Austen had insisted on going with the evacuation team. She should not have been there, perhaps, but she had presented herself to Colonel Aguilar and told him that, as a physician, she should be with the boy as the representative of the Reachdeep team. He did not argue with her.
The helicopter was crowded. Dr. Aguilar watched the boy’s vital signs. They passed over the Williamsburg Bridge. The blade noise was high, and they spoke through headsets.
“Watch him! He’s seizing again!” Dr. Aguilar said.
Hector Ramirez went into a flurry. He was buckled down, but his small body seemed incredibly strong. He turned diagonally under the straps, and his head lashed back and forth. He began biting his lips behind the oxygen mask, and a fresh run of blood spattered inside the mask.
An Army nurse named Captain Dorothy Each yanked off the boy’s oxygen mask. She took his head in her hands. She was wearing rubber gloves. She held his head steady. It was impossible to control his jaws. The helicopter shook and the boy shook, and his jaw worked. The helicopter began its descent to Governors Island.
Austen, also wearing gloves, put her hands on the boy. She grabbed his wrists. They were the thin wrists of a five-year-old. She was impressed with how strong a boy could be.
The two women bent over the boy. Austen transferred her grip to the boy’s head. “There, there, steady, sweetheart,” she said to him. She could feel his neck tightening and writhing. It was basal writhing. This was the first time she had felt it happening in a patient.
Captain Each transferred her grip to the boy’s jaw. She held his jaw tightly in both hands, clenched, to keep him from biting. That seemed to help.
Suddenly the boy arched his back. His teeth flashed, and he bit down hard on Captain Each’s left hand. His teeth tore the rubber glove.
“Oh!” the nurse said. She pulled away momentarily, but then she was back over the boy, holding his head and jaw. Austen saw how her hand bled. The blood from her hand ran onto the boy’s hair. Austen did not say anything. Nobody said anything about it.
Everyone understood that Captain Dorothy Each would be placed in quarantine biocontainment in the Army Medical Management Unit.
THE BOY’S MOTHER, Ana Ramirez, the boy’s aunt, Carla Salazar, and his ten-year-old sister, Ana Julia, were all admitted as patients in the Medical Management Unit. They had been in close proximity with the boy. They were kept in separate containment rooms, attended around the clock by Army medical personnel. There was very little that could be done for them except monitoring and supportive therapy. The boy’s mother exhibited symptoms of the common cold, with a clear mucosal effusion from the nasopharynx. Dr. Aguilar ordered IV drips of an experimental Army drug, cidofovir, given to the patients. It was supposed to work on smallpox, but there was no evidence it would work on Cobra. Dr. Aguilar also ordered a dose of Dilantin to help control the boy’s seizures but decided not to go ahead with any heavier antiseizure drug therapy for fear that it would cause Hector to go into an irreversible coma. The only member of the family who had had seizures so far was the boy. His mother was alert, although deeply afraid and almost hysterical about her child.
The doctors had set up a biocontainment intensive-care unit, a group of rooms accessible through a vestibule in the north wing of the Coast Guard hospital. Hector Ramirez was placed there, along with Suzanne Tanaka.
Tanaka lay strapped in bed, receiving cidofovir, ribavirin, and Valium drips. She drifted in and out of consciousness, but she had not suffered any seizures.
The boy was strapped into a bed, and various monitoring machines were placed around him. One of the machines was a pressure sensor for real-time monitoring of the boy’s cranial pressure. They had drilled a small hole in his skull and put in a plastic pressure sensor there. The machine could detect brain swelling. If the doctors saw signs of swelling, they might decide to put him into surgery to remove a portion of his skull, to give his brain space to swell. “The mortality rate is awful, but it may be our only chance,” Dr. Aguilar explained to Austen.
Hector uttered a sharp cry.
Austen drew closer. The boy was small for his age. His body shook as if a wind were rattling it. The nursing staff had tied soft cords of gauze around his wrists and ankles and across his chest. They’d done their best to immobilize his head, but his mouth was uncontrollable. He had torn off a part of his tongue and swallowed it. His eyes were half open, the pupils darting. “Mama!” he said. “Mama!”
