'There is Nothing Fair About ALS'

posted by Dan O'Donnell -

May is ALS Awareness Month and Dan O'Donnell and the ALS Association are raising money to combat this always-deadly disease.  Click here or on the picture below to donate

Life is at its most basic a series of moments: The moment we’re born, the moment we graduate, get married, have a child, retire. There are moments we remember, moments we forget, moments we cherish, and moments for which we spend a lifetime planning.

And then there are moments that come out of nowhere and change our lives forever.

Mine came early in the summer of 2002.  I had just finished my junior year of college and was preparing to take the Law School Admission Test.  Even though school was out for the semester, I was busier than ever taking an LSAT prep course and working full time while getting ready to take a few summer classes.

I had been preparing for that test for weeks.  That test was going to determine where I went to law school; at the time, I thought it was going to be the moment that determined the very trajectory of my life.

Today I can barely remember a thing about it.  But I remember every second of the next night. 

I went out to dinner with my family, ostensibly to celebrate being done with the test.  But as soon as we sat down, I could tell something was off.  Something was wrong.

“Dan,” my mom said haltingly.  “We didn’t want to tell you this before the LSAT because we were worried it would distract you, but Aunt Margaret is very sick.”

Aunt Margaret wasn’t just my aunt: She was my godmother, my confirmation sponsor, and out of all of my aunts and uncles, all my cousins, I was by far the closest to her.

She was my first babysitter as a child, the first person I opened up to when my parents divorced as a teenager, and now, the first loved one I would lose far too early.    

“Dan,” my mom continued. “Aunt Margaret has ALS.”

“ALS stands for Amyotropic Lateral Sclerosis,” said Dr. Allison Ebert, an assistant professor and researcher at the Medical College of Wisconsin.  “Amyotropic means ‘away from the muscle.’ Lateral means ‘away from the center part of your body,’ and sclerorsis means ‘breakdown of the muscle.’ The disease together is when the nerve fibers that originate in your spinal cord that reach your muscle degenerate, pull back from the muscle and leave the muscle without proper support.  That causes them to degenerate, atrophy, and you end up with paralysis and eventual death.”

And death is always the result, because ALS has no known cure and, perhaps even more terrifying, no known cause—striking seemingly at random; from my beloved aunt to Sarah Jazdzyk’s beloved father.

“We were big into running,” she remembered.  We always liked to do races together and that’s always how we bonded so my sisters and I would do the Thanksgiving Day run.  That was our first race together and one of our last races together.  Our last race he knew he was sick, but we didn’t know.” 

Just a few months later, Sarah’s father got the diagnosis her family dreaded.

“It was really hard up until the point where he got his wheelchair and he kind of couldn’t do anything else.  Watching him lose his ability to walk or even stand...”

Her voice trailed off as she fought off tears.

“When you see someone fall who’s not supposed to be falling, it’s very scary. He started losing strength in his legs first and then it went to his upper body, his arms.  Once he couldn’t get up from sitting down, he needed a lot of help getting up and down.  Eventually then it was food, he couldn’t feed himself.  Once that all happened, you kind of knew what was going to be next.”

“The nerve cells in the brain and spinal cord degenerate; they start to die and we don’t really know why,” explained Dr. Ebert.  “When they do that, they retract their connections to certain muscle groups, and so arms, legs, diaphragm, your breathing muscles start to degenerate because they’re not getting the signals from the nerve cells inside your brain and spinal cord to function properly.

“When tissue, including your muscles, don’t get those signals from your brain and spinal cord, they stop working properly and then they start to atrophy.  They get smaller and they get less functional.  The capabilities of those muscles become diminished and their ability to actually work declines, and when it affects the breathing muscles, you end up dying through respiratory failure.”

In a perverse twist, as the disease attacks the body, it generally leaves the brain alone.

“For the most part, your thinking and cognitive capabilities are mostly intact,” Dr. Ebert continued.  “There is some portion of ALS patients that do have cognitive decline as well, but that’s not the majority of patients.

“In many cases, patients can know exactly what’s happening to them, but they have lost their ability to speak or lost their ability to communicate, and so they know what’s happening but can’t do anything about it.”

“This disease is very unfair for the people who get diagnosed,” Jadzyk said.  “It comes out of nowhere and they deserve to live out their lives and spend time with their families.” 

Each year, tens of thousands of families in the United States battle ALS, as roughly 30,000 people at any one time have it and 5,000 each year are diagnosed.

Those numbers, however, may be misleadingly small.  By some estimates, ALS is responsible for five out of every 100,000 deaths in people aged 20 or older, but in many cases the disease is never diagnosed because testing for it is impossible.   

