Smoking Ears and Screaming Teeth - Trevor Norton (2010)

The Disease Detectives

‘Infectious disease is one of the few genuine adventures left in the world’ – Hans Zinsser, who discovered the causal organism of typhus

When a deadly disease threatens, our instinct is to flee. Surely only fools rush in. Yet several countries have specially trained teams of medics, scientists and vets on standby to investigate outbreaks of disease wherever they occur. The Epidemic Intelligence Service of the United States Centres for Disease Control is just one example of these storm troopers of disease. The epidemics that interest them most are also the most deadly.

In 1967 three patients in the clinic of the University of Marburg in Germany developed alarming symptoms. As if fever, vomiting, diarrhoea and severe pain were not enough, blood began to suffuse their entire body beneath the skin. Then blood leaked from every opening, even their eye sockets. They were bleeding to death and no one had the slightest idea what had struck them.

The clinic soon had seventeen more patients with the same symptoms. All were dangerously ill or dying. The illness was highly contagious and a doctor and nurse became infected.

Microbiologists worked night and day to identify the causal organism, which proved to be a virus. It was a previously unknown disease so they called it Marburg fever.

In Marburg and other haemorrhagic (blood-flowing) fevers small clots form all over the body cutting off the blood supply to tissues downstream, which then die from lack of oxygen. The body responds to this critical problem by flooding the system with anti-clotting compounds. This emergency reaction results in massive internal bleeding. Several other conditions are dangerous because of an excessive response to invaders by the body’s defences.

The three original victims worked for a local pharmaceutical firm. They had all been in contact with live monkeys whose liver cells were used to produce polio vaccines. The monkeys, imported from Uganda, had brought the Marburg virus with them and it had jumped from monkey to man.

There was worse to come. In 1976 outbreaks of haemorrhagic fever occurred in the Sudan and at a mission hospital in Zaire. The virus was similar to the Marburg one, but not identical. For the 318 victims in Zaire the mortality rate was ninety per cent, well over three times higher than for Marburg. They named this new disease Ebola after a tributary of the river Congo. Had even one of these patients travelled abroad it could have sparked a deadly pandemic. There was no cure; indeed, there was no treatment at all.

Disease detectives were dispatched to the outbreaks. The impact of the intrusion of western scientists can be as frightening to tribal communities as the disease itself. Imagine people wearing ‘spacesuits’ and perhaps military respirators, so as not to breathe the possibly contaminated air, walking down your street and taking throat swabs and samples of your blood.

There is a tendency for westerners to dismiss tribal medicine, with its witch doctors and belief in evil spirits, but in several African countries when the tribal elders realised this was no ordinary infection they enforced appropriate emergency measures. The infected were confined to their huts and tended by only one person. When victims died their huts were torched and their bodies were buried well beyond the village boundary and without a funeral gathering. Those who recovered remained in isolation for a lunar cycle. No one was allowed to travel to another village. Such wise measures were often sufficient to prevent the spread of even the most contagious diseases.

Haemorrhagic fevers are the deadliest diseases on the planet. They should be handled under what is called ‘hot-zone’ biosafety Level 4. This means that the researcher must be housed in a secure laboratory where all airflow is intothe lab. Body fluids that may be infected must be ‘handled’ in special cabinets with the researcher wearing gauntlets, having sealed protective clothing covering the entire body, and breathing from an external air supply.

The conditions when researchers arrive in a remote jungle village are dangerously different. They may be uncertain which disease they are dealing with, and if it is not clear how it’s transmitted it is difficult to know how to avoid it.

When processing hundreds of blood samples, mishaps are inevitable. During the outbreak of Ebola in the Sudan, Joe McCormick from the US Centres for Disease Control was taking numerous blood samples from patients. He accidentally stabbed his hand with a needle containing blood from someone with probable symptoms of Ebola. Though shocked and apprehensive, Joe reckoned that if he’d become infected he could do nothing about it, so he bravely continued to take samples. Luckily he was all right because the feverish patient did not have Ebola. There is a long tradition of researchers contracting the diseases they study. Joseph Goldberger, a pioneering disease detective in the early twentieth century, caught dengue fever and yellow fever and almost died of typhus. He also got a skin infection called Shamberg’s disease, but that doesn’t count because he gave it to himself deliberately in a self-experiment. We will hear more of his exploits later.

