We have all heard it. " Live the moment, live for today, take it one step at the time". But what seems and sounds so easy to do, actually becomes the most difficult to achieve in our lives today. How do we treasure the moment we are living right now. To soak in and appreciate what we have right now. We are bombarded by constant distractions of stress, responsibilities, work, sickness, loss of loved ones, etc, etc. It seems today with all our comfort luxuries. Everything is at our finger tips. We can get everything in moments notice. Turn on our heat, air conditioner, go on the internet and the world is a click away. But we forget to slow our down lives appreciate it and gear it all down. How we do this?

My name is William Knetsch and here is my story.

I am fifty eight years old and was born with with a rare form of muscular dystrophy called Myotionia Gongenita. It stiffens every muscle in my body like a hardwood plank at any given time. You name a bone in my body and I have probably broken it. Because when my muscles stiffen up and locks me up. I fall and it depends where I am on the severity of the injury. Because I looked healthy as a child I could not do sports or do what every child does and takes for granted. I wished then I could wear Polio type braces on my legs so everyone could see I was not "normal". But instead I would make up any story and excuse to hide the fact I have a muscular dystrophy. I would hide it as best as I could and pretend everything was just fine. It was a curse for me as a child and young man. But it took me until I was in my thirties to realize what I have is actually a blessing. I have to live the moment. Constantly be aware of my surroundings. I have learned to appreciate all that I am able to do and achieve. I never look at a staircase and see all the steps I need to take to get to the top. because it is overwhelming for me to get to the top. But I look down and check out one at the time and before you know it I am at the top. That is how I live my life, one step at the time. I became a photographer because of my Myotonia. It was the perfect profession for me. Because with photography you have to stop study and capture the moment. To slow your brain down and soak in the world around you. Once you do this everyday the world becomes this magical place. Where everything is possible and you can go as far as your imagination can take you. It has taught me not to be afraid of the world but take on any challenge before me with strength, pride and at the end a great feeling of accomplishment. So as simple when I sit in a chair and my body is completely stiff and it takes every ounce of energy to stand up and walk "normally". Or to take a cup of coffee and bring it to my lips without dropping it, is everything for me. I tell myself " Bill you did it" and I am proud of just doing something that the rest of the world just takes for granted. It empowers me to appreciate and soak in every little detail of every day. If that is not a blessing then I do not know what is.

Type of Myotonia: Myotonia Gongenita

Country: Canada

William, thanks for sharing! I really appreciate your outlook.

Are you familiar with the Muscular Dystrophy Association's art collection program? You can submit photography for consideration:

MDA Art Collection

I have been a photographer for many years - it was one of the few jobs I could handle when I was younger and very stiff all the time. It got a little more challenging when I started doing some virtual tours and real estate photography because I had to climb around hillsides and perch on rocks to get good shots, but I just took it very slowly!

I find that when you live with a chronic health challenge sometimes you feel like you have black holes in your life and it seems like the world is zooming past. Having pictures of even mundane things gives me an anchor because they capture a moment in time, and I can live the memory over and over to remind me I'm here and I'm alive!

Jan

Type of Myotonia: Thomsen's

Country: US

Hi Jan,

Thank you for your reply and the contact for anyone with muscular dystrophy to submit their art work. I wrote my letter to try and inspire and give people hope and strength that have this ailment. Especially young children and young adults. When I was growing up things were just not talked about and swept under the carpet. I always felt left out, different and had no support and thought I was the only one with this challenge. Except my parents, who tried their best to get me through it all under the circumstances at that point in time. As I said, I was in my mid thirties when I turned everything around. It was a mind set to make a positive out of a negative. I feel honoured to now be able to share this with my fellow brothers and sisters who share what I have. Knowing that life can be amazing. I want to thank you for this forum and keep up the great work. You have no idea what this will do for us, all around the world. Knowing we are not different or weird, but special. All of us have to remember, " to finish a journey it requires all of us to take it one step at the time". We have a gift. We are aware and appreciate every one of the steps we take. Thank you.

Bill

Type of Myotonia: Myotonia Gongenita

Country: Canada

Hi William!

There is a form of muscular dystrophy that involves myotonia called Myotonic Muscular Dystrophy, but it is not the same as Myotonia Congenita.

People with myotonic muscular dystrophy, on average, tend to have far more severe weakness and muscle wasting, shorter life spans, and the disorder can cause cataracts, kidney problems, brain atrophy, and heart problems, to name a few things. I think their myotonia is generally less severe....or they tend to not acknowledge it as much for some reason.

Thankfully Myotonia Congenita doesn't seem to be nearly as life threatening! Our most prominent symptom is usually the myotonia itself and it is not associated with any vital organ issues.

Myotonia Congenita is in a group of disorders called the Non-Dystrophic Myotonias, but it is still one of the disorders that the Muscular Dystrophy Association provides services for.

I know what you mean about wishing the disorder was more visible, and having to always be aware of your surroundings. Due to the severity of my myotonia growing up, and the fact that I can usually feel when I am going to have a problem with my muscles locking up, before I actually move, I tend to be a lot more calculated in my movements than others. This has served me well in laboratories I've worked in!

Type of Myotonia: Becker

Country: USA

Hi Jenna,
Thank you for this information. I think this forum is great for that. For me personally it is amazing to share our stories like this. I have always felt alone in dealing with this. Even the medical profession in my experience do not know how to deal with it. For instance. I had to have back surgery about a 1 1/2 years ago. It was supposed to be in and out the same day. They ended up paralyzing both legs from the knees down and was in the hospital and rehab for over two month's. I later found out that certain anesthesia used in operations have drastic side effects to Myotonia Congenita. As shown in a study by two doctors from Norway. You would think that the medical profession would be aware of this and warn us about this. Or do their research before putting us under the knife. To this point I have not received any real answers to what happened except they tell me that there was some access bleeding that contributed to me becoming paralyzed. All the heads from the hospital have apologized for what happened not only for the paralyzation but the treatment I received. They should have sent me to a rehab facility that specialize in neurological and muscular disorders. Instead I was placed in general rehab and they had no clue how to handle me and basically tortured me.
Thankfully because of my will to do so I can walk again but it is difficult. So what I am trying to say is be vocal with the medical profession explain best as you can to them what it is we have. Do not let them just google the disease and be fine with it. get the facts from them so you know they have all the right information. That is what happened to me.

Type of Myotonia: Myotonia Gongenita

Country: Canada

William, sorry you had such an awful experience. You're right, we have to be our own advocates and make sure our doctors (especially anesthesiologists) have the correct information. I took the anesthesia protocol from the website with me when I was in the hospital a few months ago and they put it in with my records.

However what you're describing is not an anesthesia reaction. Apparently they damaged the L-5 (5th lumbar disc) nerve at the spine and this affected the peroneal nerve. If you haven't already done so, it's worth running past a medical malpractice attorney. Hopefully you won't develop foot drop and other problems in the future, but it would be good to have someone review the records.

Jan

Type of Myotonia: Thomsen's

Country: US