I was hoping that my next blog post would be about my recent motorcycle trip through the highlands of Scotland. However, my life has taken a major turn. The Scotland blog post will have to wait.
There is no easy way to explain what I have been going
through for the past few months and the emotional journey that my
family and I have been on for the past ten days. I have decided to
share my experiences here because I will soon be admitted to the Foothills
hospital under the Neurology service and I want my friends and colleagues to
know what I have been going through. I have included my personal medical
information in this post because I hope there will be some educational benefit for my
colleagues. I have not had the time to tell many people and did not
know myself what was going on until very recently so please do not be offended
if I did not get an opportunity to tell you in person. I have told almost no
one outside of my immediate family with the exception of a few close friends (thanks for the support).
I have decided to be brutally honest in
this post. If there is a gift from this experience it is that after having gone
through it I just don’t care what anybody thinks. I feel free to do what I think is right.
Neurotic Neurology Resident Syndrome
I think that almost every neurology resident at some point
in their training worries that they might have ALS. Because we see patients at
every stage of this tragic disease, we become hypersensitized to a muscle
twitch here or there and our neurotic minds jump to the conclusion that these
normal fasciculations are in fact the first sign of ALS.
In my case I first wondered whether I had ALS while in
Toronto in July for a wedding. At the brunch the day after the wedding (July
28, 2013), I was trying to fill people’s coffee mugs and dropped the coffee pot
on the table. I never lost my grip on the handle, but my right shoulder just
gave out. Why did I have shoulder weakness? I hadn’t injured it. Although, I had
played golf a couple of weeks before and had carried my clubs using a shoulder
strap. Could it be some sort of painless rotator cuff injury? I was a bit
worried because painless weakness is also a feature of motor neuron disease.
However, I told myself that I was falling victim to the Neurotic Neurology
Resident Syndrome and put it out of my mind, expecting my shoulder to get
strong again on its own. However, my shoulder would not improve; rather it
would continue to progressively weaken.
Congratulations on your wedding Matt and Andrea. It was beautiful.
For the August long weekend, Ronak, Avidesh, and myself went
on a road trip to Lethbridge and Waterton Lakes National Park where my parents
cooked us a picnic BBQ next to Waterton Lake. We saw 6 bears in the park, and
Avidesh had several entertaining panic attacks. I’ve never seen anyone so
scared of bears!
Left: Me at Bear’s Hump, Waterton Park. Right: Avidesh points out his monster drive at Pincher Creek Golf Course (August, 2013).
On the way we played 9 holes
of golf at the Pincher Creek Golf Club. I had some monster drives approaching
250 yards and had carded several pars in a row. I finished with a birdie on the
challenging par 4 9th hole with its hilltop green and sharp dog leg
to the left. My tee shot landed in the middle of the fairway and my second shot
was a 7 iron that landed on the green. It wasn’t until I got up to the green
that I realized how close it was to the cup. Almost a tap-in birdie, but the
two footer still required concentration. When it dropped I cheered. As we were
walking off the course I joked that “now I can go into retirement”. Who knew.
Despite my right shoulder weakness, if anything my golf game was actually better
than normal. I certainly beat the pants off of Ronak and Avidesh. Today there
is no way that I could even swing a golf club, let alone hit a golf ball 250
yards. Looking back, I don’t think there was any significant functional impairment
yet at that point.
Over the next several weeks I started noticing difficulty
reaching for objects with my right arm. I also had trouble reaching for my
briefcase. It was all proximal weakness as I had no difficulty with grip or
handwriting. I started to use my left arm for reaching tasks. It was around
this time I first felt and saw fasciculations in the right supraspinatus,
infraspinatus, deltoid, and triceps muscles. Now it became harder to attribute
everything to the Neurotic Neurology Resident Syndrome. I started to seriously
consider the possibility that I had ALS. Yet I was also still in denial.
By the time we had our resident retreat, which included
archery and high rope out at Camp Horizon on September 27th, I had
significant weakness. On the high rope I had to hang on to the cables with my
left arm only. On the archery range I could not even pull the bow back with my
right arm and could not shoot an arrow. I tried to throw a football and it fell
straight to the ground. I tripped and fell, hitting my face on the pavement because
I could not break my fall with my arms. Something was seriously wrong.
I had been planning a trip to BC to explore
career options, although I was pretty sure that I wanted to be a community
neurologist in Lethbridge with a special interest in both Multiple Sclerosis
and Neuromuscular Neurology. Ultimately I decided to go on my planned trip despite
my fears of having ALS because I thought it might be my last. I stayed in a nice
bed and breakfast on a vineyard in the Similkameen valley and enjoyed two days
of wine tasting. I bought many nice estate wines. I met up with a
colleague in Surrey and then flew to Prince George where I spent a few relaxing
days touring the city.
Left: Crowsnest Vineyard, Similkameen Valley, BC. Right: Yakima Valley, Washington.
After flying back to Vancouver, I drove through the Yakima
Valley in Washington State and did another day of wine tasting, buying another
batch of estate wines. Even though I was still partly in denial, I wanted to
have a supply of good wine to help dull the coming nightmare. I left my
car in Great Falls, Montana, and flew to Las Vegas for a few days, staying at
the Treasure Island. I was obviously still partly in denial, as I spent the
afternoons reading the Continuum by the pool and making flashcards. This is not
something I would be doing if I knew for sure I had ALS – I can assure you of
that! I even bought clothes for clinic at the outlet mall. In the evenings I
played no limit Texas hold’em poker. I saw a Cirque du Soleil show at the Aria after
meeting up with a friend and her colleague who were in town for a conference. But
by now the fasciculations were almost constant and they were keeping me up at
night. I was also starting to have cramps. There was significant atrophy of my
right supraspinatus, infraspinatus, deltoid, and triceps. Perhaps most
frightening was realizing the left side was starting to get weak too, and also
starting to fasciculate.
