Mad Cow Disease - The Case of the Cherry Hill Cluster - NYT 3/28/04

[Guest guest]

Mad Cow in America - one author thinks it's already begun. Here is a

review of her book.

 

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hkdd

 

 

New York Times March 28, 2004

 

The Case of the Cherry Hill Cluster

 

 

By D.T. MAX

 

 

 

Janet Skarbek is 36 years old and lives in Cinnaminson,

N.J. She is the author of ''Planning Your Future: A Guide

for Professional Women,'' a book about managing the

unknown. It was published in 2001 by the Professional

Women's Institute, a small networking and support

organization that Skarbek and three other women jointly ran

out of their homes. ''Planning Your Future'' presents a

world where exemplary order and control are possible. It

urges working women to get ahead by thinking ahead:

choosing a career with their children in mind, timing

pregnancies so as not to lose traction at work. It tells

them to fend for themselves in a society they may sometimes

perceive as unsympathetic to their needs. Skarbek herself

turned down a plum corporate job for the sake of her two

kids. ''I knew that I wasn't willing to work the hours that

a vice president of a Fortune 500 company would require to

get the job done right,'' she said.

 

 

''Planning Your Future,'' with its emphasis on success

through self-sufficiency, was a minor volley in the culture

wars. It landed Skarbek an invitation to speak at a

Department of Labor conference in 2001 on ''the

21st-century work force.'' Skarbek is forceful and

credible, a former internal auditor for the Internal

Revenue Service who started her own accounting practice.

President Bush opened the conference. When Skarbek's turn

came to speak, she repeated her message to professional

women to buck up, take their careers into their own hands

and watch as their incomes rose.

 

 

Skarbek resembles Lewis Carroll's Alice -- the same short

stature, broad forehead, straight, long hair and grown-up

gaze, as well as the same touching, plucky personality. And

like Alice, she, too, was about to fall down a rabbit hole.

 

 

 

In January 2000, a friend of Skarbek's named Carrie Mahan

became ill. One evening, Mahan, 29, went with her boyfriend

to a party and came home unusually tired. The next morning

she started hearing songs in her head and had trouble using

her key to unlock her car door. At an emergency room in

Philadelphia, doctors gave her medicine and suggested rest.

But she was back the next day, complaining of anxiety,

nausea and hallucinations. She was admitted, then

transferred to the University of Pennsylvania Medical

Center. Things got worse quickly. She faded in and out and

began to suffer body twitches called myoclonus jerks. Soon

she fell into a coma and was put on life support. About a

month later, on Feb. 24, 2000, she was allowed to die.

 

 

Early on in Mahan's illness, one of her doctors, Peter

Crino, wrote on her chart, ''Could this be C.J.D.?'' -- by

which he meant Creutzfeldt-Jakob disease, a fatal brain

disorder often characterized by myoclonus jerks, loss of

coordination and sometimes dementia. C.J.D. is extremely

rare; the University of Pennsylvania Medical Center, which

is an unofficial referring hospital for such cases in its

region, typically sees only about one or two victims of

C.J.D. a year. So the working diagnosis was some sort of

viral infection of the brain. But when Crino saw the

autopsy and brain biopsy results, he suspected that his

first thought had been right. ''She had holes all over the

place,'' he said. ''She clearly had a devastating

neurologic injury. Her brain was just gone.''

 

 

Unlike many diseases, C.J.D. is caused not by a virus or

bacteria but by a misformed prion (a type of protein) that

no longer performs its proper function in the body. Prions

-- at least according to current scientific thinking --

cause several other diseases, most notably bovine

spongiform encephalopathy, or mad cow disease. Prion

diseases are unique because of the number of ways they can

occur. They can result from a genetic mutation that

generates the harmful prion or from an infection of prions

from an outside source, most likely in meat that is

consumed -- or simply by chance: a certain number of

proteins in the body just happen to lose their shape over

time and bring about the disease.

 

 

Mahan's doctors were not sure which of these versions of

C.J.D. she may have had. Her prion gene was found to be

normal. And since no one was known to have gotten mad cow

disease in the United States, the possibility of infection

seemed remote. Her C.J.D. was most likely the third kind.

