In times of crisis, Huntington’s and other chronic disease communities provide examples of fortitude

For individuals and families facing Huntington’s and other chronic diseases, daily living often seems like nonstop crisis. These communities, including selfless caregivers, have demonstrated great fortitude.

The current coronavirus pandemic adds another giant, unknown layer to the kind of challenges and suffering many in these communities are already accustomed to.

The country’s reaction to the pandemic and the shutdown of daily routines starkly reminded me of the national climate in the months following the September 11, 2001, terrorist attacks on New York City and Washington, D.C. Resulting in 3,000 deaths, 9/11 led to a temporary grounding of all domestic flights, the fear of subsequent attacks (including nuclear), and panic regarding other, unrelated terrorist threats such as the deliberate spread of highly dangerous anthrax bacteria. The economy also suffered serious long-term effects.

Members of the San Diego Chapter of the Huntington’s Disease Society of America (HDSA), in the bewildering days after 9/11, held a previously planned fundraiser and got ready to hold its first Celebration of Hope Gala.

With so many other, urgent demands on potential donors, we feared people might not remember our needs.

Later that fall, in an editorial in Conquest, our chapter newsletter, I wrote the following:

On September 20, less than two weeks after the crisis, members of the public and local organizations participated in the Third Annual Indy Go-Kart Challenge to raise funds for the cure of HD. Then, on October 11, the one-month anniversary of the attack, several hundred San Diegans took part in the Celebration of Hope Dinner to assist the Center of Excellence for Family Services and Research at the University of California, San Diego. We raised tens of thousands of dollars at these events. Elsewhere in the country other HDSA events went on as planned.

In this manner HDSA and its supporters have sent a resounding message that generosity and compassion are far stronger than the hateful politics of terror. We will move ahead, no matter what the odds are against us. This is the American spirit.

Unprecedented impact on daily life

So far, the coronavirus pandemic has had an unprecedented impact on the United States, with schools, universities, and many other institutions closing or switching to remote, online operations. As of March 15, at least 65 people had died in the U.S., with worst-case scenarios projecting 200,000 to 1.7 million deaths.

The pandemic has changed my workplace unlike any earlier tragedy. After 9/11, no classes were cancelled by my employer, the University of San Diego (USD). In 2003, the devastating Cedar Fire in San Diego County left 15 dead, destroyed 2,820 buildings, and charred more than 280,000 acres. In response, USD shut down for just two weeks and finished the semester normally, with everybody back on campus.

Now, because of the virus, USD has cancelled classes this week and will finish the semester (eight more weeks, plus final exams) online. The University of Pennsylvania, where my HD-free daughter Bianca studies, will do the same. My wife and I are worried that Bianca will not be able to make it home in time before potential travel restrictions.

Each hour, we learn of more victims of the virus and further curtailment of life as we know it.

Impact on the HD community

The global crisis has also impacted the mission of HDSA and individual HD families.

On March 13, HDSA CEO Louise Vetter sent an e-mail to “friends of HDSA” announcing that the organization “holds the well-being of our families, volunteers and staff as our top priority” and will therefore postpone “ALL local HDSA events (educational & fundraising) nationwide” until April 30. That complies with public health recommendations to curtail public gatherings to slow the spread of the coronavirus.

According to Vetter, HDSA still plans to hold its 35th Annual Convention in New Orleans, June 4-6, but will continue to follow the guidance from the Centers for Disease Control, World Health Organization, and state and local health agencies.

HDSA’s national headquarters in New York City is closed. However, the entire staff is working remotely and has the resources “to continue the uninterrupted support of our mission during this time,” Vetter assured.

The Huntington’s Disease Youth Organization postponed its May 2020 international congress in Glasgow, Scotland, to March 2021.

I have accepted an invitation to deliver the keynote address at the 37th National Conference of the Huntington Society of Canada in November, but have been told to hold off on reserving flights until the pandemic’s long-term effects become clear.

Visiting the afflicted

For one HD community member and blogger, Mandi, 33, the pandemic meant that on March 12 she could not visit her 58-year-old HD-stricken father, Danny, at the Missouri nursing home where he resides.

“They are locking all doors for the safety of people there,” wrote Mandi, a dedicated caregiver and herself at risk, but untested, for HD. “Was my first reaction sad I couldn’t see my dad? Sure, but I immediately checked myself. I realized my own selfish want to see my dad was tiny in comparison to keeping the people in the nursing home safe because who knew if I was a carrier since you can have it for over a week and not show symptoms.”

Mandi pointed out how the nursing home’s restriction is “terrifying for those of us with possibilities of losing family.” She wants “to do everything in my power to protect him as I always have.”