Goldberger was lucky to survive. Others were less fortunate. In 1927 alone three senior specialists in tropical diseases died of yellow fever while in the field. Deaths became so common that one scientific journal issued lists of the latest ‘martyrs to medicine’. Even today, every team that flies out to confront outbreaks of deadly diseases knows of a colleague who has not survived. Yet C. J. Peters, a veteran disease detective, grumbles not about the risks of infection but about the bone-hard biscuits, cheese that tastes like putty and a foul paste of congealed fat masquerading as meat. He also fears foreign toilet paper that is best suited for sanding down furniture. His golden rule is to take his own loo rolls. He reckons that if you’ve put your ass on the line, you should pamper it a little.

Outbreaks of Ebola and Marburg occur sporadically and then ‘go into hiding’. Discovering which animals act as reservoirs of the virus may help to devise ways of controlling the disease. With exposure to the disease over many generations wild animals may develop some immunity and can harbour the virus without displaying symptoms. Taking blood samples from such creatures is risky. Infection may be just a scratch or a bite away.

The ‘reservoir’ for Marburg was elusive until tourists visiting caves in Uganda died of the disease. At no small risk to themselves scientists caught every type of animal found in the caves and eventually discovered the Marburg virus in cave-dwelling bats. Later, other researchers showed that forest-living bats harboured Ebola.

For all the courage of the disease detectives, the unsung martyrs of lethal outbreaks are the carers who tend to the sick and dying. Of the 774 people who died during the well-publicised SARS epidemic in 2002, 162 were hospital staff who bravely stayed at their posts. During the first outbreak of Ebola in Uganda nurses working at the centre of the epidemic threatened to strike until Dr Matthew Lukwiya told them that: ‘Whoever wants to leave can leave. As for me, I will not betray my profession. Even if I am on the wards alone, I will continue.’ He knew the risks and six weeks later he became one of the 224 victims of the outbreak. In 2007 Uganda was so terror-struck by another Ebola epidemic that the country’s president advised people not to shake hands. Market vendors took to wearing gloves and priests refrained from handing out communion wafers. Again, a valiant local doctor headed for the focus of the outbreak. His boss had warned Jonah Kule that it could be a fatal mission, but he replied that he was prepared to die to help the stricken people. Within a few weeks both he and the matron who nursed him were buried.

Isolated facilities for the study of lethal diseases were established by the opening of the twentieth century when Alexander Oldenburgsky, a Romanov prince, set up a laboratory in an abandoned fort on an island in the Gulf of Finland. Its remit was to study plague and develop a vaccine. Few visitors were allowed and none could stay overnight. The researchers wore protective clothing such as rubber-lined cloaks, and were aware of how dangerous their work could be. There was the constant reminder of the isolation block. If you were contaminated and passed through its heavy hermetically sealed doors, you were unlikely to return. Two scientists died after being accidentally infected. The incineration of their bodies contributed to the in-house heating.

One of the staff, a physician and epidemiologist called Ippolit Deminsky, wanted to disprove the xenophobic notion that all plague was imported into Russia by foreigners. So he trudged the steppes searching for proof that plague lurked in the local fauna. He isolated the causal bacterium from a dead rodent (a suslik), but infected himself in the process. In a telegram to his colleagues, he instructed them to ‘take the cultures that I have isolated. All the laboratory records are in order … my body should be examined as an experimental case of a human contracting the plague from suslik. Goodbye.’

By 1981 the fort had been replaced by over a hundred laboratories ostensibly dedicated to the development and manufacture of vaccines. But they had another secret and sinister purpose.

That deadly diseases might be a weapon of war was a very early idea. In 1346 the Mogul hordes besieging Kaffa on the Black Sea coast hurled corpses riddled with plague over the city walls. The citizens who survived fled west taking the plague with them and, it is claimed, instigating the Black Death, the greatest catastrophe in human history. Once deployed, biological weapons are difficult to control.

Anthrax has long been the killer of choice for makers of biological weapons. It is a highly infectious bacterial disease of livestock that can transfer to humans. Virgil described the horrors of anthrax: ‘In droves she deals out death and in the very stalls piles up the bodies, rotting with putrid foulness, till … men bury them in pits.’ He also noted that if a person wore the fleece from an infected beast, he suffered ‘feverish blisters and accursed fire feeding on his stricken limbs’. In later centuries it came to be called the ‘woolsorters’ disease’ and once laid low a hundred felt workers in a carpet factory.

