It began with prime rib - juicy, succulent prime rib. Sam Fitz is a connoisseur of prime rib and this stuff tasted awful. He was puzzled and grew more so when he realized everyone else at the family dinner thought it was delicious. Something niggled in the back of his mind. Maybe he was sick, maybe he had a brain tumor.
The truth was even more frightening. His body was still months away from giving up its secret, but a genetic time bomb had detonated, changing life for him and his family forever. There was no brain tumor, but over the next few months Sam began to mumble and slur his words as if there was. His gait became unsteady, his movements jerky. He lost his job as a car salesman when customers complained.
"At my wedding," recalled his daughter, Cheri Fitz Williams, "people thought he was drunk. It was awful. We knew he wasn't, but we didn't know what was wrong." A short time later sophisticated new tests unraveled the mystery, leaving everyone in shock. Sam Fitz, this easy-going guy who was everybody's friend, had Huntington's disease, an inherited, degenerative neurological disease that always is fatal.
By then his younger brother, Jim, was exhibiting symptoms too. While Jim's muscle control was pretty much intact, he had trouble thinking and talking. Tough for a guy getting his life together and going back to school. Thus began a five-year physical and mental downhill slide for the brothers and an emotional roller coaster for their families.
Tears well up in sister Jan Batt's eyes as she watches the deterioration of her brothers. Tears for them, tears for herself as she sees her future unfold. Jim's twin, she also carries the insidious gene, apparently inherited from their late father, Norman. How many family members harbor the destructive gene remains uncertain. All but two have chosen not to be tested, preferring not to know. At least for now.
Until last summer this longtime Bremerton family saw little to celebrate in their future. But last Monday they wheeled 47-year-old Jim into a sterile operating room at Good Samaritan Hospital in Los Angeles for an experimental but promising operation.
Under local anesthetic doctors bored two holes in Jim's head, mapped deterioration points in his brain, then put him under all the way. Then they implanted a mixture of fetal brain cells and bits of a nerve from his foot, shooting it with a needle into the damaged areas of his brain. With luck the cells will grow, slowing or halting the progress of the disease.
On Monday, 57-year-old Sam, the oldest patient to undergo the treatment, was to follow in Jim's footsteps. But for the fifth time since early October, the surgery was delayed. "Each time it's because the fetal tissue isn't good," said Cheri. "Sunday night they canceled the surgery because the fetal tissue tested positive for Hepatitis B. Thank God they're careful and test. But it's frustrating for Mom and Dad." Sam's surgery has been rescheduled to Dec. 2, and the couple flew home Monday to wait.
Sam's wife, Eileen, said there is no long term data to predict how well the surgery works or how long the effects last. It's been tried less than a dozen times on Huntington's patients. "The first patient was operated on only a little more than a year ago," she said from Sam's hospital room. "So far he's doing really well. All we know is that it seems to help and maybe it can hold off (deterioration) until a chemical cure is found."
It's early, but already Jim has shown some improvement, Cheri said, giving the family hope. The chorea (jerky movements) has diminished, and he seems to be improving under intensive physical therapy in Los Angeles. "There was evidence of atrophy in his brain," Cheri said. "But that's the case with all the Huntington's patients."
The surgery, used successfully in Parkinson's patients, is enormously expensive - $43,500 - and since it's experimental, insurance won't pay for it. Kitsap Physicians Service did pay a portion of the $4,000 pre-operative costs. Their mother, Bertha Dinsmore, mortgaged family property to pay for the surgery, but she's in her 80s. It will be up to the family to pay it back. The financial drain comes at a time when Sam's family finances are in terrible shape.
For years he and Eileen, who resigned as Bremerton City Council president after he became ill, have been financially comfortable, operating a succession of restaurants in Bremerton. But as Sam's ability to work or handle money has eroded, it's been harder to keep things on an even keel financially.
