I have been meaning to post this one all week. I thought I would give a little update on my progression and thoughts about the ride.
In 2011 I was still standing, walking, driving and barely feeling weakness in my upper body. In 2011 I was dreading losing my legs. Today, I'm dreading losing my arms and hands.
Today I'm struggling to lift cups, button shirts, opening already loosened bottle caps, holding items between my fingers and so so much more. The past 4 years the progression of my upper body has become more alarming. To a casual onlooker they can't see the new weaknesses as well but to me they stare at me in the face daily, morphing into new and more elaborate forms every day.
In 2007 I met my very first HIBM patient. It was the ARM cofounder and HIBM research scientist. He also is an HIBM patient along with his brother.
Upon meeting him he asked Jason to grab a piece of paper for him. "A piece of paper?", I thought. He can't even grab a piece of paper?". At the time just my legs were affected so the notion of weakened fingers was foreign to me.
During my college years I vividly remember researching my disease in the basement of U of M library. I remember seeing the word "quadriplegia" but I'm not sure I paid enough attention to it. I've come to realize the textbook definition of any disease is much different than reality. You don't know anything about a disease or condition until you go through it. Not a bit.
One day I looked down at my hands and realized they looksd like other HIBM patients' weakened hands and fingers. My fingers and toes were beginning to look swollen lifeless and languid fixtures. What has happened to my legs is now happening to my arms, fingers, hands, shoulders, neck....The legs are one thing, but the arms and hands are an entirely different experience. With each level of progression I'm reminded of the severity of this condition and its depth. Pieces of me are disappearing like sand in the wind and time continuously haunts me.
We are currently driving to San Francisco and I'm excited for the ride. I'm not too worried about the trip. I have an amazing rig that was custom built for me, good company and a general belief that things normally work out. I try not to overthink the unexpected potentials too much, sometimes over planning destroys the spontaneous fun of it all.
If I had any concerns for the trip it would be being tired and in pain. When people see me they always say things like "you look great!" but in a surprising tone as if they were expecting the worse due to my condition. As if if you have a disease you should look like the most wretched person on the planet, but diseases take all forms and shapes and it's sometimes hard to see the levels of progression unless you are intimately witnessing them on a daily basis. In addition to the weakness the past 4-5 years I have developed 24-7 severe pain in just about every area you could think of. The pain is not a direct side effect of HIBM, though. Perhaps some of the pain comes from losing so much muscle mass around the joints and also me compensating my body to accommodate weaknesses but the rest of the pain and where it comes from is mysterious. The third issue besides the weakness and pain is being tired all the time. I get tired just from a simple hair wash now. I sometimes feel pain and being tired all the time is more of a battle than the weakness itself. So even though I'll be sitting in a rig while my friends pull me...8 days on the road, camping and battling it out with a porta potty will be an incredible physical strain on me, I won't lie. But despite this slight concern I'm ready and excited to see what happens. I try to avoid letting these three areas of my physical journey stop me from living life and I just simply couldn't pass on this 500 mile coastal opportunity.
Here is the column I wrote to accompany this illustration back in December 2014. Thought I would share:
"When I was a child, the last few weeks of the year felt like they would never end, and I was convinced extra hours had been added to each day. I was so consumed by the great Christmas ceremony of sinking into piles of brightly wrapped presents under the tree, and once that ritual was complete, I felt like the year had officially ended.
As we age, of course, the expression, “Where has the year/time gone?” starts to ring truer and truer. How I view the end of the year today is very different from how I viewed it 13 years ago. Now, I am not so anxious to usher out another year, but it’s not aging that has painted my perspective; it is mostly certainly influenced by my very rare and debilitating muscle-wasting condition, Hereditary Inclusion Body Myopathy (HIBM).
With the passage of time, I feel like I literally see sand cascading through an hourglass, and each grain serves as a constant reminder that, as a result of HIBM, more parts of my body have left me. Because I am still relatively young, this experience could not be more akin to the beginning of autumn, my favorite season. Like the change in seasons, something is decidedly dying, and it is bittersweet.
When my condition began to show noticeable symptoms in me in 2001, I was 22—young and active, as most people are at that age. It all began very slowly, and the most visible symptom was my most “stylish” gait. It began as foot drop, a gait abnormality in which the dropping of the forefoot happens due to weakness. Then, I slowly began to employ the use of a cane, then leg braces. Up to that point, I was too consumed with school and my search for a diagnosis to really think about how this condition could play out in my future. And, even when I was made aware of the fact that this condition would never stop and one day take my arms, hands, fingers—basically any part that was left of me hostage—I still couldn’t quite comprehend that. Or maybe I didn’t want to. One can’t perfectly understand until you experience it and travel through the milestones of degradation.
When my hands, arms, fingers and neck began to become affected, I was forced to pay more attention to the reality of my progressive condition, as well as to appreciate what I did have left a little more. I began realizing how close I was moving to the ultimate stage of immobility. I had to stop driving, a day I had dreaded so much. Driving was previously my way to escape. I began to cook incessantly and grew a great love for making meals for others, but today, I sense this beloved hobby slowly slipping away from me, too. Everything I love seems to disappear, not just parts of my body, but all the extensions of who I am. I soon began to illustrate, documenting my dying body through drawings, but even that is becoming a more challenging effort.
I think journalist and author Jean-Dominique Bauby said it best: “In the end handicap gives us vertigo.” It is a slow decline into infancy, and like the ocean waves that moisten our feet and then skips away, my past recedes while embers of my old life linger in my memory.
Of course, all of us are dying from birth, but for some of us, our personal clocks bear a much heavier weight. My disease makes me feel like I’m losing a loved one each time my condition worsens, except I keep losing that same person over and over, and each time in a new way. It is an uninterrupted coil of loss.
But all I can do is move forward. Looking back is detrimental to my personal growth. I still fondly sift through the memories of the self I once was, but deep down know, I’ll never be her again, and I reluctantly bid her good-bye.
For 2014, I don’t have New Year’s resolutions. I plan for uncertainty. Sure, reflecting on the past can be valuable and wonderful, but life is not checklist, something to be managed and controlled. None of what I would consider my great experiences were ever pre-planned. Instead, my constant “resolution” is to always stay open and adaptable.
The future is scary and at times makes me shudder, but I know the change the future holds will undoubtedly teach me something—a lesson that slowly lifts the veil to authenticity and manifests into something greater.
And, so for this year, I’ll do the same as last year. I’ll do my best to live life as it comes and always to be open. I will face fear and uncertainty, but not allow them to rob me of hope. This year there may be some great news in therapeutic development for my condition, and I will continue advocacy, including the work I do with the nonprofit organization, ARM (hibm.org), and my annual grassroots fundraiser, Bike for Kam (bikeforkam.com). I look forward to you following me in 2014, and hopefully together, more can be achieved in developing treatment that will one day slow down the progression of HIBM for everyone affected."