Entering into mainstream consciousness through the “ALS Ice Bucket Challenge”, ALS or Amyotrophic lateral sclerosis (also known as Lou Gehrig’s disease after the famous baseball player), is a motor neuron disease that adversely impacts the nerve cells that control the muscle of the body (Miller, Brooks, Swain-Eng, Basner, Carter, Casey & Tolin, 2014). Based on my personal experience with the disease, it can state that it manifests through neural degeneration resulting in the subsequent death of the nerve cells over time.
What must be understood is that the motor neurons that control the body are indispensable due to their function of enabling a person to move, breathe, speak, chew and swallow (McCluskey, Geser, Elman, Van Deerlin, Robinson, Lee & Trojanowski, 2014). During the period that I was unaware that I had ALS, I noticed that sometimes had some difficulty in grasping objects, as if my hand all of a sudden lost its strength and there were other instances where I had trouble breathing while I was lying down.
While my speech was relatively fine before my diagnosis, I noticed that a year after it seemed as if I had a slight speech impediment wherein what I was saying sometimes came out slightly garbled or it was a bit slow. I did not understand it at the time and merely chalked it up to my body acting weirdly, however, over time the problems associated with my ability to speak and my hand strength slowly worsened. I became concerned over what I perceived was a deterioration of my physical abilities and went to a doctor to get diagnosed.
When looking back at my condition, I began to realize that if degeneration of the motor nerve cells occurs, the result is a person being unable to function properly resulting in eventual death. ALS can thus be described as a “wasting disease” since it manifests slowly over time through gradual muscle weakness and eventual paralysis. When my doctor informed me that I had ALS, I initially had no idea what to expect. I thought it was some sort of condition that could be treated over time through the use of a variety of medications.
The truly frightening aspect of the disease that I learned from my doctor is the fact that while it adversely impacts the motor neurons of the body, it does not affect an individual’s memory, intellect, or 5 senses. As a result, a person becomes fully aware of what is happening to their body up till the point that they become paralyzed and become unable to move (Hansen, Bedore, Nickel, Hanowski, Tangen & Goldish, 2014). Essentially, should the disease continue to progress unimpeded, I will become trapped up till the point that the full functionality of my motor neurons stops resulting in my untimely death.
To say that I was frightened is an understatement, I was terrified of the diagnosis I was given. When presented with the finality of death, most people would break down into a form of hysteria and I, unfortunately, was not an exception to this state. I tried to get a second, third, or even a fourth opinion hoping that the doctor was wrong in some way. Regrettably, all the doctors came to the same conclusion that I had ALS and there was nothing I could do about it.
The doctors informed me that the progress of the disease can be described as “relentless” given the rapid nature of symptom progress which can drastically reduce a person’s life expectancy from 2 to 5 years once they have been identified as being affected by the disease (Jones, Jivraj, Balendra, Murphy, Kelly, Thornhill & Al-Chalabi, 2014). No one can truly be prepared to receive a life-threatening condition with the inevitability of death on the horizon. I did not ask to receive the disease, yet I, unfortunately, got it. In my search for answers, I delved deeper into the world of ALS to find out more about it.
Symptoms of the Disease
Based on my research, I learned that there is no known cure for ALS, and the only approved medicine for it, namely Riluzole, is only a temporary measure that only helps to stave off the full effects of the disease for a few months (Dietary intake of fruits and beta-carotene is negatively associated with amyotrophic lateral sclerosis risk in Koreans: A case-control study, 2014).
This is one of the reasons why I realized that it is important for people to immediately get diagnosed if they feel that they have symptoms related to the disease since a few extra months of life are incredibly precious when it comes to saying goodbye to your friends and loved ones while also putting your affairs in order. As you can imagine, the fact that there was no known cure weighed heavily on my mind and resulted in me trying to determine what would happen to me over time. The fruits of this labor came in the form of the following list which details the symptoms of the disease:
- Muscle Weakness – one of the first symptoms of ALS is related to muscle weakness in the body. This is a direct result of the deterioration of the nerves. The weakness normally begins in the hands and feet then slowly progresses to encompass the entirety of a person’s arms and legs (Morgan & Srivastava, 2014).
- Twitching and Cramping – another symptom of the disease is a mild form of twitching and cramping in the arms and feet. This is believed to be the result of nerves misfiring due to their deterioration (Yu, Su, Callaghan, Goutman, Batterman & Feldman, 2014).
- Speech problems – a person with ALS would eventually develop a problem with their speech resulting in a “thick” form of speaking and they would even have problems projecting their voice.
