A Hamlet's Tragedy: How Mad-Cow Disease Hit Home for Five Families and One Village

A Hamlet's Tragedy:
How Mad-Cow Disease Hit Home for Five Families and One Village

June 12, 2001, The Wall Street Journal by Steve Stecklow

QUENIBOROUGH, England -- In the summer of 1996, Pamela Beyless, a 21-year-old bank loan-processor, began to act strangely.

She often stumbled, and her speech slurred. Her supervisor suspected she might have a drinking problem. Her friends thought she was on drugs. Her parents figured it was a broken heart: her fiance had dumped her for another woman, leaving behind just a note. Ms. Beyless went on sick leave and moved in with her folks.

A year later, a 17-year-old new mother named Stacey Robinson also started behaving bizarrely. She complained of hot and cold sweats and often became tearful. Ms. Robinson's mother assumed it was postnatal depression. A doctor prescribed an antidepressant.

By the fall of 1998, both women were dead. So was Glen Day, a 35-year-old van driver. His supervisors had thought something was wrong when, after seven years on the job, he no longer could remember the stops on his delivery route.

Beginning of the Nightmare

So began the nightmare that has gripped the centuries-old hamlet of Queniborough, population 2,500. Ms. Beyless, Ms. Robinson and Mr. Day, all of whom grew up in or near this rural village two hours north of London, were victims of new-variant Creutzfeldt-Jakob disease, the human form of an ailment more commonly known as mad-cow disease. They weren't the first ever to contract the illness or to die from it. They never even knew each other. But they, along with two other young men from the Queniborough area, are the only group identified by British researchers as a statistically significant "cluster" of victims of the disease, which has so far struck at least 105 people, nearly all of them in Britain, and killed 98.

The story of the Queniborough deaths, told in new detail by some of the victims' families and close friends, shows just how terrifying and unusual the mad-cow outbreak has been. When the victims first took ill, everyone assumed it wasn't serious. When doctors finally identified the problem, it turned out to be an incurable, fatal disease that gradually destroys the brain. No one knew how the victims got it, or when, and how many others might be infected. Then, about three months ago, two local epidemiologists thought they had cracked the case. But now it appears their theory may be flawed, leaving scientists no closer to answering the biggest question of all: How many more people will die? Current estimates for Britain range anywhere from a few to more than 100,000.

Pamela Beyless was the first to show symptoms. An outgoing young woman who loved to go dancing with her friends, she was living in the southern English city of Southampton when a supervisor one day in August 1996 noticed her stumbling. The manager thought she might have been drinking, and suggested she visit the company doctor. After an examination, the doctor recommended time off and another checkup.

Pamela didn't tell her parents. But a few days later, when she visited her best friend, Jo Lambert, Ms. Lambert's father happened to be watching a TV documentary on mad-cow disease. As he observed Pamela leaving the house and staggering across the street, he turned to his family and said: "That looks like what's on the telly." Ms. Lambert's mother called Pamela's parents to say she was worried but didn't mention her husband's remark.

Arthur and June Beyless weren't too surprised. They assumed Pamela was still upset about her broken engagement a year and a half earlier. They drove down from their home near Queniborough to pick up Pamela and took her to a doctor who diagnosed stress and prescribed Prozac.

Mr. Beyless, a milkman, says he and his wife tried to comfort Pamela by reminding her that stress had once laid him low, too. A few years earlier, Mr. Beyless had spent a year on sick leave, often too depressed to leave the house, and hobbling around with a cane because he found it painful to walk. "We said, 'Look, Pam, this is what's happened,' " Mr. Beyless recalls. " 'It's a reaction to you splitting up with Andrew.' "

But over the next several months, Pamela's behavior became more disturbing. At night, she grew paranoid and demanded that her father lock the doors and close the curtains. She expressed fear that someone was going to break in and attack her mother. Local doctors were stumped, and she wound up at the national neurological hospital in London.

A Stunning Announcement

This was in early February 1997, only about 10 months after British officials had made the stunning announcement that a fatal brain disease in cattle called bovine spongiform encephalopathy, or BSE, apparently had jumped the species barrier to humans. Diagnosis of the human form, called variant CJD, was still very difficult, and could only be confirmed by an autopsy or a brain biopsy. The disease so far was extremely rare in humans; at first there were only 10 suspected cases. Scientists believed that the bovine form spread when cows ate feed that included ground-up parts of infected cattle. Such feed had been banned in Britain in 1988, but it was estimated that as many as one million infected cattle had entered the food chain.

Immediately, beef sales plunged, and the British government embarked on a massive slaughter of older cattle -- the most likely animals to be infected -- in an effort to safeguard the country's beef supply and stop the panic.

