Griffin died of very rare brain disease
Always-fatal disorder progressed rapidly
The first signs that a terrible and incurable brain disease was afflicting former Buffalo Mayor James D. Griffin began in January, his daughter Maureen Tomczak said.
“With my dad, it started more with movement,” she told reporters Wednesday. “Very off balance. Dizziness. Having a hard time walking. At that time, we thought it was an inner ear infection.”
It turned out to be Creutzfeldt-Jakob Disease, an extremely rare, degenerative brain disease estimated to strike one in a million people every year. It is always fatal and progresses rapidly, usually in just a few months.
The family watched helplessly as Griffin — a famously feisty politician known for his tough, combative demeanor — was ravaged by the disease. “It was really hard,” said Tomczak, to watch her father deteriorate. “But it was going so quickly we never really had time to think about how frustrated we were. He was changing so quickly, day to day. Sometimes, hour to hour, his condition would change. We were just trying to keep up with what we were dealing with.”
Griffin succumbed to CJD, as the disease is often referred to, on May 25 at age 78. Tomczak told reporters at a news conference Wednesday afternoon in the Dent Neurologic Institute in Amherst that the family had not disclosed the cause of death until now because they wanted to be 100 percent sure about the diagnosis. Only highly specialized testing, including genetic testing, could confirm that it was CJD.
That confirmation came last month, but the family waited until Wednesday to announce the autopsy results because Nov. 12 was International CJD Awareness Day.
“While my father received wonderful care from many health care providers,” Tomczak said, “we learned firsthand that more awareness is needed. . . . This disease has very deceptive symptoms. It progresses with alarming speed. It is likely that many cases of CJD go undetected. We hope that more awareness will bring better support over time.”
Tomczak also announced that the family had established the Mayor James D. Griffin Fund for CJD Research at Dent’s Neuroscience Research Center.
The family donated $10,000 to start the fund and is encouraging the public’s support.
Doctors present at the news conference said Griffin died of sporadic CJD, the most common form of the disease. About 85 percent of all CJD cases fall into this category.
There is no known cause for sporadic CJD, unlike mad cow disease, which is a contagious, but even rarer, form of the brain disease.
Dr. Laszlo Mechtler, vice president at the Dent Neurologic Institute, who helped treat Griffin during his final days, emphasized that Griffin did not suffer from mad cow disease — as some people had speculated in the days after the mayor’s death.
“This is not mad cow disease,” Mechtler said. Sporadic CJD “is a disease that is not infectious. It’s not environmental. It’s not related to diet, as we know it today.”
As Griffin grew sicker, his family had worried that he could have contracted mad cow disease while on a visit to Ireland. There have been about 150 cases of mad cow disease in England, Mechtler said.
Tomczak said that while her father struggled with his illness, he was never totally lost in dementia. “He knew he was sick,” she recalled. “He said to us at one point: ‘I’m sick.’ ”
Griffin was “in and out,” she said. “There were times when we weren’t quite sure [if he was lucid]. But for the most part, yes.”
They also noticed changes in his patterns. “He fell out of his routine,” she said. “That was another odd thing for us. He’s very much a routine guy. He fell out of that, and that was a sign that something just wasn’t right.”
The family took Griffin to the doctor’s and then to the hospital. “Things started to be ruled out, and he started to continue down that path very quickly. Very quickly.”
Doctors discussed the possibility of CJD but also wondered whether it was Parkinson’s. CJD is often misdiagnosed as Parkinson’s or Alzheimer’s, far more common ailments, experts say.
Griffin eventually was admitted to Father Baker Manor in Orchard Park, where he “began rehab for a good two weeks,” Tomczak said. But his loved ones soon saw that he wasn’t going to improve. “We realized that our goal at that point was to keep him comfortable,” she said.
The family was considering sending Griffin to San Francisco, where an experimental study for a possible treatment was under way.
“But unfortunately, the disease became so progressive at the end that we were unable to do that,” Mechtler said. “Unfortunately, even in that study the results have not been overtly encouraging.”
Mechtler said that while CJD is extremely rare, he has treated three confirmed cases in the last six or seven months. “We have just recently seen a cluster of cases,” he said.
But he was adamant that he did not believe there was any reason for Western New Yorkers to worry.
“Is there a higher incidence of CJD in Western New York? I would say at this point, I cannot say that,” he said. “We are looking into it. We are talking to pathologists who look at the cases here in Western New York. But I cannot and will not make that statement. That’s just not the fact.” Because the disease is so rare, the fact there are two or three here rather than just one is not cause for concern, he said.
He attributed the spike to more aggressive testing and the older population in the area.
Mechtler said that while there is no treatment or cure so far for CJD, strides have been made in early detection. Until only recently, the disease could only be diagnosed after death. Now, specialized MRIs can show early signs of the disease, he said, as they did in Griffin.
“We’re at the level that we can diagnose this disease while the patient is alive,” he said. “The real question is what we do after that.”
Griffin’s family members Wednesday thanked the public for its support over the last six months since the former mayor’s death.
“May God bless all of you for supporting us, for loving our father and for making our community great,” Tomczak said.
To donate to the Mayor James D. Griffin Fund for CJD Research, go to www.dentinstitute.com .
News Medical Reporter Henry Davis contributed to this report. mbecker@buffnews.com