In the years preceding the Second World War several countries were working feverishly on the production of biological weapons. The Japanese produced bombs made of porcelain, which could be shattered by a tiny charge to scatter their payload of anthrax spores without damaging them. They tested them on foreign prisoners staked out in batches of ten. Germ warfare presented an excellent opportunity to experiment on fellow human beings on a large scale and anthrax became one of the first weapons of mass destruction. During their Manchurian campaign against the Chinese the Japanese deployed their anthrax bombs, infecting entire towns and villages. Unfortunately, a mischievous wind changed direction and over 10,000 Japanese troops became ill and almost 2,000 died. The Japanese also gave contaminated chocolate to prisoners of war, then allowed them to return home taking the disease with them.

Had Germany used biological weapons, the British were ready to retaliate with five million cattle cakes produced in a disused soap factory and then laced with anthrax spores by the housewives of Salisbury. Twelve Lancaster bombers would have dropped them over Germany. Churchill was enthusiastic about using anthrax to kill livestock and plague to poison armies.

There is a more deadly form of anthrax than over-seasoned cattle cake. Handling infected animals or their pelts transmits cutaneous anthrax with nasty lesions on the skin and a mortality rate of only twenty per cent; eating infected meat that’s been cattle-caked does better with fifty per cent fatalities, but inhaling anthrax spores results in an aggressive whole-body infection that can kill ninety per cent of victims. So the wartime research concentrated on delivering anthrax by air.

In 1942 Paul Fildes, a bacteriologist who was the first head of Britain’s Porton Down laboratory, calculated the amount of anthrax required for a strategic attack. As the laboratory was close to residential areas unsympathetic to anthrax assaults, the aerial tests were carried out on the island of Gruinard off the west coast of Scotland. Its only inhabitants were 155 sheep, but not for long – anthrax bombs saw to that. Fildes reckoned that weight for weight, anthrax could be as much as a thousand times more potent than any chemical weapon available at that time.

In 1943 the American biological warfare programme produced 7,000 bombs filled with anthrax. By the end of the war the United States had a factory that could produce 363 kilos of concentrated anthrax slurry with every run of its production line. It was never used.

Such was the lingering fear of germ warfare that after the war US researchers tested the dispersal patterns of harmless powder (standing in for anthrax spores) at Washington DC airport, in the New York subway and from a plane over the Bay area of San Francisco. In 1963 they played out similar aerial attack scenarios over three other cities.

Anthrax has long been considered a possible terrorist weapon. It’s the poor man’s ‘dirty bomb’ and, according to experts, it is not that difficult to produce. A 1993 report estimated that if a hundred-kilo cloud of its spores were released upwind of Washington DC it could cause anything up to five million deaths. Decontaminating the ground afterwards would be a mammoth task. The island of Gruinard remained under quarantine for fifty years with no one allowed to land. Viable spores of anthrax were still present and the land had to be decontaminated by removing loads of the most infected soil and spraying other areas with 280 tonnes of formaldehyde and 2,000 tonnes of seawater. The clean-up of tiny Gruinard took more than nine years.

America’s fears were realised when in 2001 a letter containing white powder was opened by a newspaper photo editor in Florida. Four days later he died from inhaling anthrax. There had been only eighteen cases of inhalation anthrax in the entire United States in over a century, but within days more poisoned letters arrived on the desks of broadcasters and politicians. In Washington, postal workers at the main sorting office fell ill. Both the sorting office and the House of Representatives closed down.

Eighty investigators from the Centers for Disease Control (CDC) were soon on the scene. All the offices at NBC had to be quarantined and ‘swept’ for contamination. At the Senate Office, where the spores had been dispersed by the air-conditioning system, two entire floors were sealed off and checked. Over 400 people might have been infected. Everyone had a nasal swab that was then cultured to see if it contained anthrax bacteria.

The leader of one of the CDC teams was asked by a woman who had been feverish for two weeks whether, if she had anthrax, she’d be dead by now. The scientist reassured her that she would. Another man complained of his wife refusing to let him into the marital bed as he might have anthrax. The investigator suggested that she would have to think up a new excuse.