It had no direct cause. In medical parlance, the disease

was sporadic C.J.D.

 

 

It was not a perfect diagnosis. For one thing, Mahan was

black, and very few black C.J.D. victims had been reported.

She was also extremely young to have sporadic C.J.D.: it

usually strikes people in their 60's or older. According to

Pierluigi Gambetti, who runs the national surveillance

center for C.J.D. at Case Western Reserve University

Medical School, only 3 to 5 percent of the cases his center

sees are in people in her age group or younger. Gambetti

was sent some of Mahan's brain tissue to look at, and when

he subjected it to a test for the presence of harmful

prions, he got a negative result. But this itself was not

especially troubling to Mahan's doctors. Prion pathology is

an emerging science. Odd things occur all the time. What

Mahan undeniably had were innumerable holes in her brain,

and sporadic C.J.D. fit the pathology best. So C.J.D. went

on her death certificate.

 

 

Skarbek did not concern herself at the time with the

complexities of her friend's diagnosis. What mattered was

that her friend died. Pat Hammond, Janet's mother, who

hired Mahan at the Garden State Race Track in Cherry Hill,

N.J., had also been close to her. Mother and daughter

absorbed the bad news together. ''We were told it was

C.J.D.,'' Skarbek said. ''You know, the human form of the

mad cow but not related to eating mad cow.'' Sporadic

C.J.D. affects about one person in a million in the United

States, where there are about 250 to 300 cases a year.

Mahan had had terrible luck and nothing more. Skarbek was

an accountant; she knew how to look at numbers without

emotion. She could accept that and move on. ''We were told

she was just one of those who got it,'' she said. ''It was

a tragedy.''

 

 

Three years passed. Skarbek published her book. She gave

seminars for women in corporate life. She drove her two

kids in the family minivan to Girl Scout meetings and

karate class. ''Plan!'' she urged everyone. One day, in

June 2003, she happened to be reading The Burlington County

Times, a local newspaper. She scanned the obituaries. ''I

was just looking in the newspaper for Cinnaminson residents

because I live in Cinnaminson,'' she said. She came across

one for a woman named Carol Olive. ''The first paragraph

said she died of C.J.D.,'' Skarbek remembered. ''I'm

thinking, Oh my gosh, that's what Carrie died of. The

second paragraph said she worked at the Garden State Race

Track. That's when I almost fell over.'' She asked her

mother if she remembered Olive from the racetrack. She did:

Olive had worked as a media representative, an

administrative employee, alongside her and Mahan. Could

their deaths have been a result of mad cow, rather than of

chance? The staff had been small. ''Suddenly we had two

victims out of a hundred administrative employees,''

Skarbek said.

 

 

Skarbek couldn't sleep the night after she discovered

Olive's death. The next day she went immediately into her

home office and searched for victims of C.J.D. in

Lexis-Nexis, the online database that includes newspaper

and magazine articles. She came upon an obituary for John

Weber, who lived in Pennsauken, a neighboring town, and

died of C.J.D. in 2000. She decided to call his family,

even though she said she knew they'd think she was

''nuts.'' ''This way I'd just put it away,'' she explained.

''I'd make the call, and they'd say there was no relation

to the track, and I'd forget about it.'' Weber's brother

William answered the phone. He said his brother had a

season pass to the Garden State Race Track and ''ate there

at least once a week.''

 

 

Skarbek dropped the phone. Weber was her ''eureka moment,''

she said. ''To me, it was just sending up red flags

everywhere.''

 

 

 

There is a lot of anxiety over mad cow disease in America

right now. The fear has its origins in 1987, when British

media began to report that for two years something strange

had been happening on British farms: dairy cows repeatedly

falling over, returning to their feet and charging. After

finding the first sick cow, British veterinarians quickly

found a lot of others. The daily papers could hardly keep

pace with the rising toll. (It is now at more than 180,000

and has involved cows from nearly 36,000 farms.)

Photographs of pyres of burning British cows appeared on

front pages across the world.

