ALS Patient, Husband Use Custom Wheelchair in Boston Marathon UMass Team

ALS Cellucci Fund runner Chris Benyo and his wife, Denise DiMarzo, who has amyotrophic lateral sclerosis (ALS), are competing in this year’s Boston Marathon using a custom wheelchair designed for the race.

Illinois residents Benyo and DiMarzo make up two of the six people representing the UMass ALS team in the April 18 marathon to raise awareness and funds for ALS research at the University of Massachusetts Medical School (UMMS), seeking a breakthrough in treatment of ALS (Lou Gehrig’s Disease). This is the fifth year the Cellucci Fund has taken part in the John Hancock nonprofit marathon program, allowing UMMS to give five official race numbers to marathoners who commit to raising at least $7,500 each to support ALS research at UMMS.

ALS is a progressive, neurodegenerative, fatal, and currently untreatable and incurable disorder affecting motor neurons of the central nervous system. As motor neurons die, the brain’s ability to send signals to muscles is disrupted, leading to loss of voluntary muscle movement, paralysis, and eventually death from respiratory failure.

An estimated 5,000 people in the United States are newly diagnosed with ALS annually, with an average survival rate of three to five years. What causes ALS is not completely understood, although about 10 percent of cases are attributable to inherited genetic defects. The cause of the remaining 90 percent of cases — known as sporadic ALS — remain an unknown, although one hypothesis proposes that in the majority of ALS cases, the disease may be unleashed by a combination of genetic causes with exposure to one or more environmental factors, citing growing evidence that head trauma may predispose patients to ALS and related cognitive decline.

However, development of therapeutics for treating ALS is contingent on better understanding of how the disease works. In recognition of the critical need for new ALS treatments, the UMass ALS Cellucci Fund (formerly known as the UMass ALS Champion Fund) is a movement to drive awareness and funding for ALS breakthroughs at UMMS in the laboratory of Robert H. Brown, Jr., DPhil, M.D., one of the world’s leading and most promising ALS researchers.

Brown, who holdds the Leo P. and Theresa M. LaChance Chair in Medical Research and is chair of the Department of Neurology at UMass Memorial Medical Center, is widely recognized as a pioneer in neurodegenerative disease research. For more than 30 years he has dedicated his career to unlocking the secrets of ALS, been a leading visionary for ALS treatment, and been part of nearly every fundamental ALS breakthrough to date, including identification of mutations in the gene responsible for the disease’s rare familial form.

The Celluci Fund notes that Brown’s work has opened a window into ALS that has drastically changed the research landscape, and provided patients and loved ones touched by ALS with something very rare in this context: hope.

The Cellucci Fund aims to help Brown and his colleagues pursue ALS research leads and breakthroughs immediately, rather than potentially having to wait years to attract funding through traditional sources. As a result, the fund will better enable UMMS researchers to “seize the moment” when highly promising ALS discoveries are made.

In October 2010, Brown and his UMMS team reported evidence suggesting that the SOD1 gene, which is implicated in 20 percent of inherited cases of familial ALS, also plays a part in the more common, sporadic forms of the disease. The Brown team’s research showed that under certain conditions and absent a mutation in the gene, a normal SOD1 protein can have the same toxic characteristics that are found in mutated forms of the gene, noting that in select cases of sporadic ALS, these toxic proteins are present.

A Cellucci Fund release notes that when he began researching ALS over 20 years ago, Brown believed that one day his work to identify the genes implicated in the inherited form of ALS would yield new insights into the more common, sporadic form of the disease. These findings are the first evidence that genes implicated in inherited ALS also play a role in the sporadic form of the disease.

In 2011, UMMS and Brown were joined in this effort by former Massachusetts Gov. and Ambassador to Canada A. Paul Cellucci, who announced in January of that year that he had been diagnosed with ALS and was being treated at UMMS by Brown. Cellucci chose to use his story as a catalyst to support ALS research at UMMS through establishment of the UMass ALS Champion Fund, now the Cellucci Fund.

“I am proud to continue my career in public service by leading the UMass ALS Champion Fund,” Cellucci said at the time. “A champion helps to deliver great victories, and Dr. Brown is a true champion of ALS research.”

Cellucci died from ALS complications in 2013 at age 65. In 2014, the fund was renamed the UMass ALS Cellucci Fund to honor his commitment to the cause.

The 2016 running will be Benyo and DiMarzo’s second Boston Marathon, eighth marathon together, and Benyo’s 45th marathon. DiMarzo was diagnosed with ALS in December 2010, just six months after the couple was married. Before the diagnosis Benyo and DiMarzo had already started training to run marathons together. DiMarzo’s new wheelchair, provided by Team Hoyt, was built by Southbridge Tool & Manufacturing in Dudley, Massachusetts.

There are no pressure points, which was a problem DiMarzo had with a previous marathon chair. Benyo, who will be pushing his wife along the 26.2-mile route from Hopkinton to Boston, explains in a UMMS release, “This chair has been made to our specifications, and I’m confident it will be perfect for her.”

Michael DiDonato of Team Hoyt Running Chairs says he and his co-workers draw inspiration from the runners and their riders.

To learn more about the 2016 UMass ALS Cellucci Fund Boston Marathon team and meet the other members — Jaime Miller, Terry Nash, Kerri Floramo, and Angela Mioglionico — visit the UMass ALS Cellucci Fund websites at
https://www.umassals.com/
and
https://www.umassmed.edu/advancement/umass-als-cellucci-fund/.

Also visit UMass ALS Cellucci Fund 2016 Boston Marathon Team.