During the more advanced stages of the disease, ALS patients find that they develop shortness of breath (i.e. they cannot breathe properly) and they find it difficult to swallow food. This is due to the deterioration of the nerve clusters associated with the proper function of these activities.
What Helped to Feel Understood, Supported, Valued, and Resilient?
I believe that support from family, friends and other loved ones is necessary to remain strong against the disease. While it is true that ALS will eventually kill me, this does not mean that I should give in to despair and just wait for the end. I believe that it is important to become closer to your loved ones during this period and appreciate what has happened to your life so far. The disease may be taking your life in the future, but this does not mean that it takes everything that has happened to you.
Your trials, your joys, and everything that you have shared with your friends and family experiences that you can truly treasure when you find out that your time is short. Aside from this, it is important to note that merely focusing on the disease just makes things worse. What is needed is in this case is to focus on what you can do to make sure that you die without regrets. This can take the form of trying out activities that you originally were hesitant to do so, going to places that you planned to visit but never actually went to as well as an assortment of other things that you can do to live a more fulfilling life before the end comes.
It should also be noted though that some individuals with ALS volunteer their time towards various advocacy organizations to generate funding and awareness. The reason behind this is quite simple, by getting more people to know about ALS and actively trying to solicit donations for research towards finding a cure, ALS sufferers who volunteer their time begin to feel that they are doing something positive with their lives and that is what they do will have some form of legacy for the future. It is this desire to have some type of purpose to their existence that results in a greater degree of mental resilience to the threat of their impending death.
What is Stressful or Frightening about the Experience?
To be honest, the problem is not the fact that I will die, that is a given since there is no known cure for ALS, rather, how I will die is what stresses me the most. People always seem to think that dying is the worst thing that can happen to them, I say that a quick and painless death would be fantastic as compared to slowly feeling your impending death as you start to lose control of your motor functions.
It is the awareness that you will die slowly that gets to you; that you may eventually find yourself being unable to move, speak, or do anything yet still having the capacity to be fully aware of everything that is happening. The mere fact that you will be aware is one of the most frightening aspects of a slow death since everything that you could have done normally will no longer be accessible to you.
You will merely lie, eyes wide open as your body refuses to move while you merely stare forward in utter and complete hopelessness with the only thing to look forward to being your eventual death when your motor neurons finally deteriorate to the point that you are no longer able to breathe. The mere fact that such a future awaits me truly frightens me to the core and I am even reminded of an episode of Scrubs (a comedy/drama series that was set within a hospital) where one patient was suffering from ALS planned to commit suicide. She reasoned that she did not want to suffer a fate where her life was no longer her own to do with as she pleased.
She did not want to be trapped within the confines of her own body while she became a burden to her family. As such, she wanted to overdose on the medication she was given for ALS while she still could make a choice. For me, this is the essence of my experience with ALS wherein the future that awaits me is equally as bleak. While I may be able to stave off the full effects of the disease, I know that it is only a temporary measure.
Eventually, it will start as a form of numbness in my arms and legs, then it will progress to a sense of fatigue that permeates my entire body. I will start to feel lethargic and numb, be less willing to move, and then, I will be confined to a hospital bed as I slowly start to be unable to move my arms, legs, and body. This sense of impending absolute isolation within my own body is a depressing inevitability, so much so that I somewhat agree with the action of the patient from Scrubs were choosing to end it early would be a far better experience than to feel myself slowly slipping away and going crazy within the confines of my mind.
Access to information
Based on my own experience when it came to wanting to know more about my condition, I realized that when it comes to a patient’s patient’s psychosocial adjustment to chronic illness, actually knowing all facets about the disease and what is to come is far better as compared to not knowing at all. What you have to understand regarding humans, in general, is that we fear the unknown; however, once something becomes known and understandable, we no longer fear it (Pagnini, 2013).
The same set of conditions applies to when a person is suffering from a degenerative disease. I believe that people want to know and should know what to expect so that they can prepare for it instead of merely fearing what might happen to them. It should also be noted that the family and friends of a patient would like to know what is going to happen to their loved one. As such, information plays a critical role in the grieving process since it would allow both patients and their loved ones to properly prepare for the inevitable (Pagnini, 2013).
Influence Access to Acute and Ongoing Care
If a patient knows about what is going to happen to them, they would likely attempt to seek proper medical care to at least ease the symptoms of their condition. For example, during the more advanced stages of the disease, ALS patients find that they develop shortness of breath (i.e. they cannot breathe properly) and they find it difficult to swallow food. This is due to the deterioration of the nerve clusters associated with the proper functioning of the muscles connected to the throat and lungs. Knowing this information, people with ALS would attempt to contact the nearest hospital for some sort of care or to hire a caregiver to see to their needs.
