The Middle Aged Woman Who Lived in a Shoe: Our ALS Ice Bucket Challenges

The news and social media have been buzzing with The ALS Ice bucket Challenge. Because there has been such tremendous support there has of course been lots of accolades and just as much criticism. As of August 27, 2014 the Ice Bucket Challenge has raised 94.3 million dollars for ALS in the USA and in Canada we have raised $11, 698, 759.00 (I think that this number could have been higher, but some Canadians unknowingly made donations to the American ALS). The criticisms range from alleged misuse of funds (they contend that very few dollars actually go toward ALS research, but rather to CEOs) to graphs showing how many people die of ALS vs those who die from cancer, or heart disease. These are all valid points except.........

The above picture is of my father-in-law Don Lindsay. He died of ALS March 14 of 2009, he was 66. This picture was taken after his body had begun to deteriorate on him, but before he needed an air mask to allow his lungs to breath for him. In his younger years he was an athlete and was scouted by major league teams. He was the guy that would mow the grass and shovel the driveways of the elderly widows in his neighbourhood right up until ALS took that from him. He loved to sing Barbershop and was in a critically acclaimed group called "Area Code 519", until ALS stole his music. He and my mother-in-law Doreen were married for 46 years. He had four kids and was generous with his time and money with all of them. I remember when we first bought our house he took time off work so that he could come through for a few days and help Christopher paint the house. Not only did he help paint the house, he bought the paint. He was a good man, and ALS robbed us of him!

Let me answer some of the criticism, in my humble opinion. Prior to the ALS Ice Bucket Challenge, had you ever heard of ALS? I have taken the liberty of copying some information from the ALS site.

Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's Disease," 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.

A-myo-trophic comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment–"No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.

As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look "thinner" as muscle tissue atrophies.

Symptoms of ALS

At the onset of ALS the symptoms may be so slight that they are frequently overlooked. With regard to the appearance of symptoms and the progression of the illness, the course of the disease may include the following:

muscle weakness in one or more of the following: hands, arms, legs or the muscles of speech,

swallowing or breathing
twitching (fasciculation) and cramping of muscles, especially those in the hands and feet
impairment of the use of the arms and legs
"thick speech" and difficulty in projecting the voice
in more advanced stages, shortness of breath, difficulty in breathing and swallowing

The initial symptoms of ALS can be quite varied in different people. One person may experience tripping over carpet edges, another person may have trouble lifting and a third person's early symptom may be slurred speech. The rate at which ALS progresses can be quite variable from one person to another. Although the mean survival time with ALS is three to five years, many people live five, ten or more years. In a small number of people, ALS is known to remit or halt its progression, though there is no scientific understanding as to how and why this happens. Symptoms can begin in the muscles of speech, swallowing or in the hands, arms, legs or feet. Not all people with ALS experience the same symptoms or the same sequences or patterns of progression. But, progressive muscle weakness and paralysis are universally experienced.

Muscle weakness is a hallmark initial sign in ALS, occurring in approximately 60% of patients. Early symptoms vary with each individual, but usually include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches and/or uncontrollable periods of laughing or crying.

The hands and feet may be affected first, causing difficulty in lifting, walking or using the hands for the activities of daily living such as dressing, washing and buttoning clothes.

As the weakening and paralysis continue to spread to the muscles of the trunk of the body the disease, eventually affects speech, swallowing, chewing and breathing. When the breathing muscles become affected, ultimately, the patient will need permanent ventilatory support in order to survive.

When Don first started with symptoms he would have muscle spasms. It took a long time to get a diagnosis. They knew that something was wrong, but it took a long time to put a name to it. He would drop things, that was very unlike him. Then came the diagnosis, and the bottom fell out of everyone's world... ALS. At first the deterioration seemed slow. We had heard that the deterioration would be rapid. The average person diagnosed with ALS's life expectancy is 2 years after they receive their diagnosis. The deterioration at first seemed slow, he couldn't open jars, it then pushed through like a speeding freight train. He lost so much weight, that it was a constant challenge to get enough calories into him. He went from requiring a breathing mask at night, to requiring a breathing mask all the time to survive. Each time we would go to visit, the difference was heart breaking. We watched this independent man who helped everyone, become confined to a chair and then a bed, and then he was gone.

In Canada, the ALS Society's largest single fund raiser is their "Walk For ALS". The annual revenue for this event is $3.6 million dollars ($1 million of this coming from the Halton Chapter where my Mother-in-law Doreen is a huge supporter). As a matter of fact the annual ALS Canada budget is $7 Million Dollars, $14 Million if you include the combined ALS Societies for each province as well as Canada. ALS Canada uses their funds to give grants to researchers. The provincial societies help the families. This is not only a devastating disease, it is an extremely expensive disease. Don and Doreen had to make their home wheelchair accessible, that is expensive. The society provided Don's breathing machine, and loaned them a hospital bed. They gave them information, put them in touch with doctors. It is no accident that it is called The ALS "society". They provide a social outlet for those people who have been devastated with a very little known disease.

Now for the criticism about the amount of donations to this cause vs the amount of individuals who die from ALS. If you saw someone you loved suffer through ALS, you would not be so pompous. It is a vicious disease that steals everything from it's victims. There is no cure, they don't even really know what the cause is. Because this is considered a "rare" disease it is underfunded.

I think that the Ice Bucket Challenge is marvelous! It has brought ALS to the public's attention. It is fun and brings out the spirit of competition. It is an internet sensation. Although the nay sayers have been quick to criticize this campaign they have forgotten the most important part of this... it's a fad. This time next year everyone will remember that crazy fad, but ALS donations will return to their previous numbers. This is a one time phenomena. A one time phenomena that is allowing ALS to stand in the light for just a brief moment.