Daniel’s ALS Journey, A Not-So-Short Narrative
It’s been two years since the onset of ALS. It’s hard to believe – it feels longer…
Here’s a recap of my Journey that will bring you up to date with what’s going on.
How it began
It was the middle of June, 2012, an ordinary morning on an ordinary day. I had showered, blow dried my hair, grabbed the can of hairspray, and depressed the angled spray button when I first noticed it. The usual amount of pressure from my right forefinger tip wouldn’t depress the button. I had to position my fingertip on TOP of the button to get it to spray. “Interesting,” I thought, “it’s a tad sore, maybe I hurt it working out.”
Then, a couple weeks later in July, I noticed that, when I attempted to clip the fingernails on my left hand, the clippers wouldn’t cut - Dull… or so I thought. When I switched hands and found no issues clipping the fingernails on my right hand, I realized that something was not right. Five months, three neurologists, three very painful sets of tests and two misdiagnoses later, Pherron and I were given the news: I had the “Classic” form of ALS, the limb-onset variety that attacks the dominant arm and hand first, before spreading to the other arm and legs. That was December 18, 2012, around 6:02 pm.
Stunned, Pherron and I went home and jumped on our computers, each of us researching
anything and everything on ALS. We learned that, according to one Italian study, there are
eight different “phenotypes” of ALS. The one I have, “Classic”, has a median survival time of 2 years, 8 months. This means that half of those with Classic ALS phenotype were still alive at the 2 year, eight month point. For those with the “Bulbar” onset, the phenotype that strikes the muscles above the shoulders, the median was less – two years. Bulbar strikes the tongue, soft palate, lips, facial muscles, and neck muscles, to name a few. People with Bulbar lose their ability to speak, chew, swallow, suck on a straw, kiss… After reading that, we were relieved that my onset wasn’t Bulbar.
After the initial shock
Initially there was all the shock, profound sadness and various stages of mourning that one would expect. Sixty days later, while I continued to mentally process my new reality, I regained equilibrium and took up the Fight. I pursued alternate strategies for slowing disease progression: the “Deanna Protocol” – www.winningthefight.net. I asked my doctor for an authorization for medical cannibals because it has been shown to have neuro protective, anti-inflammatory and pain relieving qualities. BTW, I don’t smoke it, I use a vaporizer. Anything that made sense, I was going for it.
“Bucket Trip” in Europe
At the one year anniversary of the ALS onset, June, 2013, Pherron and I went on a two month “Bucket Trip”, spending time with my French family in Southern France and exploring Barcelona, Munich, Paris, London, Edinburgh and Dublin. A good friend was very gracious and gave us the keys to her apartment in the 3rd Arrondissement of Paris, thereby allowing me to fulfill my lifelong dream of living in France. As many of you know, I had a French half-brother, now deceased, whom I visited many times. Thus, I just love speaking French and my ability to speak it got better and better during those two months. Toward the end of the trip, however, in late July, changes were taking place in my mouth and my speech was beginning to slur.
Seattle Walk to Defeat ALS – September, 2013
Pherron and I learned that ALS is extremely rare: in the US alone, just 6,000 people are diagnosed each year. Additionally, in the US, there are fewer than 30,000 with the disease at any one time. Because ALS is considered an “orphan disease”, few funds are available for research. “Big Pharma” and Federal monies focus on diseases with larger target populations.
We were shocked to learn that, 72 years after Lou Gehrig succumbed to the disease that bears his name, there still is no test for ALS! My diagnosis was made after all the diseases that mimic the symptoms were excluded, leaving ALS on the table! This is really a sobering reality. Thus, ALS research is largely sponsored through fund drives and private donations like the annual “Walks to Defeat ALS”.
We put our hearts and souls into that fund raising event. It gave my family and friends an outlet to express their grief as well as an opportunity to do something about it, by creating a greater public awareness of this hideous disease.
I walked the three miles in Seattle’s beautiful Seward Park unassisted. My family and many
friends walked in support of me and the other PALS – Persons with ALS. Our team, “DANIEL LIVES!” raised $12,832! To all of you who contributed, THANK YOU!
I vowed that day to do the 2014 Walk, hopefully unassisted.
The “Bulbar Affect” – It wasn’t supposed to be this way!
After that Walk, the “Classic” ALS seemed to have slowed way down. While my right arm and hand dangled limply from my shoulder, my left arm and legs remained strong. I thought that the Deanna Protocol and the inhalation of cannabis were actually having an effect in slowing this Beast’s progression. My left arm and hand had adapted, taking over what the right ones used to do. I could safely drive, I could eat as long as the food was cut for me, and I could shower and dress myself. Life was good, having relegated the ALS challenges as “inconveniences”.
