A common theme emerged: couples become dependent, adults become like permanent children. I learned about adult teething toys. One wife complained that her husband, who was obsessed with putting things in her mouth, could chew the toys in a day. She broke a tooth on a rock, she told us.
Sarcasm took over the conversation when someone mentioned social media. People have admitted that they get jealous when they see photos of “normal” couples on cruise ships and on the golf course. “Can they really be that happy?” someone asked. Many in the group experience an extreme case of “till death do us part.” They are alone but attached.
Today, there is new hope for people with Mr. Karger’s illness. “About a quarter of cases of frontotemporal dementia are genetic,” said Dr. Grossman, director of the Center for Frontotemporal Dementia at the University of Pennsylvania. In frontotemporal dementia, unlike Alzheimer’s disease, we often know which gene causes the disease and which toxic molecule accumulates in the brain. “It allows us to build rational treatments,” said Dr. Grossman.
This summer, the biotech company Alector announced the first results of a drug given to people with frontotemporal dementia caused by mutations in a gene called progranulin.. People with the mutated gene have abnormally low levels of the protein progranulin, but those given the drug saw levels rise into the normal range. Two other trials are underway for people with a different genetic mutation that causes amyotrophic lateral sclerosis (ALS), but can also cause frontotemporal dementia. The three studies appear to validate an idea that has been bubbling up in the broader neurological community for several years: molecularly targeted therapies could be the future of care for people with dementia. Some neurologists question whether dementia should be treated like cancer.
“Because frontotemporal dementia often runs in families, we can get people into a trial before they show symptoms,” Dr. Grossman said. “By sequencing genes from a blood sample, we know which family members are most likely to get the disease. If we can slow the progression of these people, that’s pretty much a cure.
However, the science came too late for Mr. Karger. His symptoms are too severe for ongoing clinical trials and he does not have any of the mutations being studied at this stage. For Ms. Karger, most days feel like a dress rehearsal for widowhood.