What ALS means to me

ALS, also known as Amyotrophic Lateral Sclerosis, is a terminal disease that causes the neurons (nerve cells) in the brain to break down all muscles in the body as well as breathing and talking. The famous baseball player for the Yankees, Lou Gehrig, died of ALS in 1936 and this disease is often named after him (Bear et al, 2001, p. 432). Here’s a breakdown of the scientific name:
A: meaning “no” or “negative”
Myo: refers to the muscles
Trophic: refers to nourishment

Therefore, Amyotrophic stands for “no nourishment to the muscles.” The word “lateral” is the area in the spine where the brain tells the muscles what to do. Lastly, the word “sclerosis” which is often heard with Multiple Sclerosis, stands for hardening. As neural signals from the brain and spinal cord send messages to the muscles that have sclerosis, the messages are stopped due to the “non-absorbable” muscle tissue. In the picture below, you can see that the axon is attached to the muscle. Nerve fibers feed off the axon and contract when triggered by a signal. With ALS, sclerosis hardens on the nerve fibers and blocks the signal from permeating through the membrane to the muscle causing degeneration (Medicaluniya, 2012). The meaning of ALS is very important for patients and families to understand, as well as how it begins to affect the body.

Normal nerve cell and muscle vs. ALS-altered nerve cell and dying muscle.
Normal nerve cell and muscle vs. ALS-altered nerve cell and dying muscle.
In 2005, my family received the news that my Papa was diagnosed with advanced stages of Lou Gehrig’s disease. For us, this was not only a shock, but something that would ultimately turn into a life changing learning experience. Like many others in the world, this was the first time we had encountered ALS and Lou Gehrig’s disease, so we were walking into the situation blind. We were told what to expect many times from many different doctors but nothing quite prepares you for the mental and physical deterioration until you experience it firsthand.

Our family made the decision after we found out about the diagnosis to move my Papa into our house, the doctors had warned us numerous times that he would need more and more special care as the disease progressed. In the beginning, he was still my Papa, he still would pester my cats, he could still sit on the back porch and smoke a cigarette, and he would still try with all of his might to get my brother and myself to watch Alfie with him. Slowly, my Papa began to experience changes, the changes that we were warned of at the doctors, one day he was fine (or so it seemed in my eyes) and the next day he could not walk to the bathroom on his own. He was physically falling apart in front of my eyes; our next step was to get him a motorized wheelchair. You see with a disease that is so physically deteriorating, you have to do everything in your power to try and let the person suffering have what little independence they have left. For us, it was the motorized wheelchair and giving Papa my brother’s room, so he would have a space of his own and feel more comfortable in our home.

Papa and I on prom night.
Papa and I on prom night.
Maybe I was slightly naïve at the time, even after hearing the prognosis from the doctors, but I was still convinced that one day my Papa would get out of his wheelchair. I was not sure how or why something so debilitating had to happen to someone I loved so much, but I was hopeful for a cure. Sadly ALS is not something you recover from, it is something that ultimately takes your life. For my Papa he only continued to worsen after we had to get him the motorized wheelchair. At first it was little changes like his mood over silly things such as getting him the wrong sauce for his fish, then it was bigger; his speech was all but absent. My mother had become a master at reading his lips and was able to help him communicate with us, thankfully. It was not really until the last few months of his battle with his disease that we all came to realize that the only positive outcome from all of this was going to be that he would not have to suffer anymore. In his final months, his breathing was labored, the only thing we could do was place him in hospice until it was his time to go.

While there is still no exact testing for ALS, there are ways to help doctors give a more accurate diagnosis. For this a doctor must rule out other similar diseases and would then be able to tell you if you have the qualifications of ALS. With my Papa, he had suffered from a couple of strokes before receiving the diagnosis of ALS. Many military men who are veterans, like my Papa, also have a higher risk of getting this disease making it a “service-connected disease” (ALSA, 2015). I’ve always wondered if this disease could ever affect someone who has never served in the military or been a spouse of someone who has. This disease mainly affects men (due to their service), however it is not impossible for females to get ALS.

