- WELL THAT HAPPENED: Escaping Neverland
- Photo contest garners stirring moments
- MUSIC BOX: Get weird with Peelander-Z
- GET OUT: Motley crews command the desert
- FEATURE STORY: New American Anthem
- Riotous sequel pokes more fun at Jackson life
- FEATURE STORY: The Journey to Jackson
- MUSIC BOX: Sodapop’s Bottomless Well
- FEED ME: World’s best street food is made in Wilson
- GUEST OPINION: Climate Change is my fault
Local researcher may crack the code on some brain disorders
Jackson Hole, Wyoming – Dr. Paul Cox is a lot more than a local hero to the growing number of families with someone suffering from a neurodegenerative disease. He is hoping to find a cure for ALS, Parkinson’s and Alzheimer’s, diseases that afflict more than 6 million people in America, from his little lab in Jackson Hole.
The Harvard PhD who started the Jackson-based Institute for Ethnomedicine has spent the last three years searching rainforests all over the world for clues about what causes these neurological disorders and how amino acids that mimic them might help reduce the progression of these diseases.
What Cox and his colleagues found is a neurotoxic amino acid called beta-methylamino-L-alanine, or BMAA, that is produced by cyanobacteria, a blue-green bacterium that lives in soil, lakes, and oceans, and another naturally occurring amino acid called L-Serine, which appears to block it. Cyanobacteria are consumed by fish and other creatures like bats, which are ingested by indigenous populations with a high incidence of ALS.
“Our laboratory experiments suggest that L-serine can block mistake insertion of BMAA into proteins, preventing misfolding and tangles,” Cox explained in an email. “Based on these data, the FDA granted permission for human clinical trials of L-serine in ALS patients. The Phase I trials are nearly complete, and the patients seems to have tolerated the oral L-serine doses well.”
A native of Salt Lake City, Cox has partnered with Dartmouth Medical School to discover why people who live near cyanbacterially-contaminated lakes have a higher incidence of ALS.
Cox, who has received numerous distinguished awards and published nearly 189 scientific papers and four books, will answer questions at Center for the Arts on Thursday. Institute for Ethnomedicine and Jackson Hole Wildlife Film Festival are sponsoring a free screening of Running for Jim, a film about a cross-country coach who lived beyond ALS’s average 2.5-year survival rate. There will be a question and answer period afterward, as well as an art exhibit beforehand by local artist Susan Durfee Thulin, who has interpreted Cox’s research in her paintings. Thulin will also paint a model in honor of the Institute’s research for the Nature’s Beauty fashion show at 5 p.m.
Cox, who was introduced at TEDxJacksonHole in 2012 as a potential Nobel Peace Prize winner by Lisa Samford, executive director of Jackson Hole Film Festival, was the only speaker from the independently organized local TED conference invited to TED.com. In 1997, TIME magazine named him one of 11 Heroes of Medicine for his work in ethnobotanical drug discovery. In the same year, he was awarded the Goldman Environmental Prize for his efforts in preserving Samoan rain forests.
But despite his international recognition, his door in the shadows of the Tetons is always open to people like Patrick Long, 52, who could not speak on the phone because his diaphragm is paralyzed since he was diagnosed with ALS 18 months ago. Long and his sister Katsey spent two hours with Cox last fall discussing his research.
“They were very positive and enthusiastic,” Patrick Long remarked in an email. “They gave me a comprehensive explanation of their work and tour of their lab and offices. They spent well over an hour with me. I left the meeting feeling encouraged. Most everyone you meet who is involved with ALS has a positive and optimistic attitude. There is no known cure, or any long-term treatments so hope is among the best therapies available. I think the Institute’s work is innovative and aligns with my belief that cures and therapies for diseases that afflict many people will be found by individuals not bound by large institutional restraints. People looking at problems in a variety of ways, old and new. Everyone with ALS hopes for a cure. No one wants this challenge for another person or to be honest, for themselves. It affects a relatively small portion of the population so it does not receive the money and attention that more common, fatal diseases get.”
The second phase of Cox’s clinical trials, which will determine if oral L-serine can help reduce the progression of disease in ALS patients, will cost $750,000, Cox said.
For ALS patients, damage to upper motor neurons begins at the top of the brain, causing muscle weakness, stiffness and augmented reflexes. As the damaged neurons move down to the spinal cord they cause muscle atrophy, twitching, weakness, and reduced reflexes. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost.
Alzheimer’s disease, the most prevalent neurodegenerative disease affecting an aging population, also is characterized by misfolded and tangled brain proteins. But instead of attacking the body, it causes a loss of cognitive abilities including memory loss. And Parkinson’s disease, the second most common neurodegenerative disorder, causes rigidity, resting tremor and instability in a much more gradual way than ALS.
“The biggest challenge of the disease is to not let it destroy your spirit,” Long wrote. “Eventually it will shut down my body, I have no control over how and when that will happen. We all die and most of us live in denial of that. ALS has smashed the delusion that I would live forever, and if I didn’t I’d probably die peacefully in my sleep. This has given me the opportunity to consciously embrace my death. I have four children and many friends. I hope they will learn from me and from this experience to fully live without fear of life or death.”
