In the last two weeks, the Ice Bucket Challenge has quite literally “soaked” the nation. Everyone from Ethel Kennedy to Justin Timberlake has poured a bucket of ice water over his or her head and challenged others do the same or make a donation to fight ALS within twenty-four hours. Between July 29 and August 12, The ALS Association and its 38 chapters have received an astonishing $4 million in donations compared to $1.12 million during the same time period last year. The ALS Association is incredibly grateful for the outpouring of support from those people who have been doused, made a donation, or both. Contributions further The Association’s mission to find a cure for ALS while funding the highest quality of care for people living with the disease.
“We have never seen anything like this in the history of the disease,” said Barbara Newhouse, President and CEO of The ALS Association. “We couldn’t be more thrilled with the level of compassion, generosity and sense of humour that people are exhibiting as they take part in this impactful viral initiative.” With only about half of the general public knowledgeable about amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s Disease, the Ice Bucket Challenge is making a profound difference. Since July 29, The Association has welcomed more than 70,000 new donors to the cause.
“While the monetary donations are absolutely incredible,” said Newhouse, “the visibility that this disease is getting as a result of the challenge is truly invaluable. People who have never before heard of ALS are now engaged in the fight to find treatments and a cure for ALS.” Currently, there is only one drug approved by the U.S. Food and Drug Administration (FDA) to treat ALS, which only modestly extends survival by two to three months. Consequently, ALS is 100 percent fatal. In addition to acclimating to the challenges that come with losing control of voluntary muscle movement, people with the disease progressively lose their ability to eat, speak, walk, and eventually breathe. The Ice Bucket Challenge has done a lot of good. Let’s make sure the next one is even better.
[su_highlight background=”#1c58eb” color=”#fafcfc”]WHAT IS ALS[/su_highlight]
Amyotrophic lateral sclerosis (ALS)—also referred to as motor neurone disease (MND), Charcot disease, and, in the United States, Lou Gehrig’s disease—is a neurodegenerative disease with various causes. It is characterised by muscle spasticity, rapidly progressive weakness due to muscle atrophy, difficulty in speaking (dysarthria), swallowing (dysphagia), and breathing (dyspnea). ALS is the most common of the five motor neuron diseases.
ALS was first found in 1869 by French neurologist Jean-Martin Charcot, but it wasn’t until 1939 that Lou Gehrig brought national and international attention to the disease. Ending the career of one of the most beloved baseball players of all time, the disease is still most closely associated with his name. Amyotrophic lateral sclerosis (ALS) 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 death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
Most commonly, ALS strikes people between the ages of 40 and 70, and as many as 30,000 Americans have the disease at any given time. ALS has cut short the lives of other such notable and courageous individuals as Hall of Fame pitcher Jim “Catfish” Hunter, Senator Jacob Javits, actors Michael Zaslow and David Niven, creator of Sesame Street Jon Stone, television producer Scott Brazil, boxing champion Ezzard Charles, NBA Hall of Fame basketball player George Yardley, pro football player Glenn Montgomery, golfer Jeff Julian, golf caddie Bruce Edwards, British soccer player Jimmy Johnstone, 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 and U.S. Army General Maxwell Taylor.
[su_highlight background=”#1c58eb” color=”#fafcfc”]SYMPTOMS[/su_highlight]
The earliest symptoms of ALS are typically obvious weakness and/or muscle atrophy. Other presenting symptoms include trouble swallowing, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. The parts of the body affected by early symptoms of ALS depend on which motor neurons in the body are damaged first. About 75% of people contracting the disease experience “limb onset” ALS, i.e., first symptoms in the arms or legs. Patients with the leg onset form may experience awkwardness when walking or running or notice that they are tripping or stumbling, often with a “dropped foot” which drags gently along the ground. Arm-onset patients may experience difficulty with tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock. Occasionally, the symptoms remain confined to one limb for a long period of time or for the whole length of the illness; this is known as monomelic amyotrophy.
Over time, patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski’s sign also indicates upper motor neuron damage. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles that can be seen under the skin (fasciculations).
Although respiratory support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within three to five years from the onset of symptoms. The median survival time from onset to death is around 39 months, and only 4% survive longer than 10 years. Physicist Stephen Hawking has lived with the disease for more than 50 years, though he is an unusual case.
In late stages the oculomotor nerve that controls the movements of the eye, can be affected as can the extraocular muscles. The eye movements remain unaffected largely until the later stages due to differences in the extraocular muscles compared to the skeletal muscles that are initially and readily affected.
There is a known hereditary factor in familial ALS, where the condition is known to run in families. A defect on chromosome 21, which codes for superoxide dismutase, is associated with approximately 20% of familial cases of ALS, or about 2% of ALS cases overall.] This mutation is believed to be transmitted in an autosomal dominant manner, and has over a hundred different forms of mutation. The most common ALS-causing mutation is a mutant SOD1 gene, seen in North American patients; this is characterized by an exceptionally rapid progression from onset to death. The most common mutation found in Scandinavian countries, D90A-SOD1, is more slowly progressive than typical ALS and patients with this form of the disease survive for an average of 11 years.
No test can provide a definite diagnosis of ALS, although the presence of upper and lower motor neuron signs in a single limb is strongly suggestive. Instead, the diagnosis of ALS is primarily based on the symptoms and signs the physician observes in the patient and a series of tests to rule out other diseases. Physicians obtain the patient’s full medical history and usually conduct a neurologic examination at regular intervals to assess whether symptoms such as muscle weakness, atrophy of muscles, hyperreflexia, and spasticity are getting progressively worse.
[su_highlight background=”#1c58eb” color=”#fafcfc”]Public Awareness Campaigns[/su_highlight]
This year’s ALS Awareness Month Campaign allows people with ALS and their families to raise awareness of Lou Gehrig’s Disease and share what they hope for in a world without ALS. The theme is “Create a world without ALS. Speak up now to give hope.” Every individual tells their own personal story and how the disease has impacted their loved ones and them.