 

 

The disease was eventually traced to infected feed. To keep

the cows growing fast, farmers had put rendered animal

parts into their food. Some of the animal parts were

infected with prions. It had occurred to people that mad

cows might in turn infect humans -- humans do eat a lot of

cows. But public officials in Britain counseled calm.

British beef is safe, they said again and again. In 1990,

John Gummer, the minister of agriculture, coaxed his

4-year-old daughter, Cordelia, into eating a hamburger on

television.

 

 

The assurances were made in vain. Mad cow in England had a

second act. The first human to die was Stephen Churchill, a

19-year-old who fell victim to mad cow disease in 1994 and

died in May the next year. He was followed by others. In

March 1996, European officials banned British beef exports.

As of February, 156 cases of the disease, formally called

variant C.J.D., have been identified.

 

 

The epidemic seemed unlikely to strike in America. The

United States imported little British feed or beef. And the

United States Department of Agriculture tracked the 300 or

so British cattle that had been imported for breeding, and

though it eventually lost track of half of them, they were

past the usual age of onset of the disease. The U.S.D.A.

declared America's meat safe. But food safety is an

emotional issue. Many American consumers assume that

whatever England gets, we will get worse. In England there

are farms around every turn in the road. They look idyllic.

They can be watched. In America farms are isolated in vast

tracts of the Midwest and West. The logic of the market

compels American ranchers to fatten their cattle with the

help of hormones. Yet no cases of mad cow were reported

here.

 

 

In the years after mad cow struck in humans in Britain, the

U.S.D.A. changed some of its regulations. Beginning in

1993, it started testing ''downers,'' or cattle that are

too sick or injured to walk. But it did not test very many

of the approximately 35 million cattle slaughtered each

year in this country. The 40,000 or so downers that were

tested over the last decade all tested negative -- until

this December, when a cow in Washington State tested

positive.

 

Much to the relief of the U.S.D.A., the cow

turned out to be from a Canadian herd; it probably ate

infected feed in Alberta when it was a calf. But this

discovery has not entirely reassured food-safety experts,

let alone the public. Anxiety returned earlier this month,

when the inspector general of the Agriculture Department

announced a criminal investigation to determine if

documents from the slaughterhouse in Washington were

falsified to cover up the fact that the cow was not a

downer and had been tested only by accident. If the

diseased cow was walking, the entire logic of the testing

system would unravel. And without a sound testing system,

who could say with confidence that mad cow was not here in

America?

 

 

Many public health advocates and some scientists say they

suspect that mad cow does in fact exist in America.

According to an international panel that reported in

February to the U.S.D.A., it is ''probable that other

infected animals have been imported from Canada and

possibly also from Europe.''

 

The panel added that since the

animals haven't been detected, their infected tissue has

probably been rendered and fed to other animals, ''so that

cattle in the U.S.A. have also been indigenously

infected.'' And humans? ''I hope that there are no

undiagnosed cases of variant C.J.D.,'' said Gambetti of

Case Western Reserve. ''However, my hopes are

meaningless.'' He wants more testing and surveillance.

Peter Crino, the neurologist who treated Carrie Mahan,

said: ''I've got to think there's something more to the

story of sporadic C.J.D. We're getting past the lightning

strikes idea'' -- the idea that prion diseases just happen.

 

 

 

Some organic food and vegetarian Web sites track and

publicize suspicious-looking aggregations of cases of

C.J.D., like a cluster of cases in Lehigh, Pa., in the late

80's, or a more recent group in Washington State reported

to involve 35 deaths. And they never miss a chance to point

out that the U.S.D.A., like the Ministry of Agriculture,

Fisheries and Food, the agency that botched mad cow in

England, is charged with a conflicting mission: protecting

both the food supply and the business interests of food

suppliers.

 

Working on a book on prion diseases, I often run

into people who tell me, ''My mother died of mad cow'' or

''I have a friend whose aunt died of mad cow.'' In such

cases, it turns out that sporadic C.J.D. was diagnosed in

the relative in question but that the family remains

skeptical. The victim ate a lot of meat or spent a week in

England in the 80's -- and the death was so awful. Always

the awfulness of the death comes up. It's something many

families can never let go of. It fuels the debate and the

fear.