“ALS is a rare disease and there is no definitive test for it so it’s basically what’s left over after you’ve ruled everything else out,” explained Tom Kettler, the President of the ALS Association of Wisconsin’s Board of Directors.  He joined the organization he joined in 2003, when his wife Suzanne was diagnosed.

“It’s an absolutely unfathomable feeling,” he explained.  “It’s something that we never anticipated.  We were in this for life.  We were going to be together.  We did everything together.  We planned together. We worked together.  We had children together.  And this was our life and it was going to continue to be our life until we were told it would not be and that it would end.”

There is no part of the world that is unaffected by ALS; it is a disease that knows no racial, ethnic, or socioeconomic boundaries.  It is most prevalent in people over 60 and generally strikes those between 40 and 70, but it can occur at a much younger age.

Once it does occur, it ravages the body remarkably quickly.

“Unfortunately, the progression of this disease is usually death after onset within two to five years,” said Dr. Ebert.

Just 20 percent of ALS patients live more than five years, while just 10 percent live more than 10 years.  50 percent die within three years of the onset of their first symptoms.

Generally, those early symptoms include muscle cramps and twitches, especially in the hands and feet, loss of motor control in and the use of arms and legs, and slurred or thick speech with difficulty in projecting the voice.

As the disease progresses, it begins to prevent the victim from moving at all, eventually leading to paralysis and an inability to either swallow or breathe.  It is, in other words, a living nightmare.

And my family suddenly had to wake up to it.

Margaret Ann O’Donnell was born in 1947, the third child and first girl in a middle class Irish family of seven that grew up in Milwaukee.  Her younger brother and sisters had trouble pronouncing “Margaret” when they were first learning to talk, so they called her “Sis,” a nickname that stuck into adulthood.

It was fitting, because Margaret was the consummate sis—always keeping a watchful eye over her younger siblings and even her troublemaking older brother; my father, Kevin, her Irish twin born less than a year before her.    

After college, she became a teacher at Divine Savior Holy Angels High School.  When my mom and dad had me, there was no question about who would be the baby’s godmother; it would be Aunt Margaret.

She never married or had children, but she treated her nieces and nephews as if we were her own.

Growing up, we lived near Aunt Margaret, and my brothers and I always wanted her to be our babysitter.  Whether it was playing Go Fish, laughing hysterically while trying in vain to teach her how to play Super Mario Bros, Saturday night mass and then dinner at Fuddruckers’, or our annual trip to the movies on the day after Thanksgiving, she was always there for us.

When I was 15 years old, I worked a summer at my father’s subrogation firm, and he had hired Aunt Margaret to be his  Director of Human Resources.  This meant that she had to brief me on the company’s policies...including on sexual harassment in the workplace.  Naturally, I giggled uncontrollably as she turned beat red and tried her hardest not to laugh while reading from the manual and then showing me a horribly outdated instructional video. 

During lunch hours, she would help me practice for my driver’s test and we would talk about anything and everything.

A few years later, when I had to pick a confirmation sponsor, there was no question about who it would be. I remember my mom asking if I wanted a man to be my sponsor since all of my guy friends would have uncles or older brothers as their sponsors.

No, I told her, I knew I wanted mine to be Aunt Margaret.

As a confirmation gift, she gave me a silver Celtic cross necklace.  I don’t even remember many of the birthday or Christmas gifts I got as a teenager, but I still wear that cross today.

ALS has likely been humanity’s cross to bear for centuries or even millennia, but its identification and classification are relatively recent.

Scottish surgeon and medical researcher Charles Bell, who first detected the difference between sensory and motor nerves in the spinal column and the connection between nerves and the facial paralysis that became known as Bell’s palsy, is generally credited with first describing the symptoms of ALS in 1824.

In 1850, British neurophysiologist Augustus Waller first detected shriveled nerve fibers, which led to a breakthrough by a French researcher named Jean-Marie Charcot.

Often called one of the fathers of modern neurology, Charcot’s pioneering work was so revolutionary that more than 15 different diseases and symptoms are named after him, including Charcot’s Artery, Charcot’s Joint, Charcot-Marie-Tooth Disease, Charcot-Willibrand Syndrome, and Charcot’s Disease, which he termed Amyotrophic Lateral Sclerosis in an 1874 paper.

Five years earlier, Charcot first made the connection between the symptoms of the disease and the underlying neurological problem that caused them, and in 1881 his research was first translated into English.

Outside of medical journals, though, ALS was largely unknown to the world until 1939, when Yankees superstar slugger Lou Gehrig announced that he had the disease.

