The investigation was conducted because of prodding by Janet Skarbek, a private citizen in Cinnaminson, N.J., who doggedly has collected information on deaths among employees and season-pass holders at the now-defunct Garden State Racetrack in Cherry Hill, N.J. Their death certificates listed the cause of their mortality as Creutzfeldt-Jakob disease.
CJD is an incurable, fatal brain disorder that is thought to occur spontaneously, but a closely related disease, called variant CJD, has been tied to eating meat infected with mad cow disease -- which Skarbek thinks caused the cases tied to the racetrack.
Several members of Congress, including Sen. Frank Lautenberg, D-N.J., and Rep. Rob Andrews, D-N.J., also thought the possible link to mad cow deserved further evaluation and urged the Centers for Disease Control and Prevention in Atlanta, and the New Jersey Department of Health and Senior Services, to investigate.
Officials from both agencies, who insisted there was no increase or cluster of CJD cases in the state, reluctantly agreed to conduct an inquiry. Their final report, which involved reviewing the medical records of 17 cases in six states, is scheduled to be released Friday morning.
"As far as the specific details (of the conclusions), I don't have that information, but just listening to some of our experts on this there are reasons to believe this may not be a major issue," Llelwyn Grant, spokesman for the CDC, which assisted in the investigation, told UPI.
If there were a single case, let alone a cluster of vCJD victims, the CDC would notify local and state health departments as well as the media, Grant said. To date, the agency has not taken that action.
Concerns about vCJD have been heightened since the discovery of a case of mad cow disease in Washington state last December. There have been no confirmed cases of vCJD in the United States, except for a 22-year-old woman in Florida in 2002. The woman was a United Kingdom citizen and was thought to have contracted the disease while in England.
New Jersey officials declined to comment on the report before it was released, but other sources who requested anonymity suggested some of the cases identified by Skarbek as CJD did not bear up under closer scrutiny. The diseases may have been Alzheimer's, dementia or some other brain disorder.
Skarbek told UPI she would not be surprised if New Jersey officials ruled out some of the cases. She noted she only provided health officials with 15 confirmed CJD cases, whereas in other cases she brought to their attention she had made it clear the cause of death had not yet been established.
Skarbek said she plans to release at least one more and perhaps as many as three additional cases Friday.
"I could go up to 20 cases (tied to the racetrack) by the end of the month," she said, noting she still is in the process of confirming several cases that were brought to her attention by family members.
Skarbek also plans to release a report prior to the New Jersey news conference linking the 16 confirmed CJD cases to one specific restaurant at the racetrack. She said only about 15 percent of all the racetrack attendees ate at an upscale restaurant at the track called Phoenix, but according to interviews with family members, all 16 of the confirmed cases ate at the restaurant, she said. Half ate there exclusively.
She said several unnamed epidemiologists have told her "that is pretty conclusive proof" the deaths are related to something at the racetrack.
Dr. Marla Gold, an infectious disease expert and dean of Drexel University's department of health management and policy, told UPI, "It would be surprising if there were an outbreak of CJD linked to a point source, such as a race track."
Gold said the experience in the United Kingdom, where millions of cows may have been infected, but only 146 people came down with vCJD, indicates it is difficult to transmit the disease from cows to people. Nevertheless, Gold lauded Skarbek's efforts and noted that non-scientists previously have uncovered clusters of diseases, such as the parents in Connecticut who helped reveal Lyme disease was infecting children in that state.
Gold, who has not seen the New Jersey report, said she is familiar with other investigations the New Jersey Health Department has conducted. She said she has confidence the agency -- and specifically Eddy Bresnitz, the state epidemiologist who is overseeing the investigation -- will produce a thorough and accurate report.
Skarbek noted, however, that family members of all 15 victims have told her they were not contacted by New Jersey officials.
"They have never been interviewed, never asked about the victims' eating habits, whether they donated blood, nothing," she said.
If officials had interviewed the families, they would have heard the name "Phoenix Restaurant" mentioned over and over, Skarbek said. "So I'm still doing the Department of Health's and the CDC's job for them," she added.
Rep. Andrews, who has not yet been briefed on the report, said previously he would not be assured unless a thorough investigation was conducted, and he told UPI late Thursday he already is dissatisfied with CDC's response.
In a March 29 letter to Andrews obtained by UPI, CDC director Julie Gerberding said the agency had examined four of the 13 New Jersey cases for evidence of a genetic marker found in most vCJD cases and three had tested negative.
"I am going to call on the CDC to look further because they've only looked at four of the 13 cases my office forwarded to them," Andrews said. "As far as the New Jersey Department of Health is concerned, I want to see what their report says first."
Although Gerberding said in the letter that an outbreak of vCJD in the United States is unlikely, she did conclude the CDC needs to enhance its routine surveillance for CJD by increasing the number of patients who undergo brain autopsies -- the only way to diagnose CJD or vCJD definitively.
Steve Mitchell is UPI's Medical Correspondent. E-mail firstname.lastname@example.org