 

 

 

Until June 2003, Skarbek was not suspicious of meat. She

loved steak, and her family often ate at McDonald's. But

after finding the three deaths of people associated with

the Garden State Race Track, she became more careful. She

started feeding her family organic meat. She also contacted

public health officials about her discovery. She called the

Centers for Disease Control and Prevention in Atlanta and

the New Jersey Department of Health and Senior Services and

told them what she had found. ''You have to understand that

there's only a thousand season-pass holders,'' she told

them. ''And there's a hundred administrative employees. So

at this time, out of the 1,100 who ate there, the most

often we should have seen one sporadic C.J.D. case was

every 909 years. Here we've got three!''

 

She sent the same

information by e-mail and fax. She had testified about

sales taxes before the House Ways and Means Committee and

spoken about women and the work force to a Washington

audience of 6,000, but that did not mean health experts

would take her seriously. The New Jersey Department of

Health, citing Gambetti's findings, responded that Mahan

hadn't died of C.J.D. In the other two cases, it said it

was waiting for more information. ''They all blew me off,''

she said.

 

 

But Skarbek was undeterred. She continued combing through

Lexis-Nexis, looking for C.J.D. in the local obituaries.

She kept finding people. In 1997, Jack Schott, a

59-year-old dispatcher for trucking companies, died of

C.J.D. So did a jazz musician named Kenneth Shepherd, in

2003, and in the same year, a 71-year-old man named John

LaPaglia Sr. Skarbek began to get tips from friends and

families and found two more victims: Walter Z., an I.R.S.

accountant, and Alfred P., both of whom died in 1997. (In

these two cases, for privacy reasons, the families did not

release their full names to the press.)

 

 

What excited Skarbek most was that all the victims had

eaten at the Garden State Race Track: Alfred P. dined there

with a New Jersey congressman, Robert Andrews. Both Walter

Z. and John LaPaglia had been season-pass holders.

Shepherd's wife remembered him eating at the track. Jack

Schott went in the early 90's. ''He had the beef,'' Skarbek

said. ''His wife had the fish.'' Carol Olive, her sister

said, was ''a lover of hamburgers.''

 

 

Skarbek kept the C.D.C. and the New Jersey Department of

Health up to date on her discoveries, despite what she saw

as their lack of encouragement. She wrote them in January:

''The cluster of people that ate at the racetrack and then

developed C.J.D. is now at eight victims.'' She expressed

frustration at not being heard. ''My concern is that the

New Jersey Department of Health is not taking this cluster

seriously,'' she wrote. She explained that a quick look at

a map revealed that each of four victims lived in a town

that bordered on Cherry Hill, the location of the

racetrack. (In 2001, the track was closed.)

 

She looked up

the population of these towns: Cherry Hill had 70,000

people, Cinnaminson 14,583, Merchantville 3,801, Pennsauken

35,737 -- for a total of 124,121. Since the natural

occurrence of sporadic C.J.D. was supposed to be one person

in a million, a population this size should be expected to

see a case only once every eight years, she calculated.

''Yet,'' she pointed out, ''we had four people die of

C.J.D. in just over three years. The idea that it was just

sporadic C.J.D. and could be easily dismissed as just

occurring spontaneously in nature doesn't ring true.''

 

 

Skarbek's case may have seemed compelling, but it had one

obvious weakness. She was trying to prove that the cluster

of victims associated with the racetrack died of variant

C.J.D., the human form of mad cow disease -- not sporadic

C.J.D. But in humans these two diseases look very

different, even though both are caused by prions. The

sporadic type tends to produce holes in the brain. The

variant type is typically characterized by a daisylike

formation of thick deposits of prions in the brain, as well

as by holes. James Ironside, director of the National

C.J.D. Surveillance Unit in Britain, who saw some of the

earliest cases of mad cow in humans in England, said he was

''overwhelmed'' by the difference between the two forms.