At the London hospital where their daughter grew steadily sicker, incoherent and incontinent, Mr. and Mrs. Beyless didn't initially make the connection to the wider crisis. But after Pamela had been hospitalized for a few weeks, a team of neurologists told them Pamela was suffering from a rare disease that they didn't identify. Mr. Beyless says he immediately suspected mad-cow disease, but he was afraid to ask the doctors in front of his wife.

The next day, Mr. Beyless phoned the hospital and asked point-blank if his daughter had the disease. A doctor said yes. He added that he thought she would be dead within one year from the time she had first displayed symptoms. Mr. Beyless figured that meant July -- five months away.

To further confirm the diagnosis, doctors ordered a tonsil biopsy -- a new test to identify the disease. Mad-cow disease is believed to be caused by rogue proteins called prions (pronounced "PRY-ons" [According to Prusiner, the scientist who coined the term, it's pronounced 'PREE-ons'--BSE coordinator]), which attack nerve cells, gradually destroying them and perforating the brain with microscopic holes. Prions are detectable in the brain and, for reasons that are still unclear, in lymphatic organs including the tonsils.

Pamela was transferred to another hospital for the biopsy. As she sat slumped in a wheelchair in a waiting room, another patient asked the 22-year-old woman how old she was. "Fourteen years," Pamela replied. Then Pamela turned to her mother. "Do I have to go to school today?" she asked.

Through it all, Pamela never asked what was wrong with her, Mr. Beyless says. When the tonsil biopsy confirmed mad-cow disease, Mr. Beyless asked a doctor whether she should finally be told. The doctor said "it was best to be on a need-to-know basis," Mr. Beyless recalls. To his daughter, Mr. Beyless said simply: "You've got a disease of the nervous system which is going to get worse before it gets better." Pamela showed no sign that she had understood. By then she could no longer speak.

Spurred to Action

Before his daughter's diagnosis, Mr. Beyless, a stocky, gray-haired, soft-spoken man, didn't know much about science and had never been one to challenge doctors. But now his anger and grief spurred him to action. He visited a local library and scoured articles on mad-cow disease. He called the mother of a teenager who had been identified in the papers as the first victim. And he grew gradually frustrated with Pamela's treatment -- which consisted of little more than painkillers.

One day in March, the hospital staff attempted to administer a blood test to Pamela as she was shouting and clenching her arms. Mr. Beyless had had enough. He and his wife raced Pamela in her wheelchair down the hospital corridor, left through an exit and drove her home. There, he says, they tried to treat her with aromatherapy, massages and a British version of Tylenol.

Pamela survived past July -- the month her father was told she would die -- and into the summer. But she remained bedridden, her whole body gradually pulled into "a fetal position," Mr. Beyless says. A physical therapist had visited once, and when further sessions were denied, Mr. Beyless barraged Britain's National Health Service with letters and phone calls. By then, he was in touch with an informal support group of families of mad-cow victims, and another father suggested he contact a reporter at the Independent newspaper. In September, a story appeared, and Pamela's therapy resumed.

The same month, roughly five miles away in the village of Thurmaston, another young woman began to exhibit strange symptoms. Stacey Robinson, an ebullient teen who loved singing at karaoke bars, started complaining of hot and cold sweats, and then of tingling in her fingers. After a few weeks, she stopped bathing regularly and started wearing her clothes inside-out. A doctor prescribed an antidepressant, and then another, but Stacey's condition grew worse. Her speech slurred and she teetered "like a drunk," recalls her mother, Lorraine.

A few weeks later, Stacey walked into a wall in the living room while carrying her baby, Josh. Mrs. Robinson took her to the nearest large hospital, the Leicester Royal Infirmary. Neurological tests indicated that part of Stacey's brain wasn't functioning, but doctors couldn't explain it.

Soon, Stacey couldn't walk without help, required a diaper and often cried uncontrollably. In the hospital, she scalded her hands on the radiators. Even more disturbing, she often screamed or made animal sounds, which echoed down the ward's halls.

Dirk Day, an auto mechanic who had come to the Leicester hospital to visit his younger brother, Glen, recalls hearing Stacey's terrifying screams one day in early 1998. "It's a weird sensation to be sitting in the ward and then hear someone howling," he says. But he didn't immediately make the connection to Glen, who had recently checked in with mysterious symptoms of forgetfulness, mood swings and a strange stare.

In fact, even though several months had passed since Stacey was admitted, the hospital still hadn't diagnosed her problem. Doctors told Mrs. Robinson only that Stacey had a brain disease. When Mrs. Robinson, an unemployed mother of three, asked if it was mad-cow disease, she says, "I was told not to even think about that."