I knew that I could not deny this any longer. When I got
back from my trip, I would have to act on my belief that I almost certainly had
ALS. I had not told anyone about my fears. I was on my Pediatric Neurology
rotation and had continued to work even though by then I could not overcome a 4
year-old on power testing. My clinical performance was probably affected by my
constant anxiety and ruminating thoughts about ALS. I noticed little things
were off. I’ve always been a bit absent minded, but looking back I think I was
more absent minded than usual because of how distracted I was. I probably
should not have continued to work under the circumstances. But denial is
powerful.
On my way home from Las Vegas, while at the airport, I finally
texted Ronak, my friend and fellow PGY4 Neurology resident, the following text:
“Dude I know this is going to sound crazy but I want to stop by and get
NCS/EMG. I’ve become convinced I have ALS.” Ronak offered to do the studies as
soon as I got back. He has himself been afflicted by the Neurotic Neurology
Resident Syndrome and was empathetic. I suspect that he figured that I just
needed some reassurance. But by then I knew that this wasn’t going to be about
reassurance. I knew what I was going to hear when the needle went in: the
ominous sound of fibrillation potentials, positive sharp waves, and
fasciculations. These were the sounds of muscle fibre denervation from anterior horn cell loss. After further reflection, I decided it wasn’t fair to have
Ronak on the other side of the needle for this.
Instead, I waited to see Steve McNeil during the resident
half-day so that none of my fellow residents would be around to hear me get
needled. We chatted a little about hockey; Dr. McNeil had twice given me tickets
to see Calgary Flames games in what seemed like another lifetime. I wasn’t sure
how to tell him my fears so in the end I just blurted that I was convinced I
had ALS. Even though he had a full clinic, he said “let’s take a look at you.”
The nerve conduction studies were entirely normal as I knew they would be. As
Dr. McNeil examined me I could sense his growing concern at the widespread right
shoulder atrophy and weakness. His concern increased when I was hyperreflexic
in the arms despite the atrophy. When he said he wanted to stick an EMG needle
in, I wanted to run out of the room. I felt a wave of nausea. Not because of
the needle itself, but because of what I knew it would show and of what I would
hear. I fought down the nausea.
When the needle when into my right deltoid it didn’t take
long to hear the characteristic sound that some have described as “rain on a
tin roof” and others as the “ticking of a clock”. To me it sounded like gun
shots. Every time he stuck the needle in it was the same, right down into my
hand. My left arm too. The legs were normal. But I thought I could hear it again
in the thoracic paraspinals. I could not think of anything other than ALS that
could do this. Dr. McNeil was obviously concerned. He asked me who I wanted to
be referred to. One thing I’ve learned is that if I ever needed a Neurologist I
would want that person to be Chris White. With his permission I have included
his consult note in the section below.
An Urgent Consult
After I left the EMG lab I immediately texted Ronak who met
me on the 12th floor in one of the clinic rooms. I think he was
shocked by what I had to tell him. Then Chris White called me. He had dropped
everything to see me immediately. He didn’t have his neurology tools with him
but I had mine. That is how my worst fears ended up being confirmed with my own
reflex hammer. At the end of the assessment I could tell that Chris White was
worried. He told me he was very concerned that we were dealing with motor
neuron disease. More than by what he was saying I could sense his concern in
his body language. He gave me a hug. I was scared. Although I had suspected ALS
it wasn’t until I saw Chris White’s concern that it really hit home. I had dark
thoughts. I wondered if I had seen my last patient. How would I break the news
to my family? How much time did I have left?
I decided that I wanted to go home to Lethbridge to die in
my parents’ home. Initially I would be able to live in the basement, but at
some point I would have to move up to the main level when I could no longer
walk. I would enjoy my Dad’s gourmet cooking for as long as possible. I would enjoy the wine I had brought back while I could still swallow.
Dr. White told me it was important to have hope and that we needed to do
an MRI to rule out a cervical spine lesion. I said: “I think we both know what
we’re dealing with”. He didn’t argue with me. I believed with certainty at that
moment that I was going to die a horrible death. I would lose control of one
bodily function after the other until I was quadriplegic, unable to swallow,
unable to speak, and unable to breathe, and not necessarily in that order.
Consult Note by Dr. Chris White:
Tyson Brennan Peddie BRUST – October 31,
2013
PROBLEM LIST
1. Under investigation for possible amyotrophic lateral
sclerosis.
2. Spontaneous intracranial hypotension – remote.
Tyson Brust was reviewed on October 31 in the
outpatient clinic setting. This 35-year-old right-handed male, a neurology
resident, was referred for assessment of possible motor neuron disease. Tyson
felt well until this summer. He was at a wedding on July 25 and noticed that he
dropped a cup. He noticed thereafter difficulty reaching and then clear
weakness around his shoulder. This has reached the point now that he uses his
left arm for most activities. He has noticed no weakness in the hand itself.
