I’m Going on a Trip…

I’m planning a trip – not to Paris or Rome… not that kind of trip. This will be a journey to explore my inner world with the help of psilocybin (magic mushrooms). Let me explain – last summer a friend mentioned that she had a friend who was taking psilocybin as end-of-life therapy. Last fall and winter I did some online research and reading as I knew nothing at all about psilocybin; although I came of age in the 1970s, I never dabbled in hallucinogenics.

Psilocybin is a hallucinogen that works by activating serotonin receptors, most often in the prefrontal cortex. This part of the brain affects mood, cognition, and perception. Psilocybin-assisted psychotherapy is the professionally guided use of psilocybin in combination with psychotherapy.

This will be a therapeutic journey for me, in other words, the intent is not recreation. The treatment involves pre-session therapy, therapy during the psilocybin experience and an integration process afterward. I am working with a local psychiatrist who is experienced in treating with psilocybin.

I’ve learned a lot about the potential of psilocybin in therapy, especially ‘end-of-life’ therapy, and found Michael Pollan’s book quite useful (How to Change Your Mind: What the New Science of Psychedelics Teaches Us About Consciousness, Dying, Addiction, Depression and Transcendence). He states that, “existential distress is what psychologists call the complex of depression, anxiety and fear common in people facing a terminal diagnosis.” Apparently, there have not been very successful ways of treating this existential (or end-of-life) distress which contributes to a sense of demoralization and hopelessness.

In studies at NYU and John Hopkins, “some 80% of cancer patients showed clinically significant reductions in standard measures of anxiety and depression”, leading to an increased quality of life, life meaning, death acceptance and optimism. Pollan further states “…the great gift of the psychedelic journey, especially to the dying, is its power to imbue everything in our field of experience with a heightened sense of purpose and consequence.”  In the two studies mentioned, most patients rated their psilocybin experience in the top ten experiences of their lives, which is remarkable.

So how does it work? My understanding is that psychedelic drugs don’t ‘add’, they ‘clear away’ what is unnecessary.  The drugs are not the healing, rather, they help the patient do the healing themselves.

We all have habitual ways of thinking; psilocybin helps to break these habits of thinking and offers opportunity for a reset – of feeling more in the present and more connected. Patients in the two studies mentioned above typically described the experience  as mystical and spiritual.

Pollan writes that “the boundary between the conscious and the unconscious realms of the mind and the boundary between self and other…  (when) these boundaries fade or disappear, as they seem to do under the influence of psychedelics, we can let go of rigid patterns of thought, allowing us to perceive new meanings with less fear.” I have included a link to TheraPsil’s website which has content about psychotherapy using a psilocybin and links to research.

However, psilocybin is illegal (since 1974 in Canada); a federal exemption is required to access the drug for therapeutic purposes. B.C.-based non-profit TheraPsil last August helped a cancer patient secure the first exemption under Canada’s drug laws to use magic mushrooms for psychotherapy. Since then, Federal Health Minister Patty Hajdu has used the power she has under Section 56 of the Controlled Drugs and Substances Act to grant 35 patients and some health-care professionals limited exemptions from the law. The  process for applying and proving a medical need to use psilocybin is not clear, and the criteria used by Health Canada appears arbitrary (see link to Tyee article below). This is an inconsistent process; some people have been given exemptions and others have not, with no clear reasons for the decisions. Furthermore, the process is dependent on political party positions, elections or change in ministers. There is a need for government to come up with reasonable and transparent regulations for making psilocybin legal and available for medical purposes. TheraPsil continues to assist and advocate for patients wishing to access the drug.

I have received an exemption from Health Canada, and I was surprised to learn from Therapsil that I am the first Canadian with ALS to receive an exemption. 

So why would I want to have this experience?

I try very hard to stay positive and be proactive concerning all aspects of my health including my emotional state. But it’s hard, and it’s getting harder. It is a struggle to stay upbeat when my body is incrementally losing function in a way that greatly impacts quality of life. Simple tasks like dressing and moving around are becoming exhausting. Since losing the ability to speak it has become a challenge to stay connected to others. Now I can only type with one hand which has further reduced my ability to communicate with a keyboard – it is slow and tiring to peck away with one finger. Loss of connection, isolation, feeling left out – these are constant struggles, along with trying to get the #%&* mucus out of my mouth. These challenges can easily feed into depression.

Even though I may think and believe in a particular point of view that is positive and hopeful, as I try to do, unfortunately my feelings may not always align with that perspective. While I make these constant shifts as my body weakens in phases, my emotions fight against these changes. So why wouldn’t I want to access a treatment that can improve my emotional sense of wellbeing for my remaining time? I believe that psilocybin-assisted psychotherapy can help me to gain perspective regarding my future and create greater self-awareness and sense of connectedness.  The research is solid and feedback from participants is extremely positive. I am grateful for TheraPsil for providing this opportunity.

