My Huntington’s disease sister – and a hero who strives for the unthinkable

Sharon Shaffer is my Huntington’s disease sister – and my hero.

Afflicted with progressively worse HD symptoms the past 15 years, San Diego area resident Sharon decided to once again attempt the unthinkable for someone heading into the final years of the fatal, incurable disorder: she rowed the equivalent of an aquatic marathon (26.2 miles) on a gym machine in six and a half hours.

The May 10 event, with loved ones and supporters cheering her on, was called “Sharon's Marathon Row for Huntington's Disease Awareness Month.” 

Sharon’s feat was captured in a five-minute video titled Magic on Marathon Row, produced by filmmaker Nathan Apffel, who also directed the feature-length documentary The Longest Journey, about the Shaffer nuclear family’s epic battle against Huntington’s.

A scene from Magic on Marathon Row

“When disease takes your health and mobility and will take your life, you have a choice,” the video’s overlying text says. “Meet Sharon, a real-life super woman. As Sharon’s body gives out, her determination grows. As her world contracts, her commitment to raising awareness intensifies. As her body breaks down, one thing remains consistent: her determination to fight.”

Sharon’s husband Renato told me in an e-mail that, despite her advanced HD symptoms, she did the "marathon" through “sheer perseverance.”

“It is the only exercise she is able to do without assistance,” he added.

“Sharon, you truly are an inspiration to so many,” wrote E.J. Garner, the chair of the board of the Huntington’s Disease Society of America, in response to my Facebook posting of the video. “Thank you for being such a beautiful example of persistence to fight and bringing awareness to Huntington's disease.”

“She completed a feat only few in the entire world, even healthy and athletic, would not dare approach, let alone finish,” observed LeeJ Razalan, a friend and the owner of Stay Classy CrossFit, the San Diego gym where the event took place. “Sharon's statement in life and in disease will forever ring: we shall overcome and we will finish this together."

Watch Sharon in the video below.

Advancing symptoms

The “marathon” exhausted Sharon. “She could barely move when we got home and took a good three days for full recovery,” Renato explained.

With her family’s help, Sharon has remained one of the most physically active HD-afflicted individuals I have encountered. The highly demanding CrossFit workouts have been one of her favorite activities.

Renato believes that CrossFit delayed Sharon’s need for a wheelchair.

“I also believe the people she worked out with gave her motivation,” he added. “She always looked forward to working out.”

However, Renato also described CrossFit as a “barometer” of HD progression.

In recent years she has become physically weaker, and the involuntary movements caused by HD have increased.

“We have witnessed a rapid decline in the past twelve months,” Renato wrote. “She is no longer able to walk unassisted, and her speech is becoming more labored every day. We have full-time in-home care and are so fortunate they are doing a really great job taking care of her.”

Sharon still exercises, but “it gets more difficult every week,” Renato added.

Renato allowed Sharon to row in the “marathon,” he wrote, because as symptoms worsen, exercise will become impossible. That moment will be "heartbreaking."

An upbeat family

I met Sharon about two decades ago at the HDSA-San Diego support group. With others in the breakout group for presymptomatic gene carriers like us and also the untested at-risk, we shared our fears about the disease and its consequences for our children and extended families.

The Shaffers became one of the most active supporters of the cause. Between 2005 and 2015, with Sharon, family members, and others following in support vehicles, Renato and three other riders competed four times in the Race Across America, a non-stop coast-to-coast bicycle race. They always completed the race in fewer than the required eight days.

The Longest Journey documentary chronicles the 2015 race. “In contrast with most presentations of HD I’ve witnessed in the two decades since my mother’s diagnosis for the disorder, this often emotionally wrenching film left me, an HD gene carrier, feeling upbeat,” I wrote in a review of a private screening of the film in 2016 (click here to read more). 

The closing words of Sharon’s rowing video ask viewers to learn more about Sharon’s story by watching The Longest Journey. (It’s on Amazon and free to Prime members.)

According to Renato, Amazon statistics reveal that the film has been viewed 200,000 times.

The essential goal: treatments

Sadly, I’ve witnessed how HD has overcome Sharon, transforming her from a healthy and vibrant woman into someone now dependent on a wheelchair and the assistance of others.

However, Sharon has demonstrated that, despite the ravages of the "devil of all diseases," the core of her personality has survived.

As we enter the final week of HD Awareness Month, Sharon’s gutsy fight reminds us all that we must not give up in the face of Huntington’s difficult challenges.

