Comedian Rod Man’s heartfelt speech, Ramona Johnston’s death remind us of urgent need to cure Huntington’s disease

In his raw, heartfelt speech in February about the devastation of Huntington’s disease in his extended family, comedian Rod “Rod Man” Thompson reminded his audience of the HD community’s urgent need for help: “We’ve got to find a cure. Any disease: you want to kick his ass.”

On April 4, the story of yet another fallen “HD warrior” drove home how the condition often strikes in the prime of life and is ultimately fatal. That night, Ramona Johnston, the wife of longtime San Diego Chargers football team public relations director and major HD fundraiser Bill Johnston, succumbed to the disease after more than 20 years of brave struggle. Diagnosed at age 39, Ramona was just 60.

“Ramona passed away last night after fighting unbelievably hard against this horrible disease,” Bill wrote on Facebook on April 5. “As much as I knew this day would come, it hurts so bad that my girl is gone. She’s the strongest, toughest, bravest person I know. One of God’s angels on earth finally has peace and is with Him in heaven.”

Introducing Rod Man

HD affects not just individuals but families. To illustrate that, as promised earlier, I’m now providing a detailed report on Rob’s important and moving February 25 keynote address to several hundred scientists, drug hunters, and advocates at the 14th Annual Huntington’s Disease Therapeutics Conference. Sponsored by CHDI Foundation, Inc., the event took place at the Parker Palm Springs hotel in Palm Springs, CA. 

A standup comedian, Rod Man was the winner of season 8 (2014) of NBC’s Last Comic Standing reality TV talent competition. He has appeared at Caesar’s Palace in Las Vegas, the Apollo Theater in New York City, and the Gibson Amphitheatre in Los Angeles, where he resides. He has also appeared in film and TV roles, and hosts his own YouTube shows.

“That’s my passion,” said the performer known as Rod Man. “Tonight, we’re going to set a different tone. I am Rod Thompson tonight.”

A native of the small Georgia town of Villa Rica, Rod was the first African-American CHDI keynoter. His family’s story underscored the fact that HD affects people of all ethnicities, as well as both sexes.

“As an African-American, I know that sometimes we don’t get the information,” Rob commented. “I’m here as a black man.”

However, he said, he recognized that HD affects his family just as it does others. He said that he aimed to “promote conversation” and awareness about HD, no matter what a person’s personal or educational background.

Rod "Rod Man" Thompson (right) exchanges laughs with leading HD researcher Steven Hersch, M.D., Ph.D., of Voyager Therapeutics, after Rod's keynote speech (photo by Gene Veritas).

The challenges families face

Rod titled his presentation “The Cycle: My Mother’s Story.” Interlacing humor with wrenching stories of HD’s debilitating physical and mental impact on his 66-year-old mother Shirley, Rod captured the many aspects of HD that families struggle with and doctors and scientists seek to alleviate.

His speech reflected once again how, as an inheritable disease, HD affects the extended family. His elder daughter prepared the slides for his talk. It included photos of Shirley and three aunts – all now in nursing homes – and a cousin also stricken with the disease.

Rob recalled how his maternal grandmother was initially misdiagnosed with cerebral palsy. Growing up, he was unaware of HD. “I just knew my grandmama was mean,” he said, referring to HD’s psychiatric and behavioral symptoms.

Before arriving in Palm Springs, Rod interviewed his sister, the primary caregiver for his mother in Villa Rica. She described details of Shirley’s daily struggles with HD, as well as the constraints it imposes on her own life.

“I understand how it affects her life,” Rod said. “She said, ‘Yes, it’s a lot to deal with.’”

Because of her “sacrifice” to care for Shirley, Rob’s sister has for now given up her own career dreams in fashion design, Rob added. “I could see the anger build in her, because she feels like, ‘If I don’t, who else is going to?’”

Rod recognized that other HD families face a similar battle. “It’s like having a child sometimes,” he said of the inability of HD people to care for themselves and the challenges faced by caregivers.

Shirley’s decline

Shirley showed symptoms before her official diagnosis in 2016, Rob explained. 

“Once they told her, she broke down and cried, because she knows from her sisters and her mama what it does,” he said.