My ‘mushroom trip’ is booked for early September – I will report back after, but don’t expect any souvenirs.

My photos for this post are from my trip to Scotland two years ago with my daughter Rachel. These are the Callanish Stones, older than Stonehenge, located on the Isle of Lewis in the Outer Hebrides, my ancestral home on my father’s side of the family. It is an otherworldly place that had a real impact on me. Many thanks to the wonderful Kara Smith for taking us there at dusk..

(https://therapsil.ca/ TheraPsil – non-profit that works to provide access to psilocybin for therapeutic purposes. Excellent website with loads of information

https://thetyee.ca/News/2021/07/05/Fight-Medical-Use-Magic-Mushrooms-Moving-To-Court/ Good article from The Tyee that sums up current status of psilocybin

How to Change Your Mind: What the New Science of Psychedelics Teaches Us About Consciousness, Dying, Addiction, Depression and Transcendence” Michael Pollan. Penguin, 2018.

Gratitude

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Read the list below and see if you can do any of these things:

Do up the buttons on your shirt

Kiss your child

Cut your toenails

Drink a cold beer on a patio

Pick up your cat

Drive a car 

Tell a story

Kick a rock

Put on a t-shirt all by yourself

Eat some cheese on a cracker

Have a bath

Belt out a Broadway tune

Pick up something you dropped on the floor

Style your hair

Pull a weed out of your garden

Hug someone with both arms 

Run up the stairs

Tie your shoelaces

Whistle

Chat to someone on the phone

Now say thank you to God or the universe or whatever works for you.

Being able to do any of these things is a gift.

I should know because I can’t do any of them.

_____________________________________________________________

I am grateful that I can:

Listen to the birds

Cough

Brush my teeth

Stroke my cat’s tummy

Read on my Kobo

Type (slowly)

Have a shower sitting down

Go up and down stairs (slowly)

Text someone

Paint

Knit (very slowly)

Smell coffee

Thank you, God, for these gifts

____________________________________________________________________

Either you decide to stay in the shallow end of the pool, or you go out in the ocean / Christopher Reeve

What is Legacy?@

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What is Legacy?

One concept I have spent time considering over the past year is that of legacy. As we get older or become seriously ill, I suspect many people do. We ask ourselves, “What will my legacy be?”,  beyond the stamp collection or what is left in the bank.

Webster’s Dictionary defines “legacy” as “something transmitted by or received from an ancestor or predecessor or from the past.” Examples of legacy might be, “ She left us a legacy of a million dollars”, “He left his children a legacy of love and respect”, or “The war left a legacy of pain and suffering”. So legacy can be positive or negative. It can be quantifiable or not. If we base our understanding of legacy on commercials and ads it would seem that it is defined as financial or funds or assets we pass on to family or our community. But this is just a partial view of legacy – there’s much more to it.

Part of my tangible legacy is the charity I founded in 2003 – Cool Arts Society. I started this organization to serve adults with intellectual disabilities in the Central Okanagan because of the need in the community; my son wanted to take art classes and there was a lack of opportunity. So, almost 20 years later the group still exists, has a location in the civic art centre and enjoys a solid reputation in both the local arts and disabilities communities. One of my core values is to make a difference in the world and Cool Arts definitely reflects that.

I’m also proud of the research thesis I wrote for my MA about social equity in the arts for artists with intellectual disabilities. I worked really hard on this under-researched topic; the 200+ page work is published online through UBC. Individuals from different parts of the world have contacted me because of their shared interest in improving access in the arts. I feel like it made a difference.

I’m a ‘doing’ person, and it can be difficult for me to separate my values and/or legacy from ‘doing’ or what I have ‘done’. As we get older or sicker, we are not able to ‘do’ things that we think might provide legacy. In his book, Being Mortal: Medicine and What Matters in the End, Atul Gawande says the following:

“As our time winds down, we all seek comfort in simple pleasures – companionship, everyday routines, the taste of good food, the warmth of sunlight on our faces. We become less interested in the rewards of achieving and accumulating, and more interested in the rewards of simple being. Yet while we may feel less ambitious, we also become concerned for our legacy. And we have a deep need to identify purposes outside ourselves that make living feel meaningful and worthwhile.”

I really love the musical, Hamilton. This was a surprise because the premise sounded pretty dry and I wasn’t sure if the musical genre would be to my taste.  However, the energy, creativity and passion really impressed me. If you haven’t seen it, you can view the live recording of the Hamilton Broadway performance on the Disney Channel and I highly recommend it.  There are several themes that run through the production and one of them is that of legacy. Let me share some lines of the finale song that had me in tears:

“Is it enough?

Let me tell you what I wish I’d known
When I was young and dreamed of glory
You have no control
Who lives, who dies, who tells your story?

And when you’re gone, who remembers your name?
Who keeps your flame?
Who tells your story?

