ALS: A Community on the Brink

A crowd of people takes the Ice Bucket Challenge in August of 2014.

By Jacqui Neber
John Anderson and his wife, Amanda, sat at a table with me at a restaurant within the Johns Hopkins University Robert Packard Center for ALS Research. It was September 15, 2014. The restaurant was called Grille 601, and according to John, it was the hospital’s better place to eat. He had ordered a veggie burger that he was eating with Amanda’s help. She held the burger up to his chin for him to take bites. He chewed the carrots and broccoli carefully, in small round bites, looking up at the ceiling as he talked.

“A veggie burger is my favorite thing to eat at the hospital,” he said. “I don’t eat meat so my options are limited.”

John had had ALS since January 2011. He had been living with the debilitating disease for almost four years, much longer than doctors typically predict for a case like his. He was chewing his veggie burger slowly, some of the vegetables landing on the napkin Amanda had hooked around his collar. He couldn’t hold the food himself—but he was eating, and that was all that mattered.

ALS, amyotrophic lateral sclerosis, is an elusive disease. It is not the outraged, sinister sick crawl of cancer. It does not demand attention. It does not affect 1 out of 4, or 1 out of 8, or even 1 out of 100. It affects approximately 5-7 out of every 100,000 people. That’s about thirty thousand people in the United States, and not many people know about it. What people also don’t know is that the ALS community—patients, doctors, philanthropists, donors, families—is on the brink.

The brink of what?

According to ALS advocates, the brink of discovery.

Michelle Farr has been around the disease, and the Packard Center, for years. She does not know John Anderson, but the patient and the woman are loosely connected. They both know Milan Karol, a pivotal philanthropy director at the Packard Center. The Andersons’ doctor, Jeff Rothstein, is Farr’s friend (and also a micro-celebrity in the ALS community, an authority on the disease). People connect the Andersons and Farr together, and me, in an ominous but fascinating web—tangled, haunting and somehow glimmering with hope. The Andersons, Farr, Karol, Rothstein, myself, countless others—we are flies caught, searching for a way out, and our stories intersect to trap others in the search for answers, treatments, even a cure.

Farr’s father-in-law died at the hands of a mysterious illness in 2006, and since then she has pursued a tireless, complicated search for the assailant, moonlighting as a volunteer fundraiser for the Packard Center when her work as a CEO of a consulting company is done. She has helped me understand that the history of ALS is more of a non-history: the disease does not have the detailed backstory of cancer or other equally debilitating diseases. Though first identified in 1869, ALS didn’t receive much attention from the medical community until Lou Gehrig was diagnosed with it in 1939. Eventually doctors came to understand that ALS is a neurodegenerative illness, affecting nerve cells in the brain and the spinal cord that tell the body’s muscles how to move. In the decades since, they’ve gained a more sophisticated understanding of the progress of the disease, as it slowly causes patients to lose control of voluntary and involuntary bodily functions. But to this day, still not much is known about why and who it chooses. What the ALS community does know is that the disease is ultimately fatal in every single case.

Perhaps this is why Farr’s hunt for answers has been tireless. Perhaps this is why I found watching John Anderson determinedly eat his veggie burger so riveting.

John Anderson used to be a professional writer and managing editor for a software company. When he got the diagnosis, he decided he wanted to travel.

In 2013, he and his wife went to Italy. But by the fall of 2014, he couldn’t travel anymore—he was confined to his BiPAP machine, a device that helped him breathe. Sleeping with the machine was second nature, he said. But the most difficult thing was that he became immobile once he lay down. He could move his arms and legs from side to side, but couldn’t get back to vertical. His wife had to help him out of bed and into the wheelchair each morning. And she could, but, soon, she knew she wouldn’t be able to, and they would need something new.

Sitting at the table in Grille 601 she wasn’t worried about that. Her husband was still chewing the veggie burger. At this stage in the progression of the disease, they wanted to keep him eating 2,000 calories a day. A thousand came from a feeding tube. A thousand came from her husband’s regular diet.

“Usually it’s cookies, and brownies, and beer,” she said. She was genuinely enthusiastic.

Philanthropy director Karol, also at the table with us, asked if John Anderson drank the beer, or if he had it supplied through the tube.

“I drink the beer,” John Anderson said, smiling, on the verge of a big laugh, like it’s the most natural thing in the world, like he wouldn’t have it any other way.

His mindset had changed over the course of his illness.

“In the first month,” he said, “the hardest struggle wasn’t coming to terms with the diagnosis, it was dealing with the uncertainty. I’m sure you know. No one can predict the course the disease will take.”

Without any idea about how long he would have, he and his wife kicked off their traveling schedule, trying to ensure that they made lasting memories.

To protect their family from the emotionally traumatic news, both John Anderson and his wife made the unusual decision not to tell anyone they knew for a year. The symptoms were barely noticeable at first. Most days, he presented himself as a normal, healthy man. But after a year, family members began to notice.