Over the last 2-3 weeks he feels there has been a bit of weakness in the left
arm. He has noticed clear fasciculations in the right shoulder and arm, and now
to a lesser extent the left arm and both legs. He has noticed cramping in the
shoulders and perhaps some more cramping than usual in the legs. He denies any
truncal weakness. He denies any shortness of breath, dysphagia or dysarthria.
He denies any weakness in the legs or change in his gait. He has had no pain,
except for some mild discomfort around his right shoulder, which I think he attributes to the weakness that he has. There has been no
numbness, tingling or other sensory complaints. There has been no change in his
bowel and bladder function. There has been no change in vision. There has been
no change in cognition, although obviously he has been more anxious, having
suspected for the last few weeks that he might have ALS. There has been no emotional
lability. He did have an abnormally long upper respiratory tract infection with
cough, low energy and coryza in August, but his symptoms clearly antedated
that. Other than this, there have been no fevers, chills, sweats or weight
loss. His past medical history is significant for an episode of spontaneous
intracranial hypotension, which in retrospect may have related to a snowboarding
injury. He had a positional headache, which resolved spontaneously. He never
did have imaging of his spine associated with this.
His only medication is zopiclone that he will take p.r.n. to
help him sleep after a night on call.
He has no known drug allergies.
His social history reveals that he is a neurology resident.
He has a roommate, who he also considers a friend. He is not married. His
parents live at home in Lethbridge. He does not smoke. He has two or three
drinks of alcohol a week. He does not use any street drugs.
The family history reveals parents who are alive and well.
He also has a younger brother, who is well. There is no family history of
similar problems.
On examination there were no carotid bruits. His heart
sounds were normal. His chest was clear. There was no hepatosplenomegaly and
his abdomen was nontender. There was no obvious rash, nor obvious changes in
the joints.
On neurological examination there appeared an intact
sensorium. Formal mental status testing was not performed, but he gave a clear,
concise and consistent history. Cranial nerve assessment revealed full visual
fields. Visual acuity was not assessed. Funduscopy was normal. His extraocular movements
were full. He did have a mild right exophoria. There was no ptosis. Facial
sensation was normal to light touch and pinprick sensation. The jaw jerk was
normal. Facial strength was normal. I could not appreciate facial
fasciculations. Hearing was grossly normal. There was no dysarthria. The palate
appeared to move normally. The tongue was midline and displayed normal bulk,
without fasciculations. Tongue dexterity was normal. The sternocleidomastoid
and trapezius muscles demonstrated normal bulk and strength, without fasciculations.
Neck flexion and extension were normal. There was a clear decrease in bulk of
his arm and scapular region on the right-hand side, including the biceps, triceps, deltoid, supraspinatus and
infraspinatus. I think there was also a bit of a decrease in bulk of the
infraspinatus and supraspinatus on the left-hand side. Fasciculations were noted
in the right shoulder and arm. I could not appreciate fasciculations in the
left arm or either leg today. Testing of tone was normal in all four limbs.
Strength testing revealed the following: deltoid 3, 4;
biceps 4-, 5; triceps 4-, 5; wrist flexors 5-, 5; wrist extensors 5, 5; finger
extensors 5, 5; FDI 5, 5; ADM 4+, 5; APB 5, 5; hip flexors 5, 5; knee
extensors 5, 5; knee flexors 5, 5; ankle dorsiflexors 5, 5; right and left
respectively.
Sensory testing was normal to pinprick and vibration
sensation. Deep tendon reflexes revealed quite a brisk right triceps jerk when
compared to the left. There was evidence of finger flexion with testing of the
biceps and brachioradialis bilaterally, but the jerks themselves were not
brisk. The knee jerks were quite brisk, but without evidence of spread. There
were two beats of clonus at the ankle.
Plantar responses were flexor. Tyson does believe that his
knee jerks are much brisker than they have been in the past and obviously these
have been assessed as part of his medical training.
Finger-nose and heel-shin testing were normal. Stance and
gait were normal. Tandem gait was normal. Romberg sign was absent.
He has recently undergone electrophysiological studies and I
have not had a chance to formally evaluate these yet. I have discussed these
with you and there is evidence of active denervation and chronic motor
unit remodeling in the right arm and, to a lesser extent, the left arm. You
wondered whether there were fibrillations and positive sharp waves in the
thoracic paraspinals. You felt that the legs were normal. You did make specific note that there was
no evidence of fasciculations in any of the muscles that you evaluated.
He has not had any further investigations at this point.
SUMMARY
This 35-year-old right-handed male presents with a 4 month
history of progressive weakness, with prominent lower motor neuron findings in
the right arm and to a lesser degree the left arm, which is corroborated by
electrophysiology. There are suggestions on the examination of upper motor
neuron dysfunction, particularly with the brisk right triceps jerk, especially
given the weakness in that muscle and the brisker reflexes at the knees. Unfortunately, I
think that it is likely we are dealing with motor neuron disease. It is
important to note that a ventral problem affecting the cord and the motor neurons
at the cervical level could cause an abnormality like this and he has had an
episode of spontaneous intracranial hypotension, raising the possibility of an
anterior dural defect, which needs to be evaluated further. He also needs the routine blood
work and screening that would go along with any of our patients that we see in
the ALS Clinic.
I did discuss all of this with Tyson today and obviously he
has a very clear understanding of what we are seeing, what we are discussing
and what this might mean. I did get his permission to talk to Susan Munroe, our
Clinic coordinator, and get her to contact him to see how he is doing, and I
will talk to her tomorrow when she is back in the office.