The disease is also considered to be sporadic, as in not hereditary, according to the ALS foundation site (ALSA, 2015). Since there is no exact testing for this disease, there is a part of me that wonders if I or any of my family members could possibly get this terrible disease. The ALS researchers have been making strides in finding biomarkers that would help with earlier diagnosis and possible treatment for anyone might suspect having ALS (ALSA 2015). To care for someone with ALS, is not only hard on the soul but also tough on the pocket. Many households who take care of loved ones with this disease tend to spend a quarter of a million dollars on medical needs, doctor’s visits, and housing care (ALSA, 2015). Coming from experience, money has no factor on taking care of someone that you love in their last days. It is also important to remember that not only the person with ALS is suffering but the loved ones caring for the person affected also are grieving in their own way, and need special attention to help them cope with changes they are watching their loved ones go through on a daily basis.

Although this may be a rare disease among many others out in the world, this is a fatal and quick one. Doctors usually put patients in “stages” according to their illness, but with ALS, patients are given less than five years to live. My Papa lived with ALS less than two years. He also had stage 4 emphysema. To have a disease take you by storm so quickly, it is like dumping a bucket of ice over your head and having your breath taken away. This is how the huge YOUTUBE ice bucket challenge came to be. It was meant to bring awareness to the disease and to raise money for not only testing but hoping to one day find a cure so that maybe one day families will not have to deal with losing someone so quickly and painfully. I’m very proud of anyone who supported the Ice Bucket Challenge and also donated to the ALS foundation so that sooner than later, people that are living with this horrible disease can have a cure. My Papa did have a cure though. He had his family by his side at all times. He accepted Jesus, an answered prayer of my mom’s, and he went with dignity. Although it is still very hard for me to talk about, like I said before, this was one of the greatest learning experiences of my life. I now know about a disease that not everyone has the chance to learn about, and having experienced it first hand, it only makes me want to help support and educate others even more. I will forever support the ALS foundation and their journey of finding a cure!

Here’s an Ice Bucket Challenge video compiled from many users from the ALS Association website

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References
What is ALS? (2015). About ALS. Retrieved on June 8, 2015 from ALS Association http://www.alsa.org/about-als/what-is-als.html

Bear, Mark F., Conners, Barry W., Paradiso, Michael A. (2001). Neuroscience: Exploring the Brain. Baltimore, MD: Lippincott Williams & Wilkins

1st Picture: Leung, Jeremy. (2010, September 4). ALS (Amyotrophic lateral sclerosis). Retrieved on June 8, 2015 from http://len.epfl.ch/webdav/site/len/shared/import/migration/ALS1.jpg

2nd Picture: owned by self

The ALS Association. (2014, September 17). Ice Bucket Challenge: Thank You from The ALS Association. [Youtube]. Retrieved on June 9, 2015 from https://youtu.be/ObC0lcC-hLA?list=UUQD7Myz4Ssut-TNOM_N5HAw

Medicaluniya. (2012, May 21). Lou Gehrig’s Disease – Effects of Lou Gehrig’s Disease. [Youtube]. Retrieved on June 8, 2015 from https://youtu.be/pOvvW8gbWSA

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6 thoughts on “What ALS means to me

  1. What a beautiful, heartwarming, educational and insightful blog! I honestly think this is the greatest blog I’ve ready yet. I’m sorry for the loss of your Papa, but what a beautiful tribute to him to educate others and to participate in the Ice Bucket Challenge. I remember when the kids were doing this last summer – my son included – but I had no idea what it was REALLY for. I knew it was “ALS” but unfortunately, I did not understand what the disease entailed. Your blog and video was so eye-opening. I truly enjoyed this post.