About Christmas, the disease’s progression took an unexpected and dire change of direction. Remember the “Bulbar” phenotype that Pherron and I were relieved I didn’t have? Well, it arrived, big time, and started wielding its nastiness above the shoulders, in the area that it claims for itself.
Speech
By March of this year, while I believed I could speak, no one could understand me. I started using an iPad text-to-speech app to communicate. In one-on-one interactions, the conversation would go back and forth, with my tapping out my thought and the other person expressing hers. However, when conversations involved more than one other person, it got crazy. I may have had something to contribute but, by the time I was ready to have the text-to-speech engine play what I typed, the discussion had moved on to other topics making what I wanted to say moot or irrelevant. Fortunately, I am surrounded by people who are lovingly patient. For example, at a recent gathering, the person sitting beside me saw that I was ready to “speak” and called for everyone’s attention saying, “Okay, Daniel has something to say.” With the room silent, I pressed PLAY and the synthesized voice did its thing. It was awkward but it worked.
Food and Drink
March was also the month that chewing and swallowing became problematic. I decided, wisely as it turned out, to have the Mic-Key feeding port placed in my abdomen. I believed at the time that I was early with the port and would continue to eat normally. However, because I was becoming increasingly “chewing and swallowing-challenged,” it was taking longer and longer for me to eat. Because I desired a life outside of slowly feeding my face, I decided to ingest two meals via the feeding tube each day, reserving the third for eating anything I wanted, call it “Recreational Eating”.
Choking Episodes
It wasn’t long before violent and desperate choking episodes would begin. I’d take a bite of food, take a sip of wine and would suddenly begin choking, unable to inhale. I would jump from the table, run to the nearest sink, lean over and cough until I could inhale once again. One time, unable to breathe, I started to pass out before the coughing cleared the airway – that was a close one!
During this period, my salivary glands became overly productive, not uncommon for PALS, which felt like a stream of liquid was pouring into my mouth. Normally, these secretions would be unconsciously swallowed but, because of the swallowing issues, they would pool in my mouth until suddenly overflowing into the airway. These episodes began to occur more frequently, 20 to 30 times a day.
Pneumonia
Unable to sleep due to these episodes, I was exhausted and debilitatingly weak, so sick I didn’t know how I could go on. Told by a nurse practitioner that this was all ALS-related, we delayed seeing a doctor, but did start a course of antibiotics to knock out any unwanted bacteria that might be lurking in my body. I also had a chest x-ray. When the x-ray was read a few days later, it revealed that I had pneumonia, walking pneumonia. With the antibiotics on board, I began to feel better.
Most PALS succumb to respiratory issues caused by the slow paralysis of the diaphragm
muscles. These muscles not only expand the chest to pull air into the lungs, they’re also
instrumental in clearing the airway and lungs via the cough.
Eating and Drinking Are Out – So Much for “Recreational Eating”!
When I did visit a doctor to discuss the pneumonia, a specialist evaluated my swallowing mechanics. I was informed that my tongue, soft palate and lips were paralyzed and had atrophied. Therefore, any time there was something in my mouth - food, liquids, including saliva, I was at risk of choking to death or aspirating something into my lungs causing pneumonia.
It was strongly recommended that I forgo eating and drinking and to use the feeding tube for
100% of my nourishment and hydration. To avoid swallowing the excessive salivation, I was
prescribed several medication to reduce the amount and given a portable suction machine to be my constant companion.
Accelerating Progression – A Real Game Changer
For a time, it was all about Bulbar but recently, the Classic has resumed its debilitating course.
My left arm and hand are so weak that I find the iPad very heavy. Trying to text is ridiculous
with a hand that no longer has fine motor skills – I love your texts. Keep’em coming! I may not be able to text you back. I can no longer shower or dress myself without assistance. Can no longer hold and manipulate the Sonicare toothbrush – the same with the television controller.
Fatigue, oh the fatigue. It’s always there, in the morning and throughout the day and evening. While I do nap, sleep doesn’t reduce its grip.
My legs are going. I can’t walk in a straight line. When I do walk, I tend to shuffle. Stairs are a real concern: a fall would not be good. A powered wheelchair is just around the corner.
That said, I hope to be able to amble part of the 2014 Seattle Walk to Defeat ALS in Seward Park on Saturday, September 13th!
With these legs, getting into the car is challenging. With these hands, it’s difficult to fasten my seat belt. But if I can get by those obstacles, starting the car and manipulating the foot pedals are no problem… Steering might be, however. Be assured, I’ve hung up my keys!