I have talked to Mayank Goyal to see if we can
expedite an MRI of the brain and cervical spine, and we will work on getting
this facilitated tomorrow. Early next week we can arrange for the routine screening
blood tests to occur. Obviously it is to be hoped that we find an alternate
explanation for this, but I think that the odds are against it and so does
Tyson.
Tyson wanted to go home today to see his family. He does feel
that it is safe for him to drive and certainly I trust his judgment in this
regard. He does have a friend whom he can confide in and talk to, and has done
so after seeing me today.
I have sent an email to Michael Yeung, the residency
training director, telling him that Tyson is on medical leave. I also do have
Tyson’s consent to discuss the case with him further should that be appropriate.
Thank you for contacting me today and helping in providing
the care to one of our residents.
Yours truly,
DICTATED BUT NOT READ
Chris White, MD, FRCPC – Neurology
Electronically signed by Chris White, MD, FRCPC – Neurology
at 10:15 AM on 2013-11-01
CSCN – Fellow (EMG)
ALS Clinic
The Dark Ride Home
I wanted to go to Lethbridge to break the news to my
parents. I convinced Dr. White that I would be safe to drive. After all, I had
been driving around ruminating about ALS for weeks. But now it was different. I
felt it had been confirmed. I drove home in a daze to pack for my trip to
Lethbridge. I called my parents and said that I had an unexpected few days off
and wanted to come down for a visit. They were happy for what they thought was
a pleasant surprise. Ronak had been waiting for me in the resident room but I
told him I would meet him at his place on my way out of town. When I got there
I said: “It’s bad man.” He poured us each a glass of wine and I told him about
my discussion with Dr. White. At one
point I said “you’re one of the only ones that truly understands what I’m up
against.” We hugged and cried. That would become a theme.
Ronak would not let me drive home alone. He insisted that he
would drive me home. I argued but he really did not give me an option. I texted
my Dad saying Ronak wanted to come too and he replied “Great!”. My parents love
it when I bring friends home and they had met Ronak already at Waterton Lake
back in August. Still, we had no good explanation for why Ronak was driving me
in my own car mid-week. I decided to bring the bottle of wine we had cracked
and drink it on the car ride home. This would provide an explanation, albeit a
weak one. I would tell my parents I had had a few too many drinks at Ronak’s
place so he had driven me home. Also, I really just wanted to drink some wine. Arriving home in such a fashion would be way
out of character for me (believe it or not) and my parents would know that
something was up. But I would tell them in due time. I did not want to tell my
parents immediately about the possibility (in my mind the certainty) that I had
ALS. I wanted one more night of normal. I planned to tell them the following
day after Ronak had driven back.
Cruel and Unusual Punishment
Normally on our road trips Ronak is the DJ but tonight it
was my turn. I was in the mood for Metallica. I drank the beautiful Inkameep
red wine Ronak had brought back from Osoyoos right out of the bottle. A lot
became clear to me on that ride and I shared my thoughts. Ronak proved a good
listener to my rants. I realized that my greatest achievements were not being
Valedictorian of my high school, completing a PhD, publishing scientific
articles, being accepted into medical school, or being accepted into Neurology
residency. Instead I was most thankful for the life experiences I had had and the adventures I had been fortunate enough to embark upon.
Jeremy, myself, and Tom at the Great Pyramid of Cheops, near Giza, Egypt. This photo was taken on our London to Cape Town adventure in 2008.
I was thankful for the friends I had made and the time I had spent with my family. I felt grateful that I had been accepted into a residency program so close to my parents and had had the chance to visit them often. I also felt
that it was extremely lucky that I had already been living my life as if
something like this would happen to me. I have always loved the James Dean
quote: “Dream as if you will live forever; live as if you will die tomorrow.” I
had crossed a lot of items off of my bucket list already. But the grief came in waves when I thought of all the things I still wanted to do.
I took this photo of a mountain gorilla in Uganda on our London to Cape Town motorcycle adventure of 2008. Even though it likely meant missing a week of medical school to take the detour to Uganda, I was able to cross Gorilla tracking off of my bucket list. It was well worth it; the hour that we spent watching these magnificent creatures in the high mountain forests of Uganda remains a highlight of the trip and my life.
Another powerful thought that came to me is that my quality
of life during my dying process would be infinitely higher if I had control
over my own death, even if I was physically unable to kill myself. I was more
afraid of the anticipated suffering than I was of death itself. If I had the
knowledge that I could have help exiting this world when I decided the time had
come, then I would be able to actually enjoy a few moments in the present. Without
that control over my own life and death, the existential suffering of knowing
what was going to happen to me felt so profound as to eliminate any quality of
life at all. Forcing people to endure a futile existence filled with pointless
suffering when they are terminally ill and have decided that they want to end
their life is cruel and unusual punishment. That is not “sanctity of life” – it
is torture. Why should I be subjected to laws that are largely based on the religious beliefs of others when I do not share such beliefs?
Whatever happens to me I plan to join the cause of changing the
laws in Canada so that doctor-assisted death is allowed for those who are imminently dying and of sound mind. The specific conditions that would make a patient eligible for a doctor-death need to be debated openly by the medical community, but I think the Oregon model is a good place to start. Right
now anyone who was willing to do me this Final Favour would be putting
themselves at considerable risk. My mother would later tell me that she would
push the lethal injection herself even if she had to spend the rest of her life
in prison. But how could I exit in that way knowing that my mother could be
prosecuted?