Austen bent over the bed. “We’re doctors and we’re here for you, Hector.”
“¿Donde está mamá?”
She touched his forehead. Through her glove, she could feel his facial muscles tightening and twitching.
They couldn’t give the boy a brain scan, because his condition was too unstable and because he was liable to go into seizure at any moment. The Army nurses and doctors moved around the room with great speed and desperation at times, but at other times they seemed to be moving through thick water.
WILL HOPKINS ENTERED the intensive-care unit. He was dressed in a protective suit. The probe compounds had arrived from the Navy, and Hopkins had programmed them into a Boink biosensor. “I’ve got a hand-held that’ll detect Cobra, I think.”
The staff had been taking samples of the boy’s blood. Hopkins mixed a few drops in a tube containing salt water, then put a drop of the bloody water into the sample port of the device.
It gave off a chiming sound. “Cobra,” Hopkins said, looking at the screen.
Suzanne Tanaka was now suffering agonies in a bed on the other side of the unit. Hopkins tested her blood, and the answer was obvious. He stayed by her bed for a while. “I’m sorry,” he said.
She could not answer, and it was not clear that she even heard.
As he walked out of the unit, he met Alice Austen. They spoke about what had happened to Tanaka. Hopkins told Austen that when he’d been driving down to Quantico, and she had been asleep in the car, Tanaka had begged him over the telephone to include her in the mission. “I made the decision,” he said to Austen.
“Don’t think back on decisions, Will.”
“I can’t help it,” he said.
“I can’t either. I should have put Peter Talides in the hospital.”
In the spread of an infective agent, chance plays a part in survival. Hopkins went down the hall and tested the blood of Aimee Dana, the wife of John Dana, who had been infected by brain material from Peter Talides. He did not get a reading. She seemed okay. Then he went to see Captain Dorothy Each.
She had been placed in a biocontainment room. She was sitting in a chair, reading a book, a bandage over the cut on her hand. She seemed calm, but she was very pale.
Hopkins tested her blood. So far, there was no sign of Cobra. “Looks good, but it’s really too early to tell,” he said to her.
“Thanks, anyway,” Captain Each said to him.
IN HECTOR RAMIREZ’S room, Austen continued to watch the boy. She felt that she was on the verge of understanding something important. The pattern was emerging, and then it slipped away.
She turned to Dr. Aguilar. “I still don’t believe we’ve made a diagnosis,” she said.
“We know a fair amount,” he answered.
“But we don’t understand the disease process. We’re missing a diagnosis.”
“Okay, I can buy that,” he said. “What are your ideas?”
“It’s there, but I can’t touch it.”
A doctor walked in with some test results. Hector’s white-cell count, in his spinal fluid, was too high.
“His uric acid’s high also,” the doctor remarked.
“What’s the count?” Austen asked.
“Fourteen point six. Extremely high.”
“It’s probably the result of his seizures,” Dr. Aguilar said. People having muscle breakdown have high uric acid counts in their blood and urine.
Austen was remembering something. She ran the images of Kate Moran’s autopsy through her mind. The kidneys. She remembered the
golden-yellow streaks in the girl’s kidneys. That was damage by uric acid. Something moved in Austen’s mind. It was like a bird fluttering its wings, a bird with unusual markings.
“Could you please loosen the boy’s straps?” Austen asked. “I want to see how he moves his legs.”
The medical staff hesitated.
She asked them again.
They loosened Hector Ramirez’s straps. Austen got down on her knees. She took the boy’s arm in her hand, grasping it firmly.
He was looking at her with yellow eyes. It was not easy to see where the personality of the boy was. His essential being seemed to have already died or partly died.
She let his arm go slightly. He drew it to his mouth. His teeth snapped. He moaned. He began crying, “No! Basta! Vaya! Ay!”
“Oh, God,” one of the nurses said.
With the straps loosened, the boy’s body assumed a peculiar posture. One arm was bent toward his mouth, and the opposite leg was bent. The other leg was straight. The boy’s posture was like that of a fencer leaping in for a touch. One arm straight, the other bent. The opposite leg bent, the other leg straight. It was a diagonal thrashing of the human body.