Rod described the terrible physical, cognitive, and psychiatric decline that HD has wrought in his mother.

“I see depression and sadness about stuff that can be a misunderstanding to most people, and they’ll let it go, but she still holds on to it and harbors it,” Rod said.

Because of Shirley’s involuntary movements, the family also keeps kitchen utensils away from her to prevent her from injuring herself or others. She can no longer walk normally.

“Now it’s better for her to eat with her hands, because she’s a little shaky,” Rod explained. “Her body’s not the same.”

Inspiring the scientists

Untested, Rod and his two daughters – a college graduate and a high school student – are also at risk.

Rod and members of his extended have candid conversations about testing. However, he added, “Most people in my family are scared to get tested.” That reflects the majority attitude in the HD community. 

However, Rob hopes for a cure, hence his motivation to keynote the conference. “I appreciate what you guys do in your work day to day,” he said. “You all do admirable work. Hopefully, by telling my story, and others like me, you’ll get inspired.”

Rod received a standing ovation.

You can watch Rod’s address in the video below.

Celebrating Ramona’s life

Ramona died at Edgemoor Hospital in Santee, CA. The highly regarded public long-term care facility has cared for dozens of HD patients over the last few decades.

In early 2017, when the Chargers announced their move to Los Angeles, Bill made a gutsy, loving decision: after 38 years with the team, he quit so that Ramona could stay at Edgemoor. He visited her there each morning before heading to work.

“Everybody would make the same decision I am making if they were in my shoes,” Bill said at the time. “It’s just the situation I find myself in.” (Click here to read more.)

Bill now works for the San Diego Padres baseball team, which has supported his advocacy.

Ramona’s son Jared tested negative for HD. Untested, daughter Hayley is the vice president of the San Diego Chapter of the Huntington’s Disease Society of America (HDSA). She and Bill continue as leading advocates in Southern California. Their efforts have raised almost $3 million for HDSA.

A Celebration of Life will take place at 1 p.m., April 17 at Skyline Church, 11330 Campo Road, La Mesa, CA. In lieu of flowers, memorial contributions may be made to HDSA or www.HelpCureHD.org.

The Johnston team at the 2014 Rock-n-Roll Marathon raising funds and awareness for HDSA. Bill has his arm around Ramona, in wheelchair. Daughter Hayley stands directly behind Bill (photo by Andrew McClanahan/PhotoRun.net).

A time for hope: HDSA’s 50th anniversary, a record-setting gala, and the impending release of highly anticipated clinical trial results

This year, Pope Francis’ historic “Hidden No More” audience with the Huntington’s disease community has understandably overshadowed another milestone in the HD cause: the 50th anniversary of the Huntington’s Disease Society of America (HDSA).

However, on November 4 the spirit of HDSA, embodied in founder Marjorie Guthrie’s goal of a cure, helped fuel new hopes: the San Diego chapter’s sixteenth gala, recalling HDSA’s start in 1967 and honoring celebrated ESPN sportscaster Chris Berman, raised more than $250,000, a national record for chapters. It was the society’s most successful fundraising event of the last decade.

Also, for the first time, the gala attendees included top scientists from Ionis Pharmaceuticals, Inc., which, by January 2018, expects to release results of its highly anticipated Phase 1 gene-silencing clinical trial.

It was also the first time an HDSA chairman of the board attended: Chair Arik Johnson, Psy.D., the staff psychologist at the HDSA Center of Excellence at the University of California, Los Angeles, joined HDSA CEO Louise Vetter at the event.

It was a striking convergence of history, people, and research progress.

The gala was organized by HDSA-San Diego and the chapter’s former president, Bill Johnston. Johnston left a 38-year career as PR director for the San Diego Chargers football team, which moved to Los Angeles, to keep his HD-stricken wife Ramona at Edgemoor Hospital, an award-winning care facility in nearby Santee. Johnston now works as special advisor to Ron Fowler, the executive chairman and co-owner of the San Diego Padres baseball team and a long-time supporter of HDSA-San Diego.

