Celebrity Profiles April/May 2023

By Robert Firpo-Cappiello

Musician John Driskell Hopkins Turned an ALS Diagnosis into a Mission to Help Others

Musician John Driskell Hopkins has turned his diagnosis of amyotrophic lateral sclerosis into a mission to help others, starting a foundation to raise funds for research.

After a diagnosis of ALS, John Driskell Hopkins started Hop on a Cure to fund research. *Photographs by Jolie Loren*

It was in 2019 that John Driskell Hopkins first noticed an odd symptom. “I started having balance issues. I even took a tumble onstage,” says the 52-year-old vocalist, multi-instrumentalist, and founding member of the Zac Brown Band, a Grammy Award–winning country music act. Other symptoms cropped up. “My speech was sometimes slurred, and strumming with my right hand started slowing down,” Hopkins says. “I was finding it harder to perform to the standard I set for myself.”

His doctors initially attributed the symptoms to side effects of the statins he was taking to lower his cholesterol. (The medication's side effects can include weakness and muscle pain.) When going off statins for a few months on his doctors' recommendation didn't make a difference, Hopkins, whose nickname is Hop, tweaked his diet and increased his exercise. Still no relief. Hopkins knew something was seriously wrong, but the advent of the pandemic and suspension of nonessential medical visits put his quest for answers on pause.

Finally, in December 2021, Hopkins underwent an electromyography (EMG)—a diagnostic procedure that helps determine the health of muscles and the motor neurons that control their movements—and received a heartbreaking diagnosis: amyotrophic lateral sclerosis (ALS). “I learned that my motor neurons were progressively deteriorating,” Hopkins says. The world-renowned musician, husband, and father of three was facing a fatal disease he'd only vaguely heard of. (An EMG is one in a range of options—including a neurologic exam, blood and urine tests, spinal tap, and MRI—for testing for ALS.)

The Progression of ALS

A neurodegenerative condition that affects motor neurons in the brain and spinal cord that control voluntary movement, ALS causes weakness and muscle atrophy. As the disease progresses, people lose the use of their hands, arms, and legs and the ability to breathe without a ventilator. Most people die within three to five years of diagnosis, with about 10 percent surviving 10 years or more. In rare cases—such as those of physicist Stephen Hawking and Cathy Wolf, a former writer for Brain & Life and an experimental psychologist who worked at IBM—people can survive and function for decades with the help of ventilators, feeding tubes, and communication devices.

ALS is more prevalent among men than women and is more common in people older than 60, according to the ALS Association. Exact figures are not available, but the U.S. Centers for Disease Control and Prevention have estimated that 31,000 Americans have the disease.

The first two weeks after his diagnosis were “brutal,” Hopkins says. “I had trouble sleeping. I lost weight. I began thinking the disease progression and physical decline were all happening right now.” He was frightened for himself and worried about his wife, Jennifer, and daughters, Grace, now 14, and twins Faith and Hope, 10.

Hopkins gained a new perspective when he traveled from his home in Marietta, GA, to the Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital in Boston. “They explained that my progression was slow and will likely stay slow,” says Hopkins. Proof of that came a year later when another EMG showed little change. “But I do notice that my walking is not as good as it was a year ago,” he says.

So far his other symptoms, including slightly slurred speech and stiffness in his legs, have remained relatively mild. “They're slowly getting a little more apparent,” he says. He's able to maintain a daily yoga practice to ease stiffness, and he keeps his singing voice in good shape with a vaporized saline treatment. “My speech is a little slower,” he says, “and I'm losing control of the flap between my nose and mouth. My singing is more nasal, which makes me angry. Regardless, I'm on tour with the band, and I'll keep playing as long as I can.”

Hopkins performing with the John Driskell Hopkins Band in Nashville in 2022.

Hopkins also makes sure to get plenty of sleep. To try to reduce inflammation, which can exacerbate fatigue and muscle and joint pain, he changed his diet. “I'm gluten-free, dairy-free, and sugar-free—except between Halloween and Thanksgiving,” he says, half in jest.