Legacy, what is a legacy?
It’s planting seeds in a garden you never get to see
I wrote some notes at the beginning of a song someone will sing for me”

Is our legacy also a reflection of our values? I think so… in fact I believe the way we have lived our lives may be the most significant part of our legacy. Values are what others will remember about us beyond our favorite joke, our signature drink or the way we did our hair.

I have identified my own values as follows:

            Integrity & honesty

            Seeing beauty in all things

            Connection with others

            Justice

            Making the world a better place

Identifying our values can be a difficult exercise (in my experience). However, at this point in my life  I’ve done enough personal development work to observe that these five components always come up in discussions about values. I hope this is how I am remembered.

Sara’s gang


Gawande, Atul, Being Mortal: Medicine and What Matters in the End. Doubleday, 2014.

https://www.coolarts.ca/





Measuring reality

Ruddy Duck / Munsen Pond

I love gardening. It makes me happy to be outside mucking around in soil, planting flowers and then watching them grow. It’s one of those hands-on activities that I enjoy and one of the reasons I don’t get manicures. Last year I was able to tend to my little townhouse garden without any problems.

Last month on a lovely sunny day, I thought I would do some garden cleanup and spread some of nice moist compost I had picked up at Buckerfields. I raked and pruned and sat on the edge of a raised garden bed to get closer to the plants, which was fine until I realized that I could not get out of a sitting position. The strength in my large leg muscles and torso are weakened enough that I could not get myself up from such a low height. There is a chain link fence close by and after skooching very slowly along the edge of the raised bed I was able to grab onto the fence and after a few attempts pulled myself up. Good grief. No more sitting on surfaces lower than a standard chair unless there is someone around to assist.

I continued to work on the other side of the raised bed by standing and bending over. Scrabbling in the soil trying to find a hosta that may have kicked the bucket over the winter, reaching farther and farther, I felt myself tipping forward, and then it was a slow motion fall into the garden. Like a Douglas Fir felled in the forest, I fell with a thunk. What I felt like was a beached whale – this raised bed is where I have many of my beach rocks collected over decades of vacationing on Vancouver Island. Hard and bumpy with some barnacles.

I lay on the dirt thinking that surely someone would see me from one of the neighbouring townhouses. I wondered what they might think if they saw me laying in the garden on top of the plants. Heart attack? Close inspection of roots? I’ll never know, because on this beautiful sunny day no one was outside and no one was home at my unit either. Since I was already down there I figured that I might as well take care of that part of the garden, so I cleaned up debris while I was horizontal in the dirt. Then it took me about half an hour to inch over to a step  by the chain link fence, with lots of little rests, to right myself. My arms have also weakened so pulling myself up is not easily done. Good grief again. Obviously I cannot work in the garden unless someone is close by.

Did I feel humiliated? No… the only dumb thing I did was to try gardening when no one was home. It’s just one more thing I can’t do by myself anymore. It was a bit shocking to discover how easily I could fall while standing and not even walking. A week later I tipped over again reaching my closet and landed on top of a bunch of shoes. I have now ordered a fanny pack so that I always have my emergency button and phone with me.

I have participated in a non-profit research program through TDI (ALS Therapy Development Institute) since July 2020. A major part of the Precision Medicine Program at TDI is to try and improve on the current system of patient reported outcomes (ALSFRS-R) using an unbiased data collection system to measure movement. The ALSF-R is a short series of questions about basic functions like eating, dressing, walking, etc.; every month I complete the ALSFRS-R test online. Last July my score was 42, and in April 2021 it was 21 – quite a significant difference.  It’s a quantifiable way to understand the rate of progression. My score this spring was lower than I thought it would be, but like anything that is slow and gradual, it is difficult to assess change while in the middle of it. Experiences like my gardening day and my closet mishap a couple of weeks ago are a reality check, in this case confirmed by data.  It’s very difficult to predict progression as ALS progresses differently in each person.

I also wear accelerometers (monitors) on my wrists and ankles for 5 days per month. At the end of the 5 days I send the monitors back to TDI in Massachusetts where the data in downloaded. Why do I do this? It’s a way to document my progression in a tangible way and also to contribute to ALS research. The Precision Medicine Program (PMP) allows people with ALS to become partners in ALS research. Participants also gain access to helpful data and tools regarding their disease progression. The website has diagrams that chart my ALSFRS progression and progression according to the data received from the accelerometers. Here are two of the diagrams that chart my progression:

ALSFRS-R Tracking graph

Accelerometer Activity graph

There you go – probably more than you wanted to know about measuring ALS progression, but actually fairly basic stuff. If you are a nerd about this kind of thing, please check out the link below:

https://www.als.net/about-als-tdi/

Cry Me A River

Pseudo-what?

Pseudobulbar affect is a condition I had never heard of until I started reading up on ALS.  It is characterized by episodes of sudden uncontrollable laughing or crying that have no relation, or seem out of proportion, to how the individual actually feels. Pseudobulbar affect (PBA) is not limited to ALS; it typically occurs in people with certain neurological conditions (stroke, MS, etc.) or  brain injuries. In a nutshell, PBA affects the way the brain controls emotion. Obviously, the condition can be unsettling and embarrassing, and for some people, PBA is very extreme to the point of avoiding being in public.