A diagonal crisscross.
The posture indicated damage to areas of the brain where signals cross. That would be the midbrain. A deranged midbrain.
The boy squirmed, and his back arched. His legs crossed in an abrupt scissoring motion.
The diagnosis clicked.
“They eat themselves. They’re children,” Austen said with sudden clarity and horror. “They pull out their eyes. Lash. Lesch. What is it called, Dr. Aguilar?”
“Oh, Jesus,” Aguilar whispered. Suddenly, he had seen it too.
“High uric acid,” she said.
“Yeah,” he said. “This kid looks like he has Lesch-Nyhan syndrome.”
“I had forgotten what it was called,” Austen said.
Lesch-Nyhan
LESCH-NYHAN SYNDROME is an extremely rare disease. It occurs once in a million births, and it occurs naturally only in boys. It is caused by a genetic mutation. Alice Austen did not make the diagnosis alone. She began the diagnosis. It was made by a team of physicians.
Frank Masaccio immediately flew to Governors Island with senior managers of his joint task force. They arrived just as Austen and the other doctors began giving a presentation to the assembled Reachdeep team.
Austen was speaking: “Lesch-Nyhan syndrome may be the most terrible genetic disease known.”
Lesch-Nyhan syndrome is caused by a mutation on the X chromosome, which is the chromosome that every child inherits from his or her mother. Lesch-Nyhan boys lack an enzyme that breaks down a metabolic waste product, and the end result is a huge excess of uric acid in the bloodstream. The enzyme they lack is called HPRT.
Lesch-Nyhan syndrome was first identified in 1964 by Michael Lesch and William L. Nyhan. Michael Lesch was then a sophomore medical student at Johns Hopkins University, in Baltimore. Bill Nyhan was his research adviser.
A boy with Lesch-Nyhan syndrome seems normal as a baby, except that the parents begin to notice what they sometimes describe as “orange sand” in the diaper. These are crystals of uric acid being passed from the kidneys. By the baby’s first birthday there is something definitely wrong. The boy becomes spastic. He does not develop normal coordination. He does not learn how to crawl or walk. His limbs become stiff. The baby’s body tends to assume the characteristic “fencing” posture of Lesch-Nyhan disease—one arm bent, the opposite leg bent. This is a sign of damage to nerve fibers in the midbrain. As the boy’s teeth come in, he begins chewing on his lips. The chewing is uncontrollable. The child begins to eat off his lips. He begins to eat his fingers. He concentrates his gnawing on certain parts of his body; no one knows why.
The parents can’t control their child. Often doctors have trouble making a diagnosis. The boy may not be retarded. He may have normal intelligence, but it’s hard to tell, because his speech is poor. He can’t speak well, although his eyes are bright and alert, and he seems to be taking in the world with understanding and intelligence. The boy may pull out his fingernails with his teeth. He attacks his body. As he grows older and stronger, he attacks the people he loves, lashing out with his arms and legs, biting at them, and using obscenities. It is clear that he is capable of love, and he forms strong attachments to his caregivers, even while he is attacking them.
The pain of self-injury is excruciating for Lesch-Nyhan children. It troubles them when they attack people, yet they can’t resist doing it. They cry out with pain as they chew themselves. They know what they are doing but can’t stop. They feel the pain, but the biting continues, and the more it hurts the more they bite themselves. They fear the pain, and their fear makes them bite themselves more violently. Thus the Lesch-Nyhan cycle of behavior literally feeds on itself. When they feel an episode of self-mutilation coming on, they beg to have their hands tied and their bodies restrained. The sudden appearance of a stranger in the room may make them bite themselves. They vomit upon themselves. They may blind themselves, tearing out their eyeballs. Self-enucleation, tearing out of the eyes, is rare, but it happens. There are not many Lesch-Nyhan adults. A Lesch-Nyhan boy may survive to young adulthood, but at some point he will die of kidney failure or self-injury.