For me, Sharon’s devotion to exercise is a cue to spend more time swimming.

At the same time, exercise, although certainly important, has not stopped Sharon’s HD from progressing.

We need effective treatments – urgently.

In the HD movement, advocates create moments that capture the essence of the cause.

In defying seemingly insurmountable obstacles, the Shaffers remind us of the immense scientific, financial, political, and social challenges that the HD community faces in seeking to find the first effective treatment – and perhaps even a cure – for a neurological disorder.

Accentuating the positive: Olympic medalist Sarah Winckless’s strategy against Huntington’s disease

As a bronze medalist in rowing in the 2004 Summer Olympics in Athens and chair of the highly successful British athletes’ commission in the 2012 London games, Sarah Winckless pursued her goals based on strenuous training, teamwork, and effective thinking.

However, Sarah faces a challenge far more daunting than most Olympians could imagine.

Since 1997, when she learned at just 22 that she carries the gene for Huntington’s disease, Sarah has faced the difficult reality of knowing that she will likely follow in the footsteps of her HD-afflicted mother Val, who requires full-time care.

Sarah has kept upbeat by focusing on athletics and her major leadership roles in the British sports and HD communities.

Huntington’s is always a family story. Sarah was the first of four siblings to undergo genetic testing. When Sarah began to feel isolated from her two brothers and sister after all three tested negative for HD, she turned once again to the “thinking challenge” she had learned in sports.

“Suddenly my siblings didn’t know what is was like to open the door of your genetic counselor and know that you’ve got a bad result,” Sarah told an audience of several hundred scientists, drug company representatives, and Huntington’s disease advocates the evening of February 24 in her keynote address at the Ninth Annual HD Therapeutics Conference, sponsored by the CHDI Foundation, Inc., in Palm Springs, CA. “And I didn’t want them to know that. But it made me different. It separated me.”

Sarah said that she “hated” herself for “thinking and feeling that way.”

“I had to choose a thought that brought me back in, that made me part of my family – they’re so important to me,” she continued. “And I did what I always do when I need to think.”

Sarah went out for a long session of rowing, which helped bring clarity to the issue at hand. She had in mind the fact that every child of a parent with HD has a 50-50 chance of inheriting the genetic mutation.

“I came up with a thought: three out of four, we’ve beaten the odds,” she said. “And for the next two weeks, every time I caught myself thinking in a negative way, I ran that thought into my brain. And people would come and go, ‘We’ve heard the news about John (the last to be tested). How do you feel?’ And I’d go: ‘three out of four, we’ve beaten the odds!’

“And it helped, because suddenly I was part of it, and I was with them.”

Sarah struck a similarly optimistic, sometimes even jovial chord throughout her speech. (You can watch an excerpt in the video below.)

 

At dinner, Sarah regaled a group of conference attendees with other stories from her athletic career as well as her mother’s spirited fight against HD. Though HD patients rarely live two decades beyond onset, Val has now lived with the disease for about 25 years.

I felt infected by Sarah’s optimism.

“In all of my years of dealing with HD, you are the most positive individual I have ever met,” I told Sarah as I prepared to retire for the evening.

I hugged her and gave her a brotherly kiss on the forehead. “You are not going to get HD,” I declared. She smiled broadly as she thanked me.

As an HD gene carrier, I live with the stark knowledge of the disease’s inevitability. However, scientists believe that personal enrichment and staying active and positive might very well help delay onset. And there is the very real hope of an effective treatment for Sarah, me, and the entire HD community.

Sarah Winckless and Gene Veritas (photo by Simon Noble, CHDI)

That night and into the next day, I thought of how I need to maintain my own positive attitude – not just to bolster myself in the fight against HD, but to become a better husband, father, coworker, and citizen.

As the HD therapeutics conference proceeded, Sarah’s spirit lived on as the participants presented new research demonstrating a deeper understanding of HD and continued progress in the quest for treatments.

We all need to become infected with Sarah’s optimism.

Like the Winckless family, we must all bond together in this fight.

As difficult and terrible as it may feel, we are never alone.

“Three out of four, we’ve beaten the odds” – despite her own adversity, Sarah found joy in her siblings’ good fortune.

She has reminded us of a key lesson: no matter how difficult our situation, our lives mean so much more than the fight against HD.

Next time: a report on the therapeutics conference.