FSH Muscular Dystrophy: I am a hero... shhh.. don't tell :)

Another late post. Hoping to get better at this now that I have installed the Blogger app on my phone. Technology is grand.

I wanted to update those of you who are following along on this journey with me... thanks for that by the way. Adventures such as these are always better with others. 

The complexities of this disease leave those who have it, and those who are trying to care for people that have it, confused and frustrated. You are not alone. This is surely a steep journey with lots of bumps along the way. But the view can often be enlightening.

It is only recently that I realised that this disability makes me, and everyone else with a disability, a hero albeit a reluctant one. We feel that being born with something that you have to live with isn't heroic. But now, I believe it is.

I often thought I had to do something extraordinary to be inspiring if I had a disability. I read about amazing people who have turned this disease into a great opportunity to be motivational speakers, become public advocates and lobby governments for change. They invent things and break boundaries and I didn't think my getting up each day and living meant much. But it does. 

A person with a disability faces their own struggle each day with each task. I know now that the determination not to give up, not to just stay in bed is actually a really big deal. When walking up a driveway is like hiking a mountain and lifting your coffee cup is like lifting a boulder, it is a big deal to get up, face the world with a smile and move past all the hurdles each day brings.

So I am going to give myself a pat on the back and quietly accept I am a hero too. Just like the thousands like me who do not know what each day brings with their disability.

Since my last post, I have experienced a great deal of muscle loss in my legs and arms. My left arm doesn't function properly and I can't bend at my knees well because of the muscle loss in my calves and thighs. FSH keeps you guessing, you never know what you can't do until you try to do it and realise.. well, you can't. But on the upside, I am still independent, still working and still doing as much as I can.

On the research front, I recently attended the Friends of FSH research and patient meeting at the University of Washington. I heard from a number of leading researchers in FSH. These are our FSH champions and I have no idea where we would be without their dedication to unravelling this disease. They are moving forward in leaps and bounds in my opinion, studying drug therapies and creating viable mouse models and learning more and more about the evil DUX4.

Until next time, stay happy and at peace. You are heroes too!

The Silver Lining

Yes, it sounds cliche I know. But for this post, I have to share some cliches, or fortune cookie wisdom, as I call it. No bad thing should ever go without a good thing taking place or a lesson of some kind, hence the silver lining.

Sometimes you really have to search for it. The key is that you should go search for it. Each thing, even a bad thing, leads to something else... often something much bigger. Some call it the butterfly effect.

In any case, I broke my wrist in September. This may not seem to be a big deal, lots of people break their wrists. But for someone with FSH, it can lead to much more, as I soon found out.

How I broke my wrist is stupid and small and who would have thought something so insignificant like trying to move a fan could have such ripple effects, but it did.

First of all, let me say that the fan in question should not have been there. Lesson #1: Don't put off for tomorrow what you can do today. I had been meaning to get rid of that fan for the past two years. But kept waiting for the perfect replacement and was too lazy to dispose of it. I should have gotten rid of it. But I didn't and so in trying to move it, I tripped on one of the awkward "accident waiting to happen" legs, and tried to break my fall on the unstable stem of it which broke in half, sent me down to the ground where I landed on my wrist and shattered my radius bone.

So now I'm on the ground in the unceremonious "I've fallen and I can't get up" mode. Phones nowhere in reach and all alone. Lesson #2: Women are like tea bags, you don't know how strong they are until they get into hot water. So I regrouped and bum-walked my way to a phone, pulled a McGyver move with a broom handle and managed to open the door. 

The rest is the usual trip to the ER followed by a cast followed by an xray that said the cast didn't work followed by surgery followed by heavy drugs and a the life changing moments that ensued.

As I said, broken wrist.. not such a big deal. But when you have FSH and are at the point in your "adventure" where you need both hands to transfer from seats, this is huge. Seats, by the way, includes toilets and the car.

Imagine that. Now I had to get someone to lift me off the toilet at the hospital because I was stuck. Embarrassing. Lesson #3: Always have clean underwear and shave your legs. OK, that second part was just for the girls.

Talk about your independence gone, in the snap of a finger. Lesson #4: One moment changes everything.

