Conn. woman had signs of human mad cow !

Submitted by Mr. KIM on 03/29/2004 at 11:45. ( ) 205.188.116.13

Just want to let all of you know what comes across my desk here at our Taxidermy shop and business. Information that I HOPE will inform you and give some information on questionable matters.....KIM


Conn. woman had signs of human mad cow
By Steve Mitchell
United Press International
Published 3/26/2004 3:44 PM


WASHINGTON, March 26 (UPI) -- A Connecticut woman who physicians initially suspected in 2000 of being the nation's first case of human mad cow disease appears to have been overlooked by state and federal health officials, United Press International has learned.

Jodi Tharp, who was 50 when she died in March 2001, seems to have slipped through the cracks of the Centers for Disease Control and Prevention's monitoring program for the disease, and the exact nature of her condition probably will never be known.

Jim Tharp first noticed there was something wrong with his wife in late October 2000.

Jodi called him from a tile store located about five minutes by car from their home in Andover, Conn., saying she didn't know how to get back. Concerned something was seriously wrong, Jim said he would come get her. Jodi declined his offer and said she'd find her way. She finally arrived home 30 minutes later.

That event led to visits with as many as 10 different doctors to figure out what was wrong with Jodi.

On Christmas day, 2000, she was tentatively diagnosed with Creutzfeldt-Jakob disease, an incurable condition that destroys the brain and ultimately causes death. Although doctors could not be certain, they suspected variant CJD, the form of the disease linked to mad cow disease.

Jim told UPI he was never approached by CDC or Connecticut health officials. In addition, a copy of Jodi's death certificate, obtained by UPI, shows an autopsy -- the only way to diagnose the condition conclusively -- was never conducted, despite the fact Jodi's relatively young age put her in the population most likely to have vCJD.

Dr. Tanya Bilchik, of Hartford Hospital, initially suspected that form of the disease based on electroencephalogram readings of Jodi's brain waves.

Although the Connecticut Department of Public Health added CJD to the list of diseases physicians are required to report in 2000 -- a full 10 months before Jodi first developed her condition and more than a year before she died -- it is unclear whether state or federal health authorities were ever notified.

Neither agency will discuss the case and Bilchik declined two requests for comment from UPI.

Bill Gerrish of the Connecticut Department of Public Health in Hartford told UPI that patient confidentiality restrictions prevented him from discussing whether the department investigated Tharp's case. Gerrish did not respond to two separate requests asking whether state health officials looked into any CJD cases in 2001, the year Jodi died.

The CDC's protocol for vCJD surveillance in the United States is to "follow up on cases that come to our attention in those under age 55," agency spokesman Tom Skinner told UPI.

In 1997, the CDC set up the National Prion Disease Pathology Surveillance Center in Cleveland for the specific purpose of autopsying cases like Jodi's that could be vCJD.

Skinner said the agency's surveillance program is based primarily on death certificates.

"Basically, every case of CJD in the U.S. listed on the death certificate as the cause of death -- that will be picked up by our surveillance system," he added.

The surveillance system appears to have overlooked Tharp's case, however. Her death certificate cites CJD as the cause of death.

Asked if the agency investigated any CJD cases in Connecticut in 2001, Skinner responded, "Off the top of my head, I don't believe so."

Skinner declined to clarify why the CDC did not look into Jodi's case, saying, "Due to patient confidentiality, I couldn't provide you with information on an individual case like that."

Jim Tharp said Bilchik told him during the course of his wife's illness that one of the reasons she suspected vCJD was Jodi's age. There have only been 153 cases of vCJD worldwide, with nearly all of these occurring in England and in people younger than 55.

In contrast, spontaneously occurring CJD -- also called sporadic CJD or, simply, CJD -- has not been linked to mad cow disease and it typically strikes people over age 55.

Jim said he still believes his wife suffered from vCJD.

"I definitely think it is variant CJD, especially in light of her age," he said.

Only one vCJD case has ever been detected in the United States, but the CDC suspects the woman -- a 22-year-old Florida resident who was born and raised in the United Kingdom -- contracted the disease while in England.

At the time Jodi Tharp developed her condition, no cases of mad cow disease had been detected among U.S. herds. However, a case was discovered among a Holstein in Washington last December. Also, an international panel of experts commissioned by the U.S. Department of Agriculture said in a report issued in February that infected cows could have been brought in from Europe, suggesting diseased animals might have been circulating among U.S. herds as long ago as 1997 when European cow imports were banned.

Jodi's condition rapidly deteriorated after October. She had difficulty speaking, lost control of her muscle coordination and by the end, the only word she could say was Jim's name. A hospice worker was hired to help care for her at home.

Jim says Bilchik called him on Feb. 10, 2001, requesting permission to conduct an autopsy on Jodi once she died, "because they were so sure this was vCJD."

Five days later, however, an article appeared in the Hartford Courant, in which Bilchik dismissed the possibility of vCJD, saying further tests had ruled it out.

Jim said he was surprised by this because, to his knowledge, the additional tests had never been conducted. He subsequently obtained Jodi's medical records and said they did not show the tests Bilchik mentioned.

In the article, Bilchik said although initial EEGs showed a pattern consistent with vCJD, further recordings showed Jodi's entire brain was involved, which in her view was more consistent with sporadic CJD.

Jim disputes this account and said there were no additional EEG recordings in Jodi's medical records that showed entire brain involvement.

"Furthermore, the part of her brain and which side is involved has nothing to do with whether it's vCJD or regular CJD," Jim added.

According to diagnostic guides from the United Kingdom CJD Surveillance Unit, located at the Western General Hospital in Edinburgh, Scotland -- considered the world's expert source on these diseases -- patients with sporadic CJD will often, but not always, show characteristic EEG patterns. However, the guides noted, vCJD patients also can show EEG abnormalities.

The encephalogram, combined with other tests, can help predict which form of the disease the patient has, but this is not considered conclusive until an autopsy or brain biopsy is performed, the surveillance unit guides said. If an autopsy or biopsy is not conducted, "then one cannot be absolutely sure as to the diagnosis," it added.

Bilchik, who specializes in headaches, declined to comment on Jim Tharp's accusations, but issued a statement via Lee Monroe, director of public relations for the hospital.

"She just says she's got nothing to say about this," Monroe told UPI. "There's nothing she believes would add or subtract from the facts of the case themselves."

On March 26, 2001, Jodi died.

"I had just walked into the room that morning, she looked at me, took a breath and that was it," Jim said.

--

Steve Mitchell is UPI's Medical Correspondent. E-mail sciencemail@upi.com

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