Part of me imagined that if I were to take up Sue Rodriguez’s cause it would give
me some purpose during my final months. It is past time that the laws were challenged again in this country
to legalize doctor-assisted death. But I also knew that this would bring
greater scrutiny and could eliminate any chances of an anonymous
assisted death here. My fear of not having that option was overwhelming. I was not
sure I had the courage to be the next Sue Rodriguez. No, to ensure I had the
option to die when I wanted, I would probably go to Switzerland for a legal doctor-assisted
death if it came to that. It would be a tough decision because I wanted to die at home, but not if I did not have end of life rights.
A Cruel Cosmic Joke
By the time I got to the bottom of the bottle of wine, I had
become angry. Why couldn’t I have died in a motorcycle crash? I had certainly
had many near misses. What a cruel cosmic joke to come through all them just to
die a horrible slow death now. In Germany I had taken Ted’s Fireblade up to 270 km/h on the
autobahn, but even though there was still more left on the throttle I didn’t
have the nerve to take it to its’ top speed of 300 km/h. Why the hell not?
Crashing at 300 km/h would have been a good death. Instant nothingness.
This is a photo I took of Ted on the Honda Fireblade 900RR in Romania (2008). I think he had had the nerve to take faster than I ever did, at least up to 290 km/h. But did he ever get it up to 300 km/h? *Edit: Ted has since told me that he did top in out in Turkey. Good job man.*
In
Honduras I had come around a corner on a mountain road with a cliff on one side
an a sheer drop on the other and came face to face with two semis; one trying
to pass the other on a blind corner. I threaded the needle and rode the yellow
line between them being buffeted by their draft. There must have only been
inches on each side. If I had been in a car it would have been a fatal head-on
collision. That would have been a good spot. Another near miss happened in Peru
when my fork seal broke on a mountain road pouring fork oil onto my front
tire causing the bike to high-side on a corner and slide across the highway.
Why was there no oncoming traffic at that moment?
I created my own oil spill when my fork seals failed spilling oil onto my front tire which caused the bike to slide out from under me and skid across the road into the potential path of oncoming traffic. You can see the scrape marks on the pavement where my bike slid across. Right after I picked up my bike and took if to the side of the road a semi truck came roaring around the corner and drove over where my bike had just been lying.
Another good spot would have
been in Ethiopia when I had been overtaking a semi and a dog ran right out in
front of the semi into my path. I hit the dog full-on the poor animal got wedged under my bashplate and my bike was launched skyward
and came down on its nose, breaking the front cowling and splitting my windscreen. I was thrown down onto the highway into the path of oncoming
traffic. If it had happened anywhere else I would have almost certainly been run
over but it just so happened that there was a railway crossing right there that
everyone was slowing down for. The oncoming trucks were able to stop in time. My
only injury was a laceration to my elbow.
Jeremy, is that a first-aid kit or a toolbox you’re holding? It seemed we always needed one or the other; often both. These photos show us patching my bike and I up after I hit the dog in Ethiopia just outside of Addis Ababa and was thrown from the bike. Jeremy would suture up the front cowling of my bike with zip-ties that night. A surgeon already.
And if not there, why not in rural
Kenya on the isolated Marsabit road when I had come across a vehicle that had just been shot up by machine
gun fire in an ambush in progress? I was able to turn around and ride away. There
have been so many opportunities for a good death. My family would still be
devastated of course, but at least they could take some comfort in knowing that I had died
doing something that I loved. I’ve always said that I ride a motorcycle not
because I want to die, but because I want to LIVE.
We were almost in Lethbridge by the time I got into the Talisker and was singing along to Fade to Black: “Death
greets me warm, now I will just say good-bye”. I had brought the Talisker back from the Isle of
Skye, having stopped there during our motorcycle trip through Scotland this past July.
I had been saving it. Saving it for what? Be damned if I was going to leave any
good Scotch behind.
Above are photos taken by Tom Smith and depict our recent motorcycle trip through the highlands of Scotland. This trip was supposed to me my next blog post but I have not had the time and life has intervened.
When we pulled into my parents’ driveway I admit I had a
meltdown. I knew the news I was bringing would devastate my family and the
thought of them having to watch me waste away and become my caregivers filled
me with so much sadness. I was supposed to be their caregiver one day. Not the the other way around. Ronak suggested we go somewhere for coffee first, but
I said: “we’re going to go in there and put on a show”. And we did. I got my one
last night of normal. Happy Halloween.
Breaking Bad News
The next day (Friday, Nov 1) I found out that I had an MRI
scheduled for the following morning at the Foothills hospital. Ronak had stayed
the night at my parents house and had driven my car back to Calgary that
morning because he had an afternoon clinic at the South Health Campus. Our plan
was for him to drive back down to Lethbridge after work to pick me up. I would
have to tell my parents what I was dealing with before he got back to
Lethbridge to get me.
The moment to break the news came when I was sitting with my
mom and dad in the garage with the door open watching the sunset with a nice
glass of red wine I had brought back from my recent trip. My mom actually started the conversation by asking: “what do
you think of intuition?”. She knew I had something to say. It was an opening.
