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Mad cow raises awareness of another mysterious killer

April 8, 2001 NewJersey.com by Bob Groves

Three years have gone by and Patricia Carlin's ashes are still stored in an urn at a Hasbrouck Heights funeral home.

Her children are afraid to bring her remains home. They fear the disease that destroyed their mother's brain somehow withstood cremation and could threaten their lives.

Carlin suffered from Creutzfeldt-Jakob disease, a rare illness with symptoms similar to the mad cow disease sweeping Europe. Like mad cow, the disease riddles the brain with sponge-like holes, swiftly killing its victims.

Patricia Carlin died at age 62 within weeks of falling ill in late 1997. Her family first noticed changes in her personality when she served Thanksgiving dinner in her Lyndhurst home. She said she felt disorganized. She had trouble setting the table.

"I walk into a room and forget what I came for," Carlin told one daughter. "I think I must have had a stroke in my sleep."

In the following days, Carlin accused a daughter of smuggling a kitten into her house and complained that others were talking behind her back. She needed prompting to get bread and sandwich meat to make lunch. The active, working woman suddenly could no longer turn the ignition key in her car.

Doctors at one hospital said Carlin had suffered a stroke. Those at a second diagnosed Creutzfeldt-Jakob.

The disease has gotten global media attention recently because nearly 100 Europeans have died from another form of Creutzfeldt, called "new variant," after eating beef infected with mad cow. Headlines about the 180,000 infected cows and the deaths in Europe have set American nerves on edge, despite extensive U.S. government measures to protect people and animals here.

The United States has had no such mad cow-related cases in humans. But there are instances of people, like Carlin, who contract the so-called general form of CJD by other means. It can be inherited, occur spontaneously, or spread through infected transplant tissue or surgical instruments. There are often no explanations: Patricia Carlin's family doesn't know how she got CJD.

Creutzfeldt-Jakob disease is so rare that patients and their families have long complained about the lack of information on the disease. But now, because of the effort to keep mad cow out of America, scientists are paying more attention to CJD. At a lab in Cleveland, researchers are evaluating the brain tissue of Creutzfeldt victims. If new variant CJD reaches this nation, the Cleveland scientists will likely be the first to know.

Both forms of CJD are fatal, and doctors can do little beyond trying to keep a patient comfortable. The disease attacks the nerve tissue, killing normal cells, causing seizures and progressive dementia.

The disease kills about one person in a million worldwide. About 4,700 people died from the disease in the United States between 1979 and 1998, most of them elderly.

"It's a terrible, horrible disease," said Dr. Damon Fellman, a Hackensack neurologist who has treated several cases. "It has a devastating effect. Dementia and senility develop, combined with twitching in the body. The person is progressively more confused. Many people have mistaken this for a psychological disorder."

The disease left Carlin bedridden. She developed the shakes and shocking episodes of uncontrollable strength, according to her daughters. She raged and tried to bite. Then, she lapsed into silence.

In her last days in a nursing home, their mother could only communicate by raising her eyebrows, "so we knew she was still in there," said a daughter, Siobhan Carrasco, 41, of Bogota. The doctors said she was in no pain. All the children could do was keep their mother's lips moist with teaspoons of water, and wait at her bedside while she dozed in a fetal position.

"It was a horrific death," said Cathy Kundert, 44, of Ridgewood, another of Carlin's six children. "Nobody deserved a death this tragic."

Her children didn't even have the comfort of bringing her ashes home because a doctor told them the remains could be infectious, family members said. At the time of her death, Kundert watched a television documentary that said CJD was virulent enough to be active years after cremated remains were buried.

Scientists say there is no evidence that ashes pose a risk, but the disease is rife with such misunderstandings. Mad cow and Creutzfeldt-Jakob are surrounded by the misinformation and fear that accompanied AIDS when that virus first appeared in this country 20 years ago.

Marie Kassai, a nurse who counsels CJD patients and their families, has heard of doctors who refuse to treat CJD victims and funeral directors scared to embalm them. Physicians say that often when they try to explain the disease, families leave thinking their loved one has mad cow.

"There's a lot of stigma attached right now. We need to look at this logically, rather than with a lot of fear," said Kassai of Elmwood Park, whose mother died of CJD 10 years ago at age 72.

When her mother died, doctors could tell her little about this disease. When her father died in 1995 with signs of dementia, Kassai asked for an autopsy. The examination found Alzheimer's. "We just wanted to make sure he didn't have CJD for our own peace of mind," she said.

