Jessica Pierce used to have conversations with her big brother, Josh, about her life and career.
Now when she visits 40-year-old Josh in the assisted-living facility where he lives in Vernon Hills, she asks him questions that one might ask a child: What are his favorite foods? What DVDs does he like to watch?
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"Definitely, it's been hard watching him deteriorate," said Jessica, who is 12 years younger than Josh. "Even though I'm the little sister, I feel I'm the caregiver now when I'm with him."
Josh Pierce was diagnosed two years ago with frontotemporal degeneration, a rare form of early onset dementia that can strike people as young as 20. It took 18 frustrating months to find out what was happening to his oldest son, recalled Josh's father, Don Pierce of Aurora.
"He would forget where he was," Don Pierce said. "He was just acting silly."
Until then, Josh Pierce had been a regular guy who enjoyed ballgames, a couple of beers and golf with his buddies, his father said. The unassuming young man had moved to Chicago's Wrigleyville neighborhood with a few fraternity brothers after graduating from Bowling Green University in Ohio. Later, he opened a successful health insurance brokerage company in Libertyville, where he lived with his wife, also named Jessica, and twin daughters, Maddie and Chloe.
But four or five years ago, Josh began acting strangely. His assistant in the insurance company called Don Pierce one day to ask him to talk to the management of the building where Josh leased office space. The management had threatened to evict Josh because of his disruptive behavior. He couldn't be left alone with his twin daughters.
A family member went to sit with Josh in his office every day for three or four months to prevent him from wandering the building. Don Pierce's wife, Josh's stepmother, stayed in Josh's home for a few months to ensure the safety of the children.
Then Don Pierce and his wife took Josh to live with them until his behavior became such that he needed 24-hour care. The family placed him in Autumn Leaves, a memory care facility in Vernon Hills, in January.
"We were turned down in 22 places because of his age," Don Pierce said.
FTD, once known as Pick's disease, strikes earlier than Alzheimer's and more common forms of dementia.
Rather than being primarily a memory disorder, it affects the frontal and temporal lobes of the brain, resulting in a loss of self-control, inappropriate social behavior, lack of empathy for others, deterioration in language and sometimes of motor control.
The disease is marked by an abnormal accumulation of protein in the brain cells. Only in a minority of cases have hereditary factors been found.
Because patients are relatively young, the disease may be misdiagnosed as mental illness or a motor disorder, such as Parkinson's. It also is sometimes mistaken for Alzheimer's.
"My guess is that there are a large number of young people locked up in facilities that have this disease," Don Pierce said. "One of the frustrations is that nobody knows about this disease."
The Pierces contacted specialists in top institutions -- even medical facilities that receive research funding from The Association for Frontotemporal Degeneration -- but they all failed to diagnose what Josh had. Finally, a psychologist at Alexian Brothers Medical Center said she thought it might be FTD and referred the family to Dr. Michael Schrift, head of neuropsychiatry at the University of Illinois Medical Center in Chicago.
Schrift diagnosed Josh, but Pierce said he has yet to meet another person with FTD, and many medical professionals seem unaware of the disease.
"They don't know a darn thing about it," Pierce said. "It's rare."
According to The Association for Frontotemporal Degeneration, the disease affects an estimated 50,000 to 60,000 Americans and represents 10 percent to 20 percent of all dementia cases. Subgroups of FTD exist, but research has not yet sorted all that out.
No treatment exists, although initial clinical trials are under way. Patients may live between two and 20 years, with eight to 10 years being the typical life expectancy. The Pierces don't know just how long Josh has had FTD, but they estimate four to five years.
Josh recognizes family members and is unaware that he has FTD. Because the disease is progressive, family members never know quite what to expect during their visits. During one recent visit, Don Pierce took his son out to lunch, only to look up after receiving a text message to find Josh eating his napkin.
"He's eating anything he can get in his mouth," he said.
His sister, Jessica Pierce, said her brother retains his love of music.
"He can sing every word to The Killers, his favorite band ... He can remember that, but not what happened 20 minutes ago," she said.
Hard on family
The disease has taken its toll not just on Josh, but his family as well. No longer able to work, Josh receives Social Security disability that helps pay for his care. His wife, Jessica, has taken over his business, and she and their twin daughters continue to live in their Libertyville home.
Josh's wife said she has come to terms with what has happened to her husband, but their twins daughters, now 5, are receiving professional help.
Jessica said she found support in reaching out to the wife of a man with FTD who was featured in a New York Times article; Josh's stepmother, Michelle; and a doctor who recognized what she was going through. All of them assured her she would make the right decisions, she said.
"When we got the diagnosis, it was like being hit by a truck," Jessica Pierce said. "It took me a full year. I can talk about it now."
There have been family arguments and hard feelings, Don Pierce said. The owner of a nonmedical home health care company, Pierce said he now understands firsthand the stress caring for a loved one puts on families.
"Everybody processes it differently," said Pierce, who has three daughters and one other son. "It's very, very difficult on families."
Prompted by his wife, Don Pierce has chosen to make it his mission to raise awareness and research dollars for FTD. He founded the Help Josh Find a Cure for FTD foundation three months ago and held the first golf outing/dinner fundraiser Aug. 2 at Ruffled Feathers Golf Club in Lemont.
He thought the event might draw about 60 golfers and bring in $2,000 to $3,000. Instead, 140 golfers turned out and more than 200 people attended the dinner. Pierce estimates that after expenses, the event will clear $15,000 which will go to FTD research. Dr. Schrift was one of the speakers at the event.
"It turned out much better than I anticipated," Pierce said. "To bring awareness is more important (than the dollars) because no one has heard of this disease."