“I have something I have to tell you.” I told them I had been getting weaker
for the last 4 months. I explained that there was a good possibility it was
ALS, or Lou Gehrig’s disease, but that we had to do an MRI to rule out other
causes. My mom immediately became emotional. She started to shake uncontrollably, spilling her wine in splashes on the garage floor. She knew more about ALS than I
realized as she had just read a book where one of the main characters had the
disease. She said “we’re with you until the end,” and started sobbing. My dad,
the optimist, said “there’s still a chance it’s something else, right? This is
not for sure yet.” My parents insisted on driving me to Calgary for my MRI. I
called Ronak and told him he need not come. I would need him to meet us at the
hospital the next day to comfort my parents while I was in the scanner and
because I knew I would look at my images. And I didn’t want to do that alone.
An Incidental Finding
I’ve ordered countless MRIs for patients over the years but
this was my first time in the tube. I had no idea how confining it would feel
or how loud it would be. I was getting imaging of my brain and C-spine with
contrast and it look a long time; probably about an hour. I didn’t mind being shut off from the world. For me, the worst part about
the experience was that the fasciculations never let up the entire time I was in the scanner. Lying there fasciculating on the cold slab I could not help but think, over and over again: “I’m a corpse.”
After the MRI was completed I had my parents wait in the lobby
and Ronak and I went down to radiology for the moment of truth. We found a
deserted corner in the darkness. How many scans had I looked at there while on
my Neuroradiology rotations? I felt like I was living a surreal nightmare. I
had Ronak drive. When we first pulled up my scans none of the images had loaded
so we had to keep refreshing, prolonging the agony. Finally they were there.
“Brain or C-spine?” Ronak asked. Of course we both knew where the money was. Save
that for last. “Brain,” I said. I thought there was diffuse dural enhancement,
but other than that it looked normal. Surprisingly there was very little
cerebellar atrophy. Now it was the moment of truth. My life was in the balance.
We pulled up the C-spine images. On first inspection things looked grossly
normal on the sagittal T2. There was no big syrinx, disc herniation, or tumour.
There did not appear to be any abnormal signal within the cord. I said “part of
me was still hoping for some kind of miracle”. Ronak said “sorry man”. Then I
saw it: the dura seemed to be suspended within the CSF both ventrally and
posteriorly. “What’s this outside the dura?” I asked. Ronak flipped to the T1.
Dark on T1, bright on T2. Not fat then; it was CSF intensity. In one posterior
location the collection appeared to have eroded through the ligamentum flavum
to outside the spinal canal itself. On the axials, the dura seemed to be
adhered to the cord with CSF-intensity collections outside of the thecal sac
circumferentially surrounding the cord. “Is the cord flattened here?” I asked.
I had a sudden surge of hope. It was the first time in weeks that I had felt
any hope. What a beautiful feeling. What I was seeing was clearly abnormal.
Could it explain my symptoms? Later Ronak would tell me that he thought I was a
spine imaging whiz. I would reply that I was not – I had just had a figurative
gun to my head. The truth was that neither of us knew how to interpret what we
were looking at. We needed a Neuroradiology opinion.
I knocked on the door to the reading room and was invited
inside. I asked for a personal favour. The neuroradiologist pulled up my scan
immediately. As he was scanning through the C-spine images, he stopped. A surge
of hope. “Have you had any orthostatic headaches?” he asked. I replied that I
had had an episode of spontaneous intracranial hypotension 8 years before. He
told me that what he saw was evidence of the sequelae of spontaneous intracranial
hypotension but that it would not explain my ALS-like symptoms. My hope was extinguished.
Despair welled up. He shook my hand and he wished me luck. Ronak and I stumbled
out of the room and hugged and cried outside the closed door. Like I said – a
theme. To have my hope dashed was crushing and horrendous. I walked through the
hospital in a daze to find my parents. I saw people I knew who smiled and nodded
at me. I felt like I was drowning in a surreal nightmare. I found my parents
and said: “it is what we feared.” I could barely find my way out of the
hospital. The place where I had worked so hard seeing countless patients over
the years now seemed like an alien landscape. Now I would be a patient. I would
never see another patient. I was going home to die.
My parents rallied to my support. We would enjoy every
moment. We would drive to Saskatoon to tell my brother and his wife in person.
I would spend some precious time with my twin nieces, Ella and Lily.
The above photos were taken at Thanksgiving this year. I was already too weak to lift Ella and Lily on and off of my lap. Although they were pretty big for being only 15 months old!
If I wanted, we could go
to Hawaii while I was still mobile. We went to the River Café. There was a
heavy snow falling. We ordered a beer and made a toast “to life”. While my
parents planned our trip to Saskatoon, I thought about my impending death. It
was comforting knowing that I had a one million dollar life insurance policy
and that my parents would be taken care of and I would be able to leave a
legacy for my twin nieces. It saddened me how much of their lives I would miss
out on. But what was worrying me the most was whether there was a clause in my life
insurance policy that would prevent my family from getting paid out if I were
to choose a legal doctor-assisted death. I had already been thinking about going to
Switzerland for this purpose, and just the thought of having this final control
over the end of my life was bringing me comfort. My parents were supportive of
this and would take me to Switzerland if I wanted. I would have rather died at
home, but just having that option was taking away some of my fear of my
impending suffering. It suddenly became extremely important for me to make sure
that my family would still get paid if I chose this option. I would need to go
through my life insurance policy line by line. I had a project. But if there was a
clause that would prevent a payout in the event of a legal doctor-assisted
death, I realized that I was willing to tough it out for my family.