My face is slowly becoming paralyzed, the most obvious impact being on my smile. The good news is that now I wear my smile on the inside and, if you look closely, you can still see it in the twinkle in my eyes :).
How am I dealing with all of this psychologically?
Better than I thought.
Following the diagnosis, there was a period of shock, of “Why me?”, “I’m actually going to DIE!”, “This world will go on without me?” and “It’s not FAIR!” At some point during that time, after I realized that “fairness” doesn’t exist in the natural order of the Universe, I had an epiphany: “Okay, the ALS Beast has declared he’s going to destroy my body. Fine, while he’s not going to get it without a fight, there are two things that the he can’t steal from me: my dignity and my mind’s ability to control my perception of this reality.
So I resolved not to waste my precious time feeling sorry for myself for, whenever I find myself going there, it scores one for the Beast! Whenever I mourn the loss of things I could do yesterday or imagine how ugly it’s going to be tomorrow, it racks up points for the Beast. Sure, I’ve had my emotional “moments,” but I quickly move away from those dark places. I don’t want him run up the score! I shake my head once or twice and get back on my game!
Looking at all the “inconveniences” that I must face as a result of the disease’s debilitation is
definitely challenging - God how I miss eating and drinking, tasting and swallowing!! - BUT I have developed a strategy to deal with it.
Consider this analogy: Imagine your FM music system has a persistent hum and your favorite song comes on. If you let the hum annoy you, chances are you’ll not enjoy your song: the hum will be all that you hear. But if you were to change your focus away from the hum and move the song to the forefront…
The music is my JOY of living each day and, obviously, the Beast is the hum. As my body wastes away and the hum gets louder, I will need to boost the Joy. How will I do that? By expressing gratitude, smiling, joking, laughing and doing the things that produce joy.
And there are so many things to be grateful for. I live as an American in the Western World (Reading the news, I am sure I’m not alone with that sentiment.) I am fortunate to have been able to get a first class education that has opened doors to incredible experiences and financial reward.
I have wonderful friends who take me to baseball games, or walks on the beach, or to their
homes for stimulating one-on-one conversations. People are traveling to visit me, a few from far away. My niece traveled with her family from Montpellier on the French Mediterranean. The mother of my other French niece will soon be flying in from Toulouse. Cousins from Orange County, a niece from Maine, wonderful friends from Portland and Salt Lake City – these are the things that make me smile, that provide joy.
I’m married to my very best friend and the love of my life. We are very proud of Devon and Michaela, both very focused on developing themselves while displaying solid values along the way. I’m blessed to be a member of a large, loving family both here and in France. While everyone has been traumatized by what’s happening, all of us have pulled more closely together. With death on the doorstep, we all look at one another in a different way, with the realization that being alive is a gift that is temporary in nature.
As I’ve traversed this life, I have crossed paths of many hundreds of people, a few have become friends - former colleagues, healthcare professionals, neighbors and parents from our 25 years living on Mercer Island. I am truly a mosaic, comprised of the influence, the impact that each one had on me. I’ve been blessed with a richness of life for which I am enormously thankful.
I’ve noticed that when I’m grateful, I can’t help but smile. When I do, there is a release of
endorphins, natural pain killers, and serotonin that feel warm and relaxing. And the amazing
thing is that, when I smile, the world seems to smile right back at me.
Pherron Muchmore-Mulhaney – Uber-Wife, Uber-Partner and now, Uber-Caregiver
When we said our vows that included the words, “Until death do us part,” we never envisionedthat mine would be such a drawn out and laborious affair. But it is, and Pherron has stepped up to the challenge. She spends three to five hours each day preparing and delivering my “feedings”- the only way for me to receive the nourishment and hydration that I need. Pherron washes me, dresses me, combs my hair, brushes my teeth, the list goes on. Each time she does I nod in gratitude and we exchange smiles. She grabs my face between her hands, looks into my eyes and reassuringly says, “I love you. I’m here for you. I’m not going anywhere.” Of course, that brings tears to both of our eyes.
I’ve been told how courageous, how inspirational I am. Pherron, however, is the one who really deserves those accolades, for she not only has to take care of herself, she has the added burden of taking care of me. All the household responsibilities that I used to do, have been shifted to her shoulders. Without her, I’d be lost.
And courage? All the dreams of travel, freedom and lightness we both had envisioned for our senior years have evaporated, gone. But even with all this, all the stress, she manages to hold it all together. I married a very strong woman.
So, in closing this not-so-short narrative, I would like to share something with you that I posted on Facebook:
Pherron,
All of me loves all of you.
Thank you for giving me all of you, especially in these challenging times.
I'm so grateful.
I will love you eternally.
Daniel
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