 

 

Skarbek did not know how to surmount this objection. But

she was a go-getter. She wasn't about to give up on her

cluster so easily. Fortunately, she was in contact with

Terry Singeltary. She had seen his name quoted often on the

Web in articles on C.J.D. and mad cow. Singeltary lost his

mother to an extremely rare strain of sporadic C.J.D. in

1997. Soon after, he learned that a year earlier to the

day, the mother of his next-door neighbor died of the

disease. Since that time, he has become convinced that

these sporadic cases are not sporadic at all, that mad cow

is now a disease of humans in America. He said he believes

that his mother was accidentally infected during surgery

and the mother of his neighbor from taking nutritional

supplements made from high-risk bovine tissue, which he

calls ''mad cow in a pill.''

 

 

Singeltary has a sloping face and slicked-back hair. He is

nearsighted, with small blue eyes. He looks like Lewis

Carroll's White Rabbit. From his living room in Bacliff,

Tex., he dominates the listservs and message boards of an

online debate over sporadic C.J.D. -- the scientists who

say it exists; the heartbroken family members who doubt it.

Early, deep in his grief, he would sign his e-mail messages

to scientists, ''I am the madson of a deadmom who died of

madcow.''

 

Singeltary turned out to be helpful for Skarbek.

He pointed her to a paper that was published in 2002 in the

journal of the European Molecular Biology Organization by

John Collinge, the premier prion researcher in England.

Collinge argued that experiments conducted in mice suggest

that infections with mad cow can sometimes look like

sporadic C.J.D. Collinge accepted the implications: he

recommended that ''serious consideration should be given''

to the idea that some of the more recent sporadic C.J.D.

cases in Europe were in fact related to mad cow disease.

 

 

Skarbek said she thought that the same was true in New

Jersey. It is hard to guess what a disease will look like

based on laboratory experiments in mice -- ''The fact that

you saw it in animals does not necessarily mean that it

happens in humans,'' said Ermias Belay, the coordinator of

C.J.D. surveillance for the Centers for Disease Control and

Prevention -- but Skarbek was convinced.

 

A new strain of

mad cow, she argued, possibly domestic in origin, had been

present in the meat served to people who had gone to the

Garden State Race Track. ''Everyone ate at the track

between 1988 and 1992,'' she said. ''I think the mad cow

was probably served maybe during a one-week period during

those years is what I'm suspecting. As we get more people

who ate at the track and died of this, I think we'll be

able to narrow it down maybe to the exact month.''

 

 

Is it possible that meat from an infected cow got to the

Garden State Race Track in the late 80's? There are a lot

of assumptions built into Skarbek's theory. There has to be

a strain of mad cow disease in America that is rare enough

or different enough to have avoided detection. (Belay of

the C.D.C. said he thought that if there were a new variant

of mad cow, it would have already shown up in England,

where there has been far greater exposure.)

 

That strain has

to infect humans in a way that mimics the symptoms of

sporadic C.J.D. but also differs from the well-documented

symptoms of variant C.J.D. in Britain. And it had to be

present in no more than a few cows whose meat was consumed

by only a small group of people who ate at a single venue

over a short period of time more than a decade ago. It does

not help Skarbek's theory that during those years the

racetrack food was provided by more than two dozen

suppliers.

 

 

There is also the matter of the cluster itself. Is it

really what it appears to be? Skarbek is not trained in

this line of work. She effectively made up the rules of her

investigation as she went along. Originally she was

interested in victims who ate at the track often.

Eventually she was interested in victims who ate there even

once. This increased her pool considerably. Attendance at

the racetrack from 1988 to 1992 was at least four million

people. It is true that eight cases of sporadic C.J.D.

would still be a lot in a group this size, but even that is

not necessarily meaningful.

 

 

Averages are tricky. You can

assume that a chance occurrence like sporadic C.J.D. will

happen at a steady rate over vast periods of time and among

large populations. For instance, the overall number of

cases of C.J.D. in the country has held steady for more

than 20 years. But as you focus in on a smaller part of the

picture, local aberrations are common -- even likely. Flip

a coin a hundred times and you should expect heads and

tails to come up about even. But during those hundred flips

you are likely to see a long run of all heads or all tails.

If that's the only time you happen to be paying attention

-- or if you happen to live near Cherry Hill, N.J. -- you

may well think something strange is going on.

 

 

Peter Crino, Carrie Mahan's neurologist, said he admired

Skarbek's effort but had reservations about her technique.