“It was so rewarding to see the extended HD community having a wonderful evening and enjoying a series of heartfelt and humorous stories about Marjorie Guthrie, the Johnstons and the 2017 Guthrie Awardee, Chris Berman,” HDSA-San Diego President Beth Hoffman, Ph.D., said after the event. “Generosity was in the air, and we’re so grateful to everyone who dug deep to support HD families and to find a cure for HD.”

HDSA CEO Louise Vetter addressing the gala (photo by Derrick Tuskan)

Marjorie Guthrie’s vision for a cure

More than 300 people attended the gala, held at the Pendry San Diego Hotel, including members of the Padres organization, sports and local celebrities, drug company representatives, HD researchers and clinicians, and HD family members.

In her speech, Vetter described HD’s devastating symptoms, its impact on families, and the story of Guthrie’s founding of what would become HDSA after her husband, folk singer and social activist Woody Guthrie, died of HD in 1967. Guthrie began in a time before the Internet: she placed a classified ad in The New York Times seeking contact with other families affected by a disease mainly unknown, even to New York’s medical community.

“In three months, she had 35 families affected by Huntington’s disease that were meeting around her kitchen table and dreaming about developing a cure for HD,” Vetter said. “That’s how the Huntington’s Disease Society of America was born. And at 50 years, we mark the amazing vision that this woman had to really set a course for a community to come together and provide support for one another.”

Today HDSA provides education about HD, supports research, and advocates for better care for HD-affected individuals, Vetter added. It now has more than 40 Centers of Excellence (COE), which provide a variety of family services and involve the community in research studies and clinical trials.

In a pre-gala interview, board chair Johnson outlined some of the ways in which HDSA and COEs are assisting affected individuals and families with HD’s daunting psychological and behavioral symptoms. “We have a Telehealth program for people to call in and talk to a trained social worker or psychologist who has been educated in Huntington’s disease,” he said.

You can watch Vetter’s speech in the video below. Click here to watch my interview with Dr. Johnson.

At 50, HDSA striving for founder Marjorie Guthrie's goal of a cure from Gene Veritas on Vimeo.

‘A heart for people that need a voice’

After the audience watched an ESPN report on the Johnston family’s struggles and leadership of the cause, Johnston received a standing ovation as he approached the podium. Johnston’s daughter Hayley, who has not tested for the inheritable genetic defect that causes HD, appears in the video.

“If my wife were here, then it would make sense that you’re standing up,” Johnston told the audience, explaining that Ramona’s advanced symptoms caused her to miss the event for the first time. “My wife is my hero. I appreciate everybody being here tonight. Hayley said it in that video about this disease: people don’t really know what it is until you see it. I wish Ramona could be here. She would be here, if she could.”

Johnston noted that Berman and Fowler have been the “biggest” and “most consistent” contributors to his fundraising efforts. Fowler was honored at the 2013 San Diego gala.

Johnston recalled his friendship with Berman, begun in the 1980s when he started at the Chargers and Berman was at ESPN. Starting in the 1990s, around 6 a.m. every Sunday during football season, Johnston updated Berman on Chargers news for the sportscaster’s reports.

“We just talked football,” Johnston said. “And then when Ramona got diagnosed, I could not have that conversation without him asking first how was Ramona, how are the kids. Every year, at the end of the year, a check would show up in the mail, unannounced, unrequested, a donation. Our honoree has been a huge friend of our organization and the effort to find a cure, find a treatment, for this disease.”

Berman does more than write checks, Johnston added. Berman, he said, “shows to me a commitment and a caring and a heart for people that need a voice, and that’s what this disease needs. It needs people to talk about it, to learn about it. And that’s why we’re doing what we’re doing here tonight.”

Bill Johnston (left) and 2017 Guthrie Award Honoree Chris Berman (photo by Derrick Tuskan)

‘Advancing the ball’ for HDSA

In accepting the Guthrie Award, Berman praised Johnston’s commitment.

“Billy is as loyal as they come,” he said, referring to Johnston’s decision to stay in San Diego. “Is there anything more admirable than that?”

Berman recalled that, after his wife’s death in a car accident in May, Johnston flew to the East Coast for the funeral. Johnston and other “true friends” have helped him overcome his grief, he said.