Getting Help for the Disease
While I was one of the “lucky ones” to have an immediate ALS diagnosis, there are others who are not as fortunate. The reason behind this is that one of the inherent problems with ALS is that it is difficult to diagnose since there is no single test available that can immediately tell if a person has ALS or not. A proper and comprehensive diagnostic for ALS is normally composed of the following procedures:
- An X-ray and MRI image is taken to evaluate the current state of the nerves and muscles
- a nerve conduction velocity test (NCV), as well as electromyography test (EMG), is conducted to determine whether neural degeneration has occurred
- A myelogram of the cervical spine is conducted to determine whether the nerves are firing properly or if there is a degeneration in the nerve clusters there
- a biopsy (i.e. getting a sample of the muscle and nerves) is conducted to determine whether the muscle or nerves have degenerated
- a normal spinal tap examination is conducted to assess the condition of the spine
- Lastly, a neurological examination is conducted to assess the overall responsiveness of the patient
All in all, these procedures are some of what can be done to diagnose ALS, however, as mentioned earlier, since what is done is merely a process of elimination instead of an absolute confirmation, the discretion of a proper physician is necessary to properly diagnose the disease. In other words, a general physician may not be the most appropriate when it comes to determining whether a person has ALS or not. In the absence of a proper diagnosis, though in my case a general physician was able to diagnose it, however, this was merely an exception since the doctor had experience with ALS patients in the past.
The following are links to appropriate clinics, testing centers, and support organizations that are recommended by the ALS Association that I have used in the past. These organizations have the necessary experience and facilities to properly determine whether a person has ALS or not and, as such, it is recommended that if your diagnosing physician thinks you have ALS, it is important to get a second opinion through these organizations to get absolute confirmation.
- Links to a directory that has a list of diagnostic centers that have experience in diagnosing people with ALS
- Contains a comprehensive list of clinics that have the necessary specialists to properly diagnose ALS
- List of local ALS chapters that can help a person find a local physician that knows how to perform a proper diagnosis of ALS
- Contains a contact form that enables someone to communicate with the ALS foundation to obtain the necessary resources to find a local physician that can help in determining whether you have ALS or not
Given the sheer amount of contacts these sites contain, finding an adequate physician should be simple enough. Do note though that a diagnosis involving ALS should always have a second opinion due to the potential for clinical errors. Given the lack of an absolute test for the disease, a diagnosis, in this case, is more of a subjective opinion rather than absolute confirmation, and, as such, a second or even third opinion must get absolute confirmation.
Dietary intake of fruits and beta-carotene is negatively associated with amyotrophic lateral sclerosis risk in Koreans: A case-control study. (2014). Nutritional Neuroscience, 17(3), 104-108.
Hansen, A., Bedore, B., Nickel, E., Hanowski, K., Tangen, S., & Goldish, G. (2014). Elastic head support for persons with amyotrophic lateral sclerosis. Journal Of Rehabilitation Research & Development, 51(2), 297-303.
Jones, A. R., Jivraj, N., Balendra, R., Murphy, C., Kelly, J., Thornhill, M., & Al-Chalabi, A. (2014). Health utility decreases with increasing clinical stage in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 15(3/4), 285-29
McCluskey, L. F., Geser, F., Elman, L. B., Van Deerlin, V. M., Robinson, J. L., Lee, V. Y., & Trojanowski, J. Q. (2014). Atypical Alzheimer’s disease in an elderly United States resident with amyotrophic lateral sclerosis and pathological tau in spinal motor neurons. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 15(5/6), 466-472.
Miller, R. G., Brooks, B., Swain-Eng, R. J., Basner, R. C., Carter, G. T., Casey, P., & Tolin, F. P. (2014). Quality improvement in neurology: Amyotrophic Lateral Sclerosis Quality Measures. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 15(3/4), 165-168.
Morgan, R. H., & Srivastava, A. K. (2014). Clinical relevance of stem cell therapies in amyotrophic lateral sclerosis. Neurology India, 62(3), 239-248.
Pagnini, F. (2013). Psychological wellbeing and quality of life in amyotrophic lateral sclerosis: A review. International Journal Of Psychology, 48(3), 194-205.
Yu, Y., Su, F., Callaghan, B. C., Goutman, S. A., Batterman, S. A., & Feldman, E. L. (2014). Environmental Risk Factors and Amyotrophic Lateral Sclerosis (ALS): A Case-Control Study of ALS in Michigan. Plos ONE, 9(6), 1-9.