Then came a coincidence that would confirm Mrs. Robinson's worst fears. In April, a new patient moved into the room next door: an emaciated young woman, just like Stacey, who couldn't speak or eat. It was Pamela Beyless.

Mrs. Robinson approached Mr. Beyless and spoke briefly with him. When she saw Pamela for the first time, she says, she was convinced that her daughter was dying of the same disease.

Stacey died the following August, after a long coma, at the age of 18. Pamela, whose first symptoms had appeared more than a full year before Stacey's, managed to be released from the hospital and to survive past the summer. But in early October, she entered the Leicester hospital again. On Oct. 11, two days after her 24th birthday, a machine at her bedside beeped; Pamela had stopped breathing. Mr. Beyless ran into the hall, shouting for help, and medical staff rushed into the room. But it was too late. As her parents held Pamela up on each side, her eyes popped open. Then she slumped back and died.

Glen Day, who by now had also been diagnosed with mad-cow disease, was still in the Leicester hospital. The staff had been trying for months to get him transferred to a nursing home because they felt there was little more they could do. The 6'6" van driver's condition had deteriorated so badly he needed to be fed by a tube directly into his stomach.

On Oct. 29, Glen finally was transferred by ambulance to a local nursing home. His brother, Dirk and Dirk's wife, Kathy, and their six-year-old daughter, Lucy, visited him in the late afternoon. "At one point he clutched my wife's hand and he wouldn't let go," Dirk recalls. Early the next morning, he received a telephone call that Glen had died overnight.

It didn't take long for Mr. Beyless, who by now was well plugged into the grapevine of mad-cow victims' families, to learn about Glen's death. Mr. Beyless had also kept in touch with Mrs. Robinson and knew that her daughter, too, had died. The fact that three young people from the area had been stricken with such a rare disease struck him as a disturbing coincidence, and he decided to notify a Leicester newspaper and radio station.

This was news to Philip Monk, an epidemiologist at the regional health authority in Leicester, who first read about the cases in the paper. Dr. Monk quickly contacted the National CJD Surveillance Unit in Edinburgh, which tracks human mad-cow cases in Britain. But the Edinburgh unit concluded that they weren't a statistically significant cluster.

Then, about a year and a half later, in May 2000, Dr. Monk learned about another Queniborough-area case from a local doctor. This was 24-year-old Christopher Reeve, a farmhand who several months earlier had begun to show signs of absentmindedness and by now was emaciated and could no longer speak.

Dr. Monk and a colleague, Gerry Bryant, again contacted the Edinburgh unit. This time, they were told that Edinburgh had also identified a fifth case in the area, a 19-year-old male who had just died. Moreover, all five victims had lived within a three-mile radius between 1980 and 1991. Still, the Edinburgh unit said the cases didn't appear to be a cluster, defined as a statistically meaningful group that epidemiologists study to try to track down a disease's source. "It can be extraordinarily difficult to assess the statistical significance" of a group, says Robert Will, the unit's director.

'Alarm Bells'

Dr. Bryant, who was still completing her training in epidemiology, says she jotted down some numbers on an envelope. At the time there were about 70 suspected human cases of mad-cow disease in Britain, and she calculated that Queniborough-area residents were at a roughly 45-times-greater risk of developing the disease than other Britons. "That just rang the alarm bells," she says.

In early July, the Edinburgh unit reversed itself and said the five Queniborough-area cases did indeed represent a statistical cluster of mad-cow victims -- the first ever. Soon after, on July 13, a member of Britain's House of Lords publicly confirmed it. The next morning, Queniborough's Main Street swarmed with satellite trucks and television crews. A tabloid paper dubbed the quaint collection of mostly affluent, thatched-roof homes "the Village of the Damned."

Drs. Monk and Bryant were authorized to conduct a study to determine the source of the disease. They distributed questionnaires, asking residents everything from what kind of pets they had to whether they drank unpasteurized milk. In addition, they interviewed members of the victims' families and more than two dozen people connected with the local beef industry.

The two epidemiologists quickly ruled out blood transfusions as a possible source, since none of the victims had undergone major surgery. School meals were also ruled out, because the victims had attended different schools. So was drinking water, since the families had gotten their water from different suppliers.

But two patterns emerged, the epidemiologists said. One was that the victims apparently ate more beef than most people. The other was that the victims' families shopped at local butchers more than did most residents, who generally purchased their meat from supermarkets. Drs. Monk and Bryant decline to disclose how much beef the victims' families ate, or the names of others they interviewed, saying they promised confidentiality to their subjects.

'Eureka Moment'

In mid-October, the epidemiologists say, they had a "Eureka moment" when a former butcher-shop employee described local cow-processing techniques. Two traditional practices struck researchers as problematic, even though they weren't illegal at the time.