“I got busted around Christmas time,” John Anderson chuckled like he was caught stealing a cookie from the jar. “My friend noticed something was wrong. He didn’t know what it was, he just knew something wasn’t right.”

Amanda Anderson joked that they blamed the shaking hands on carpel tunnel. Everyone—me, Karol, the couple—laughed. It was a desperate type of laughing because we were sitting in a hospital cafeteria and I was trying to tell the story of a man coping with a disease, a man who was still laughing, even if it was a circumstantial laugh.

Farr also laughed a circumstantial laugh in our many interviews. In her years of working to help solve some of the mysteries surrounding ALS, there have been little waves of hope and discovery, but they have belonged to the larger, murkier pools of misinformation and difficulty. Even calculating the number of people currently living with ALS in the United States is a complicated process.

“The belief is that any point in time there are 30,000 people living with ALS. Every 90 minutes someone is diagnosed with ALS and someone dies from ALS,” Farr said. “If you look at it like a revolving door—every time someone comes in, someone goes out.”

The Center for Disease Control keeps a registry on their website specifically for people diagnosed with ALS. It’s designed to paint a better picture of the different niches ALS might more commonly affect, leading to more innovative research. The registry asks patients many questions that are designed to help researchers understand those niche situations that researchers suspect might be linked to the disease. “Is it about being in the military, or about living next to a lake in New Hampshire? The registry is not a final word on it by any stretch of the imagination. It’s a government [initiative] to get as much information as possible,” Farr clarified.

Because patients are not required to self-report their diagnosis, the registry is not an accurate portrayal of how many people are actually living with ALS in the United States. Despite this challenge, the ALS community is still encouraging its members to engage in conversation. Most families affected by the disease don’t have much except for oral history – but, according to Farr, ALS advocates are hoping that there is value in that oral history.

“You are right in the thick of our current conversations,” Farr told me. “We don’t have answers and we don’t have consensus on the answers that we do have.” But in the last few years, with the help of the database, people have begun to come together and recognize each other’s contributions to the conversation in an organized way.

One family in particular has had a large impact on the community’s search for answers. Steve Gleason is a former NFL New Orleans Saints player, and though his fame might make him an atypical ALS patient, according to Farr, the support he has gotten from his family since his diagnosis has made him a model for many families in the community. “The family is trying to deal with this in the public eye. It’s so inspiring to me that they’re willing to put these stories out there. Steve Gleason gets on Twitter and writes about his incontinence…and it’s funny. He’s bringing to light all of the issues that these families are suffering through and doing it with humor,” Farr said.

Since his diagnosis, Gleason and his family, dubbed “Team Gleason,” have partnered with ALS stakeholders representatives to create the Answer ALS Campaign. Answer ALS is marketed as “A $500 Million Effort to End ALS in our Lifetime” on its Facebook page, which touts 3,215 likes. According to its website, the initiative is the largest coordinated and collaborative effort to end ALS in the history of the disease.

The Answer ALS campaign, like the registry provided by the ALS Association, is an attempt to connect the web of people that this disease impacts. The campaign aims to find more creative ways to approach finding a cure for the disease. They engage in “Venture Altruism,” a method designed to “invent a technology-rights marketplace where investors can bid on the development of patented but undeveloped technologies.” Campaign spearheads also aim to create a funding pool that can be used to aid research and technology development. Perhaps the most radical component of the campaign is its goal of creating The National ALS Awareness Act “to help shape a governance model to influence a structural war on ALS.” Additionally, Answer ALS will collaborate with the ALS Residence Initiative to help patients with ALS find places to live most comfortably and conveniently. These efforts set Answer ALS apart from other disease-fighting crusades. Answer ALS is not an attempt to be huge or all-encompassing, as are the campaigns inspired by other more well-known diseases. Like the people leading it—Steve Gleason, Pat Quinn, Seth Christensen—Answer ALS is humble and hard-hitting in its emotional impact. It is an intense campaign aimed at raising awareness for a disease known by few.

The Answer ALS campaign comes on the heels of the ALS Ice Bucket Challenge, which took the country by storm during the summer of 2014. To complete the challenge, participants had to donate to the ALS Association or risk dumping a bucket of ice water over their heads. The campaign flooded Facebook, Instagram and Twitter with posts by individuals, families, sports teams and even President Barack Obama—all looking to help raise awareness for the cause. With awareness came $113 million in donations, according to the Association.

The ALS community is hoping that that $113 million will help them fund groundbreaking research as well as connect that research to current patients. $113 million could be the amount needed to finally allow the ALS community to jump off the cliff of discovery.

Janet Wells was my Girl Scout troop leader throughout elementary and middle school. She helped shaped my childhood with trust games and deep talks and lots of cookies. Her husband, John Wells, was diagnosed with ALS in March 2012. Though he passed away last November, he survived with ALS for two and half years—longer than most, considering he had the more detrimental form of the disease.