In addition to making lifestyle changes, Hopkins takes three daily medications—riluzole (Rilutek), edaravone (Radicava), and sodium phenylbutyrate/taurursodiol (Relyvrio)—that have been approved by the U.S. Food and Drug Administration to slow ALS progression. He's been fortunate enough not to experience medication side effects and has not required treatment for other ALS-related symptoms such as spasticity, sleep disorders, and trouble swallowing.

Looking Ahead

Just as powerful as prescription medications and dietary modifications, however, is Hopkins' attitude. “Instead of manifesting disaster, I manifest positive thoughts and a new sense of purpose,” he says. Almost immediately, he wanted to establish a foundation, and he knew he would share his diagnosis with his fans, but his wife was cautious and concerned about their children being in the spotlight. “My argument was that I wanted our daughters to see that we did everything we could to solve this problem,” says Hopkins. “I wanted them to be proud of their mom and dad for hitting the most difficult challenge of their lives head-on. It felt like a responsibility.” Together, the couple agreed to start a foundation.

Hopkins—with his wife, Jennifer, his oldest daughter, Grace, and twins Hope and Faith—says he wants his children to be proud of their parents for facing this difficult challenge head-on.

In May 2022, Hopkins and his bandmates released a video that revealed Hopkins' diagnosis and announced the launch of Hop on a Cure, with a mission to support “research to prevent, reverse, and cure ALS while raising awareness, building a compassionate community, and unleashing the healing power of hope.”

A group of Hop on a Cure supporters at the Braves game.

Since its launch, Hop on a Cure has raised approximately $800,000. The first grant went to the Healey Center in January 2023. The foundation is reviewing additional proposals for promising research.

Although new medications slow progression of ALS, no current treatment can stop or reverse it, and there is no cure for the disease. Furthermore, researchers have not yet discovered the cause of ALS or why some people get it and others do not. About 5 to 10 percent of cases appear to be linked to ALS-related mutations in certain genes, but the rest of the cases seem to occur randomly with few identified risk factors.

“There is a slightly higher incidence of ALS among those who served in the military and athletes who played football or soccer,” says Catherine Lomen-Hoerth, MD, PhD, FAAN, a neurologist and director of the ALS Center at the University of California San Francisco Medical Center. “Trauma and environmental risk factors such as head injury, electrical burns, and exposure to lead have been proposed as potential reasons,” she says.

His actions and positive attitude are inspired by his children, Hopkins says. “My legacy is for my daughters, these three beautiful young women who are with me every day in my home.” He remains hopeful for a cure—and is working hard to make it happen.

Hop on a Cure aims to identify research to slow, stop, and ultimately cure ALS. “I'm not a scientist,” Hopkins says. “But I have a platform, and I'm using it to make a difference.” The Zac Brown Band has connected with millions of fans through its multiplatinum albums and chart-topping tunes like “Chicken Fried,” “Toes,” “Colder Weather,” “Jump Right In,” and “Knee Deep” (featuring Jimmy Buffett).

“I want to sing for as long as I live,” Hopkins says. “And to that end, I just think about that next song.”

With twins Faith and Hope before throwing out the first pitch at the Atlanta Braves Lou Gehrig Day game in 2022.

Treatment Arsenal for ALS

Before the approval of riluzole (Rilutek) in 1995, people diagnosed with amyotrophic lateral sclerosis (ALS) had no way to slow progression of the disease. Now there are two additional medications, edaravone (Radicava) and sodium phenylbutyrate/taurursodiol (Relyvrio), which were approved by the U.S. Food and Drug Administration (FDA) in 2017 and 2022, respectively. “The effect of these two drugs is modest, slowing progression only by a few months, and patients may not notice it,” says Mark B. Bromberg, MD, PhD, FAAN, professor of neurology at the University of Utah in Salt Lake City.

Three edaravone trials in Japan did not initially convince the FDA that the drug was effective, but the agency reconsidered because one of the trials showed a 33 percent slowing of disease progression in a subset of participants.