Nicknamed “The Iron Horse,” Gehrig had played in a record 2,130 consecutive games and was considered one of baseball’s greatest players.  But early in the 1938 season, his play had declined so rapidly that on May 2nd he benched himself for what he said was “the good of the team.”

He never played another game.

On June 19th, 1939, Gehrig’s 36th birthday, doctors at the Mayo Clinic diagnosed him with ALS.  Two days later, he retired from baseball.

The public was stunned.  How could one of the healthiest, most athletic men in the world be so ill?

A few weeks later, on July 4th, the Yankees held a “Lou Gehrig Appreciation Day” ceremony in between games of a doubleheader.  His farewell address to the crowd would echo through history, especially the line “Fans, for the past two weeks you have been reading about the bad break I got. Yet today I consider myself the luckiest man on the face of the earth.”

The Yankees retired Gehrig’s number 4, making him the first baseball player ever to be so honored, and a few months later he became the youngest player ever inducted into the Hall of Fame.

But ALS gave the great ballplayer no special treatment, and on June 2nd, 1941, less than two years after his diagnosis, Lou Gehrig died.

So beloved was he and so shocked was America by the mysterious illness that killed him that ALS became known as “Lou Gehrig’s Disease,” the name by which it is still perhaps better known today.

Research continued throughout the 20th century, and while neurologists and geneticists gained a greater understanding of the genes that may play a role in the disease, its cause remained an enigma and its cure remained elusive.

In 1985, the national ALS Association was established to raise awareness of the disease, raise money to fight it, and provide support and services for victims and their families.  

But there was no chapter in Wisconsin, at least not until a determined woman named Carmen Downes got to work.  

“In December of 1984, my dad was diagnosed with ALS,” Carmen’s son Curt Downes recalled.  “At that time, he was told to go home, take some vitamins and that there was nothing else that really could be done.

“My mom, being the feisty Italian, wouldn’t take no for an answer, wouldn’t just sit still, and wrote to doctors all over the world.  She kept calling the national ALS Association out in California saying ‘We need help here.’  Those cries for help were followed by a suggestion to start a chapter.

“My mom said, ‘Okay, what do I do?’  So she found a couple of people around here that came onboard with her and in the late ‘80s she started the ALS Association Southeast Wisconsin Chapter, that’s what it was called at the time. I did the first fundraiser.  I was in college at the time; I was playing volleyball, so I did a volleyball fundraiser.

“ALS became part of who we were and we were going to fight it as much as we could.” 

Today, the ALS Association of Wisconsin is a thriving non-profit that raises funds through its annual “Chasin’ a Cure” tailgate party at Miller Park, “Stickin’ it to ALS” hockey game, “Walk/Run to Defeat ALS,” and a black tie dinner and silent auction called “An Evening of Hope.”

“The first Evening of Hope took place on the evening that we buried my dad,” Downes remembered.  “We had all arranged that we were going to hang out at the house with my dad so that my mom could go to this.  It was on a Friday night, January 29th, and my dad actually died very suddenly on Tuesday morning so we had his funeral on Thursday evening and Friday morning.

“My mom said, ‘Well I’m not going to go to the Evening of Hope and we just said, ‘Oh yes you are.  I mean, this is your second family, this is your support.’  She was at the support group meetings every month.  She helped run those and it was her way of learning about the disease and also just being there for other patients.”

That, in a nutshell, is the ALS Association’s mission: To be there for patients and their families.  And companies like Scott Bucher’s marketing and advertising agency Traction Factory are right there with them.

“As a business owner and a business owner like many business owners who are approached to be supporting non-profits and to be supporting the cause du jour, I judge them on fairness,” he said.  “There is nothing fair about ALS.

“A person wakes up one day, having done nothing wrong their entire life, walks into a doctor’s office and is told that they’re going to die.  I don’t know how much more unfair life can be.”

In the spring of 2004, I was in my second semester of law school.  I had known this day was coming since Christmas, but now I finally had to accept it.

I drove home from school, and my aunts and uncles flew in from across the country to gather at Aunt Margaret’s bedside.  She didn’t want to be at a hospital; she wanted to be home.

It was quite possibly the longest weekend of my life, and when I had to head back to school on Sunday night, it appeared that I wouldn’t get a chance to say goodbye.

Her caretaker said she had slipped into a coma.  Fighting back tears, I said one last time, “I love you, Aunt Margaret.”

Surprisingly, she opened her eyes and, through, her oxygen mask, answered, “I love you, too, Danny.”

Aunt Margaret died early the next morning. 

Life is a series of moments.  Some are almost unbearably difficult, but others can fill us with almost impossible hope; because sometimes life’s most difficult moments can teach us that there is always hope, which can sometimes come from the most unexpected places—like a viral social media challenge in which people across the globe poured buckets of ice water on their heads.