John Wells was diagnosed with sporadic ALS with bulbar onset, a particularly aggressive form of the disease. Though sporadic ALS shares the same symptoms as inherited ALS – the type Farr’s father-in-law had – the sporadic form is more common and is not passed down through generations of the family through genes and mutations. According to the ALS Hope Foundation, approximately 2/3 of people affected by ALS have the sporadic version of the disease. John Wells’ symptoms were made more complicated by the fact that he experienced bulbar onset ALS, which hits patients with symptoms that worsen and multiply at a faster rate. Respiratory failure causes the death of all ALS patients, and those with bulbar onset experience respiratory problems more quickly than other ALS patients. A website called “ALS Fighters” compares bulbar onset ALS to Niagara Falls—“relentless in their assault as they erode the foundation of that which supports them.”

Still for Janet Wells, one of the hardest experiences in the whole ordeal was the moment the doctors gave John Wells’ symptoms a name.

“I thought the diagnosis when we received it was rather cold,” she said. “There wasn’t even a box of tissues in the room.”

The coolness the couple experienced is not unusual for members of the ALS community. Because the disease is relatively unknown, some doctors are underprepared when giving this kind of terminal diagnosis – as well as discussing the nonexistent treatment options. John Wells symptoms progressed very fast, his wife said, and their struggle to find a good support group right away made the process more difficult. As Janet Wells explained, the mysterious, murky circumstances surrounding each ALS diagnosis create a fragmented patient community, where people are unsure of how to reach out and help each other.

However, that is changing.

Across the country, many small-town philanthropic groups have been partnering with the ALS Foundation and the Packard Center to achieve the same goal. In Northport, New York, my high school’s community service program, A Midwinter Night’s Dream (AMND), hosts an annual charity gala in order to fight back against ALS, in memory of a Northport teacher diagnosed with the disease in 2004. Each year several notable celebrities attend the gala and help raise funds for the cause. AMND allocates its funds to different patients, research programs and ALS foundations. The group of students has raised more than $3 million to date. AMND student alumni have also partnered with the Packard Center as part of Team Packard groups that raise funds to fight ALS through different collegiate charity events around the country. AMND has its own philanthropy center on Main Street of my hometown. It is the largest community service effort Northport has ever seen.

Everything is connected.

ALS is not currently recognized as a global disease. It is too small; it affects too few. But, with the help of ALS is rising in both numbers and awareness. People are talking about it. People are learning from it, using tools like registries and foundations and family histories to piece together a devastating puzzle. A cure isn’t what Karol or Farr or any foundation is looking for right now—you can’t find an effect without first knowing the cause. What the ALS community is looking for, maybe even about to fall over the edge of discovery in search of, is where ALS comes from and why it chooses certain people.

After Karol introduced me to the Andersons, he brought me to the Johns Hopkins Brain Science Institute (BSI) to introduce me to Jeff Rothstein—the “famous one,” the Director of both the Packard Center and the BSi, the man who has the most knowledge about ALS in the world.

Rothstein had slightly rumpled salt and pepper hair. He wore a white dress shirt and a navy tie with a crocodile pattern. He founded the Packard Center in 2000. I had a question for him—as a doctor, why go into this field? Why choose to give people a diagnosis they will never recover from?

“Most of us do it for the challenge of the disease. The challenge of trying to find a cure,” Rothstein said. “Do I believe in my lifetime that we’ll find a cure? No. But I want to find a treatment. It’s not depressing. It’s uplifting. You see a family and that gives you hope. Most of us look at life and carve out chunks. You focus on what you think is the appropriate way to look at a terrible disease.”

He looks through the web from a different point of view, seeing how it entangles ordinary and extraordinary people with diagnoses.

Scientists, researchers and doctors at the Packard Center conduct research around the proteins implicated in ALS, relying on research grants to fund their efforts. Since its founding in 2000, the Center has received 138 grants, with several more pending—a grand total of more than $25 million. This sum is part of the pool of money various organizations and groups like the Packard Center will use to fund new research initiatives, social media crusades and patient support programs. Jeff Rothstein, as one of the most knowledgeable men in the world when it comes to ALS, is at the shores of this vast pool. His colleagues have already dipped in to fund their own discoveries, and Rothstein seems to watch with an all-knowing gaze, even if he doesn’t actually know everything.

I met John Anderson and documented his veggie burger adventure in September 2014. In February of 2015, Karol told me he had passed away.

That’s the thing with ALS—one minute someone like John Anderson is eating a veggie burger and smiling about Coke and brownies, and the next he is gone. On that day in the Packard Center’s restaurant, I remember he was wearing a navy blue “Anna Maria Island” shirt with a graphic of a yellow spike-toothed manic fish on the front. He wore black sweatpants, fuzzy socks, no shoes. There were bright rubber bracelets around his wrists. He looked like someone who is experiencing a disease a lot of us don’t know much about; he looked like someone who was eager to tell his story.

As the Andersons turned to leave Grille 601, they left me and Karol awestruck. John Anderson said, “If I can help you capture the story you want to tell, I’d be honored to do so.”

And so I began.

A previous version of this article misidentified Steve Gleason as a former minor league baseball player. He was actually an NFL player, for the New Orleans Saints.