 Crying appears to be a more common sign of PBA than laughing.

No kidding. A few months ago I noticed that if I started crying, even a little, it was difficult to stop. People have said to me, “You should cry. Let it out. You’re going through a lot.”  However, there are some challenges with doing that:

1) Once I really cry, it’s off to the races – I cannot stop. It develops into sobbing and awful wailing, and can go on for quite a while. Afterwards I have a headache and don’t feel any better about life.

2) As indicated above, the manifestation of emotion might look like a 9 or 10 out of 10 (e.g. loud sobbing and weird noises), but inside my emotion level might be  a 3 out of 10. It doesn’t match up.

3) Full on crying can make me gasp or even choke (on saliva). Don’t want that.

Anything can trigger me, in particular, something on TV or music. I sobbed during the first half of the musical Hamilton (stayed clear of the second half). I sobbed during the Mr. Rogers movie. I cry watching stupid sitcoms and reality fashion shows. Any display of emotion sets me off.

The laughing part of PBA is not as extreme for me, but I do find that there are times when I will start to laugh at something  inappropriate or that no one else would find funny.  I usually catch myself on that. If I do find something genuinely funny, I’ll laugh really hard for several minutes and that’s ok. There is a theory that the character of the Joker in the 2019 film Joker, played by Joaquin Phoenix, had pseudobulbar affect. He displayed uncontrollable and uproarious laughing, and cried when happy.

PBA has also been called emotional dysregulation, emotional lability or my personal favorite, emotional incontinence. A study designed specifically to survey for prevalence found that 49% of patients with amyotrophic lateral sclerosis (ALS) had PBA. There are medications available if deemed necessary.

Norman MacIsacc, Canadian author of  The Best of the Worst News, writes that PBA“messes with our ability to control the physical expressions of our emotions…now those levees have weakened, and they burst at the slightest emotion with  unruly surges so ungainly it’s difficult to distinguish laughter from sobs.”

So what to do? Sure, there are times when I am feeling genuinely sorry for myself and could shed some tears, but I tend to nip that urge in the bud. I know myself well enough to know it’s a slippery slope with not much of a payoff. Sometimes I write it down instead – seeing my feelings visually on the page gives me clarity. Or I might do or read something to take my mind in another direction.

Of course I feel sad and upset at times. Disappointed. There are many experiences that I was hoping to have. I want to see my grandchildren grow up. I want to make sure that my son who lives with a disability is ok. I want to see all my children grow and thrive in their lives. But we don’t get to choose some things and that’s life. I’m grateful for the experiences that I have had and the family that I have now. I’ve come to terms with living with ALS as well as I can for now, although it is an ongoing process.

__________________________________________________________________________________________________Norman MacIsaac   The Best of the Worst News.   Insomniac Press 2019 MacIsaac is a leader in the Canadian ALS Advocacy movement.

“What Can I Do?”

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January 25 marked the one-year anniversary of my ALS diagnosis.  Obviously there have been many enormous shifts and changes in my life. It’s been a very long year that included stopping work, losing my voice, losing my ability to eat and drink, a visit to the hospital, an excess of saliva and waning strength in my limbs. However, 2020 also offered opportunities for gratitude and a more acute ability to prioritize, find clarity and further value relationships.

People very often ask, “What can I do?”, or “How can I help?”.  Well, if it wasn’t STILL the season of COVID, you could come for a visit or have me over for an outing, but, really, there’s isn’t much anyone can do for me right now. There will come a time when my daughter and I will welcome practical help, but we’re not there yet. I’ll let you know when…

However, here is actually something you can do – sign up as a supporter on the ALS Action Canada website. A group of ALS patients and family members  across Canada have been working hard to improve access to treatments and clinical trials for Canadian PALS (People with ALS). I encourage you to please check out the ALS Action website, in particular the Take Action section: https://alsactioncanada.org/take-action/I am a part of this group, but not in the leadership. The main players are a talented and dedicated group of ALS patients and family members using their formidable skills and valuable experience in the area of policy change and advocacy.

May I rant here? It makes me feel angry that people living with ALS  who have limited time left in their lives should have to spend their precious time and energy doing this. I think it’s truly selfless that they do so. Make no mistake – it’s unlikely that they will live to see the benefits of their persistence. Positive changes will chiefly benefit ALS patients who likely haven’t been diagnosed yet.

ALS moves fast. It’s also hitting younger people – although the typical patient is still over 50, there are more and more people in their 20’s and 30’s being diagnosed, and I’ve met quite a few in their 40’s.  I recently read about a fundraiser in the US for an 11-year ALS patient, which I found shocking. Unfortunately, the rate of ALS is rising – might be the aging population or environmental factors, but no one knows for sure.