Such anxiety is inevitable -- and growing -- as mad cow and new variant CJD continue to be a scourge in Europe. Mad cow, formally called bovine spongiform encephalopathy or BSE, first appeared in Britain in the mid-1980s. Cattle are believed to have contracted it by eating feed made from sheep with a similar disease.

Adding to the concern, leading scientists in England now think the disease could have an incubation period of 30 years, and may claim tens of thousands of victims in the coming decades.

As a line of defense, the United States has banned imports of cattle or meat products from England and much of Europe, and prohibits the use of feed made from animals. It has also banned imports of blood products and blood donations from people who have recently lived in Europe.

Those efforts have not calmed a worried public. Mad cow has hit the headlines here in scary stories about everything from European candy made with beef gelatin to whether elk and wild deer in several Western states were infected with a similar virus and a posed a health risk to people who eat venison or take supplements containing ground elk antlers.

Despite the controls, officials have found mad cow on U.S. soil. Four sheep from Belgium recently seized in Vermont tested positive for the disease. Because they had been quarantined, though, there is no fear the disease spread. Meanwhile, Texas health authorities will begin destroying 21 cattle that may have been exposed before they were imported from Germany.

"With every diseased cow they find in Europe, theoretically there is an increased risk to humans from the new variant CJD," said Kassai, 57, manager of infection control at the General Hospital Center at Passaic.

"More cases are popping up in different countries, so people in Europe are getting very nervous," she said. "That can create fear in the rest of the world."

Kassai has made it her "crusade" to combat that fear. She lectures undertakers on the signs and symptoms of CJD and precautions to take. She works with families, helping them find answers and comfort.

Patricia Carlin's children are also speaking out. "I think God wanted us to be a voice" for CJD patients, Kundert said.

Carlin's children still complain about the lack of information they encountered when her mother became ill. Her family got more news from the Internet than from hospitals or doctors.

"I'm bitter. We had absolutely no help. We had to do all the footwork" to learn about the disease, Carrasco said. "I don't know if other people out there getting this disease have the same information."

The family made sure Carlin's death certificate specified CJD as the cause, rather than hide the nature of her illness out of fear or shame, she said. "A lot of people put 'pneumonia' as the cause," Kundert said.

And, while there is no known case of mad cow in humans here, "needless to say, we're all vegetarians" now Carrasco said.

As Carlin's family discovered in their search for answers, doctors find CJD difficult to diagnose. The answer traditionally came in an autopsy. In living patients, the disease can now be determined through electroencephalography -- measuring brain waves -- or a spinal fluid test for CJD, which is about 90 percent accurate, said Fellman, the Hackensack neurologist.

The disease is baffling because it can take anywhere from a few years to decades to appear. The damage to the brain is caused by protein particles in the body -- called prions -- that mysteriously enlarge, change shape, and wreak havoc.

The highly infectious prions are neither viral nor bacterial and do not have the genetic material needed to reproduce. Inexplicably, some of them become destructive, like cells that suddenly turn cancerous.

General CJD runs in some families. Others contract the disease through tissue transplants from someone who had the disease. CJD has also been linked to people who took a growth hormone before the government banned the harvesting of the hormone from human cadavers.

Prions are difficult to destroy by cooking or sterilization, which makes disposable surgical instruments preferable to ensure the disease is not spread. Surgeons have contracted CJD when they nicked themselves during a procedure with an infected patient, Fellman said.

"What makes this disease different is the difficulty of eradicating the prions. That's how come there is the fear," Kassai said.

Fellman believes the risks to morticians is "very small." Recent World Health Organization recommendations on preventing the spread of mad cow and CJD say "cremated remains can be considered to be sterile."

George Kelder, assistant executive director of the New Jersey State Funeral Directors Association, calls the idea of prions surviving in cremated ashes as "paranoia." The organization's handbook instructs members to wear gloves and goggles and use disposable instruments when treating the body of a CJD patient.

Rodger Powell, the funeral director in Hasbrouck Heights who is storing Patricia Carlin's ashes for her family, said he is not afraid of contracting CJD from the remains. "They're on the shelf with 15 others," Powell said.

"If you have the fear, you're going to have a problem," he said. "You're going to make a mistake, nick that glove, or put a needle into your finger. With respect you'll never have that problem -- respect for what you're doing, for the disease, and for the deceased."


For more information about Creutzfeldt-Jakob disease and support groups log on the Web at www.cjdvoice.com, or www.cdc.gov

This article contains material from news service reports. Staff Writer Bob Groves' e-mail address is groves@northjersey.com


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