Thankfully, I had not hastened to sort out some mobility issues at home in anticipation of what may come. Lesson #5: Do all you can do while you can.

So I was, in fact, able to go home where I stayed for the next eight weeks. Eight long weeks. Thank god I was able to work from home or I would have gone out of my mind. I had to get my mom to move in with me which was really hard for me, but I think it finally made her feel like she was doing something, anything to help me. For me, it was humbling. Lesson #6: It's ok to ask for help sometimes.

Sadly, the laying on my back, the trauma of the fall and the surgery for which I was wide awake, the effects of the nerve block and far too much time to think, left my FSH in a what felt like a rapidly progressing state. My arms got weaker, my legs got weaker and my core got weaker. I had to get a walker and a boosters for the bathrooms at work and at home. I couldn't get past imagining a life of asking for rides, waiting fro help and no freedom. Lesson #7: Suck it up. It could always be worse.

I was so worried about what people would think of me in this new state. The walker, the booster, the limping... but I got to work and was embraced by all those I left. And was greeted by another colleague who, sadly, herniated a disc and had to use a walker after a five day hospital stay. Lesson #8: Shit happens to everyone.

So I am back at work and struggling a little, but I am back at work. More importantly, I am back to spending time with friends and doing some of the things I enjoy. I had to buy a new car so I could get in and out easy (how painful was that... not!) and have my VIP parking pass (disabled sticker) which really came in handy during the busy shopping season.

Most of all, it reminded me of how resilient I, and all those with FSH, are. I learned I had developed a pattern for when bad things take place. I cry, I mourn, I take a deep breath, I pull up my socks, I get on with it. Lesson #9: Life is too short to be on the sidelines. Get in the game.

And, my parents who as you know from reading previous posts, found some strength too. The broken wrist and all that came after it moved them slightly out of denial and thankfully made them realize, I'm doing ok. Lesson #10: Reality is what you believe it to be.

So out of all that came some good. I could do without the ugly 3" scar that came along with this. But I am grateful for the lessons it reminds me of.

The things I miss. The things I don't.

I miss looking up when I walk. 
I miss holding the beautiful children in my life.
I miss running.
I miss dancing.
I miss being spontaneous.
I miss playing.
I miss competing in sports.
I miss walking up stairs.
I miss being able to exit a room with defiance.
I miss wearing whatever I want.
I miss my pretty shoes.
I miss dreaming about being a mother.


I don't miss taking life for granted.
I don't miss putting things off for tomorrow.
I don't miss believing I am worthless.
I don't miss believing I am weak and feeble.
I don't miss lying about the sum of who I am.
I don't miss seeing the glass half empty.
I don't miss worrying about the small things. 
I don't miss living in a small world with defined possibilities.

Disneyland Part 1 - The Big Dream

I just returned from my dream trip to Disneyland. A dream trip because I had the chance to plan a wonderful week-long adventure with my nephew. 


I love that kid.


But I also wanted the trip to be really memorable for a number of other reasons. I needed to do this trip while I still could. I wanted to be able to be mobile and create a fun experience with him that I would never be able to share with my own child. I also wanted to do something memorable with the last of my adoption money since the adoption wouldn't be completed.


I suppose to some that would seem a frivolous way to spend the money, but I really needed to do this. I needed to have that memory and I needed him to have a memory of me that was "normal".


I planned the trip sparing no expense to ensure the trip was easy, given the limitations with FSH. I knew I would never be able to take him on my own as he has more energy than a flock of rabbits. So his other aunty came along. Thank god for her! She is a very kind person who loves him as much as I do, so I knew this would be a great experience for her too. 


I insisted on paying for everyone as I felt I had to have that independence and ensure I didn't feel like a burden. I guess that is something many people go through with a disability. It also gave me this great freedom and euphoria to be able to get him anything he wanted and do whatever we wanted to do.


We had a week of fun and we packed it! Disneyland, Knott's Berry Farm, Legoland, Seaworld, all the swimming he could handle, parks and movies every night.


I don't think I have ever laughed so hard. Kids are so amazing and this one has a sense of humour you would not believe.


