I’ve fallen and I can’t get up

My diagnosis and progression.

I sat in my room for what seemed like the thousandth night cast away in a deep dark dungeon of thought.

“I have to say something”, I told myself.

I knew that something wasn’t right with my body and it was getting worse.

A startling event a couple of years earlier was the first time that I became fully aware of my progressing muscle weakness. I had walked over to St. George, my 9th grade  Jr. High School at the time, dribbling a basketball on the way there, it was a nice summer night in Winnipeg and I figured I’d shoot a few hoops.

As I shot at the basket I kept missing. Except I wasn’t missing in the usual way – too far left or too far right. I just wasn’t able to reach the height of the basket. To me it felt like someone had snuck into the schoolyard and secretly raised the height to 12 feet. It sparked some panic into my heart and I couldn’t stop replaying it in my head.

I was wondering what was the best way to approach my mother. It had started with basketball but now I was having trouble riding my bike, climbing stairs, rising from seated positions and was having numerous slips and falls on the ice not to mention fracturing my shoulder falling down the basement stairs.

From all of the information I could gather in this pre-internet world I was living in, I had deduced that I most probably had some kind of muscular dystrophy or inflammatory disease.

I sat down with my mum trying to hold back a flood of emotions as I began to explain the physical symptoms I had been experiencing over the last couple of years. She had thought that I was simply too lanky and tall for my age and that it was due to  teenage awkwardness. I must admit that I did a good job hiding it from everyone.

Shame and embarrassment were the main emotions I had been experiencing. You would have thought that it was fear but at the age of 15-17 the last thing a teenage boy wants to deal with is looking like a freak.  

I find it hard to recall my mother’s reaction to the news. My mother was generally a pretty anxious person and I’m sure she spent a lot of time obsessing about it. We called my pediatrician and set up an appointment to meet with him as soon as possible.

My pediatrician’s name was Dr. Beasant . He was a tall, older, heavy-set man with rosy cheeks and he liked to wear his glasses on his nose. Dr. Beasant had a very dry sense of humor it was often difficult to tell if he was joking or not. My mother and I were anxiously waiting to get into the exam room. Once in the room, we waited a few minutes until the doctor walked in. The first thing he wanted me to do was to strip down and sit on the floor. Now, this wasn’t something that I was really excited to do because it was extremely difficult to get off the floor and I didn’t really like the cold tile on my body too much. I was then asked to stand up from the cold tile floor. One of the earliest signs of muscular dystrophy is something called the Gowers Maneuver which is the way a person compensates for muscle weakness to get up from the ground.  

People with MD usually get on both knees first and have their palms on the ground then we put our feet out behind us and typically lock the knees we then use our hands to push our upper bodies off the floor because of weakness in the abdominal and back muscles.  

After having all that fun getting down on the floor and back up like some sort of laboratory monkey we went through a complete physical and the doctor asked a lot of questions about my physical abilities. He noticed that my calves were enlarged. After checking me out thoroughly he told us that he thought I either had some kind of muscle inflammation or muscular dystrophy and that he was going to refer us to a neurologist.

Electromyography or EMG for short is a fun little test that sends electrical pulses into the muscles and measures the responses. It wasn’t awfully painful but it felt strange being poked and shocked from head to toe. This was one of the first tests that I remember having along with a blood draw to check my CK levels. The CK levels in my blood were elevated in the 500-600 range. I believe 80-100 is normal range. The CK levels can show elevated enzymes caused by muscle deterioration. The date was July 29th, 1994.

At this point the tests were showing enough abnormalities to schedule a muscle biopsy, a procedure I was not looking forward to.

In August of 1994 I went into St. Boniface hospital in Winnipeg MB Canada to have a muscle biopsy. It was an outpatient procedure and was not really painful but very uncomfortable, I recall needing some orange juice and a few minutes to get my head together afterword.

I sat down in a dentist like chair, the nurse shot my shoulder with something to numb it and then they went to work. They sliced open the shoulder and got in pretty deep to get a sample of my right deltoid muscle. The doctor joked that he wasn’t sure if I had enough to find any, which my keen sense of humor didn’t find hilarious at the time. After they finished I got stitched up and to this day I have a beautiful scar, except now it’s hidden under a lone wolf tattoo.

