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 LeschNyhan 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 LeschNyhan 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. Selfenucleation, 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 LeschNyhan 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. If 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 drugresearch 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 software 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 fight-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 LeschNyhan 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 Exchang
e 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 LeschNyhan 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 stard 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.
They could do what was known as a `freeze and seize' white-collar raid on Bio-Vek. They could move in with a huge white-collar-crime-analysis team, freeze the company's operations, and take over the company in its entirety as federal evidence. That would be an extreme measure. In order to do a freeze and seize of a company, federal investigators must show probable cause that a crime has been committed. They must get a search warrant from a federal magistrate, a warrant that enables them to enter the premises and seize evidence. That was impossible in this case. There was no probable cause for thinking a crime had been committed - no evidence whatever to link Bio-Vek to the Unsub or to any crime. No federal magistrate would permit a raid on Bio-Vek.
The right way to do things - the way the F.B.I. would proceed under normal circumstances - would be for the federal investigators to take their time to develop evidence, perhaps going undercover. They would conduct quiet interviews with low-level employees. They would contact the company's bankers for information. They would check out the company's dealings with suppliers and customers. They would try to get a sense of how the money was moving.
Masaccio understood that the movement of money is the blood supply of crime. Just seeing the way this company's name popped out so easily once Dr Austen had identified the type of disease the virus was causing, he now understood, he knew in his heart, with a lifetime's experience as an investigator, that money was somehow involved with the deaths in New York City. It was there, somewhere. The long green had entered the picture - but where?
Since everyone wanted the Unsub found and arrested
in a matter of days, before any more people died, there was extreme pressure on Frank Masaccio to fly fast and hard into the case. There was no time to mount a careful investigation into Bio-Vek, no time to profile the company. Yet there was zero evidence to justify a raid. There was a good chance the company itself might be blameless. An employee or a former employee could be the Unsub. The company might not have anything to do with it, and they might be eager to cooperate. He decided to ask the company for help. Carefully. He would use some of the Reachdeep people for this, since they would know the right questions to ask.
Bio-Vek, Inc. GREENFIELD, NEW JERSEY, FRIDAY, MAY 1
Will Hopkins, Alice Austen, and Mark Littleberry took a Bell turbo helicopter across Raritan Bay and touched down on a grassy airstrip in a town not far from Greenfield, a few miles east of Bio-Vek. They were met by three F.B.I. agents from the Trenton field office in unmarked Bureau cars. The Reachdeep team got into a car driven by a female agent. The two other Trenton agents took the other car, and they moved discreetly to a remote part of the airstrip, where one of the agents wired Hopkins with a micro-tape recorder, hung down his back behind his jacket. Hopkins was wearing a charcoal-gray suit, with a blue shirt and a muted silk necktie, and he had on sunglasses. He looked every inch a federal agent. Austen thought: He's showing off. The only thing that spoiled the image was a lump under his jacket. He wore a SIG-Sauer nine-millimeter semiautomatic pistol in a holster. But that wasn't what made the lump. It was the pocket protector.
They drove over suburban roads to a business park, some low buildings constructed during the office boom of the 1980s. The buildings were not old, but they didn't look particularly new. They contained a mixture of businesses. There was a printer in one of the office blocks, with a civil-engineering firm next door.
The Bio-Vek, Inc., building had coppery dark windows
that concealed what was behind them. The investigative team cruised past, keeping a low profile. Littleberry pointed to some tall silver pipes coming out of the roof. 'Vent stacks,' he said. 'Looks like they're venting a biocontainment lab. Level 2 or Level 3.'
'That's not unusual,' Hopkins said.
The two F.B.I. cars parked in a back lot beside a Dumpster, near the printing business, out of view. Hopkins, Austen, and Littleberry got out of the car. Mark Littleberry was carrying a small Halliburton case. It contained a hand-held Boink biosensor and a swab kit.
The Reachdeep team walked casually down a sidewalk. It was a faultless day, white clouds puffy and changing in a sky as blue as dreams. The air smelled like Colorado at nine thousand feet. The ornamental cherry trees had gone into fierce bloom, and though the bloom was past its peak, the trees flashed and moved brilliantly in the breeze. The trees and plants around the business park seemed to ache and sway with life. Above Bio-Vek, a sailplane swooped and banked on rising thermals under the clouds; a pilot having fun, and below the sailplane red-tailed hawks floated and turkey buzzards moved in slow circles, people and birds enjoying the air.
