ALS is now synonymous with the Ice Bucket Challenge. Before the challenge made nationwide news, the only other way people had heard ALS was through the famous pitcher Lou Gehrig. Most had never heard of ALS, but had heard of Lou Gehrig disease.
The Ice Bucket Challenge has done a great job in raising awareness. Unfortunately, a lot of the awareness was about the ice buckets, and less about the disease, and what the challenge was all about.
ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.
Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control.
There are two different types of ALS, sporadic and familial. Sporadic which is the most common form of the disease in the U.S., is 90 – 95 percent of all cases. It may affect anyone, anywhere. Familial ALS (FALS) accounts for 5 to 10 percent of all cases in the U.S. Familial ALS means the disease is inherited. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease. French neurologist Jean-Martin Charcot discovered the disease in 1869.
Recent years have brought a wealth of new scientific understanding regarding the physiology of this disease. There is currently one FDA approved drug, riluzole, that modestly slows the progression of ALS in some people. Although there is not yet a cure or treatment that that halts or reverses ALS, scientists have made significant progress in learning more about this disease.
ALS usually strikes people between the ages of 40 and 70, and approximately 20,000 Americans can have the disease at any given time (although this number fluctuates). For unknown reasons, military veterans are approximately twice as likely to be diagnosed with the disease than the general public. Notable individuals who have been diagnosed with ALS include baseball great Lou Gehrig, Hall of Fame pitcher Jim “Catfish” Hunter, Toto bassist Mike Porcaro, Senator Jacob Javits, actor David Niven, “Sesame Street” creator Jon Stone, boxing champion Ezzard Charles, NBA Hall of Fame basketball player George Yardley, musician Lead Belly (Huddie Ledbetter), photographer Eddie Adams, entertainer Dennis Day, jazz musician Charles Mingus, former vice president of the United States Henry A. Wallace, U.S. Army General Maxwell Taylor, and NFL football players Steve Gleason, O.J. Brigance and Tim Shaw.
ALS shattered the life of a Holly Springs mother in August of 2011, when her son Louis was diagnosed with the disease at the age of 29. The former Lacrosse captain had just gotten engaged 3 days prior to the diagnosis.
In addition to his passion for lacrosse, he was an all around athlete, and played hockey and football as well. He was forced to quit his job at the date of diagnosis, leaving Lou wondering if the disease progressed quicker without working his physically taxing job as a concrete foreman. Could there be correlation with athleticism and the development of ALS? There were some symptoms for months before the MRI, but he was still walking and talking freely. His first symptoms? Tripping and falling at work. He began falling while playing in his weekly hockey league. His legs no longer able to support him in skates. He also fell suddenly at home, shattering a glass coffee table. His voice garbled at times and a fall from a four foot wall on a job site left them wondering what it could be.
The disease started to show itself quickly.
At 3 months, he had difficulty tying his shoe. At 6 months, Louis would need a wheel chair to get around. It became hard to walk and stairs required great strength and endurance. At one year, his deterioration evident in his speech and in his limbs.
One year after his diagnosis, Lou and his wife Nicole were expecting.
Lou lost his battle with ALS in November of 2014, just over 3 years after his initial diagnosis. During the biggest snow storm in Buffalo’s history, he took his last breath. A hero in his courageous battle, a hero to his daughter Zoey Hope, and a hero to the Buffalo firefighter who received Louis’ kidney. Despite his struggle with the disease, he always kept his sense of humor, and was able to make his mark.
From Buffalo to Long Island to North Carolina, TEAM DiGiacomo fought ALS nonstop. Last summer, rallying the Buffalo Bills, the only professional NFL team to take the #ALSicebucketchallenge in honor of Louis DiGiacomo!
Lou’s mother, Karen Bessel DiGiacomo has made it her mission to do her part to help raise money and awareness of this awful, debilitating and deadly disease. She spend a lot of time canvasing, and will talk to anyone and everyone to get her message out. Although she has lost her son, she’s determined to help others battling the disease.
The typical cost of someone battling ALS is roughly $200,000 annually.
Team DiGiacomo has events locally in Holly Springs to raise money to help fund the effort to find a cure. You can find their website at
http://loveforlouis.com