''She's not an epidemiologist,'' he said. ''She's not

terribly sophisticated.''

 

 

 

One day in February, Skarbek traveled down to Washington to

meet with aides to senators and congressmen, including

those of Senator Frank Lautenberg of New Jersey and Senator

Edward Kennedy of Massachusetts. She took documents that

testified to what she saw as the failure to protect the

public from mad cow. She had made poster-board versions of

them at Kinko's.

 

One quoted from an internal memo from the

U.S.D.A. on how to avoid a public-relations debacle of the

sort Britain had experienced over mad cow. Another

excerpted a General Accounting Office report from 2002

noting that mad cow disease ''may be silently incubating

somewhere in the United States'' as well as the

international panel report to the U.S.D.A. from earlier

this year warning that if that were true, those cattle

would have been slaughtered, rendered into feed pellets and

fed to other cattle, amplifying the infection.

 

 

Perhaps reluctantly, the New Jersey Department of Health

has continued to look into Skarbek's concerns. The C.D.C.

is advising the agency as it gathers the victims' autopsy

results and medical and laboratory data on them. ''I'm not

calling it a cluster,'' said Eddy Bresnitz, the chief

epidemiologist for the state. ''The individual in South

Jersey is calling it a cluster.''

 

 

''They are investigating trying to disprove it,'' Skarbek

insisted. She is past being mollified, anyway. Recently,

after she was bounced from a television talk show (for more

urgent reports on Rush Limbaugh and Michael Jackson), she

sent an e-mail message to a C.J.D. listserv, saying, ''I

believe the national beef lobby put pressure on them, just

like they have in the past.'' She has filed so many Freedom

of Information Act requests that a C.D.C. doctor told her

she's known among his colleagues as ''the witch.'' (A

spokesman for the C.D.C. denied this, calling its contact

with her ''quite cordial.'') She recognizes the

transformation that her worldview has undergone. ''Before

June and July, when I started looking into this,'' she

said, ''I would have never believed these things went on.

Maybe I was just naive to it, but I never knew.''

 

 

''Planning Your Future'' takes a dim view of impulsive job

changes. ''Do your research ahead of time, so you aren't

regretting your career decisions later on,'' it counsels.

But after Skarbek became alarmed by the Cherry Hill

cluster, she closed the Professional Women's Institute.

When the infected cow was found in Washington State in

December, she suspended her accounting practice to devote

herself full time to the cluster. ''It's not that I think

I'm special,'' she said. ''I just feel that God was getting

me ready to do this: the book, speaking in front of groups,

speaking to Congress. I was picked to do this. And when

it's done, I'm going to go back to my job.''

 

 

Last month she found two more possible C.J.D. victims who

ate at the track -- one a longtime employee at The

Philadelphia Inquirer, the other a contracts manager who

was 72 and a season-pass holder. She continues to pass the

information on to Bresnitz at the New Jersey Department of

Health. He remains skeptical. ''Even if it turned out that

all the victims ate hamburger at the same time at the

racetrack, now what?'' he asked. ''What do I do with that

information?'' Tracing the meat further back than the

racetrack would be almost impossible at this point.

 

 

Just over a week ago, Skarbek found yet another victim, a

woman named Jodi Tharp, who died of C.J.D. in 2001 and had

owned a horse that raced at the track. She had eaten there,

too, according to her husband. ''It fills in a missing

hole,'' Skarbek said. ''We had 1997, 2000, 2003 and 2004.

Now we have 2001.'' Bresnitz responded that Skarbek's case

still wasn't good science. Without ''a reasonable

hypothesis,'' he said, any investigation is a ''dead end.''

 

 

 

Skarbek has a different ending in mind. She sees herself

unraveling a conspiracy of government and business

interests to hide an epidemic. The idea pleases her. She

recited a line from ''The Pelican Brief,'' the movie about

a female law student who uncovers a government conspiracy:

''So you're the little lady who started this great

brouhaha?''

 

 

 

 

 

D.T. Max is a frequent contributor to the magazine. He is

at work on ''The Dark Eye,'' a cultural and scientific

history of mad cow and other prion diseases, to be

published next year by Random House.

 

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