It’s a revolving door and a numbers game. People often don’t realize that the ALS incidence rate is as high as Multiple Sclerosis (MS) because ALS patients die so quickly that there is never as large a population of ALS patients as other conditions. PALs who get really involved and contribute to advocacy have a limited time to do so and so the leadership must constantly shift and change. The lifetime risk of developing ALS is 1 in 400, which is about equal to MS and is five times higher than Huntington’s disease. However, the rapid progression and deaths of people with ALS have prevented the establishment of a critical mass and a loud collective voice. This dynamic is driving our demands for urgent action.

The irony of the fact that I am advocating for myself after many years of advocating for others is not lost on me. One of my children has a disability, so I have spent decades advocating for him and others with developmental disabilities (DD). Some of the same issues apply to the population of people with DD – lack of a collective ‘voice’ and that of being an invisible group. Individuals with DD often cannot represent themselves in order to advocate. I am familiar with the complaint of ‘not being heard’, and the situation of being dependent on others to advocate.

If someone in BC is diagnosed with MS, Alzheimer Disease or Huntington Disease, and directed to the provincial clinic, this is where they go:

The Djavad Mowafaghian Centre for Brain Health on the UBC campus in Vancouver.

But newly diagnosed ALS patients who are directed the ALS Provincial Clinic are sent to windowless rooms in the basement of GF Strong Rehabilitation Centre:

The BC ALS Clinic cannot give information regarding ALS clinical trials because BC does not have any ALS clinical trials, and no information is given regarding possible participation in trials outside BC. Clinical trials are of critical importance to ALS patients.  Clinical trials offer a sliver of hope, and are also a way to contribute to research and finding a cure. There are only 2 approved medications available in Canada – both only work to extend lifespan by a few months, so clinical trials are the only way to try out a drug that may help. There isn’t even a full-time neurologist working at the BC clinic, and no respirologist at all.   Imagine how disheartening it would be to go for the first time to a provincial clinic like this and realize that there is no hope being offered?

I have not been to the Provincial ALS Clinic. At the same time I initially went to my GP about my slurred voice, a neurologist with ALS experience moved to Kelowna from Calgary, so I have been his patient. Interior Health provides other support like respiratory, speech and occupational therapy, and there is a respirologist in Kelowna with experience in ALS.  I’m very thankful that I can stay local with my care.

Some BC ALS patients travel regularly to other cities in Canada (Montreal in particular), and prior to COVID, to American medical centres so that they can  participate in clinical trials. Usually all travel costs are covered by the patient, so many patients are automatically disqualified for financial reasons. And, of course, after all the travel and expenses, there is a good chance that the patient may have received a placebo – patients don’t find out whether they really received the drug until the trial is over. I was accepted into the Healy Platform trial in both Seattle and Los Angeles, but travel to the US is not desirable at this time. I thought I was accepted into a trial in Calgary but the deadline was cut off early and I didn’t get in. It just shouldn’t have to be so difficult…

So lending your support for policy changes is no small thing – it really helps. Future lives depend on it.

Tell your MP and MLA that you support better access to treatments and trials for ALS patients. Send an email to Adrian.Dix.MLA@leg.bc.ca , cc your MLA and  Renee.Merrifield.MLA@leg.bc.ca  (Critic for Health).  Request that immediate action be undertaken to significantly improve ALS treatment options and care in BC. The email doesn’t have to be lengthy or scholarly – it’s the number of responses that make a difference.

ALS isn’t incurable, it’s underfunded.

Thank you for your support,

Sara

an update…

Happy New Year to you all! And good riddance to 2020!

My intention was to post in my blog much sooner, but the best laid plans… This entry is an update on how I’ve been the last couple of months.

Unfortunately I really crashed emotionally the last part of November. I think the reality of losing my voice, as well as the ability to eat or drink really sank in – it was a very difficult transition. I just can’t risk putting anything down my throat anymore in case of aspiration or choking. Sometimes my mind just goes to musing about some of my favourite foods… like the different flavours and textures of Pad Thai… or the comfort of a really good cheese sandwich with pickles, mustard and mayo. It’s not just the taste of food, it’s the sensation of biting into it or sipping or chewing. There is so much I took for granted that I can only dream of now. I put a few drops of soy sauce on my tongue a couple of days ago and that was a real sensory treat! (Note that I’m a salt person, not a sugar person).

And I felt so tired… and continued to feel very fatigued into December as well as having nausea. Gifts lay unwrapped and holiday cards unwritten. I didn’t even have the energy to brush my teeth, let alone work on a blog post. Couldn’t even knit… which is pretty shocking for me.
What looked initially like adjustments to meds and feeding tube formulas ended with me going into ER with failing kidney function and off the chart hypothyroid levels. I came home Christmas Day from the hospital feeling much better, although it will take some time to get the thyroid levels and consequent energy levels corrected.
So I’ve spent most of my days napping, reading, watching TV and staring out the window. My family has been absolutely terrific, in particular my daughter Elisabeth who has been caregiver extraordinaire with her trusty sidekick Michael. I am extremely blessed to have the support that I have.