Humanizing disease through art
Artist Susan Durfee Thulin is captivated by dying cells and cycads, a seed plant that may be the key to better understanding neurodegenerative diseases and finding a way to cure them. In her work interpreting the research of Dr. Paul Cox at the Institute for Ethnomedicine, she mixes greens and blacks and swirls blood-colored paint with black shadows on her mixed-medium canvases, creating images that humanize diseases such as ALS, Parkinson’s and Alzheimer’s.
Thulin, Central Wyoming College director, has been painting all her life and has always been interested in science. When she heard Cox speak at TEDxJacksonHole two years ago, Thulin immediately offered to paint his laboratory. In addition to her role at the college, Thulin is on the leadership team for TEDx, an independently organized speaker’s conference promoting ideas worth spreading. Cox is the only local speaker whose talk was chosen by TED, an elite conference that challenges people to give the talk of their lives in 18 minutes or less.
“He is such an inspirational speaker,” she said. “It got me very excited. [Their lab] is tiny and clean and has the highest scientific standards. They were so gracious with their time explaining things to me.”
Thulin’s work is an abstract representation of disease cells, not a scientific illustration. It humanizes the cells by bringing them face to face with the people they attack.
For example, in her painting Alchemy: Mary/Malignancy, a profile of Mary Magdelene emerges from globular green and brown cells.
“I’m trying to pull together an understanding of what it means to be human when you have this disease,” Thulin said. “Trying to find peace and understanding; I use the painting process to come to an understanding myself.”
Like most Americans, Thulin has a family member who suffers from a neurodegenerative disease. In her case it is her uncle who lives at River Rock Assisted Living Center and struggles with Alzheimer’s.
Her paintings will be on display at Center for the Arts on Thursday with a reception at 6:30 p.m. At 7:30 p.m. there will be a free screening of Running for Jim, an inspirational story of a coach with ALS and his fight to succeed followed by a talk by Cox. Fifty percent of all art sales will be donated to the Institute for Ethnomedicine to research a cure for ALS, Parkinson’s and Alzheimer’s. The work will be displayed through July 5.
At 5 p.m. on Thursday, Thulin will paint a young model as part of Nature’s Beauty, a fashion show that beckons artists and designers to create beautiful designs from nature as part of the inaugural Jackson Hole Wild Festival.
Running for Jim buoys ALS patients
Cross-country running requires an ability to set a pace and push through when muscles fatigue. So does ALS, a neuro-motor disease made famous by New York Yankee Lou Gehrig.
In the sad, yet hopeful film Running For Jim, which screens at 7:30 p.m., Thursday at Center for the Arts, this mindset is explored. Co-producer and local artist Carlyn Hunter pitched the idea to tell Jim’s coaching story during a film class she was taking at the San Francisco Film Society in 2011. Her professor, David Brown, fell in love with Jim’s perseverance and the inspiration he gave his team against all odds. One of the longer living patients with amyotrophic lateral sclerosis (ALS), Jim died peacefully in his bed earlier this year. Despite the slow deterioration of his motor skills, he never gave up coaching.
Hunter’s daughter, Grace, ran on Jim’s team and was captain of her San Francisco High School cross-country team for three years when they rose to the California State High School Cross-Country Championships. Hunter collaborated with Brown and Robin Hauser Reynolds, the mother of Holland Reynolds, who ironically collapsed from severe dehydration near the finish line of the championship race, where Jim waited.
“I really wanted to encapsulate his message, which I think was summarized in his belief that each person has potential and he taught them always to give their best effort,” Hunter said. “He knew what each one of his team members could achieve and he was honest with them.”
The film, which took two years to make, has since traveled the world winning 13 awards at film festivals from Moscow to Jim’s home in San Francisco.
Like Lou Gehrig, who earned a reputation for the most consecutive games played, Jim set his own record by coaching nine state champion teams, more than any coach in the country, Hunter said. Grace and her team won the title all four years of high school.
As a part-time coach, however, Jim did not have a home or health insurance so he lived with Hunter and her family for 12 years until a percentage of the money raised to make the film afforded him disabled housing near the school.
Research indicates that men, military veterans, and sometimes athletes are more likely to be diagnosed with ALS. Most of the 30,000 Americans who suffer from the disease are in their 40s through 60s and die within three to five years. According to the ALS Association, half of all people affected with ALS live at least three or more years after diagnosis. Twenty percent live five years or more. Less than 10 percent live more than 10 years.
And then there is Stephen Hawking, author of A Brief History of Time, who was diagnosed at 21 and is still one of the brightest scientists at 72 years old.
“I think children want goals to live up to and Jim set his own till the day he died,” Hunter said. “He never stopped coaching and endured his disease with dignity. He would be pleased that our goal is to find a cure and we will get there whatever the crawl to the finish line is like.”