HIDDEN VALLEY LAKE, Calif. – It was 3 in the morning on a Pennsylvania highway when Charlie Breidenfield saw the flashing red light in his rear view mirror.
So he eased up on the accelerator and backed off the Ferrari’s speed by 40 miles per hour to 100 miles per hour.
Then he stopped and engaged the state police officer in a chat about the virtues of the car before receiving a ticket.
Breidenfield had lead-footed exotic European cars at such speeds before because (1) he wanted to test-drive them before he and his younger brother Bill, who were partners in a business, sold them and (2) he didn’t give a damn.
Why should he? Sometime in the future he knew that he would succumb to a condition in which he could not speak, swallow or even breathe.
“I had no fear. That’s why I drove like a lunatic,” Breidenfield said. “I’d do anything. I tried everything, just raised hell. I never worried about tomorrow because I figured sooner or later I’m going to get Lou Gehrig’s disease.”
This was a more-than-reasonable assumption for Breidenfield. As certain as sundown.
That is the cold reality for Lou Gehrig's disease – amyotrophic lateral sclerosis, or ALS – which currently is the beneficiary of a fundraising program sweeping the nation that involves pouring a bucket of ice and water over someone’s head.
A study released earlier this year by the Centers for Disease Control and Prevention estimated that, while deadly, Lou Gehrig's disease still remains rare in the general population. For the period from Oct. 19, 2010, to Dec. 31, 2011, there were approximately 12,187 people in the United States identified by the National ALS Registry as has having the disease.
However, for Breidenfield and his family, the disease has appeared with frightening frequency.
The reality for Breidenfield and his family is a chart created in 1993 to track its genealogy and deaths resulting from the ALS that Breidenfield and the rest of his family have come to know as “the family disease.” For a good and substantial reason.
“The people’s names in black are the ones in our family who died from it,” said Breidenfield as he unfolds his chart. “There are 111 that we know of.”
Or, about one out of every three names on the chart.
Generations on the chart, Breidenfield said, date back to the 1860s when his great-great-grandmother, Anna Herman, came to America from Berlin and married into a family named Reich.
It is believed that Anna Herman Reich introduced the condition – then known as Pott's disease-- to the first American generation of Breidenfield's ancestors.
“Doctors will say it’s 50-50 (fatal), but doctors are lousy on odds. The majority that I found – like in my father’s generation – died from it,” said Breidenfield. “During my grandfather’s generation there was only one who didn’t die from it.”
Devastating a family
For the past half-century ALS has been called “Lou Gehrig’s disease,” named for Babe Ruth’s iron-man teammate who was an ALS victim in 1941, two years after he played his final game for the New York Yankees.
In Europe ALS is still known as Charcot disease, named for the pioneering and preeminent 19th century French neurologist Jean-Martin Charcot.
By whatever name, it is caused by a dominant – and for the most part dooming – gene, a genetic time bomb that makes people prisoners in their own bodies and kills them in as little as two years.
“You only need one person with a gene mutation to spread it to the next generation,” said Breidenfield.
For that reason, he and Donna, his wife of 24 years, elected not to have children. The two have lived in California – most recently in Hidden Valley Lake – for the past 11 years after coming here from Michigan.
Within the past couple of years, Breidenfield learned of another branch of the family that he hadn’t realized existed when a cousin called him from Long Island.
She told Breidenfield she was in a wheelchair and a half-dozen members of her branch of the family, including her mother, died from Lou Gehrig’s disease.
Then another cousin – this one living just outside Philadelphia – contacted him to tell him about six of his relatives who were Lou Gehrig's disease victims.
In his immediate family, Breidenfield lost older and younger brothers and his father to ALS. His grandfather died as a result of the disease at age 46, his father was 67 when he died.
Charlie Breidenfield's older brother John would succumb to the disease at age 66. His younger brother Bill, an aspiring actor, was on a ventilator for 12 years before dying at age 42. Bill’s death and the death of their father, John, were separated only by a few weeks. Both died literally in Charlie Breidenfield’s arms.
Back up a few years to the top of the stairs in the Breidenfield family home in Michigan where Charlie Breidenfield, not 5 five years old, and his brother, John, older by two years, sat and for the first time learned about the debilitating neurological disorder that would take on such importance in their lives.
At the time, 1954, they were listening to their father in a long-distance, late-night phone call with one of their favorite aunts, talking about how another aunt, then suffering from Lou Gehrig’s disease, would not make it through the summer.