When it was first available, edaravone was administered as an infusion. In 2022, an oral formulation was approved. A German study published in JAMA Neurology in January 2022 found that progression of the disease and survival rates were no different for people taking edaravone with riluzole than for those receiving only riluzole.

Despite the mixed results shown in studies, many neurologists stand by the newer drug. “Edaravone slows disease progression by working to combat oxidative stress,” says Catherine Lomen-Hoerth, MD, PhD, FAAN, director of the ALS Center at the University of California San Francisco Medical Center, who believes it is most beneficial for people in the early stages of ALS.

Relyvrio is a combination of two medications—sodium phenylbutyrate and taurursodiol. Sold as a powder that gets dissolved in water for ingestion, the drug blocks stress signals in cells to prevent cell degeneration and death. The approval of Relyvrio also was controversial: An initial study demonstrated that it slowed ALS progression by 25 percent, extending life by a few months. Based on this study alone, the FDA initially declined approval, but reversed its decision several months later.

A follow-up study is in progress, and Relyvrio's manufacturer, Amylyx Pharmaceuticals, has pledged to withdraw the medication if the trial fails to demonstrate efficacy. (Results are expected in 2024.)

Taking all three ALS medications is seen by many practitioners as the best way forward. “While not a cure, it is a treatment ‘cocktail’ that we hope will have a cumulative effect,” says Ghazala Hayat, MBBS, FAAN, professor of neurology at Saint Louis University School of Medicine in Missouri. “The mechanisms of each are different, so our hope is that combining them will further slow progression and extend life,” says Dr. Bromberg.

Alternate forms of riluzole and sodium phenylbutyrate/taurursodiol are available for those who have difficulty swallowing, says Dr. Lomen-Hoerth.

Besides prescribing drugs that can slow progression, doctors can offer a range of medications to treat ALS-related symptoms, including modafinil (Provigil) for fatigue, mexiletine for muscle cramps, gabapentin (Horizant) for chronic pain, antidepressants for depression, and dexmedetomidine (Precedex) for sleep disorders. (The relationship between adequate sleep and slowing ALS progression was evidenced by a study published in Brain Science Advances in 2021.)

For people with ALS who experience pseudobulbar affect (a condition that causes bouts of uncontrollable laughing or crying), neurologists may prescribe dextromethorphan/quinidine (Nuedexta), says Merit E. Cudkowicz, MD, MSc, FAAN, director of the Sean M. Healey & AMG Center for ALS at Massachusetts General Hospital in Boston. “A small trial suggests Nuedexta could help with speech and swallowing function as well,” she adds.

Researchers continue to develop and test new drugs, says Dr. Cudkowicz, citing the Healey ALS Platform Trial, which allows for testing of more than one drug at a time, with a single placebo group for comparison. In addition, gene therapies are being explored to help the 5 to 10 percent of ALS patients with the hereditary form of the disease. Under the Accelerating Access to Critical Therapies for ALS Act, passed in 2021, a five-year plan by the FDA will support development of new medications for ALS and other neurodegenerative diseases.

The current ALS drugs are expensive: Edaravone and sodium phenylbutyrate/taurursodiol each cost more than $100,000 per year. Delays in treatment caused by the insurance approval process could be dangerous for ALS patients, who may have only months or two to three years to live, says Dr. Bromberg. Patients and their doctors must answer a range of questions and provide documentation as part of an insurer's review process—and should persist if denied. “Contact the pharmaceutical company to see if it offers a support program,” suggests Dr. Hayat.

Amylyx has a program to cover Relyvrio co-payments for patients with private insurance but not those on Medicare or with Veterans Administration benefits. Dr. Hayat recommends contacting the manufacturer's patient liaison, who may be able to suggest other organizations that provide support, such as the Assistance Fund, which can help eligible patients with out-of-pocket treatment expenses.

Resources for ALS

Listen Now!

To hear more about John Driskell Hopkins, tune in to the Brain & Life Podcast where he shares his story of being diagnosed in 2021 and what he is doing to spread awareness about this rare disease.