This is part 1, of 3-part series of reflections on my journey with ALS.
I recently had a birthday and reached 44 years of life on this planet. Although more pertinently, I had reached the 10-year milestone with ALS.
ALS is a neurological disease that destroys the motor nerves responsible for controlling all the voluntary muscles in the body. That is, the muscles for movement, eating, speaking and breathing. Both the nerves in the motor cortex in the brain and the spinal cord are affected. Once the nerves die, the muscles that they control will atrophy and, therefore, lead to full paralysis. Generally, patients have a 2 to 5-year prognosis. However, some die faster, while others last longer; but these are the minority. There’s no known cause, treatment or cure. Care is usually palliative.
Reaching the 10-year milestone was a bitter-sweet occasion. Bitter, because I am still deteriorating with paralysis, towards a distressful and undignified end, with no cure in sight.
It’s also sweet because I’m still intellectually and professionally functional. Moreover, I’ve had time to reflect:
• on what it is to feel the dying, and,
• how to remain rational and purposeful, through the constant deterioration and paralysis, towards death.
Generally, from these sad reflections you may ask two questions: how do you do it, or, why bother?
I will reflect on this existential quagmire in three parts.
This first reflection will be about the physical ALS process and how it affects my life.
The second reflection will expose the emotional tsunami of the constant loss in dying of ALS. Particularly, how it feels every day, to have that notion at the forefront of every moment. Because it’s a deteriorative disease, it never stops taking something away. It’s always reminding you, that you’re one step closer to its goal.
The third and last piece will be about how I live, despite the abysmal prognosis. After all, the only reason to write this online is so it’s read, shared, and helps someone, somewhere, to keep going. Whether that’s you, or someone you know. Moreover, I hope to help those involved with someone with ALS to understand what’s happening and be of genuine service. That is, know what THEY need to survive psychologically, and thereby, keep going. I hope, therefore, it’s a source of strength in any capacity the way its helped me.
I was in the prime of my life at 34.
After so many years of personal struggle to find my passion, I was finally enjoying an incredibly fulfilling career as a manual therapist in Structural Integration. I was teaching bodywork workshops nationally and abroad, trained in Aikido three times a week, and feeling extremely fulfilled. I had a great life.
By the time I received my diagnosis in late 2005, I had already suspected something was seriously wrong. In fact, I remember asking the Neurologist if it was ALS, even before I had received confirmation from the battery of tests. I had been experiencing an unshakable exhaustion all the way back from 2000, regardless of how much sleep or rest I had. For the year before diagnosis, I had constant and increased muscular twitches on my neck, right shoulder, and arm. But none of that was as alarming as the growing weakness in my right arm and the occasional, but increasingly regular, tripping.
It’s truly a bitch of a disease.
It started off slowly. I wouldn’t be able to sustain a grip on my partner’s gi, during BJJ and Aikido training. Considering how strong my hands were from years of Gymnastics, Martial Arts and Manual Labour, this was an initially confusing problem that I was attributing to age.
I would drop objects, and tire after a short session of writing or drawing, which I did in my free time. But the most disconcerting sign of dysfunction was in my clinic. My hand would weaken quickly and cramp when I was working on someone. I thought that maybe this was a case of Thoracic outlet syndrome; whereby the nerves and blood vessels of the arm become entrapped in the junction between the neck and shoulder. However, it wasn’t going away, despite all the bodywork and therapy I had to release tension in the area.
The loss of my beloved career was such an emotional blow that I found it almost impossible to recover from. I loved being a manual therapist. I mean, it was my entire universe. I had found my calling in Structural Integration. It wasn’t work; it was my life. “Work” was what happened when I was away from anything related to my vocation. I treasured that sacred space with my clients. That space where my hands would mould their connective tissues into an optimal state.
It slowly, but surely, systematically stripped me of independence. The paralysis of the arms and hands meant I couldn’t eventually even feed, clothe, groom, wash, write or perform any manual function myself.
The atrophy of my tongue and speech muscles meant that from initially sounding drunk or mentally challenged, it progressed to not being understood at all. This made fluid conversation impossible. The frustration at not being able to speak and be understood, or be able to write because my hands can’t grip a pen is indescribable.
Strangers assuming I’m mentally challenged when I slur my speech is confronting. But it’s heartbreaking and demoralising, to slowly watch friendships slip away because the ability to simply chat is gone. What were once passionate and vivacious conversations, are now predictable, one-sided, and awkward moments. Both parties implicitly realising, the camaraderie once sustained through particular activities or interests, is ebbing away. How can you have a face to face conversation with someone that you can’t understand?
I was becoming a desolate observer in my life. A silent passenger in a strange and foreign body.
Speech loss was just the beginning. With my mouth muscles gone, eating actions such as chewing and swallowing, were also a problem. I could no longer use my tongue to turn and move food in my mouth, as is done automatically in chewing. Nor use my tongue to push the food back to be swallowed.