The staff at KGH were wonderful and I have great admiration at both how important their jobs are and how hard they all work. When you’re feeling awful the world becomes very small and anyone that offers competent and respectful care is your hero. However, someone could inform a certain nurse in ER that just because a person cannot speak, it doesn’t mean that they are deaf. She insisted on yelling at me, but I didn’t take it personally.

I’m looking forward to having more energy in the next month or two. I am very grateful that all my children and their families are in good health as well as my mother and siblings and their families. There is much to be grateful for. Many people sent me holiday cards, emails and texts and although I haven’t responded to many, please know that I really do appreciate your support and encouragement. It really helps to know that others are thinking of me and wishing me well. Thank you.

Best of the New Year to you all! I sign off with a quote from Simon Fitzmaurice:

I am not a tragedy. I neither want nor need pity. I am full of hope… Hope is not about looking for a cure to a disease. Hope is a way of living. We often think we are entitled to a long and fruitful Coca-Cola life. But life is a privilege, not a right. I feel privileged to be alive. That’s hope.

It’s Not Yet Dark by Simon Fitzmaurice. 2017, Hachette Ireland. This is an excellent memoir by Irish Filmmaker Fitzmaurice. Died of ALS in 2017, aged 44 leaving his wife Ruth (who has also written a memoir) and his 5 children. There is also a documentary about him narrated Colin Farrell.

Happy 2021 from me and my gang

Reflections on a disappearing voice

I’m gradually but steadily losing my voice. Although I knew this would happen, the last month or so has been more difficult emotionally than I anticipated. I’m not a choir member or auctioneer or orator; I’m not a person that screams at hockey games or that reads the entire menu for the benefit of the next table. I’m a visual person and a maker, so my eyes and hands have always been what I thought were essential parts of my body as far as defining who I am in this world. And yet, I’m realizing that in losing my voice I am losing part of who I am. Anyone who knows me well knows that I enjoy chatting and telling an anecdote or a joke. I have loved telling stories to my grandchildren, especially about ‘Kevin’, a fictional boy who is generally up to no good. I like to sing in the car and talk to my cat; teaching printmaking was a very satisfying experience as was the very occasional public speaking gig.  I’m starting to comprehend how significant my voice is in relation to my identity.

ALS leads to speech problems when it attacks bulbar neurons. These are the nerve cells responsible for bringing messages from the lower parts of the brain (bulbar region) to the muscles that move the lips, tongue, soft palate (back of roof of mouth), jaw, and vocal folds (voice box). As nerves are lost to the disease, the muscles they control become weak and tight and cause dysarthria (motor disorder of speech characterized by abnormalities of the articulation and intelligibility of speech). Weakening lung muscles affect speech as well. Speaking can make me really tired, especially later in the day.

There is a difference between dysarthria associated with ALS and dysarthria resulting from other conditions – like a stroke. In ALS the muscles that are receiving signals from the brain must compensate for the muscles that are already weak; this means the functional muscles are doing extra work all the time to compensate and therefore, require more frequent and extended rest. Rest can often help temporarily to improve speech. People with other kinds of dysarthria often find that oral exercises can strengthen their muscles used for speech, but unfortunately this does not help ALS patients with dysarthria.

I was advised to start  voice banking last spring, and I’m so glad that I did. A wonderful provincial organization, Communication Assistance for Youth and Adults (CAYA), provided me with equipment and expertise to voice bank in order to create a synthetic voice made from my own voice. I can now type a message on a mini-iPad and it will ‘speak’ for me. It sounds a little like me, but is still very robotic, the sentences have odd timing, and, of course, no sense of expression. But still – I’m very grateful for this tool and the services that CAYA provides (free of charge). The ‘synthetic voice’ is made up from over 3000 sentences I recorded last spring. And I can still use my hands to write words, so that’s another thing to be thankful for.

But… I hadn’t counted on this sense of loss. I can’t read to my grandchildren anymore or tell them stories. Participating in a conversation is difficult for all involved and feels forced. Speaking on the phone is almost impossible now – an exercise in frustration for me and the other person. I can’t even hum. I understand why people with speaking difficulties find it easier to stay quiet and just observe instead of engaging.

As with eating or drinking, before I speak I have to arrange my mouth (including the excess saliva); I call a meeting, make sure all players are there and get them organized as they don’t seem to able to arrange themselves. My tongue is definitely not a team player and seems to follow some other game plan. My throat will suddenly just give up- cough, cough, sorry – cough.  Strangely, even crying is affected… it turns into an awful gasping sobbing experience that won’t stop.

I’ve noticed that some people watch my lips if I speak and mouth the words themselves in order to understand what I’m saying. But, of course, if I’m wearing a mask they can’t see my mouth. Stupid STUPID COVID.

