. Hour upon hour traveling up steep mountain slopes by foot in driving rain to reach hidden villages. One of Gajdusek’s guides became incapacitated by intense pain after his leg brushed against a stinging plant; he could not walk for hours. Gajdusek himself was tortured by cuts from the razor-sharp grasses that lined the path.

  Not that Gajdusek minded: give him a strange and remote area, hot and sticky weather, a six-month-long rainy season and the constant irritation of mosquitoes and flies and snakes, and he was just fine—even happy to be there. He was known among his colleagues as a man who was oblivious to creature comfort; he was grandiose, driven, a visionary. He had an uncanny memory but was distractible, with a short attention span.

  He was also very smart. “The peculiar sensation I had when first meeting him I have found described best in The Autobiography of Alice B. Toklas,” explained one of Gajdusek’s students. “‘A little bell went off in Alice’s head when she first met Gertrude Stein th
at said, “genius.”’ This is what I felt when I first met Carleton.”

  And he knew it: at age ten, Gajdusek had stenciled the names of twelve renowned microbiologists, the likes of Louis Pasteur and Robert Koch, on the steps leading to the attic of his house. He left the last step blank, for himself.

  On this trip to New Guinea, starting the work that would make him famous, Gajdusek sought children with kuru. He had spent years treating children with infectious diseases and considered himself to have a special bond with children—something that would later prove to be his downfall as, over the years, he began bringing dozens of young boys, and some girls, back to the United States to live with him, promising them an education as they left their parents and families behind. He said later that he had adopted them, but there were no formal adoption papers. Eventually this practice would lead to troubling accusations of inappropriate behavior and, finally, prison. But, in the beginning, in the New Guinea highlands, the Fore children adored him, following him around, calling him “Docta America.”

  There were advantages to studying kuru in children. If there was a toxin or a microbe involved, it could not have been something they were exposed to decades ago, like shingles, for example, the excruciating nerve inflammation that can follow chicken pox forty or fifty years later. In addition, children would not have chronic diseases like Alzheimer’s or Parkinson’s that could complicate analyses with their own neurological symptoms.

  Gajdusek began to review the possible causes of kuru. It could be a hysterical illness, like the seventeenth-century outbreak of “witchcraft” in Salem. Or it could be a genetic disease, like Huntington’s. Or it could be the result of a brain inflammation caused by an infection or toxin or allergic reaction.

  But every hypothesis he tested, based on everything he knew from his decade of medical practice, offered the same result: nothing.

  To make matters worse, there was no equipment in his rudimentary lab. Even surgical gloves—needed for handling specimens or treating yaws—were lacking while he waited for the shipments of medical equipment he’d ordered to arrive. If kuru was caused by a microbe, he’d risk infection himself if he worked without gloves. But then, he’d worked in laboratories handling tissues infected with rabies and polio and viral encephalitis, all incurable diseases, and nobody in those labs had ever got infected. He decided to forge ahead. He knew how to be careful when handling possibly infectious tissues and examining patients. He’d be safe, he told himself, rashly ignoring the real risk he was taking.

  The first step was to try, in a hit-or-miss way, every possible therapy he and Zigas had on hand. They administered an antibiotic, chloramphenicol, to three children and the two middle-aged women he’d met soon after he arrived. If kuru was caused by a bacterial infection, it would clear up quickly the way a strep throat gets better when a person takes penicillin. But there was no change. In truth, Gajdusek had not really expected an antibiotic to work with a disease so unlike any bacterial infection he’d encountered or read about. But he had to work by a process of elimination.

  Next, they gave another group of kuru victims phenobarbital, an epilepsy drug. They thought perhaps it might at least quell the tremors. But again, no effect.

  They tried aspirin—it might tamp down any inflammation—and vitamins B and C, and fish oil. They improved their patients’ diets. Nothing.

  After weeks of experimenting, the only thing Gajdusek and Zigas had to show for their efforts were more kuru victims. Gajdusek sent a pleading letter to Port Moresby’s director of public health, Roy Scragg, asking if he could send any or all of the following: tranquilizers that might affect the neurological symptoms; drugs like cortisone or antihistamines that might squelch immune reactions; testosterone (a wild guess, particularly since kuru’s preferred victims were women); and other types of anticonvulsants. He got them all—and still no changes.

  Gajdusek even requested to have two girls, ages eight and ten, who were in the early stage of the disease sent to Australia, where, he thought, first-world medical facilities might make the difference. They are “completely cooperative, friendly, and easily manageable,” he wrote in a letter to Scragg, pleading their case. “Most important now is haste,” he wrote, “for one case is now at the earliest recognizable stage—a really hard thing to find.” Nothing came of his suggestion—the children were not sent to Australia.

  Perhaps kuru was caused by a virus that had entered the area when Europeans first came to New Guinea. If it was a virus the Fore people had never encountered, it might devastate them much the way smallpox devastated Native Americans. But no—kuru had first appeared among the Fore around 1920. And the first European to enter the area, Ted Ubank, a gold prospector, did not arrive until 1936.