I have to chew with my mouth open, so as to be able to move the food around to be chewed and swallowed. If you’ve ever watched someone eat with their mouth open, and their sticky wad of a meal being rolled around in their mouth with saliva, you know how disgusting it is to look at. As you can imagine, watching me eat wouldn’t be a pleasant experience. Which brings me to the next annoyance: saliva.
With ALS comes this thick, gelatinous saliva, that is continuously flowing due to the nerve firing on the muscles surrounding the salivary gland. This saliva leaves a coat all over the inside of the mouth, and the back of the throat. Food particles often become trapped in this salivary web at the back of the throat, and over the airway. This causes involuntary coughing, gagging, and choking with such ferocity, that at times, I felt myself blacking out. It’s a terrifying thing to experience and disconcerting to witness.
Because I can’t feed myself, I need to be fed. Being fed and eating separately from the table, saves others from being put off their food from watching me eat. Furthermore, it’s best to eat in another room due to sanitary reasons from coughing, and potentially spluttering saliva and food, all over their meals if I choked. This, however, meant further disconnection from family and friends at not sharing a meal together. Therefore, as you can understand, I chose always to eat privately, and away from others.
I also can’t walk unaided. I can stand and shuffle if I’m holding onto someone for a few metres, but otherwise, I require a wheelchair to move around. My legs have been the slowest and will be the last to deteriorate to the point of uselessness. This is handy because it means I can still be assisted to walk to the bathroom, saving me from another blow to personal dignity. Besides that, an electric wheelchair takes care of mobility quite adequately.
Lastly, there’s the weakening of other body muscles.
As the motor nerves die, so too do their corresponding muscles lose function and atrophy. My back muscles, for instance, are becoming weak to the point that my spine sticks out, and I can’t hold myself up for too long. The neck muscles, are also too weak to lift my head if I’m lying on my back. Most dangerously though are the weakening of the respiratory muscles.
Most ALS patients die primarily from complications due to respiratory muscle problems. Firstly, there’s the distinct and terrifying prospect of not being able to breathe adequately on one’s own, and slowly suffocating to death. For some, mechanical ventilation will be required just to stay alive, as the chest muscles are too weak to draw air in.
However, the main danger is an infection. Whether viral or bacterial, an infection in the respiratory system can be the quickening of the end. The body is too weak to cough or blow out phlegm and any foreign material. Thus, it sits in there festering and re-infecting. Each infection, causing more harm and further weakening the system than the last. Eventually, pneumonia sets in and finishes the job.
You would think with all this going on, that we’d get a moment of peace during sleep; a reprieve from the physical symptoms. Not so. The motor nerves don’t stop dying then either. In fact, they seem to be felt at a higher intensity. Whether it’s because of the horizontal position increasing circulation to the head, or the change in brain neurotransmitters, one thing is certain; sound sleep is not on the cards. Besides being awoken with violent spasticity at any slight movement in position, there’s also the muscle cramps.
These are incredibly painful. A dying nerve will fire a burst of excitatory neurotransmitters, which will make the corresponding muscles contract so hard that they feel like stone. That cramp is bothersome in three ways. They’re constant and frequent throughout the night and day. They will sometimes cramp together with its opposite muscle; so stretching one out to relieve it will increase the other muscle’s pain. Lastly, being unable to reach down or stretch it yourself, means having to rely on someone doing it for you. If there’s no one, then you’re in for an excruciating time.
Sleep deprivation then becomes part of your life. Usually, I’d be awoken every two hours, every night. No exaggeration. I haven’t slept a solid, deep, uninterrupted, rejuvenating 8-hour sleep in over eight years. The problem of sleep is compounded by the fact that although ALS means paralysis of movement, it doesn’t mean paralysis of sensation. We feel everything. Furthermore, as the muscles wither away, the joints become weaker and more exposed, from the lack of muscle mass and support. So, laying on one’s side, compresses the hip and shoulder joints, irritating the bursa (a fluid-filled sac between the tendon and a joint). The weaker the muscles get, the more the joints come under stress and strain, causing further discomfort. It never ends.
Every pALS (patient with ALS), will experience its deterioration differently. Some will have symptoms start in their arms, others in the legs, and the rest in the mouth. Wherever it starts, that’s the function they’ll lose first. For me, it was the arms and hands. For some, they’ll lose the ability to walk first. Others, it’s the speech, swallowing, and breathing. If you’re “lucky”, a function will remain unaffected while the rest deteriorate. Some will keep their speech and eating function perfectly, while the rest withers away. Others can walk but will have no upper limb or speech function.
In the end, however, each of us will have differing environmental and social hurdles to overcome; while the daily battering of symptoms and terminal status take their toll on the body and mind. It’s a physical and emotional onslaught that can strip away at the spirit, leaving us bitter, despondent, devoid of hope, and even suicidal. It’s the psychological storm that I’ll be exploring further in the next reflection.
See you soon.
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