“You realize that, when times are the toughest, your friends and people bonding together is the way we all can advance the ball, if you will, to use a football term. They’ve helped me do that. And that’s where we are with Bill, and Hayley, who’s here tonight, and the Johnston family and countless other families just like this family.”

You can watch Johnston’s and Berman’s remarks in the video below.

Chris Berman: 'Let's advance the ball to cure Huntington's disease' from Gene Veritas on Vimeo.

Baking the clinical trial cake

Earlier, I watched as Ionis scientists bid on silent auction items using a mobile phone app. I greeted several of the Ionis officials, including Frank Bennett, the senior vice president for research, who attended the gala for the first time.

Dr. Bennett has overseen the ten-year development of IONIS-HTT_Rx, a gene-silencing drug that aims to alleviate HD symptoms by reducing production of the huntingtin protein in brain cells. The drug entered a Phase 1 clinical trial in September 2015 in Canada, England, and Germany. The company expects results to become available in the next two months.

Currently, to protect against bias as clinical trial administrators analyze the results, Dr. Bennett and the Ionis HD team cannot access the data. That’s standard clinical trial protocol.

Recalling a metaphor used in formal interviews about IONIS-HTT_Rx, Dr. Bennett referred to the project as a “cake in the oven.” The team is awaiting the opening of the oven to learn the results.

If successful, IONIS-HTT_Rx would mark the first effective treatment – though not a cure – for a brain disease.

The Ionis Pharmaceuticals table: (clockwise from left) Chris Bragg, Stacy Raysin (Dr. Bennett's assistant), Eric Swayze, Ph.D., Carolyn Swayze, Lisa Lane, Roger Lane, M.D., Brad Smith, Anne Smith, Ph.D., Frank Bennett, Ph.D., and Paula Bennett (photo by Gene Veritas, aka Kenneth P. Serbin)

Close to treatments

Jody Corey-Bloom, M.D., Ph.D., the director of the COE at the University of California, San Diego, also attended with her husband Floyd Bloom, M.D., staff, and members of the HD community. So did representatives of Teva Pharmaceutical Industries, a major sponsor of HDSA galas and the manufacturer of AUSTEDO, a recently approved drug – developed in San Diego – that alleviates HD’s involuntary movements but does not slow progression of the disease.

On October 30, Dr. Corey-Bloom gave her annual Huntington’s disease research update at the HDSA-San Diego support group. Most of her talk focused on gene silencing and the Ionis trial.

She also spoke on her groundbreaking research on the presence of huntingtin protein in saliva – a potential marker of both disease onset and progression and also of medications’ impact. Saliva is easier and far safer to collect than blood and cerebrospinal fluid, which are also under study for markers. (Click here for a scientific presentation on this topic.)

“This is an incredibly exciting time for HD,” Dr. Corey-Bloom concluded. “I think really we’re on the edge of really discovering something here, and being able to treat this disease. If we can’t cure it, we can slow it down.”

To watch Dr. Corey-Bloom’s presentation, click here.

Dr. Jody Corey-Bloom's table: (clockwise from front) HD family member Linda Pohl, Pablo Garcia, Lily Garcia (Dr. Corey-Bloom's assistant), Steve Granger, Ph.D., chief scientific officer at Salimetrics, LLC, Beth Thomas, Ph.D., The Scripps Research Institute, Dr. Corey-Bloom, Floyd Bloom, M.D., and HD family member Margaret Schroeder (photo by Gene Veritas, aka Kenneth P. Serbin) 

A true celebration of hope

I’ve attended every San Diego gala but one. As always, it was simultaneously exhilarating and emotionally draining.

I was thrilled to see the especially large group of scientists, researchers, and clinical workers. I was able to introduce several of these mutual contacts to each other. I also enjoyed listening to Berman talk about his career as a broadcaster, one of the highlights of the evening (click here to watch an excerpt).

When I greeted Johnston, I told him that my recent, annual neurological checkup showed no signs of HD. I’m 57; my HD-stricken mother’s symptoms started in her late 40s.

