Well it is May again. Five years since Jon's diagnosis and on the 27th it will be 3 years since he passed away. It does not get any easier for his family. If anything it gets harder with each passing year. I am reminded of a day at work when I caught a sweet exchange between an elderly mother and her son and thought I will never know that...I always thought we would grow old together.
I have been writing little snippets of memories on Facebook for the Ironman Germany 2010 Blazeman Foundation site. Each and every tale is so fresh in my mind. Jon was a "handful" growing up. He had so much energy and sometimes that energy got him into trouble with his parents. As he grew older and finished college he set off for California to teach and found the school he called home at Aseltine.
Over these past few years Bob and I have come to know of so many young, healthy and energetic young people that have been diagnosed with this horrific disease. I never know what I will find each day when I open my computer. Why has this disease gotten so little press and research dollars? It is quite disheartening to read about the disease that have treatments and even some that are quite preventable that continue to receive funding. It is time to change that!!!!!
There is a lot going on in our foundation these days and in the coming months we will share everything we are working on...stay tuned! Thanks to all your wonderful efforts there is more movement in the world of research...I firmly believe it will be the grassroots movement that will crack this disease...I have always felt that...right from the beginning. That said most of this newsletter will be related to ALS research and stories and I will report more on the races in next month's newsletter.
Bob and I just came back from Ironman St. George where we spoke to more people at the expo than ever...witnessed at leased a dozen rolls and two marriage proposals. Blazeman Warriors Yair Shalev #179 and Fausto Rodriques were among the rolls and Fausto made one of the marriage proposals! Jon was definitely looking down and smiling that beautiful smile of his.
We look forward to seeing you next month at Rev3 Quassy and the EagleMan 70.3. The Blazeman Spirit Award will be given to a worthy applicant at EagleMan where there are 20 select BMF slots.
As I asked last year...on May 27th at 8:30PM a shout for FREEDOM will be appreciated for all the PALS who have gone before us and for those we are seeking a cure.
In Strength, Honor and Freedom,
Mary Ann Blais
Blazeman Foundation for ALS
(We recently received a letter from Jana Ross, a Boston Marathon runner whose life has been touched by ALS. We were so inspired by her story that we wanted to share it with you, our readers.) In Jana's words;
"The marathon was an amazing experience. It was incredible to see how far you can push yourself and what you can accomplish when you're committed to achieving your goal. I have always been involved in sports but my love for running truly grew after my mom was diagnosed in September 2008. Running became a way to keep me calm and provided me some sense of peace. As my mom gradually lost her physical capabilities, I had the desire to run further and longer simply because I feel lucky that I can. My favorite mantra that I always repeat to myself when I hit a low point is that "I get to run." I think it's easy to complain about all the things you "have to do" but then I think about all the people, my mom included, that would give anything to be able to go out for a three hour run. Ultimately, I have no control over my mom's illness but I can control how I'm going to respond to the situation my family has been dealt. This is our reality now and all we can do is make the best of it! She is an amazing person whose strength inspires me to appreciate every day I have. She made the eight hour drive to Boston with my dad to see me accomplish what I've been training for the past year, and it meant everything to me to have the most important people in my life supporting me."
"Running is often considered a solitary or individual sport but I've found that the running community is a special breed. People who are not runners really cannot understand the happiness it gives you. When you cross the start line of a race that thousands of people have dedicated their lives to train for, all differences fade away. Throughout the 26 miles, I had various runners tap me on the shoulder and ask about the Blazeman Foundation simply because they care. There is nothing more inspiring than being surrounded by a community of all different types of people, each with their own personal struggles, and working together to reach the finish line."
Blazeman Warrior, Jenny Hansen, a 17 yr old from California started training five months ago for the Wildflower 70.3 on May 1st to honor her basketball coach Nancy who has been battling ALS. Jenny embarked on a very successful fund-raising campaign, raising a considerable amount of research funds for the Blazeman Foundation.
Not only did Jenny complete the Wildflower half-ironman distance triathlon, she broke the 11 year old course record for 17-19 year old. She was the only female participant under age 20 to attempt and finish the race. We are proud to say, the new course record in the F 17-19 year old group is 6 hours, 26 minutes and 58 seconds.
More importantly, the day was a wonderful celebration of determination and spirit. She raced in her Blazeman gear and rolled across the finish line to salute ALS patients, to raise awareness, and to raise money to find a cure. Before the race, Jenny decided that she would give her finishers medal to Nancy. Congratulations, Jenny. You are an inspiration to us all!
