Monday, April 30, 2012

Lou Gehrig’s disease stills man’s voice, but not his spirit, will to live

This article mentions my friend and colleague, Lisa Bruening.  Lisa coordinates the assistive technology program for the Northern Ohio Chapter and is extremely knowledgeable about communication devices and assistive technology for people with ALS.

April 30, 2012, 2:13 a.m. EDT
The Blade
OAK HARBOR, Ohio — Tim Brooks was a boy when his father died young.
That was only the start.

Years later, the Oak Harbor resident lost two brothers within six months; one of electric shock while working, the other, like their father, in an auto accident. More recently, his mother and another brother died of Lou Gehrig’s disease, which causes progressive muscle weakness, paralysis, and ultimately death. It is more formally known as amyotrophic lateral sclerosis, or ALS.
Now Mr. Brooks is living with ALS, which in his case has hindered his speech and swallowing to the point that most nourishment comes through a feeding tube.

Still, he and his wife of nearly 34 years, Andrea, are enjoying life and are trying to help researchers find the abnormal gene causing ALS among relatives that could be present in more — including their two daughters and 2-year-old granddaughter.

“It is hard to correct something if you don’t know what caused it,” Mr. Brooks said through an iPad, a device he uses to communicate.

Such helpfulness, one Northwestern University neurologist who has studied ALS for roughly a quarter century said, is vital to advancing research to find the cause and ultimately a cure for the neurodegenerative disease. The Chicago-area university’s research team recently discovered details about the underlying disease process for all types of ALS patients, including those with familial ALS, said Dr. Teepu Siddique, a neurology professor with the university’s Feinberg School of Medicine.

“We’re very hopeful,” Dr. Siddique said. “For the first time, we’re very hopeful.”

He added: “I’m thankful, very thankful, to Tim’s family for helping us out.”

Commonly named for former New York Yankees baseball player Lou Gehrig, who died of ALS in 1941, the disease stops signals sent by motor neurons in the brain and spinal cord from reaching muscles.

As a result, weakened muscles atrophy and eventually become paralyzed, and most patients die of respiratory failure within three years or so of symptoms showing up.

ALS in Lucas County

Six to eight people per 100,000 live with ALS, according to the ALS Association northern Ohio chapter, which provides support groups, equipment, and other services to northwest Ohio residents.
That means an estimated 30 Lucas County residents are living with ALS, part of roughly 60 people in a nine-county area of northwest Ohio, according to the association.

In about 90 percent of ALS cases, the disease is sporadic and not inherited. The other roughly 10 percent of cases involve familial ALS, caused by a gene abnormality.

A number of gene mutations causing familial ALS have been identified, but the correct one has not been found in Mr. Brooks and his family.

Typically, mental abilities are not affected by ALS, although there are some familial cases linked to dementia. Mr. Brooks’ aunt, his late mother’s twin, 81-year-old Joan Nesteroff of the Chicago area, was diagnosed with ALS more than 20 years ago, and she started showing signs of dementia at about 70, said her daughter, Kathi Boothe, who cares for her mother.

Some patients describe ALS as like being buried in sand because they are unable to move their bodies. The typical case resembles the centuries-old beliefs of French philosopher Rene Descartes, who thought a person’s mind and body were separate, Dr. Siddique noted.

“This is a terrible disease,” he said. “It separates out mind from body. The mind is there, but the body’s not there.”

He added: “It’s sort of like living in a dream world.”

Northwestern researchers discovered that the protein recycling system in the neurons of the spinal cord and brain is broken in ALS patients. As a result, cells cannot repair or maintain themselves.
A lack of funding, however, is delaying work on finding an effective treatment by testing for medications that would regulate the protein pathway, Dr. Siddique said. Northwestern researchers have less than $1 million a year to work with, and they need $10 million to $15 million over the next two to three years to come up with treatment answers, he said.

“Now we’re at a point where we need an infusion of funding and resources,” Dr. Siddique said.
Donating DNA

Relatives of Mr. Brooks have donated DNA to research efforts both at Northwestern and the National Institutes of Health, which is studying ALS genetics.