Twenty-two people were affected, some just from handling the envelopes and therefore getting the much less dangerous cutaneous anthrax. Five died, but no one was arrested.

It was almost seven years before the FBI homed in on the culprit. DNA taken from the fatalities showed that the strain of anthrax originated from the Army Medical Research Institute of Infectious Diseases at Fort Detrick in Maryland. The researcher who had created this strain and maintained it was Dr Bruce Ivins, who headed a group developing a vaccine against anthrax. It was alleged that he had sent ‘defective’ samples to the FBI when involved in the investigation of the anthrax-contaminated envelopes. A few days before the anthrax attacks, he sent an e-mail warning that Bin Laden’s terrorists had access to anthrax. On being informed that he was to be prosecuted for the attacks, Ivins took a lethal dose of painkillers. His premature death ended the investigation and started the inevitable conspiracy theories. As one FBI agent remarked, ‘There’s always going to be a spore on the grassy knoll.’

The Institute at Fort Detrick is the centre for bioweapons research in the United States. It has the highest level of security. The regulations covering scientists working on lethal microbes state that they must be mentally and emotionally stable, physically competent and trustworthy. This bears little similarity to the description of Ivins given by the FBI. Allegedly, he had confessed to a friend that he was suffering from scary paranoia. His psychiatrist classified him as being a homicidal sociopath and his brother said he thought of himself as God. I once saw a sign on the door of a high-security research laboratory that said:

Caution! May contain nuts

Further insight into the effectiveness of the vetting procedure at Fort Detrick comes from the strange affair of Steven Hatfill. He held high-security clearance at the Institute and had access to deadly strains of anthrax, Ebola and plague. After being designated a ‘person of interest’ in the anthrax attacks, though not prosecuted, he sued the US Department of Justice. His reward was $2.4 million up-front plus an annuity of $150,000 a year for twenty years.

A joint enquiry by the New York magazine SEED and the British newspaper The Observer revealed that Hatfill’s immaculate CV was less than accurate. He had not graduated from the university he cited, nor was he a fellow of the Royal Society of Medicine, nor had he been a member of the SAS and NASA. His references from distinguished professors were faked. Clearly there are excellent career prospects for candidates with an inventive mind and imaginary qualifications. An interest in the deadliest germ on the planet is an advantage.

The United States alone has around 400 labs with over 15,000 people handling dangerous biological agents. At Fort Detrick four workers have died from accidental contamination, two of them from anthrax. Three are commemorated in the names of local streets.

One takes it for granted that such dangerous research activities are carefully monitored. Yet in 2007 and 2008 alone several of these institutions failed to ensure adequate safety precautions, and some even permitted unauthorised personnel to handle anthrax and Ebola. Others failed to report lab-acquired infections. Institutions have their own internal committees to assess safety in laboratories. But one committee had not even felt it necessary to meet when it approved the re-creation of the Spanish-flu virus that had once killed two per cent of the world’s population. Researchers at a children’s hospital in California were exposed to anthrax when a supplier accidentally sent live bacteria instead of the dead ones they had ordered. In 2005 another laboratory inadvertently sent out 4,000 virus-testing kits that included a virulent Asian-flu virus that had killed millions.

One would like to think that security at the 800 universities, institutes and commercial laboratories in the United Kingdom is better, but the revelation in 2008 that the firm responsible for security in government departments had not even vetted its own employees is not encouraging. MI5 and MI6 claim to have intercepted up to a hundred suspects posing as postgraduate students aiming to acquire skills in handling biological and chemical toxins. One pupil who graduated with a PhD in Britain was Rihab Taha, otherwise known as ‘Dr Germ’, the mother of Saddam Hussein’s biological weapons programme.

Even in the best-run laboratories accidents occur. Anna Pabst died from meningitis when the animal she was injecting squirmed, causing the serum to squirt into her eye. Jeff Platt was injecting guinea pigs with Ebola virus when he accidentally pricked his thumb. He instantly removed his glove, bled his thumb and washed the wound with bleach. He became seriously ill, but prompt action saved his life. In 2009 a German researcher also accidentally stabbed herself with an Ebola-laden needle. She was given an experimental vaccine and survived.