  Gajdusek, for all his boundless energy, was no closer to an answer. He was trying to care for kuru patients, feeding those who could no longer eat, while also doing spinal taps and collecting blood and urine and keeping up with his various scientific investigations.

  Gajdusek began to reach for more eccentric explanations. Cannibalism? This would explain the gender imbalance of the illness: a full two-thirds of the children with kuru were girls; in adulthood, the ratio of women to men with kuru was 14 to 1. Fore men typically got protein by eating the animals they’d hunted, whereas women and children got by on spiders and other insects—and by eating dead relatives. Cannibalism was part of Fore funeral ceremonies, which were conducted by women and children, usually girls. Could it be that kuru was present in the brains and organs of its victims and spread when they were eaten? Or, more likely, during those funeral ceremonies, as the Fore touched the organs and brains of kuru victims and then rubbed their eyes or scratched a bite on their skin, did they infect themselves? It would be easier to spread a disease that way than for a microbe to survive digestion in the stomach and then make its way to the brain. But there was no sign of an infection, so what was being transmitted?

  He then turned to the idea that a toxin caused kuru. But again he and Zigas hit a dead end. They found nothing unusual in the dyes the Fore used to paint their skin or the smoke they breathed from their fires. Nothing in their diets. Nothing in their blood, urine, cerebrospinal fluid, or tissues obtained at autopsy. If there was a toxin, Gajdusek admitted, it would have to be “mighty unusual to explain why 15 women got the disease for every man.”

  “What is urgently needed is expert neuropathology of an entire well-fixed brain,” Gajdusek wrote once again to Scragg, “with all types of staining techniques and long-term intensive study.” The pathological exam could reveal areas of destruction in the brain that might explain the symptoms of kuru and give hints about what sort of disease or poison might be causing the disease. But if there was a new virus, for example, destroying the brain, staining would not show it.

  Gajdusek could try to get a brain from someone who died of kuru—though this would not be easy given how carefully the Fore guarded body parts, believing sorcerers could use them to cast evil spells.

  Gajdusek had to know, he went on in his letter, “exactly (with exact formulae etc.) how brain material should be prepared for such a study.” When Zigas had sent a brain to Melbourne earlier, it was not prepared properly for scientists to study it intensively. Gajdusek wanted to do better.

  In mid-May, Gajdusek had his chance. A little girl had died and her mother agreed to an autopsy in return for axes, salt, and a lap-lap (a large handwoven bag). Gajdusek did the autopsy in secret, in a hut, at two in the morning, by the light of a kerosene lamp as driving rain and a fierce wind raged outside. The only tool he had to remove the brain was a carving knife, which meant that the brain was mangled as it came out. Gajdusek removed the girl’s organs too. If the organs were diseased, that could give hints about the sort of disease he was dealing with: a poison, for example, might ravage the liver and then travel to the brain. But the girl’s organs looked normal to him and the only thing wrong with the brain that Gajdusek, who was not trained as a neuropathologist, could decipher was that
its hard coating, the dura, looked thick.

  “We got the ‘dastardly deed’ done without awakening much local curiosity or attracting too much attention to our butchery,” he wrote in a letter to colleagues. He sent the brain to Melbourne.

  He ordered an autopsy knife from Melbourne as well, and the next time he performed an autopsy, he was able to remove the brain in good enough shape for a thorough exam. Gajdusek once again sent it on to the experts in Melbourne. The brain was full of holes, they reported back, like a sponge. Gajdusek sent another brain. It had the same unusual appearance. If kuru was an infection, it was like no infection they had seen before.

  * * *

  By July, Gajdusek and Zigas had met 150 kuru patients and had more than a thousand file cards detailing their clinical history. It enabled them to establish an epidemiology of kuru. Those files, Gajdusek boasted, “represent hundreds of miles of walking and climbing in rugged New Guinea mountains.”

  There was enough information for Gajdusek and Zigas to write a paper describing kuru, chronicling the blind alleys they’d ventured down trying to understand the disease. They submitted their manuscript to the New England Journal of Medicine. Gajdusek entreated his mentor and patron, Joe Smadel, to use his influence to get the paper published.

  “Joe,” he wrote, “please urge the NEJM to get our paper out.… They have a chance at the ‘first report,’ and I think it is worth taking!!” A month later, he wrote to Smadel again: “Please, should you hear from the NEJM, assure them that kuru is no figment of my imagination but is a really exciting disease of great importance, for I am sure that is the case.”

  Their paper was accepted; it would appear in the November 14, 1957, issue of NEJM—eight months after Gajdusek had arrived in New Guinea. It described the disease in the patients Gajdusek and Zigas were following and their efforts to zero in on a cause. The disease pattern made it look like kuru was an infection, but every test for an infection had come up negative. But they also could not find a toxin. Nor could they find a clear genetic pattern of inheritance.