I also wondered about trying to go on one last motorcycle
adventure, but the truth was that I was already too weak. Instead, I told my
parents that I wanted to send my friends on an epic motorcycle adventure once I
was gone. I would join them in my own way. I wanted Tom, Jeremy, and Ted to
take my ashes to Mongolia by motorcycle. That’s a mission I think they just might have had to choose to accept.
I hope you guys would have a good Scotch and toast me somewhere along the way. The Glengoyne 21 is a good one, but my favourite may actually be the Talisker Distiller’s edition. Left: Bulgaria, next to the Black Sea (2008). Right: My fellow adventurers Jeremy, Tom, and Ted in a Scotch bar in Edinburgh, Scotland, at the outset of our recent motorcycle trip (July, 2013).
A New Hope
These were the darkest hours of my life. The snow fell wet
and heavy all afternoon. I was lost in dark thoughts. Then Chris White called
me. “Have you looked at your scan?” he asked. I said that I had and that I had
seen extradural collections but that I had reviewed it with neuroradiology and that
it was felt to be an incidental finding. He told me “I don’t think it is an
incidental finding.” He went on to tell me that this was reason to hope. “This
is what we were hoping for when we ordered the MRI”. He told me there was
reason to be optimistic. He was going to get a spine surgery opinion. He had
seen a couple of cases like this before. He sent me a link to a case report in
Neurology that was similar. My life changed. I was flooded with the sweetness
of hope. I told my parents there was reason to hope and there was a seismic
shift. We went out for a beautiful Thai dinner and enjoyed the taste of food
for the first time in days. Even if I end up having a dual diagnosis, I thank
you Chris White for that phone call. No matter what happens, you gave us the
gift of hope.
The day after hope was restored the sun came out and my parents (Walter and Susan) and I walked around Prince’s Island park and the Peace Bridge. Our world had changed.
I went back a couple of days later for MRI imaging of my
entire spine. Below is the formal report:
FINDINGS:
The study
confirms the presence of a fairly extensive extradural fluid collection throughout
the cervical and thoracic spine. The
collection extends from approximately the C1-2 level ventrally to the L2-3
level. In the cervical region there is
also a component that is located dorsal to the thecal sac terminating approximately
the C6-7 level. The collection dorsally
displaces the thecal sac effacing the subarachnoid space ventral to the cord.
In terms of
a possible site of CSF leakage, this is difficult to accurately ascertain. This
being said, there is an irregular appearance of the collection on the right at
the C2 level where I have the impression of a focal defect in the thecal sac
communicating with a compartmentalized appearance of the extra-axial
collection. At the T12-L1 level, the
ventral extradural collection extends towards the foramina bilaterally, left
greater than right though I cannot see a definite connection of this collection
with the CSF space of the thecal sac.
The cord
itself appears grossly unremarkable.
IMPRESSION:
Extensive
extradural fluid collection is seen in the cervical and thoracic spine as
described. This terminates at
approximately L2-3 level with no evidence of an extradural fluid collection
more inferiorly. In terms of potential
site of origin of CSF extravasation, this may be at the C2 level as described
above or in the lower thoracic spine as noted.
A Hard Landing; A Life Changing Event
The above photos were taken of me riding my snowboard by Tom Smith at Whistler/Blackcomb on one of our many snowboarding trips (circa 2005). I used to love to just let myself fly. I didn’t always have a soft landing.
I would not
have applied to medical school in the first place had I not become a patient in
the early spring of 2005. At the time I was 4 years into my PhD in Neuroscience at
the University of British Columbia. I had gone snowboarding with friends at
Whistler for the day as we often did. I loved doing rails and jumps. Towards
the end of the day I went off of a table jump badly and ended up landing hard
on my back from a height of about 5 feet. I was winded but there was no
immediate back or neck pain and certainly no neurologic deficits at the time. I
continued to ride for the last couple of runs of the day.
I actually did think it was a good idea to ride off a cliff. These rapid fire photos show me doing just that. I did it multiple times. At least I was wearing a helmet. This was an example of a hard landing (circa 2005 at Whistler/Blackcomb). However, this was not the fall that would cause my problems. That one wasn’t captured on film.
The next
day in the lab I had a thunderclap headache. It was a sudden severe bilateral
occipital and cervical headache that reached maximum intensity in seconds. I
had never before experienced pain even close to that level. I had never even
had a headache prior to that. It was 10/10 constant stabbing pain but I could
get it down to 7/10 by lying on the floor in the lab. When I sat up or stood up
the pain was so intense that I became nauseas and vomited twice. Of course in hindsight I should
have called EMS. Instead, I tried to walk back to my graduate residence. I
lived on campus and I thought I could get someone from my residence to drive me
to the hospital. I had to stop and lie down on the grass multiple times on my
way home. What normally was a 10-minute walk took me 45 minutes. My friend from
my lab ended up driving me to the emergency department at Vancouver General
Hospital. The only way I could ride in the car without vomiting was to have the
seat completely flat. In the waiting room I had to lie flat across several
seats. I was seen by Neurology and a CT and CTA were completed and
were normal. I had a normal neurological exam. Because of my nausea and
photophobia (I was indeed sensitive to light) a diagnosis of migraine was made.
I was started on intravenous fluids and a cocktail of abortive migraine
medications. Of course none of this had any impact on my severe orthostatic
headache.