So on it goes. As Joe Hammond wrote, “It’s hard to live the losses moment to moment, accepting them as they arise, dispensing with pieces of the self fluently like a bag of birdseed strewn into a flock of pigeons.” ALS is often described as a progression of losses… and this feels like a big one. Adaptions are being made and will continue to be made, but I feel sad about losing this part of me.

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CAYA (Communication Assistance for Youth and Adults): https://cayabc.net/ CAYA is a province-wide service program funded through the Ministry of Social Development and Poverty Reduction. CAYA supports independent communication through the use of technology. Clients have either never had or have recently lost the capacity for functional speech. The origins of these problems stem from problems at birth (e.g. cerebral palsy) or genetic conditions (e.g. Down’s Syndrome) or acquired conditions (e.g. traumatic brain injury, stroke, ALS).

Hammond, Joe. A short history of falling. (Fourth Estate, 2019) British writer and playwright Hammond chronicles his journey with ALS.

ALS Diagnosis: the worst of news

In Paul Kalanithi’s beautifully written memoir, When Breath Becomes Air, he describes receiving a diagnosis of terminal illness: “I began to realize that coming in such close contact with my own mortality had changed both nothing and everything. Before my cancer was diagnosed, I knew that someday I would die, but I didn’t know when. After the diagnosis, I knew that someday I would die, but I didn’t know when. But now I knew it acutely.”

Late last October I noticed a slight change when I answered my work phone; when I came to the end of a phrase my voice seemed to lose a bit of steam, but in a barely noticeable way. A month later I picked up on some very minor slurring. I had recently had dental surgery and assumed that was the cause; however, the dental surgeon reported that all was ok. By Christmas holidays the slurring had increased, although still minimal and unnoticed by most people. Or else some folks thought I had started drinking during the day…

I decided to get checked out, made an appointment with my GP and mentioned it in a casual fashion, “By the way – it’s no big deal, but…”. She perked right up and got me into the Quick Access Stroke Clinic pronto. The nice neurologist there assured me that I had not had a stroke and sent me to the new neurologist in town who was able to conduct an Electromyography (EMG) test. He also mentioned the possibility of Myasthenia gravis, an autoimmune disorder.

Bingo. I already have 3 autoimmune disorders (Addison’s Disease, Hypothyroidism and Vitiligo), so that seemed like a very obvious answer as people with an autoimmune disorder can have a tendency to develop additional autoimmune disorders. Problem solved. I was sent for lab work,  a CT scan and Electrocardiogram – very quickly (surprisingly quickly given the usual wait times for tests).

Before I went to see the 2nd neurologist I googled ‘slurred speech’ and found that there were five possibilities that could apply in my case:

Brain tumor

Myasthenia gravis (autoimmune disorder)

Stroke or TIA (“mini stroke”)

MS (Multiple sclerosis)

ALS (Amyotrophic Lateral Sclerosis)

Not a list that anyone would want as possible diagnoses.  But Myasthenia gravis was apparently manageable with treatment, so I wasn’t panic stricken – it made sense to me (Sara  the medical expert) that myasthenia was the answer.

I met with the second neurologist a few days later and was with him for about an hour and half – poked with numerous little needles, subjected to little ‘shocks’ and a lot of pushing/pulling. I was chattering away and asked some questions about myasthenia. He said, “Let’s wait until I’m done and then we’ll talk”. Oh. Then he asked me if I had googled my symptoms. Oh-oh. That’s when I started to wonder.

We sat down after the tests were done and the doctor told me that I likely had Motor Neuron Disease, also known as ALS or Lou Gehrig’s Disease. I want to emphasize that he was very kind and gentle in the way he communicated this to me and answered the questions that came to me in that shocked state. What? WHAT? How could this be? He explained why it wasn’t some of the other possibilities and said that I would have an MRI to rule out a brain tumor. The key factor appeared to be evidence of both lower and upper brain neuron degeneration.

And then I had to make the long walk from the far end of KGH back to the parkade by myself. Of course I should have bought someone with me… but I wasn’t expecting the worst of news.

Sometimes the expression, ‘hit by a truck’, is used as a way of describing surprise or shock. At that moment, I truly felt like I had slammed into a brick wall. We often wonder, at least I had wondered, what it would be like to receive a diagnosis of terminal illness. Now I know. I felt numb for about a month and then gradually began the mental and emotional shift towards my new reality. Because it is a giant shift… a colossal paradigm shift. One could choose to ignore or deny this new reality, and some do,  but I don’t see the point in that – I’d rather face it directly and in the most honest and positive way possible.

In his memoir, Joe Hammond described the experience of receiving his ALS diagnosis as having a “brute effect [that] comes from blunt power”, and writes that, “Really bad news is a little like medieval weaponry. It isn’t precise like a bullet or a machine-sharpened blade.”  His accurate and graphic way of putting words to the experience  resonated with me.