On Saturday, April 3rd the Hartford Track Club along with race director Kelly Burns, under sunny skies and warm temperature had a great race with 500 runners. The race charity was the Blazeman Foundation for ALS. Our daughter Jennifer and her family attended the race.
Thank you all for choosing the Blazeman Foundation for ALS as your continued charity!
Northwestern University Research Updates
Blazeman Foundation ALS stem cell project underway Thanks to all the Blazeman Warriors and supporters!!!
We are very excited to report we have begun collection of our first skin samples from ALS patients for use in transformation to pluripotent stem cells. These stem cells in turn are capable of generating motor neurons. The study will compare molecular level activities of these motor neurons from patients with different types of ALS and different presentations and courses of ALS. We will see whether there are differences between these activities in neurons from patients with younger vs. older ages of onset, from patients with a shorter vs. longer course of disease, from patients with different genetic mutations, and so on. If such differences are found it may be possible to use that information to develop treatments that delay onset and slow or arrest symptom progression. This very exciting, cutting edge work is being funded by a grant from the Blazeman Foundation, founded in memory of triathlete Jonathan Blais, who died from ALS at age 35.
The President of Northwestern University recently thanked the Blazeman Foundation for their support. Read his letter to Robert Blais here. Also, see all of Northwestern's press releases here.
More Recent Findings at
Northwestern University Researchers from Northwestern University Feinberg School of Medicine have discovered a link between sporadic and familial forms of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease also known as Lou Gehrig's disease.
Researchers found that a protein called FUS forms characteristic skein-like cytoplasmic inclusions in spinal motor neurons in most cases of ALS. Mutations in this gene have been previously linked to a small subset of familial ALS cases. Researchers thus linked a rare genetic cause to most cases of ALS, clearing the way for rational therapy based on a known molecular target.
ALS is a disease in which muscle-controlling nerve cells in the brain and spinal cord (motor neurons) die, resulting in rapidly progressive paralysis and death usually within three to five years of the onset of symptoms. Most cases of ALS are of unknown etiology and appear as sporadic ALS. About 5 to 10 percent of ALS cases are familial. Some forms of familial ALS are caused by genetic mutations in specific genes. Mutations in the Cu/Zn superoxide dismutase gene (SOD1) account for approximately 20 percent of familial ALS cases. Mutations in the TAR DNA-binding protein gene (TDP43) and FUS gene occur in about 4 to 5 percent of the familial ALS cases. Altogether, mutations in specific genes have been identified in about 30 percent of familial ALS cases. In contrast to familial ALS, the etiology and the pathogenic mechanisms underlying sporadic ALS -- 90 percent of all ALS - has remained largely unknown. Understanding the causes and pathogenic mechanisms of sporadic ALS is the major challenge in this disease.
For this study, researchers examined the post-mortem spinal cords and brains of 100 cases, 78 with ALS and 22 in a control group. They found FUS pathology in the spinal cords of all the ALS cases, except for a few cases with SOD1 mutations. But FUS pathology was not present in control cases without ALS.
"This is a game changer because it establishes a connection in the development of sporadic ALS with a known cause of familial ALS," said senior author Teepu Siddique, M.D., the Les Turner ALS Foundation/Herbert C. Wenske Professor of the Davee Department of Neurology and Clinical Neurosciences at Feinberg and a neurologist at Northwestern Memorial Hospital.
"Our finding opens up a new field of investigation for rational therapy for all of ALS," Siddique added. "This is the holy grail of researchers in this field."
"There hasn't been a therapy for most of ALS, because the cause was unknown," Siddique said. "Three genes have been identified in ALS, but the problem has been connecting inherited ALS to sporadic ALS."
"We identified the FUS pathology in sporadic ALS and most familial ALS cases," said Han-Xiang Deng, M.D., associate professor of neurology at Feinberg and lead author of the paper. "The patients with the FUS pathology may account for about 90 percent of all ALS cases. Our findings suggest that pathological interaction of FUS with other proteins is a common theme in motor neuron degeneration in the vast
majority of the ALS cases. We believe that this is a major step forward in formulating a common pathogenic pathway for motor neuron degeneration. Importantly, it may offer a novel avenue for developing therapies through targeting these FUS-containing inclusions."
The one exception to the new finding is when familial ALS is associated with a mutation on the SOD1 gene. In those patients and in the mutant SOD1 transgenic mouse models, researchers did not find evidence of FUS pathology.
"This tells us that it follows a different pathway of pathogenesis, so treatment for this form of the disease would have to be different," Deng said.
A Warm Welcome to our newest Warriors!
We are pleased to welcome the following newest warriors to our family. Thank you, and everyone else, for all you do to help our mission.
Dr. Jeffrey Galvin