However, seeing “HD brothers and sisters” like Tim Schroeder and Sharon Shaffer, both deeply affected by the disease, reminded me of my likely future unless a treatment is found soon.

Yet this gala lived up to its name: a celebration of hope. My heart jumped as I realized it would set a fundraising record.

I’m hoping it will jump even more when the Ionis Phase 1 results come out.

Above, from left to right, Fran Walker, daughter Sharon Shaffer (seated), Taylor Shaffer, Renato, Shaffer, and Alexa Shaffer (photo by Gene Veritas, aka Kenneth P. Serbin). Below, Gene Veritas with HDSA-San Diego supporter Mary Wisco (photo by Bob Walker).

(Disclosure: I hold a symbolic amount of Ionis shares.) 

(Scroll down for other photo highlights.)

Chris Berman (left) and Ron Fowler, executive chairman and co-owner of the San Diego Padres (photo by Derrick Tuskan)

HDSA-San Diego president Beth Hoffman, Ph.D., (left) and Beth A. Thomas, Ph.D., The Scripps Research Institute (photo by Gene Veritas, aka Kenneth P. Serbin)

Mike and Jan Neil, HDSA-San Diego supporters (photo by Gene Veritas)

Nina Detrow (left), KGTV news anchor Kimberly Hunt, and Lori Ello (photo by Gene Veritas)

Bill and Hayley Johnston (left) with Liya Sharif and Pete Lancia of Qualcomm (photo by Gene Veritas)

The HDSA San Diego Center of Excellence/UC San Diego/Teva table: clockwise, starting in the foreground, Ameera Haque (UCSD research assistant), Gayle Paddison (Teva clinical nurse educator), Mr. Paddison, Amy Rahilly, Jeff Rahilly (Teva), Aeri Kim (UCSD research assistant), Chase Snell (UCSD research coordinator), Sungmee Park (UCSD research coordinator), Ayesha Haque (UCSD research assistant), and Rina Patel (UCSD research assistant) (photo by Gene Veritas)

HDSA-San Diego board member Paul June (middle) with supporters Amy and Cam Stephens (photo by Gene Veritas)

Scott Yoffe and HDSA-San Diego board member Nan Pace (photo by Gene Veritas)

HD family member Doug Schulte (photo by Gene Veritas)

Former HDSA-San Diego president George Essig (standing) toasts with friends (photo by Derrick Tuskan).

Staying when the Chargers leave: a leading Huntington’s disease advocate sets a gutsy, loving example

After the San Diego Chargers’ recently announced move to Los Angeles, team public relations director and Huntington’s disease advocate Bill Johnston made a gutsy, loving decision: after 38 years with the Chargers, he will quit so that his HD-afflicted wife Ramona can stay at the highly-regarded San Diego nursing home where’s she spent the last decade.

Bill made his decision after thoroughly researching nursing homes in Orange County, which is much closer to the Chargers’ new Los Angeles headquarters than San Diego County. He visited seven facilities, paying special attention to their ability to conscientiously care for someone with HD. As the HD community is all too painfully aware, such facilities often provide poor care.

Bill did not find what he wanted. He opted for Ramona to remain at Edgemoor Hospital in Santee, located next to San Diego. A public nursing home, Edgemoor has cared for dozens of Huntington’s patients over the past several decades.

“Everybody would make the same decision I am making if they were in my shoes,” Bill told the San Diego Union-Tribune. “It’s just the situation I find myself in.”

Bill awakes at 4:55 a.m. daily and visits Ramona at Edgemoor before heading to work. She was diagnosed with HD in 1999 but had showed symptoms earlier. She is now in the late stages of the disease, confined to a wheelchair and unable to care for herself. The native San Diego couple met in high school and married in 1983.

“She can’t talk anymore, but she’ll make some sounds,” Bill told the Union-Tribune. “Sometimes, I think she’s trying to say my name. Maybe it’s just wishful thinking.”