However, she concluded: “Until we really know what we are dealing with let’s just take the precautions seriously.”

Danny (left) and Mandi (family photo)

Especially resilient

With the coronavirus pandemic, the world can learn from the example of the HD community and our generous supporters.

We in the HD community, and other neurological disease groups, face relentless adversity. HD grinds down the brain, leaving the affected like my late mother unable to walk, talk, or care for themselves. Caregivers like Mandi and my late father are true “HD warriors.” 

However, because of this, we have developed a very realistic view of life.

“We are all in a race to death, but people at risk for life-shortening diseases know that their time to the finish line is painfully fast and troubled,” I wrote in 2009 in an article titled “HD: hurtling towards death.”

At the same time, we also have a special appreciation for the “preciousness of life.”

Confronting HD makes us especially resilient. I am hopeful that the HD community will successfully meet the challenges of the coronavirus pandemic and, as in facing past challenges, become even stronger.

‘Alive and Well’ captures struggle against untreatable genetic brain disorder

Watching the recently released documentary film Alive and Well this past Monday evening, I re-experienced the torrent of emotions involved in the fight against Huntington’s disease.

Filmed on three continents, the 75-minute Alive and Well takes us on an odyssey through the lives of six families affected by HD and one HD researcher whose “reason for getting up in the morning is just to do something to solve this problem.”

The stories embody the deepest fears and highest hopes of the HD community.

Alive and Well portrays the utter helplessness of HD patients in the final stages of the disease. The filmmakers visit a medical facility where several HD patients reside. Their bodies appear almost lifeless as they sit in chairs, unable to care for themselves. Their faces are half-frozen, revealing only a wisp of the personalities they once expressed.

These HD people are in a movie, but they cannot even speak their lines.

Honoring HD people, creating an advocacy tool

“Huntington disease has been described as the most devastating disease known to man, and it’s devastating because it robs you of who you are,” says world-renowned HD scientist Dr. Michael Hayden, interviewed in his native South Africa. “You lose the ability to speak. You lose the ability to communicate. Yet your perception is fine. You can perceive things around you.”

Dr. Hayden uses “Huntington disease,” the way people spell the term in Canada, where he has spent much of his career.

“The other devastating part of this is that it’s progressive,” he adds. “There is no way to intervene in the course of the illness. And what’s particularly ironic is that it’s continuous from one generation to the next.”

In their struggles, Huntington’s families provide great inspiration for humanity, “so-called ordinary people doing extraordinary things,” Dr. Hayden continues.

“If people knew the stories that are in these families with Huntington disease, these would fill books and books of adventures and sources of inspiration for the rest of the world. The stories are really profound.”

As the filmmakers say, Alive and Well is about a disease that exists everywhere but is still unknown. It’s also about human resilience.

“We made this film to honor the people who trusted us with their stories, to have this film seen by as many people as possible and to raise awareness of Huntington's disease,” says director Josh Taft, first introduced to HD by Seattle advocate Liz Weber, in a press release. “We wanted a way to share these very personal stories with compassion, strength and beauty. We wanted to create a solid tool for the community to share their stories and to be proud of.”

No regrets

Viewing the film in a downtown San Diego movie theater at a screening organized by Misty Oto of the local chapter of the Huntington’s Disease Society of America (HDSA-San Diego), Alive and Well carried me through the peaks and valleys of my own experience with HD.

My mother was diagnosed with HD in 1995 and died in 2006 at the age of 68. I tested positive in 1999; thankfully, I have yet to show any of HD’s classic symptoms. Our daughter tested negative in the womb and is today a thriving eighth grader.

In the first profile, of 19-year-old Heather Alimossy of Medford, OR, I saw how innocence is lost and life changed forever when someone tests positive for the HD mutation – confirmation that Heather will follow in the footsteps of her HD-afflicted mom.

As shown, Heather forges on in the quest to live a full life. She continues to ride her dirt bike – and she lovingly cares for her mother.

“I don’t want to regret anything,” she says, the film then showing her on a ride through the countryside.

Taking a chance

I was heartbroken by the story of Katy Bradley and her family, of Olympia, WA. Katy married Scott despite learning that his father had HD. At one point, the couple visited him in a nursing home in California.

“I knew by then, especially, what this could turn into,” Katy says. “I guess I took a chance.”

The couple refused let HD “ruin” their lives. They decided to bear children without Scott getting tested. However, without their knowing it, Scott passed on the HD gene to their son Matthew. Scott’s symptoms didn’t begin until well into his adult years, but Matthew developed juvenile HD as a toddler.

