The scientific term for the disease that will end Senator Pete Domenici's political career is frontotrmporal lobar degeneration. What it means for Domenici and his family is a very difficult future.
Experts in the disease, which is a rare form of dementia, said Thursday that frontemporal lobar degeneration, or FTLD, eats away at a patient's brain, causes dramatic personality changes, progresses rapidly and ends in a difficult death.
Domenici, in announcing his diagonosis and political plans on Thursday, called the disease "apparantely erratic and unpredictable. It may well be that seven years from now, it will be stable," he said. "On the other hand, it may also be that it will have incapacitated me. That's possible." Progress of the disease varies from person to person, agreed Catherine Pace-Savitsky, executive director of the Association for Frontotemoporal Lobar Dementias, a non-profit based in Philadelphia.
But, she said that, at some point in its course, the disease causes a change in personality. Symptoms commonly include a lack of inhibitions and understanding or others, inappropriate behavior in social situations, the inability to process information and difficulty speaking. "This is a really, really tough disease on families," said Adam Boxer, a neurologist at the University of California, San Francisco, who specializes in dementia and FTLD.
"In the course of the disease," he said, "the person's personality really changes. They are not the person you married or grew up with." Some 250,000 Americans are believed to suffer from FTLD. The disease is not well understood, but researchers believe it is caused by accumulations of proteins on cells in the frontal and temporal lobes of the brain. The brain cells die and the brain shrinks over time.
Those areas of the brain, control personality, emotions, judgement and decision making. "Our frontal lobes are what make us human," said Pace-Savitsky. Pace-Savitsky and Boxer said FTLD patients something use course language, steal, act out sexually, neglect personal hygiene and develop food obsessions. "The person's personality will really change," Boxer said. "If they were kind and warm, they can become cold and mean. They don't retain the core aspects of who they were."
The Association for Frontotemporal Lobar Degeneration offers support for families who try to provide loving care for a family member they often don't recognize. "It's absoluetely devastating to these families," Pace-Savitsky said. "It can be very traumatic."
Like the more common form dementia, Alzheimer's disease, FTLD is untreatable and fatal. The disease progresses more rapidly than Alzheimer's and the average life span after a diagnosis is eight years, Boxer said. Boxer said there is variability in life spans, depending on whether a patient also develops ALS, or Lou Gehrig's disease. Developing ALS usually hastens death, he said.
People can live for as little as two to three years after diagnosis or they can live for 10 to 15 years," he said. A UCSF study found that a patient's age at onset, gender or level of education did not affect survival rates. Domenici's diagnosis is unusual in one regard, Pace-Savitsky said. The average age of diagnosis in 60.
The disease was identified only about 15 years ago...before then patients were thought to have Alzhermier's...and research into treatments in only beginning. Boxer is undertaking the first large clincal trial of FTLD patients, using a drug approved for treating Alzheimer's disease to see if it will have any effect on FTLD.
Pace-Savitsky said she hoped Domenici's diagnosis might bring attention to a little known disease. "We're hoping that some good can come out of this in terms of raising awareness." she said.