The Johnston team at the 2014 Rock-n-Roll Marathon raising funds and awareness for the Huntington's Disease Society of America. Bill has his arm around Ramona, in wheelchair. Daughter Hayley stands directly behind Bill (photo by Andrew McClanahan/PhotoRun.net)

‘I’ll always do what I can’

By interrupting his career and staying in San Diego, Bill is rolling with one of the many punches thrown by HD. His son Jared, 31, tested negative for HD. Daughter Hayley, 28, remains untested; she has a 50-50 chance of having inherited the HD genetic defect from Ramona.

Other HD families have adapted their lives dramatically to meet similar challenges. In my family, my mother died of HD, and I carry the gene. Since my mother’s diagnosis in 1995, HD has frequently dominated my family’s life. Fortunately, our daughter tested negative and is today a healthy teenager.

Bill’s situation reminded me of my own. In 2007, in a wrenching, career-changing decision, I turned down a major job offer in Miami to remain in California, a biotech state with crucial public support for stem cell research. California also has HD-involved companies such as Ionis Pharmaceuticals, Inc., which is currently running a historic Phase I clinical trial of a gene-silencing drug. Most important, remaining in California allowed my wife to keep her relatively well-paying teaching job and pension, our financial lifeline if I become disabled.

I had also bonded with Bill and many other members of the San Diego Chapter of the Huntington’s Disease Society of America (HDSA-San Diego). Through chapter events, Bill and his family have raised nearly $3 million for HDSA. Their leadership and fortitude have inspired people in the local HD community and beyond.

In response to my e-mail query about his plans for future advocacy, Bill wrote: “I’ll always do what I can.”

“Bill has been a ‘tour de force’ in advocating for the HD community in San Diego and nationally,” HDSA-San Diego president Beth Hoffman, Ph.D., wrote in an e-mail. “Bill has tremendous energy and passion, and brings wonderful and new ideas to our fundraising efforts. He’s always been there to drive the chapter’s success. We are thrilled that Bill will remain in San Diego and look forward to expanding our efforts towards the HDSA mission with him.”

“I am not surprised by Bill's decision to stay,” long-time HDSA-San Diego board member Misty Daniel wrote. “His dedication to Ramona and our HD community has never faltered over the years. Bill's decision to stay reaffirms what most HD families know: that family truly is everything.”

Ramona with Chargers star and HDSA supporter Antonio Gates at the 2007 Celebration of Hope Gala (photo by Gene Veritas)

Change means new opportunities

After 56 seasons in San Diego, the Chargers’ departure angered local football fans and civic leaders. “The Los Angeles Judases have betrayed us for 30 pieces of silver,” wrote Union-Tribune sports columnist Nick Canepa, who is also a member of HDSA-San Diego’s advisory board, in reference to the move

The team’s’ exit has also posed a huge challenge for HDSA. Bill’s involvement since 1999 added the team’s high-profile pro-football brand to most major fundraising events, including the chapter's annual gala. For years, HDSA-San Diego board meetings took place at Chargers’ headquarters, and team owner Dean Spanos allowed use of that facility for fundraisers. In 2003, Spanos and his wife Susie received HDSA’s Harold Leventhal Community Service Award at a dinner in New York City.

However, Bill’s decision to remain has helped offset the feelings of desertion resulting from the Chargers’ move. His connections, creativity, and dedication will help the chapter strike out in new directions. As Bill has always made clear, he’s also in this fight for Hayley – and for all families affected by HD.

Bill and Hayley Johnston exchange ideas at an HDSA-San Diego event in May 2016 (photo by Gene Veritas).

Over the years, other chapter members and even Bill himself recognized the danger of relying too heavily on the Chargers. As a result, the chapter has strived to diversify its sponsor and donor base.

The Chargers’ exodus might also provide unforeseen benefits such as distancing HDSA-San Diego from the uncomfortable connection to a sport now linked to brain diseases similar to HD.

“The Chargers organization has been stellar in its support,” Dr. Hoffman wrote, noting the chapter’s gratitude for the players’ “enthusiastic participation” at fundraisers. “We will miss our Chargers.

“That said, the HDSA-San Diego board and all of our wonderful volunteers are hard at work attracting sponsors and making our events even more exciting. Whenever there are changes, there are new opportunities. Our job is to find and leverage these opportunities to their maximum potential.”