Then two years ago the couple's East Bremerton restaurant, Sam and Dan's Bridgetender Restaurant on Lower Wheaton Way, was badly damaged after some heavy rains. The Fitz family blames it on a city contractor's shoddy work; the city denies culpability. The couple since has filed for bankruptcy - and filed claims against the city.
"Mom and Dad always had enough money for anything we kids wanted," explained Cheri. "They were generous with their friends too. If Dad had a buck, his friends had a buck. Now they need help and I've sent letters to their friends, asking for it. It's hard for Mom and Dad, but it has to be done."
Cheri said it's sad to see the changes resulting from the disease. "My parents always have been very independent," she said. "If my mom wanted to buy a car, she didn't discuss it with dad. She just did it. He was the same way. Now he looks to her for decisions."
Remarkably, Sam has maintained a quirky sense of humor. "I'm falling apart," he said, grinning. "I need a little tune up." That's what he calls the operation. His $40,000 tune up. "It's frustrating, very frustrating," he said more seriously over coffee at the restaurant recently. "This financial stuff really bothers me and look at the damage here. But you just take each day as it is and hope for the best."
Sam's involuntary muscle spasms are increasingly obvious, his reflexive swallowing reminiscent of someone gulping air. Telephone conversations are hard to understand because of the slurring. "But you know, I go to work every day (at the restaurant) and I keep track of things. This surgery really looks like it can make a big difference and I'm excited about it. This might be the ticket." The alternative is too bleak to think about.
Sam's world has become smaller as the disease has progressed. He just can't do some things anymore. "I miss driving," he said. "I like to drive and now I can't. So somebody, friends or family, have to take me wherever I have to go. If I could just drive again, that would be great."
Cheri, because she wanted to have children, was tested immediately after her dad's diagnosis and doesn't have the gene. Her brothers, Wes and Dan, have put off being tested.
"It was a hard decision to make," Cheri said. "Do you want to know your fate? For me, I couldn't live with not knowing. I knew I'd wonder every time I stubbed my toe or dropped my keys. And I wanted kids." She and her husband, Shawn, have a 18-month-old son named Quaid, and expect a second child in December.
"Right now Dad can't even pick Quaid up anymore," she said. "My dad was such a great father and I always expected to grow up and see my parents taking care of my kids, playing with them. It breaks my heart." Recently, she said, Quaid was sitting on his grandpa's lap, feeding him grapes. "Dad bit down on Quaid's fingers," Cheri said. "It hurt, of course, and Quaid was crying. But dad didn't realize he was biting Quaid's fingers. Even when he did, he couldn't make his jaws work to let go. We told Quaid grandpa didn't mean to bite him, but it's really sad."
Genetic counseling, now that the they know about the Huntington's gene, could halt the march of the disease in their family. But only one cousin has been tested. She, like Cheri, is negative. One of frightening elements of the disease is that it rarely manifests itself until the victim already has had children, even grandchildren.
The progress of the disease in their family is shrouded in mystery, but they believe it came through Sam's father, Norman Sr., who died a few years ago from cancer. The lineage began locally with Samuel Fitz, who came to Bremerton around the turn of the century. He opened a tailor and uniform shop in the heart of early Bremerton, one of its first merchants. When he married, his wife already had a child, Sam's dad Norman, from a previous marriage.
Not much is known about Norman's mother, other than the fact that the marriage didn't last. She moved to Arizona and died there at the age of 35. "We never could find out much about her or her death," Cheri said. Samuel adopted Norman after her death, and he grew up here. He and Bertha than had four children: Sam, Jim, Jan and Norman Jr., who the family says has not been tested. Among the four siblings, they've had 10 children.
"Dad was exhibiting some strange behavior when he was so sick with cancer," said Jan, a drug counselor who nursed him through his final days. "We just chalked it up to his being sick. But now we realize he probably was exhibiting some signs of the Huntington's. He had these unexplained rages (patients often undergo distinct personality changes) and I see expressions in my brothers' faces that I remember from Dad. Nobody knew."
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