    Liked by 1 person

  2. Just to reply to all three of you, I did do some research to see if cigarette smoking or heavy metal exposure could possible increase the risk of ALS. What I found in a study done in the 1980’s (I’m an 80’s baby), was that many females who were either former or current smokers had a higher risk of getting ALS than men who were former or current smokers (Weisskopf et al, 2004). When doing this study, the researchers found that the increased risk in women was caused by hormonal effects, such as oral contraceptives or menopausal issues. The researchers found that men did not have this problem (obviously) and that even with increased packs of cigarettes per day/week did not effect the increasing risk of ALS (Weisskopf et al, 2004). I actually find it very odd that this study showed women being the higher of the two genders. With women, back in the 1980’s staying at home majority of the time unless out at a bar or concert, I would have thought men were the higher exposed due to work environments.
    The study also did mention that men with ALS who also smoked, could be exposed to oxidative stress and heavy metals from the cigarette or in their occupation. My Papa was a veteran in the 1980’s but did serve time when he was younger and started smoking when he was a preteen. I don’t know which war(s) he served in exactly but I do know slightly from memory that the times were hard and the Navy camps were tough. In the article I will post at the end and where my info has come from, you can scroll down to the “Discussion” section and I found that to be extremely helpful with the questions you all have raised.
    Thanks!

    Reference
    Weisskopf, M.G., McCullough, M.L., Calle, E.E., Thun, M.J., Cudkowicz, M., Ascherio, A. (2004). “Prospective Study of Cigarette Smoking and Amyotrophic Lateral Sclerosis.” Am. J. Epidemiol, 160 (1): 26-33. doi:10.1093/aje/kwh179

    Here’s the link for a quicker look:
    http://aje.oxfordjournals.org/content/160/1/26.full

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  3. What a heartbreaking story, Victory. Thanks for giving us all such a personal view of ALS and the effects that it had on your life. I have heard and read about ALS, but I do not have any personal experience with the disease. All I really knew about it was that it is a disease that causes your body to deteriorate and then eventually takes your life. After reviewing some articles online, I did discover that some researchers believe that it could be caused by a defective gene that inhibits the body from producing the right amount of superoxide dismutase, which is an enzyme. Without the right amount of this enzyme, free radicals are able to damage body tissue. Of course, they mentioned a host of other possible causes such as exposure to heavy metals and fertilizers. Another study reported that glutamate may be overstimulating nerve cells to the extent that the nerve cells die, eventually causing ALS (webmd). It seems as though there are a lot of questions that need to be answered about this disease so that we can effectively treat the disease. I’m glad the Ice Bucket challenge was such a huge success. I hope it generated a lot of money to be put into researching and studying the cause and effects of this disease.

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  4. Thanks for sharing such a personal story. It’s quite a mystery as to what causes it. To expand on the chemical theory, a pretty high percentage of veterans smoke. There is a higher percentage of people who smoke in the military compared to the general civilian population. Amongst male veterans, more older veterans smoke as apposed to younger veterans. We know that there are tones of chemicals in cigarettes. Maybe there is a correlation between smoking and ALS?
    http://www.cdc.gov/tobacco/campaign/tips/resources/data/cigarette-smoking-in-united-states.html

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    1. I’m actually not sure but I will definitely check into it today. My papa started smoking as a preteen and all throughout his life until he lived with us with ALS. It’s an interesting thought and I’ll definitely get back to you either on here or through email!

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  5. Thank you for sharing your experience with us, Victoria. I know the heartbreak never ends. My father-in-law’s decline from ALS was unusually rapid, and he passed away within months of his diagnosis. The possibility of a genetic link makes me worry about my husband’s health. There does seem to be an environmental aspect as well. My father-in-law was exposed to a lot of chemicals through his work, and what you mentioned about veterans makes me wonder whether they were exposed to chemical warfare. This article talks about a possible correlation between repeated exposure to formaldehyde and later development of ALS:

    http://www.sciencedaily.com/releases/2008/04/080416151952.htm

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