Eventually
I was discharged home without being admitted or receiving further imaging. I
spent the next 3 days unable to leave my room because every time I sat up I
had 10/10 pain and associated nausea. Lying down the pain was 6 or 7/10. When my parents found out what was
going on, my Dad booked the next flight to Vancouver and another friend of mine picked
him up at the airport. The two of them took me back to the VGH emergency
department. Again I had to lie flat in the waiting room. There had been no
improvement since the headache first came on. Again I was seen by Neurology.
The neurologic exam was still normal. Again I was started on migraine
treatments and again there was no improvement. I spent the night in the
emergency department but was not admitted and no further investigations were
undertaken.
In the
morning I was discharged home to my residence and spent the next month lying
flat on my back. My Aunt Alice took me into her home in White Rock and her and my Dad helped me with meals because I just
could not stand up. When my Mom got time off work she joined us there as well. At one point I went to a chiropractor. I shudder to think of
the neck manipulations now. After a month my orthostatic headache slowly
started to improve. I went for cranial sacral massage and this actually made me
feel better. Around this time I saw a Neurologist as an outpatient. I was referred
to a brain injury clinic where I was given information about concussions. Although
after a month I was able to start working in the lab again, it would be about 2
or 3 months until I was completely free of orthostatic headaches. Since then I
have been symptom-free.
My
labmate’s wife happened to be a Neurologist and when she heard my story she later told me that it sounded like a dural tear. By then I was much improved and did
not go back for any further assessments. I looked up “dural tear” on the
internet and realized that of course that’s what had happened to me. The resulting
intracranial hypotension would explain my persistent orthostatic headache that
resolved over weeks. In my case I had probably had a traumatic CSF leak caused
by landing hard on my back after failing to land a jump on my snowboard.
I have a
confession to make: my application letter to medical school was not entirely
accurate. In it I explained how the above experience shaped my desire to be a
doctor. This part is true. I started studying for the MCAT while I was lying
flat on my back at my aunt’s house. I would never have applied to medical school had it not been
for my experience as a patient and the time I had to reflect. What I loved
about science was figuring out the right question, generating hypotheses,
testing those hypotheses with experiments and laboratory investigations, and
then experiencing the thrill of discovery. As a patient I had been amazed at the power of
the neurological exam to test hypotheses and the power of diagnostic testing to
rule out a life threatening diagnosis. I realized that a process that could
take years in the lab could happen with every patient encounter. An added bonus
for me was that I enjoyed interacting with people. I had spent too many years
interacting with rats. It may sound trite, but I wanted to help people. I
applied to medical school knowing that I wanted to be a Neurologist.
What was
not accurate about my application letter was the positive spin that I had put on my
experience with the health care system. I lied and said that I had been diagnosed with a dural tear much earlier, when in fact I had been treated for migraine and concussion and it was only during an informal conversation with a Neurologist after the fact that the true diagnosis emerged. In retrospect I think that my treatment
by the health care system was suboptimal to say the least. One of the
motivations I had for applying to medical school was that I wanted to do
better. That did not go into my application letter. I wanted to stay positive.
Occam’s
Razor
Where am I now? The hope is
that my traumatic CSF leak 8 years ago and resulting ventral extradural CSF
collection has been causing a progressive bibrachial amyotrophy mimicking ALS for
the past 4 months. There is some precedent in the literature for this http://www.neurology.org/content/76/16/1439.extract. There have been cases reported in the literature with delays between the initial CSF leak and the onset of neurologic deficits as long and sometimes even longer than my 8 year delay. The mechanism is thought to relate
to chronic dynamic pressure upon the ventral cervical spinal cord by the encapsulated
fluid collection. The next
step in my case is a dynamic subtraction myelogram to better elucidate the area
of CSF extravasation. Surgical options will depend on whether the site of the dural
tear can be identified. There may be major spine surgery in my future, but that is a truly wonderful possibility. There is some chance that I could actually improve, or that at the very least the progression of my weakness could be halted.
There is a chance that I have both ALS and an incidental ventral dural abnormality. However, this would be an affront to Occam’s razor.
Nonetheless, I must face the possibility that if I do have ALS I could be
spending some of the last of my mobility undergoing neurosurgical
interventions. Still, I feel so lucky to have hope. It is a gift.
“Whose body
is this?”
I realize
that doctor-assisted death in terminally ill patients is an extremely divisive
ethical and legal debate and that there will be people who will be deeply
offended by me coming out in favour of it. However, after believing with
certainty that I was dying of ALS and drawing so much comfort from the idea of
being able to choose the timing and manner of my exit from this world, I
believe that to not advocate for this same comfort for others would be the
height of hypocrisy. I’m not sure if the universe is trying to tell me
something or whether circumstances aligned to so that I could tell the universe
something. Either way, I cannot ignore the feelings I went through. I am not
advocating breaking the law; I am advocating changing it. I agree with what Dr. Low recently said to those opposed to changing the law on doctor-assisted death in his powerful posthumous video:
“I wish they could live in my body for 24 hours and I think they would change that opinion. I’m just frustrated not to be able to have control of my own life. Not being able to have the decision for myself when enough is enough.”
I will end this post
with a quote from Sue Rodriguez who was diagnosed with ALS in 1991 at the age of 41 and ultimately lost her
Supreme Court challenge to legalize doctor-assisted death in Canada in a
landmark 5-4 decision in 1993 before dying with the help of an anonymous doctor
in 1994:
“If I cannot give consent to my own death, whose body is this?
Who owns my life?”
Sue Rodriguez at a press conference in 1993.
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