On September 1st, 1994 the results came back to my doctor and they were another disappointment confirming muscular dystrophy.

After his office received the report the doctor referred Mum and I to the genetics clinic at Health Science Centre for November 14th, 1994 to go over all of test results. This was basically going to be the big reveal. I had not yet known the results of my muscle biopsy nor did my mother.  We didn’t know if I had a treatable condition or a terrible neurological disease. We were very anxious and hoping to hear the best news, I remember praying for a curable muscle inflammation diagnosis. It would be difficult to explain all of the thoughts and emotions that I had leading up to this appointment, I mean, I was basically about to be told that I had something a pill could treat or an incurable disease that was going to affect me forever. For an 18 year old it was the most important meeting of my life.

The neurologist was sure I had a type of muscular dystrophy but he was only 80% sure I had Limb-Girdle MD as he believed my enlarged (hypertrophic) calves  pointed to the possibility of Beckers MD which is a more aggressive type of muscular dystrophy. He referred us to a genetic specialist.

The lab sent the results of the further dystrophin studies to him on November 16th 1995. We received a final letter dated November 27th, 1995 concerning the further test results of my biopsy and a summarized prognosis.

Finally. It was confirmed that I did not have Beckers Muscular Dystrophy and I officially had a clinical diagnosis of Limb Girdle Muscular Dystrophy. 

My simplest definition for Muscular Dystrophy is that the muscle cells leak the protein needed to maintain and build muscle thus resulting in progressive muscle loss. There are many different types of MD, the kind they think I have is not supposed to affect any internal muscles, such as the heart. After being diagnosed, there was nothing that they could do for me except tell me to check in once and a while to see how I was progressing. 

I was able to walk without assistance until about age 32. By then I had begun falling and injuring myself quite often and I was only able to walk short distances. I was also becoming unable to stand up from chairs and needed to sit at bar stool hight. I got a scooter and used it for about two years until I could no longer stand at all before I got an electric wheelchair.  I have been in a wheelchair since around age 35. I am still progressing slowly and need help with everyday tasks that most people take for granted. I am basically in the same needs category as a quadriplegic would be in as far as the ability to perform daily activities.

As I am writing this, I am looking out of my kitchen window at the lake and realizing how blessed I am. I never let MD kill my dreams and have a pretty normal life considering the fact. I have experienced a full life for someone my age, from running a business to having children it has been very complete. Now that I am fully disabled I still find myself so fortunate -living with the love of my life and her sweet daughter while still being able to enjoy nature and write.

I hope this blog post helps you understand what being diagnosed and living with a progressive disease is like. My hopes are that it wasn’t too depressing and answers questions that most people are too afraid to ask someone like me. 

Signing off for now, 

-Brent

Worry. What is it good for? Absolutely nothing.

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Worry. Such a dastardly word that carries with it too much weight.

“Worry refers to the thoughts, images, emotions, and actions of a negative nature in a repetitive, uncontrollable manner that results from a proactive cognitive risk analysis made to avoid or solve anticipated potential threats and their potential consequences.” -Wikipedia

Some of us were raised by worriers, while others have anxiety encoded in the DNA. The fact is that no amount of worry can change how the story of our lives play out.

I’m not a licensed therapist but I have had to cope with having a degenerative disease since childhood along with enduring the loss of my parents, closing a business, divorce, and having to sell my home and property. For me, coping with anxiety has truly been a part of life.

 I’d like to offer 5 steps I take to cope with anxiety and worry:

1. Size it up.

You need to take a step back and look at your problem from an objective viewpoint. Worry tends to enlarge problems, most of the time they are much smaller than we make them out to be. We tend to look at our troubles through a magnifying glass. Ask yourself, In the scope of 10 or 20 years is this thing going to have really mattered? Or, how many other humans have dealt with the same issue and come out just fine? I tend to ask myself, when I’m on my death bed will this have even mattered to me? So take some time to process and size up your worries.

2. Have a meditation spot.

Everyone needs a location that they can go to where they feel they can mute the rest of the world for a while and have time to think, meditate, or pray. We need to go there as often as necessary to consider the problem at hand.

Taking 20-30 minutes to process our problem in a peaceful surrounding helps us size it up and quite often we come out with a solution.