I’ve discovered a large literary genre of non-fiction – memoirs by terminally ill people. No doubt that sounds like a real downer, but in fact the best written of the lot are not so –  on the contrary, I’ve read some wonderful writing that is authentic, sensitive, incisive and honest, and sometimes very funny. This writing helps to light my path. Learning of someone else’s journey and how they navigated the difficult  terrain of terminal illness lets me know that I’m not alone. Others have gone before and shared their fears and joys, struggles and triumphs.  That these writers share these deeply personal and profound moments of the end of their lives is an incredible privilege. I will share some of this writing and these authors in my posts.

 Hammond, Joe. A short history of falling. (Fourth Estate, 2019) British writer and playwright Hammond chronicles his journey with ALS with humor, insightful awareness and breathtaking candour.

 Kalanithi, Paul. When Breath Becomes Air (Random House, 2016).American neurosurgeon and writer who died at age 36  of lung cancer; this memoir was nominated for a Pulitzer prize and is well worth reading.

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Diagnosing ALS:

There is no single diagnostic test to indicate ALS. The most conclusive test is the Electromyography test.

Determining an ALS diagnosis usually becomes about ruling out other possibilities, thus the MRI rules out a brain tumor, an antibody test rules out myasthenia, and so on. In some cases a diagnosis can take several months or over a year. Sometimes a spinal tap and /or a muscle biopsy is done as well. Genetic testing would occur if a familial case of ALS is suspected (a family member has ALS).

There are three types of ALS diagnosis: definite, probable, or possible ALS.

  • Probable or definite ALS – loss of both upper and lower motor neurons detected in two or more regions of the body.
  • Possible ALS – loss of both upper and lower motor neurons is detected in only one region of the body.

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The El Escorial criteria is commonly used to aid diagnosis, which requires the following:

  1. Evidence of lower motor neuron degeneration
  2. Evidence of upper motor neuron degeneration
  3. Progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination.

Choosing to adapt

The big change in my life recently has been the placement of a feeding tube. The procedure itself is fairly minor, but the implications are significant. I won’t lie – I was more sore after the insertion than I expected to be. However, as my daughter replied to my whimpering, “You did have a hole put in your stomach”. Ouch.

My feeding tube is called a PEG tube, which stands for percutaneous endoscopic gastrostomy. A hole was made into my stomach through my abdominal wall, and a flexible tube inserted. There is ‘stopper’ on the inside of the opening and on the outside to keep the tube in place (don’t tell the specialist, but I think it actually seems kind of low-tech). Liquids can now be fed directly into my stomach, thereby bypassing my mouth and throat.

PEG Tube

Why? I’m finding it more and more difficult to swallow. Thin liquids like water (and wine!) slip slip away down the windpipe instead to the esophagus; thicker liquids like tomato juice or smoothies are better, but I still have to really focus while taking small sips. It’s a challenge to get in enough fluids to stay hydrated – that’s a big deal, but the REALLY BIG DEAL is getting enough calories inside me. Instead of watching for excess calories, I now have to look for ways to ADD calories. I get to pile sour cream on everything..

For now I’m hydrating 3x/ day with 5 – 50 ml syringes of water, and introducing prepared liquid ‘meals’. I’ll work up to creating home-made tube-worthy meals. Soon I’ll graduate to a gravity bag drip system.

No – I can’t taste anything that goes into my stomach from the tube.

Yes – I can still eat by mouth (mushy stuff).

No – I don’t feel liquids go into my stomach (just a ‘full’ feeling)

Yes – I could put wine into my feeding tube if I was desperate enough.

No – I probably won’t slide a plate of Thanksgiving dinner into the Vitamix and then into my tube as some PALS (people with ALS) have done. Or a steak. Strangely, it doesn’t sound terribly appealing.

At my first local Zoom Support group meeting, someone mentioned the importance of the word adapt. It is becoming more and more clear to me what this word means in a life with ALS. Life as a PAL is a constant series of change and adaptations, and the feeding tube feels like a really big adaptation. M A J O R. Feedings have to be scheduled, and probably need to take place at home, at least for now. I have to accept that there are many foods, including my favorite foods, that I will never ever eat again.

But NOT adapting is not an option. There are people with ALS who choose not to adapt, but this is a war where a strong will is not enough.

There are big upsides to the feeding tube – less choking for one thing, and that’s a big plus. I had a nasty choking episode last week while my sister and daughter were visiting and it was a bit scary for them. It wasn’t the first time for me, so I wasn’t as rattled, but it is very unpleasant and something I would very much prefer to avoid.

I’ll end off with a video link for anyone interested. Comedian Jim Gaffigan aired a clip of himself assisting his wife Jeannie with a tube feed. After surgery for a brain tumor, Jeannie was left with swallowing difficulties and required a tube: https://youtu.be/fQjKJYh_WLw. (length – just over a minute, but there is a longer video on youtube as well).

Photos below from ‘Sara’s Pond’ (Munsen Pond)