The Reachdeep investigators stopped before the company's nondescript brown door. There was a galvanized box by the door, for holding clinical samples.
Hopkins led the way in. He gave the team members' real names to the receptionist. He said that the group was from the Federal Bureau of Investigation and was there to see Dr Orris Heyert, the president of Bio-Vek.
'Was he expecting you?' the woman said. 'I don't see your names on the calendar.'
'No, but this is important,' Hopkins said.
She called Dr Heyert on her telephone. In a moment he came out through a door into the lobby, with a puzzled expression on his face. He was a handsome man
in his forties. He had dark hair, a smooth haircut, lively features. He wore a white shirt and a tie, but he was jacketless, and his sleeves were rolled up, and there were many cheap pens in his pocket. He had the start-upcompany look.
In Dr Heyert's office - small and cluttered, with pictures of his wife and children on the shelves - they got down to business.
'I realize this is unexpected,' Hopkins said. 'But we need your help. I am with the Federal Bureau of Investigation, and my colleagues here are with the Centers for Disease Control and the United States Navy.'
'Can I see some identification, before we go any further?' Dr Heyert said.
Hopkins showed his creds. Austen showed her C.D.C. card.
'Do you guys want some coffee?' They said they did.
He called his secretary and asked her to bring coffee. He had an informal way about him that made Hopkins look stiff and uptight.
Hopkins did the talking. 'We need your help in an investigation.'
'My company is not the subject of this investigation, I hope?'
'No. We are searching for an unknown suspect who has been making terroristic threats involving an infective biological agent. We have reason to think that he's knowledgeable about Lesch-Nyhan disease. We need your expertise and advice.'
'This is very strange,' Heyert said.
'Why?' Hopkins said. He looked calmly at Heyert. Time passed. More time passed.
Heyert clearly expected Hopkins to say something more, but Hopkins did not. He just watched Heyert. Finally Heyert answered, 'Well, it just seems strange.'
'Have you fired any employees lately? Has anyone quit? Because we're wondering if by any chance a disgruntled former employee of yours might be the person making these threats.'
'Nobody has left the company in quite a while. Our employees are very loyal.'
Hop
kins watched Heyert carefully, observing the man's body and his eyes at least as much as he listened to the words. The tape recorder would get the words anyway. 'Can you describe the research your company is doing?'
'A lot of it is proprietary,' Heyert said mildly.
'Are there areas you can talk about?' Hopkins asked. 'We are trying to find a cure for the Lesch-Nyhan syndrome,' Dr Heyert said. 'We are using gene therapy. Are you familiar with that?'
'Not totally. Could you explain it to us?' Hopkins said. 'Gene therapy is where we replace a damaged gene in human tissue with a working gene. This involves putting the new genes directly into cells. We use viruses to put the genes into the cells. These viruses are called vectors. If you infect tissue with a vector virus, it will add genes or alter the genes.'
'What kind of virus are you using?' Hopkins asked. 'It's just a construct,' Heyert said.
'A construct? What's that?' 'It's an artificial virus.'
'Is it based on a natural virus?' 'Several.'
'Which?'
'Principally the nuclear polyhedrosis virus.'
'Oh,' Hopkins said. 'Doesn't that virus live in insects?' 'Normally, yes.'
'Can you tell me, Dr Heyert, what strain you are using?'
'Autographa californica. It has been modified to enter human brain cells.'
'I'm curious, Dr Heyert,' Hopkins said. 'Could this virus be engineered so that it not only enters the brain but replicates there? Could it then spread from person to person?'
He laughed in a way that seemed to Austen rather forced. 'Good grief! No.'
'There have been indications that the suspect has such a use in mind. We're trying to evaluate the credibility of the threat.'
'Nothing has happened, then?'
'There's been what is perceived as a threat.' 'To do what?'
'To injure people with this insect virus.' 'Who's making the threat?'
'As I said, Dr Heyert, that's what we're trying to find out.'
'I don't think it's much of a threat,' Heyert said. 'The virus couldn't be used that way.'
Cobra Event Page 27