The human genetic code consists of about three billion bases of DNA. A single change of one base in the entire human genome, at a particular location on the genome, causes full-blown Lesch-Nyhan disease. Scientists understand how the change in the DNA changes the resulting structure of the enzyme. That is simple. What is a complete mystery is how a change in one enzyme causes a radical shift in behavior. What kind of brain damage could cause an organism to try to eat itself? No one knows.
Austen told the group that the Cobra virus appears to trigger a kind of Lesch-Nyhan disease in humans, in both men and women. Lesch-Nyhan had become a contagious disease. Cobra probably had the ability to knock out the gene for the enzyme HPRT, and that somehow led to self-injury and autocannibalism. Natural Lesch-Nyhan disease was a progressive disorder that came on slowly as the child developed. “No one understands the exact kind of brain damage that causes Lesch-Nyhan children to engage in self-injury,” she said to the group. “Cobra apparently causes the same general type of brain damage but very rapidly. The virus seems to engage in a massive burst of replication, just as the moth virus N.P.V. does, and that last burst almost melts the human brain, triggering the wild change of behavior in the hours leading up to death.”
Frank Masaccio had been listening to this. Listening with his hands in his pockets, and staring at the piece of fax paper on the wall of the meeting room that showed the face of an American tourist who might or might not be the Unsub. Masaccio had been trying to see how to use that information to move the investigation forward. Now he saw a new move in the chess game. He turned to his senior people.
“I see where we can go. We need to look at every biotech company that’s doing research into this disease. We get lists of employees at these companies. We see if the name of an employee matches a name of any of the thousands of tourists to Kenya who were issued visas. It we get a match there, we’ve got Archimedes.”
HECTOR RAMIREZ DIED late Thursday afternoon. By that time Hopkins and Austen were working in the Reachdeep Core, confirming that Cobra virus disease was a type of Lesch-Nyhan syndrome.
Meanwhile, the investigation had moved into financial territory. The New York field office’s joint task force on Cobra studied recent Securities and Exchange Commission filings by companies in the biotechnology industry. They found nothing there. Agents telephoned the headquarters of the Food and Drug Administration in Maryland and asked for information on any new drug-research applications involving Lesch-Nyhan disease.
There are three major geographic areas where biotechnology companies have settled in the United States. One is the San Francisco Bay area of California, where biology is mixed in with Silicon Valley and the high-tech computer and so
ftware industry. The second area is in Massachusetts, around Boston. The third area—the largest, the deepest—is a belt of biotech companies hidden away in small buildings scattered from central New Jersey south through Pennsylvania and down into Maryland to the outskirts of Washington, D.C. This is the Middle Atlantic Biotechnology Belt, and it is where some of the leading-edge start-up companies in genetic engineering and biomedical research are situated. In all three geographic areas, the biotech companies are fueling economic growth, bringing jobs, making people rich, and developing drugs that help people live longer and have more productive lives. As a group they are light-years ahead of the rest of the world in biotechnology.
In a matter of hours, investigators had determined that there were only two companies in the United States currently doing F.D.A.-reviewed research into Lesch-Nyhan syndrome. One was a publicly held company in Santa Clara, California, outside San Francisco—a medium-sized company with public shareholders. The other was a privately held company in Greenfield, New Jersey, an hour’s drive west and south of New York City. It was called Bio-Vek, Inc. Since it was a private company, it did not file financial statements with the Securities and Exchange Commission. But Bio-Vek had recently submitted a filing with the Food and Drug Administration for permission to go ahead with Phase I clinical trials of a bioengineered treatment for Lesch-Nyhan disease in children, a so-called gene therapy protocol, in which healthy genes would be inserted into the brain tissue of sick children.
The Cobra investigators from New York enlisted the help of the Trenton, New Jersey, field office of the F.B.I. The Trenton office looked at the company’s financial filings and registration statement with the state of New Jersey, and they looked at the company’s state department of labor filings. Bio-Vek was a very small company. It had just fifteen full-time employees. The president of the company was Orris Heyert, M.D.
“This feels right,” Frank Masaccio said. “This Bio-Vek is where we want to look.” He discussed with his senior investigators and with Hopkins how they should proceed.
The Cobra Event Page 27