Research to identify abnormal genes has opened up in just the last few years, said Dr. Erik Pioro, director of Cleveland Clinic’s Section of Amyotrophic Lateral Sclerosis and Related Disorders.
At Cleveland Clinic, research includes comparing neurological tissue from patients before and after their deaths to identify changes on MRIs, Dr. Pioro said. “It’s just really so important that families are willing to donate blood and are willing to participate in these types of research projects.”

Mr. Brooks, who is treated by Dr. Pioro, has agreed to donate his brain and spinal cord to Cleveland Clinic after his death.

His late brother, Gary Brooks of Genoa, made an anatomical donation after succumbing to ALS last year, the Brookses said.

Providing information and genetic material to researchers including Dr. Siddique and helping raise money are the family’s legacy, they said.

“We realize we have a genetic gold mine, not only for our family, but for other families who suffer from ALS,” Mrs. Brooks said.

In tracing Mr. Brooks’ family tree, it looks as if his maternal grandfather, Ben Wunderley, died of ALS at 47.

Mrs. Nesteroff, Mr. Brooks’ aunt, is the first of current generations to be diagnosed with ALS, and a cousin, Toledo native Bob Textor of Iowa, is living with the disease.

All of Mrs. Nesteroff’s siblings donated blood to Dr. Siddique’s research efforts a decade or so ago, said Mrs. Boothe, her daughter.

Whether any more relatives in that branch of the family, including Mrs. Boothe’s four children, has ALS not been determined, Mrs. Boothe said. They know ALS is a possibility, and it makes them appreciate what they have, said the grandmother, who called her mother’s longevity and positive outlook inspiring.

“Of course, we all hope we don’t have it,” Mrs. Boothe said. “I wish my mom had her health.”
She added: “We’re all going to die of something someday.”
Life with ALS

Brooks family members also are continuing to live their lives.

“Our children may have inherited this,” Mrs. Brooks said. “If this befalls them later in life, we want them to look back at us and say, ‘Mom and Dad handled this the best they could.’

Although finding a cure for ALS is the ultimate goal, the Brookses hope research advances enough in coming years so their offspring and other relatives can be tested if they want, they said. At least their daughters knew while in their 20s that ALS was a possibility, about two decades earlier than they did, they said.

In October, family members raised nearly $30,000 for the ALS Association northern Ohio chapter during a Toledo walk, and they plan to continue fund-raising efforts annually. FirstEnergy Corp., from which Mr. Brooks retired, provided half that sum by matching donations the family raised, the Brookses said.

Besides offering a monthly support group in Toledo, the chapter lends patients walkers and other equipment, helps with technology to aid communications, and assists with government advocacy, among other services, said Lisa Bruening, patient services coordinator.

For example, Ms. Bruening recommended Mr. Brooks get an iPad, the couple said.
“I will be 55 in July, and I’ve had a good time over the years,” Mr. Brooks said with the device’s help. “Fifty-five is not bad for a Brooks.”

Every patient has a different experience with ALS. Some start to show symptoms at a young age and die quickly. Others live for years.

The type of ALS Mr. Brooks has initially affected his tongue rather than his limbs, interfering with swallowing and speech.

He is able to use a walker to get around, and ALS symptoms overall have progressed slowly but steadily since he was given the diagnosis in September, 2009.

Symptoms can hit a plateau at any time, a grinning Mr. Brooks said through his iPad.

Despite his condition, the couple are able to spend time in Florida. Mr. Brooks continues to fish and do other activities, but he admits struggling with being unable to do yard work and other chores.
The latest blow for the couple is that their older daughter, Lindsey Borjas, is moving to Japan for three years because her Marine husband, Curt Borjas, is being transferred there from Chicago. Still, it’s a good opportunity for the young family, including 2-year-old Juliana Borjas, and they will use Skype and other technology to communicate, Mrs. Brooks said.

Several years ago, the young couple were stationed in Japan, and the Brookses’ younger daughter, Ashley Brooks, who lives in Florida, was able to do student teaching there. The sisters also traveled, one of many ways they have made the best of life despite the threat of ALS, Mrs. Brooks said.
“You only get one chance sometimes,” Mr. Brooks said with the help if his iPad.

Contact Julie M. McKinnon at:, or 419-724-6087.

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