Think of somewhere you feel away from the world and distractions. A place where you can can just let your mind slow down. I like to go out on my back deck, everyone should have a spot.

3. Have a plan of action.

Failing to plan is planning to fail.  Even if the plan is short or needs more attention later – at least think about how you can take action. It may be as simple as saving money by eating out less. Coming up with a plan gives you a sense of control back.

4. Breathe.

Take time, especially when stressed, to make sure you are breathing deeply. We tend to take smaller, shorter breaths under stress. So breathe deeply to help relax and oxygenate the body.  A breathing technique that I like is what I call the 1234 technique. Simply count to 4 slowly while breathing in, then hold your breath while counting again to 4, then exhale while counting to 4. Make sure you are breathing from your diaphragm and not just your throat. Try it right now, it should relax you. Take as many breaths as needed but do it at least 4-5 times.

5. Distract Your Mind.

If you find yourself ruminating, have a good go to that distracts you. For me I watch movies or tv but everyone has a way that works best for them. Books, Facebook, a hobby, going out with friends, working out, time spent in nature is also very therapeutic- there are many ways to develop distractions for an over active mind. Find a few.

Winnie-The-Pooh said, “Promise me you’ll always remember: you’re braver than you believe, stronger than you seem, and smarter than you think.” My wish for you is to never have any worries, but you’re human so we know some things are bound to come up. When they do I hope you’ll take this advice to heart and face it with courage.

-With Love, Brent.

On Nature

“Forget not that the earth delights to feel your bare feet and the winds long to play with your hair.” – Khalil Gibran

In the early morning, I am often captivated by watching the small, humble, green bird-feeder perched on its black post in our yard. Our pint-sized feathered friends find shelter beneath its wooden green roof as they feast on the assortment of seeds it has to offer them. One by one they land and take off as if on an airport runway, taking turns sharing of its bounty while my furry chipmunk and squirrel friends wait below for left-overs.

During my newfound hobby as an amateur bird watcher, I have spotted a wide variety of feathered creatures: Blue Jay, Cardinal, Finch, Red-Bellied Wood Pecker, Humming Bird, Blue Heron, Cow Bird, Carolina Chickadee, Downy Wood Pecker, Egret, Hawk, Crow, Buzzard, Sparrow, Mallard Duck, Owl, and Canadian Geese.

From my vantage point above the lake cove, I have also had the opportunity to do some fish watching. Bass, Catfish, Crappie, Carp, Blue Gill, Perch, and other Brim all come near the shoreline.

Rachel Carson said, “Those who contemplate the beauty of the earth find reserves of strength that will endure as long as life lasts.”

What is it about nature that provides such a grand sense of serenity?

A person cannot find it at the mall or in downtown New York -you wouldn’t go there to relax. Nature offers a feeling of connection that nothing else can. Maybe it’s evolutionary, our very genes having the long-term memory of the outdoors encoded within. Whatever it is; being around other living things keeps humans grounded. Standing Bear said it best when he stated, “Man’s heart away from nature becomes hardened.”

The other day I was enchanted by a local nature trail. The babbling brook with its shallow, white, stony rapids trickled next to an immense variety of plants and flowers. A natural spring we spotted had water bubbling out of a hole in the earth. Climaxing with a lakeside view timed perfectly with the setting sun. The pallet of color dancing across the evening sky along with everything else we experienced was like the earth was singing us a symphony. It left me with a sense of wonder.

It’s like recharging your batteries. Just observe all of the places people go on vacation. Why do you think so many rehabilitation centers are near water or nature? Pythagoras exclaimed, “Leave the road, take the trails.” Maybe we are closest to God when we are close to nature, maybe we are simply closer to our origins. But for all of the architecture man has created nothing can match the woodlands.  Man’s greatest symphony is but a clanging cymbal when compared to a late summer’s night on the lake.

Even today, nestled in our small quaint cove, I sat on the aging wood of our gray deck looking out over the still waters. I took notice of a green-headed mallard on our dock- drowsing in the sun and couldn’t help but feel a part of nature.

Allow me to leave you with the thought of Henry David Thoreau when he recorded, “I went to the woods because I wished to live deliberately to front only the essential facts of life, and see if I could not learn what it had to teach, and not when I came to die, discover that I had not lived.”

When was the last time you felt the grass under your feet and between your toes?

-Brent