One was called "pithing." According to the epidemiologists, workers at small slaughterhouses would stun cows with a bolt gun, then plunge a steel rod through the brain and into the spinal column to destroy the nerves and prevent the animal from kicking. The epidemiologists theorized that this likely burst the thin membrane that surrounds the brain, potentially putting bits of it in contact with other cow parts.

The other risky practice was the removal of the brain itself. In Britain, before the selling of brains as food was outlawed in 1989, some people liked to eat them fried, served on toast. Indeed, the British government during World War II had encouraged the eating of brains as a cheap source of protein. But in diseased animals, the brain harbors more rogue prions than do other body parts and is thus presumed to be the most infectious organ. Drs. Monk and Bryant said that out of 22 butchers they identified in the Queniborough area, only four had removed brains. And of those four, they said, two had sold meat to four of the victims' families.

In addition, the researchers said, those two butchers typically used a cloth to wipe the cow carcass after they removed a brain, which could further spread infected material from the diseased organ. By contrast, they said, large slaughterhouses hosed down cow carcasses with water after brain removal.

Finally, the researchers calculated that the disease had incubated for 10 to 16 years, based on the dates the two butchers had stopped working. The report didn't name the butchers, but the local press quickly identified them as Ian Bramley, of Queniborough, who was killed in a car crash in 1982, and John Smith, of nearby Rearsby, who retired in 1989 and later was disabled by a stroke. Members of Mr. Bramley's family couldn't be reached for comment. Mr. Smith's wife, Jennifer, says her husband's poor health prevents him from commenting.

Announcing Results

On March 21 of this year, Drs. Monk and Bryant announced their conclusions at the Queniborough rugby hall. In the audience were Mr. Beyless, Mrs. Robinson, Christopher Reeve's mother and more than 100 other residents. The meeting was carried live on British television, and newspapers around the world reported the findings. A Sydney, Australia, newspaper crowed, "Disease Sleuths Solve the Mystery of England's Mad Cow Cluster."

But did they?

On closer inspection, the report seemed to raise more questions than it answered. Ray Pratt, a slaughterhouse-industry consultant who managed Leicester's largest abattoir from 1981 to 1992, says pithing rods were used in nearly all killing operations, large and small, and weren't unique to Queniborough. "It doesn't matter where you were, a pithing rod was used," he says. And Chris Hollis, a butcher in nearby Syston who used to work at slaughterhouses, says brain removal couldn't likely have contaminated other parts of the cow because, due to its inherent messiness, it was always done separately, using different tools. If mad-cow was spread simply by brain removal, Mr. Hollis says, "we'd all be bloody dead."

Hugh Pennington, an expert in foodborne illnesses at the University of Aberdeen, Scotland, says the epidemiologists' report is fundamentally flawed because people often can't accurately remember what they ate only a few days ago, much less 20 years ago. "The evidence they have produced is not particularly strong," he says.

The report also doesn't address the issue of why none of the two butchers' hundreds of other customers are known to have fallen ill. Scientists say it's possible that some people are more genetically susceptible to mad-cow disease than others. It may also be that some people take a lot longer to show symptoms. Or it could simply be that, for whatever reason, no one else ever ate any infected meat.

Drs. Monk and Bryant stand by their findings. "This is an explanation," Dr. Monk says. "It won't be the only explanation. It might not be the right explanation. But it stacks up."

Little Comfort

For the victims' relatives, the report has brought little comfort. "This report has only added to our grief," says Mr. Beyless. He says he believes Pamela was infected by eating beef, but doesn't think it's fair to blame two local butchers "who can't answer back." Echoes Mrs. Robinson: "It's not their fault that Stacey died."

Dirk Day, the brother of Glen Day, says the report didn't explain why the outbreak occurred in Queniborough. "These practices were done throughout the country," he says. "Why just a cluster here? Why not more?"

Since their family members took ill, Mr. Beyless, Mrs. Robinson and Mr. Day have all stopped eating beef. But most of their neighbors, convinced that British beef is now safe, continue to do so. In fact, beef consumption in Britain last year surpassed the level in 1995, the year before it was announced that humans could get mad-cow disease.

In Queniborough these days, one of the most successful businesses operates out of the same building on Main Street where Mr. Bramley, one of the butchers in question, worked until he died. It is a gourmet food shop, and is now called David R. Clarke & Son, after the man who took it over from Mr. Bramley. Awards adorn the walls, and people come from miles away to shop there.

Its best-selling product: British beef.

-- Marc Champion contributed to this story.

Write to Steve Stecklow at steve.stecklow@wsj.com


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