“The ALS Ice Bucket Challenge was an absolute blessing,” said Kettler.  “There were so many people that opened their hearts and opened their wallets that it generated a tremendous amount of money.”

“It was wonderful to watch,” added Bucher.  “It was magical to see the momentum that was built over time and, frankly, I didn’t think it was going to become what it ultimately became.”

What it became was a global phenomenon, and within just a month of the challenge going viral in early August, 2014, the ALS Association reported that it had received more than $100 million in donations.

Within two years, that led to a major breakthrough in ALS research.

“The Ice Bucket Challenge money had funded a really large group of investigators from both the U.S. and European countries to do a large project together,” explained Dr. Ebert.  “What they identified was a gene that showed mutations in ALS patients that were not in normal, healthy control patients.

“Now this gene had been identified previously, but what was so exciting about this new paper that came out was that it was from a much, much larger group of individuals across the world essentially.  They found that this gene’s mutations were over-represented in this ALS population.

“Because of the Ice Bucket Challenge money, they had access to so many more patient samples on a much larger scale to make it a really in-depth, large collaborative project.”

And that has given hope to ALS patients like John Jaeckel.

“I always felt like I was invincible, like I was never going to die,” he said with a laugh.  “In fact, I used to talk about if I died.  From that moment on, it changed my language from an if to a when.  That moment was the first time that I really got in touch with my own mortality.

“It was pretty bleak for a while.  I was an athlete: I skied the Birkebeiner, I ran marathons, but now all this stuff was going to be taken away from me.”

But the disease could never take away hope, either from John or his wife Deanna.

“Why worry about tomorrow, next year, or six months from now, when right now we can do things?” she asked.  “Let’s do it.  Every single day is all we know for sure.” 

“In the world of ALS, I’m doing way better than most people,” John explained.  “I’ve been diagnosed over six years.  Life expectancy is two to five, so I’m pretty blessed to be here.”

“We have been able to plan things, we still go to Florida in the wintertime, we still do a lot of things, we just do them differently,” added Deanna.  “It’s not impossible, it’s different, and I’m pretty optimistic.”

The Jaeckels are a living example of the power of hope, even in life’s darkest moments, because living with ALS means never knowing how many moments one has left.

So the Jaeckels are making each one count, working with the ALS Association of Wisconsin to raise awareness, raise money, and raise the spirits of those affected by the disease.

And while the funds raised from the Ice Bucket Challenge have gone a long way in helping them, there is still far more work to be done—especially toward finding a cure.

“For one drug to actually become available for public consumption, from start to finish the cost will be between one and two billion—with a ‘b’—dollars,” Kettler said. “So while we’re eternally grateful for the $115 million that we received from the Ice Bucket Challenge and we’re judiciously using that money and leveraging it as much as we can, there’s still a tremendous amount of cost involved in the development of a treatment.”

And that’s why the ALS Association is working harder than ever.

“Every ounce of volunteerism, every minute of time, every dollar contributed to this organization benefits real live people who did nothing more than get up one day and get diagnosed with what is essentially a death sentence,” Bucher said. 

It was for my beloved Aunt Margaret, but not for my hope.  I joined the ALS Association of Wisconsin on the invitation of its executive director, Melanie Roach-Bekos, who happened to be one of Aunt Margaret’s students at Divine Savior Holy Angels High School.

A few months later, my wife gave birth to our third child and first daughter.  On the day she was born, I held her as my wife slept.  As I looked down at her, I caught a reflection of light off of my silver Celtic cross necklace and smiled at my beautiful baby girl.

We named her Grace Margaret.

Holding her in the moments after she was born was a moment I’ll never forget.  It was a moment of joy tinged with a hint of regret that her namesake wasn’t there to see her.

But that’s why we work to fight ALS: In the hope that one day, if not in my lifetime then in Gracie’s, the disease won’t rob any more families of any more moments together.

“I have a lot of hope that this will eventually be solved,” said Kettler.  “We are going to continue to work until we do find it, until we do solve a problem and get that cure.”

“Just get up and do whatever you can every day and make it a good day,” said Deann Jaeckel.  “Don’t hide.  I mean, it’s a disease where I can see where you’d want to, but I think it’s really important, if you are able, to talk and be a part of the cause and the cure.”

“There are folks with ALS out there,” added her husband, John.  “Connect with them, don’t lose hope.  Hope is the only thing that can keep you going I think.” 

Read more: http://newstalk1130.iheart.com/onair/dan-odonnell-37717/there-is-nothing-fair-about-als-15162518/#ixzz4ggiuOXbV

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