In a matter of a few years, Matthew’s symptoms worsened to the point where he was “in a constant state of seizure,” Katy says. “His brain is constantly firing.”

“Today’s it,” says Katy. “So let’s have fun today. So let’s do what we can. Let’s make muffins.”

The vignette finishes with people quietly attending Matthew’s burial. The couple’s small daughter Anna is also at risk for HD. Meanwhile, Scott continues to decline.

A very hopeful note

The other profiles in the film focus on Courtney Rifkin, a gene-positive woman shown climbing Mount Kilimanjaro to raise awareness about HD; Brooks and Dunn drummer Trey Gray, portrayed in his desperate struggle to maintain his skills after HD onset; Mandy Kipfer, a young woman who wants to start a family, filmed as she receives her HD-negative test result; and the middle-aged former NBC News war correspondent Charles Sabine, presented in his role as the HD community’s global advocate while striving to avoid onset.

I could empathize deeply with Trey, because I fear losing my ability to write. I felt terribly jealous of Mandy, although ultimately happy for her, because I want to be free from the shackles of HD. I felt strengthened by Courtney and Charles, because they refuse to give up and are willing to share their stories with the world.

During the film, I sat next to HDSA-San Diego president George Essig, whose extended family is affected by HD. He, too, was moved by Alive and Well.

“It’s been the best representation really of the disease I’ve seen on a variety of levels – physical, emotional, and in terms of the devastation,” George said afterwards, as the audience of 140 filed out of the theater.

The film features an original score plus songs from Radiohead, Pearl Jam, Fleet Foxes, and Sigur Ros. The overall effect is saddening but also upbeat.

“I would also say it has a very, very hopeful note,” George said.

HDSA-San Diego president George Essig with daughter Julia and wife Theresa at the screening of Alive and Well (photo by Gene Veritas)

Spreading the word

George added that he is “anxious to get copies out, because I think we could spread this virally as well as in movie theaters and a part of the whole education process and awareness process for the Huntington’s community.”

Alive and Well is not yet available on DVD and currently can only be viewed in small screenings, but event organizer Misty pledged to advocate for greater distribution of the film.

Alive and Well has shown in a number of other U.S. cities. Advocates can arrange for showings in their communities by contacting the Theatrical on Demand organization GATHR.

“We’re incredibly proud that Liz Weber and the team in Seattle developed this film,” said HDSA CEO Louise Vetter in a phone interview today. “There are a lot of multimedia tools to raise awareness about HD. We’re supportive of all the efforts.”

The producers aim to “bring the film to life” by encouraging HD families to share the opportunity to view it, Louise added. It’s also a way for HD community to “learn how to be alive and well with HD,” she said.

“That’s a very powerful effort,” she observed of the film’s promotion, which has relied heavily on social media. “They’ve been very committed to that grassroots effort from the beginning.”

In recent years, a number of advocates have been producing other films on the disease. Notable examples include: Chris Furbee’s just-completed, 89-minute Huntington’s Dance, chosen to appear at the 2014 edition of the highly competitive Slamdance Film Festival; Kristen Powers’ still- in-progress Twitch; and James Valvano’s still-in-progress The Huntington’s Disease Project: Removing the Mask.

(To read more on HD’s place in the news and entertainment media, click here.)

Yesterday, polio – tomorrow, HD?

Alive and Well begins – and ends – with a message of hope from Dr. Hayden.

“For each of us, we have to find our own passion,” he says at the outset. “That’s what makes life meaningful.”

We all can and must contribute, Dr. Hayden urges us.

“When you grow up in (apartheid) South Africa, you learn very quickly not to accept dogma,” he recalls of his youth in the closing minutes of the film.

Nobody believed HD existed in Africa, he adds. “Unfortunately, it’s alive and well throughout Africa.”

He recounts how, in conducting his Ph.D. research on HD in South Africa, he visited every mental hospital in the country to attempt to measure the frequency of HD among the populace.

Today Dr. Hayden is focused on the pathways to treatments that, although they may not cure the disease, could delay onset to offer people a longer life. (In 2012, Dr. Hayden became the president of global research and development and chief scientific officer for Teva Pharmaceuticals, Inc., a large, Israel-based drug firm, where he continues to promote HD research.)

“I’m really hopeful that we are going to be able to change the course of this illness,” he concludes, “and I think it’s in the near as opposed to distant future. I don’t know what ‘near’ is, but I’m convinced that with the … incredible donations of organs and blood and stories and financial support that’s come from so many quarters that we’ll be able to do something….

“Who would have thought in the late ‘50s there’d be treatment for polio? We can and will overcome this.”