Personal accidents are one level of risk, but institutional incidents are quite another. In the 1940s a joint US/Canadian biological weapons laboratory was beset with problems. Perhaps because of its remote location, there was low morale and creeping paranoia. The scientists became convinced that local flies were contaminating the canteen food with anthrax. The real problem with the Canadian lab was sloppy safety procedures and frequent contamination accidents. Fortunately the facility was closed before there was a major mishap, but some have occurred.

In 1979 there was an outbreak of anthrax in the Urals. Sverdlovsk (now with its original name of Yekaterinburg restored) was known as the place where Czar Nicholas II and his family were executed in 1918. Its factories forged the T-34 tanks that enabled Russia to repel the German invasion in 1943. It now became the focus of interest for epidemiologists abroad. Russian officials announced that the Sverdlovsk outbreak resulted from people eating infected meat, and stressed, perhaps a little too vociferously, that the incident had no bearing on the Soviet Union’s compliance with the international convention banning bacterial and toxic weapons. Observers abroad were unconvinced, but it would be thirteen years before glasnost enabled an American team to visit Sverdlovsk to investigate the 1979 outbreak.

On their arrival a welcoming official advised them that there was ‘no reason to disturb the skeleton in the cupboard’. The team were encouraged to hear that there was indeed a skeleton awaiting disturbance. If disaster strikes, the reflex response of governments is to lie. When a flood exhumed the skeletons of executed dissidents in another Russian town, they were dismissed as animal bones. When nobody swallowed that, they were said to be the remains of deserters. Similarly, when a dead sheep ‘anthraxed’ on Gruinard washed up on the Scottish mainland and infected a flock of sheep that then had to be slaughtered, the British authorities came up with an implausible tale of diseased sheep being heaved overboard from a Greek ship.

The investigation at Sverdlovsk was hampered because all the patients’ records were destroyed by the KGB on orders from the Council of Ministers. The skeleton wasn’t just rattling against the cupboard door, it was positively dancing to get out. One team of pathologists had bravely carried out autopsies on forty-two victims of the outbreak. Their only ‘protection’ was flimsy gauze mouth masks. Fortunately, they had retained tissue samples and anatomical photographs of each of the victims that revealed the classic symptoms of inhalation anthrax. But where could an aerosol of anthrax have possibly come from?

The American team plotted the exact location of every victim on the days immediately before the first people fell ill. Almost every one lay in a narrow zone running from north-west to south-east. Six villages where animals had died from anthrax at the time also lay along the same line. At the northern end of the zone was Military Compound 19. On the critical date the zone lay directly downwind of the compound. This indicated that a plume of anthrax spores had been liberated from somewhere in the compound. Five victims who’d lived and worked well outside the killing zone had the misfortune to be attending military reservist classes in the compound on the fateful day.

Beyond the ornate metal gates of Compound 19 there was a research laboratory ostensibly developing vaccines against deadly diseases, including anthrax. Testing such vaccines usually involved immunising animals with the vaccine, then ‘challenging’ them by exposure to a virulent strain of the disease. Such experiments would be carried out in a sealed chamber and after the experiment the contaminated air should have been expelled through highly efficient filters that removed the microbes. But the filters had to be well maintained.

Nine people in the compound died from anthrax and sixty more were killed in the city. Most died within three days of the first symptoms appearing. The surprisingly low number of fatalities might indicate that the amount of anthrax released was very small, but the prompt actions of the authorities probably greatly reduced the number of victims. Eighty per cent of the local population were vaccinated against anthrax and were also given a variety of antibiotics. Buildings were hosed down with disinfectants, hundreds of stray dogs were shot, infected livestock were killed and burnt, healthy animals were vaccinated. Human victims who died were hastily buried without ceremony in coffins filled with chlorinated lime.

Curiously, the military took no part in these operations. They never emerged from their barracks. Many of the relatives of victims had no doubt that the outbreak had something to do with what went on behind the walls of Compound 19, but the military never explained or said they were sorry. Indeed, a letter from an anonymous worker at the laboratory published in the newspaper Izvestiya described his research as being patriotic and dismissed the outbreak with the phrase ‘accidents happen’.

In 1992 President Yeltsin, in a sober moment, blamed the military for the anthrax incident and the chairman of the KGB was told to scrap all germ warfare research at Compound 19. It was merely transferred to a new location. Yeltsin also decreed that all the relatives of the anthrax victims should be given a special state pension. It is claimed that no one received a single rouble.