“I was devastated,” Breidenfield said.
Groundbreaking research
The most incredible part of Breidenfield’s story is that medical researchers have traced the ALS gene mutation he carries – a marker on the Y chromosome – back through 93 generations. That's 2,000 years on the Common Era calendar.
The worldwide study has established that Breidenfield is a member of the third largest family in which the ALS gene – known scientifically as A4V-SOD1 – has been a dominant factor.
It recently was estimated that the A4V mutation occurred 540 generations – or 12,000 years – ago. Researchers conclude that the mutation was carried by the Asian ancestors of American Indians who reached the Americas via the Bering Strait land bridge.
Even so, ALS is not primarily a familial disease. Researchers say 90 percent of the mortalities caused by the gene are sporadic.
Breidenfield has spent interminable hours and funds in his effort to combat and survive with ALS.
Twice a month he makes contact with neurologist Dr. Teepu Siddique of Northwestern University, who along with Dr. Robert Brown of Harvard discovered the first ALS mutant gene more than 20 years ago.
The major news media – including Tom Brokaw of NBC and CNN – heralded Siddique’s discovery, which Siddique said at the time “is the first time we’ve found a cause and identified what may lead to a to a treatment.”
With a treatment of choice still to be found, Breidenfield has adopted his own system of medication. Every hour on the hour he consumes a variation of compounds that presumably have warded off the disease.
“I’ve come a long distance from where I was,” he said. “Late in 2011 and early 2012 I was in a whole lot of pain. My brother John, who didn’t come back from it, was pretty sure that I was on the same road as he was.”
His brother John died at age 66. Charlie Breidenfield’s 65th birthday is in November.
“We got to the point where we were almost identical in what was happening to us,” Breidenfield said. “He had lost 18 pounds of muscle off his legs and was using a cane and I knew I was real close to needing a cane or not walking.”
Tragic as his brother’s death was, Breidenfield was motivated by it.
“It drove me like mad,” said Breidenfield. “After dinner I would sit down and stay on a computer until I blacked out. Now, I feel blessed that I can do as much as I can.”
In the time since his older brother’s death, Breidenfield has busied himself in an interminable search for medicines that can aid a Lou Gehrig’s victim. He also attempts to contact other victims.
He estimates he has tried or reviewed more than 100 compounds with mixed results. He has written to hundreds of other ALS victims including Hall of Fame and former Oakland A’s pitcher Jim (“Catfish”) Hunter who died, a victim of ALS, in 1999.
Most likely because Breidenfield is not a doctor, indeed not even a licensed practitioner, hardly anyone takes him serious in his effort to share information.
“Nobody responds,” he said sadly, “They’ll just say ‘who the hell’s Charlie Breidenfield?’ Until you see the clip from Tom Brokaw you‘d think I was just another bull-----er like you find on the next bar stool.”
He estimates he’s sent out hundreds of unanswered letters to ALS victims. Some of the victims, he believes, cling to the hope that, “If I change my name it will all go away.”
Universities and drug companies react in a entirely different way to Breidenfield's calls.
“When I call them and identify myself they know right away they’re talking to someone who knows what’s going on,” he said.
Breidenfield, along with his sister Pam, has also spent countless hours tracing his ancestry.
“I don’t know how many times I’ve gone through the family graveyard in Philadelphia,” he said. “I also went to a 200-year-old funeral parlor to find some family members and I’ve spent a lot of time digging up dead bodies – not really digging them up, but finding paperwork regarding relatives who died from Lou Gehrig’s disease.”
Donna Breidenfield has had to watch her husband struggle with the effects of ALS and gamely wage his battle against it.
“He’ll have very good days and very bad days,” she said. “The trouble is when I see him having a bad day I feel I can‘t control anything. I’m just helpless at that point.
“It can be very stressful,” she continued. “Two years ago there was a point where he couldn’t get in or out of the car, he was dropping a lot of things, tripping over things, didn’t do much and had no energy.”
In terms of the battle to find a cure for Lou Gehrig’s disease, Charlie Breidenfield is indeed somebody.
“I asked Dr. Teepu a year ago how many (ALS-) involved family members talk to him like I do. Turns out I’m the only (ALS victim) who works with him and talks with him about what he’s found,” Breidenfield said.
A moment after Breidenfield said that the phone at his Hidden Valley Lake home rang. It was Siddique returning a call.
“How important was my family in finding the gene mutation?” Breidenfield asked him.
“It was very important,” Siddique replied.
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