November 24, 2002 News-Journal by Andrew Lyons (email@example.com)
SOUTH DAYTONA -- Out of nowhere, Kathlyn Sandlin asked her husband if she could
quit her job at the mall. She wanted to know
if they could live off his income.
No one knew the 53-year-old woman's brain was deteriorating, that her body was wasting away. Sandlin knew she wasn't the same.
Over the next three years, she grew clumsy, confused, frightened. Seizures jerked her body and hallucinations haunted her. On bad days, her mind was so far gone she couldn't recognize her sisters. She even got lost in her own home.
The CT scans, MRIs and spinal taps offered few answers as neurologists made their best guess: perhaps advanced Alzheimer's disease, although Sandlin's condition worsened far too fast. But in the end, family members from South Daytona latched onto the prevailing medical opinion: Creutzfeldt-Jakob disease or CJD, what one medical professional calls "Alzheimer's on fast forward."
"The doctors made it clear this was not going away," said Sandlin's husband, Bob.
A variant of the disease, vCJD, stunned the world after spreading through Europe and killing about 80 people. Scientists concluded it was caused by eating beef tainted by mad cow disease. Both mad cow and Creutzfeldt-Jakob are neurological diseases; chronic wasting disease in deer and elk are also related.
Sandlin ate venison for decades from the deer her husband and his hunting buddies killed in Flagler County.
Her family may never know if venison played a part in her illness.
But across the country, medical experts are trying to determine whether chronic wasting disease has crossed from animals into humans, just as mad cow disease did in Europe. No documented case exists of a person contracting a brain-destroying illness from eating wild animals with chronic wasting disease.
And the only known case of the variant in this country was diagnosed this year in a 22-year-old British woman living in Florida, who is thought to have contracted the disease in England.
Still, some medical professionals fear the variant is already here. The absence of evidence does not mean that evidence is not out there, said Dr. Michael Greger, a general practitioner from Boston considered an expert on mad cow disease.
"When (scientists) say there is no proven link, that is a true statement. But by the time it's proven, it's a little too late," said Greger, who is on a cross-country tour raising awareness of CJD and warning people to avoid venison. "I don't think anyone should be eating venison in North America unless it's been conclusively tested and unfortunately, we don't have conclusive tests."
On Oct. 7, Sandlin, described as a vibrant woman who loved to dance and travel, died at age 56. Neurologists at the Florida Brain Bank at Arnold Palmer Hospital in Orlando are dissecting Sandlin's brain, searching for clues of CJD. They expect to know what killed Sandlin by next month; her family is anxious to hear.
"It happened so fast," said Sandlin's sister, Teri Smith. "She lived in a state of absolute fear."
The classic form of Creutzfeldt-Jakob, which lies dormant in the body for decades, typically strikes between one person -- maybe two -- for every million living in the United States. Other medical professionals say the disease is much more common. One study, conducted by Yale University, found that 13 percent of suspected Alzheimer's patients had actually died of CJD.
Initial signs of CJD and vCJD are blurred vision, followed by a loss of balance, hand or arm tremors and dementia.
In nearly nine of every 10 cases, the cause is unknown. But death is certain, and usually strikes within three to six months once the initial symptoms appear, although one CJD expert said its not unusual for a patient to suffer for three years or longer.
"It's hard to imagine a worse form of death than CJD," Greger said.
The disease killed Betty Ranney in less than three months. In December 1996, Gloria Earl of New Smyrna Beach received a Christmas card and realized her sister-in-law's handwriting looked sloppy, unusually poor. Within a month, Ranney lost the ability to talk, walk and swallow; then her sight and hearing faded. Finally, her mind left her.
"It was a pathetic thing, a terrible thing, to see a person just deteriorate and just waste and not be able to do the things you're supposed to," Earl said.
On Feb. 28, 1997, Ranney died in North Carolina. Her doctor diagnosed her with a rare, relatively unheard of disease: CJD. Her family hasn't a clue how she contracted the illness, although Ranney's brother said she visited Ireland in the late 1970s and could have eaten bad meat.
Doctors in Cleveland will likely be the first to know if and when a variant of CJD reaches the United States.
The National Prion Disease Pathology Surveillance Center, based at Case Western University, investigates 70 percent of all cases in the United States, which amounts to about 240 a year. The center has already investigated more than 700 cases since it opened in 1997.
Dr. Shu Chen, a CJD expert for the center, cautions people not to eat venison from the nine Midwestern states where chronic wasting disease has been detected. Hunters should also avoid eating parts of deer that have been known to carry the disease, including the brain and spinal chord.
Epidemiologists say every state in the United States should declare CJD a reportable disease so the center can investigate every instance of infection, thus increasing their chances of catching a new variant.
Every day, the disease seems to be earning more respect.
The U.S. secretary of health and human services promised earlier this month to spend about half a million dollars to study whether chronic wasting disease can jump from deer to humans. Days later, President Bush signed into law a rare disease act in which doctors would develop new treatments and cures for several obscure and mysterious diseases, including CJD.
In Florida, where deer hunting season is in full swing, state health officials are months away from taking action to make CJD a reportable disease, said state epidemiologist Dr. Steven Wiersma. Meanwhile, state wildlife officials plan to test 500 deer killed by hunters for chronic wasting disease.
As of now, scientific evidence says the disease does not exist in Florida. Still, "we need to be cautious," Wiersma said.
Because as Europe knows, the deadly disease has a tendency of lingering for years, undetected.