I had lots of advice from friends with FSH and friends who had been to Disney. I myself had been to most of these places a few years ago. I didn't realize what a difference a few years could make.


Therein lies the second part of this post - The Reality. Reality comes and gets you every time. The question is, would it be enough to knock me off my game?

My Circle of Influence Just Got Bigger

Today was a good day.

We often don't take the time to celebrate all the good things that happen in our lives. It seems to be human condition to gravitate towards the negative, the drama, the conflict. But I think one way to create a positive life is to recognize the good moments and spread those moments around - let them infect other people.

I met a few people today who are either affected by FSH or have family affected. We've been trying to create a local support group to share stories, ask and answer questions and gain insight to the differences and similarities of this mysterious condition.

As always, I find similar stories to my own but packaged slightly different. There is comfort in knowing someone understands what you are feeling. It's a hard thing to articulate sometimes.

We had a great time chatting, not just as people who have this 'thing' in common, but just people who enjoyed each other's company. It's always good to surround yourself with the right people.

That's a good day.

A Needle in a Haystack

Can you imagine what it would feel like to have a disease attack your body while you helplessly watch, only to find out you could have done something to stop it, or at least least slow it down?

While there is no cure for muscular dystrophy (yet) there are some things one can do to help.... I think.

I say that with uncertainty because the suggestions out there of things to help seem to have equal voices saying they don't help.

Exercise, how much and what type. What works for one, doesn't work for another. I attended a muscular dystrophy conference a few years ago where a researcher provided results from a study on exercise and the impact on muscular dystrophy. She said the one type of exsercise that proved to help increase strength in patients was aerobics, 20-30 minutes three to four times a week. This would be great if you don't have a foot drop, aren't already suffering from decreased mobility or fatigue.

I was also told once you experience the 'burn' when you are exerting yourself (swimming and cycling seem to be the preferred sports) you must stop as your are irreversibly damaging you muscles. Then I was told by some of my fellow FSHers, their doctors said you have to push yourself past your boundaries to move them.

Take this article I just came across as I was surfing the net. Calcium and CoQ10? Easy and inexpensive to purchase. No medical professional told me that.

How about the effectiveness of stem cell treatment? My neurologist had no information for me. What I learned, I learned from Russ Kleve's blog who went to China for treatment and documented each step of his treatment. Thank goodness he took the time to share his experience.

What to do but keep researching and sadly, not just relying on your doctor. Above all, listen to your body. Everyone with this condition is different and there seems to be different solutions at different points in the condition.

I have tried alternative medicines to help. It's not for everyone as there is a lot of blind faith that goes along with it. I am currently in Ayurveda treatment which involves taking four pills twice a day and an intense massage with strong herbal oil. The pills are made up of various herbs and spices. The basis of this treatment is increasing circulation and making your stomach function properly.

I am in the second month of treatment and while I haven't noticed a difference (except joint pain which he says is good as it means my muscles are getting stronger) he does see a difference in the back of my legs. He wants to see this work as much as I do. He believes more than I do, saying I will see a huge difference in nine months. Nine months, can you imagine?

I'll keep you posted.

Or, you could do nothing. I have a good friend with FSH who wouldn't know anything about the research or any other stories if I didn't tell her. She is progressing faster than me in some ways and not in others. She is happy and in all other respects, very healthy. The greatest joy she has is her little girl who is as feisty as her mom and as energetic as she once was. She hopes for a cure, but waits for nothing.

In the meantime, I share my experience of what has worked for me above all else. Being positive. I spent a lot of time figuring out what makes me happy and taking every opportunity to do those things. Spend as much time laughing as you can, and surrounded by people who make you feel good. It makes all the difference.

Circle of Influence

For those who know me, you have heard me say a million times how important a positive circle of influence is. Someone in my circle sent this post to me yesterday. I did my part by re-sending it to everyone else in my circle. The entire post is excellent and worth reading, printing, framing, pretty much anything short of tattooing. Surrounding yourself with the right people is top on the list.

For me, the 'right' people include those who support me through this challenge and all other challenges I face that fall in the 'part of life' category. But, they also include the people in my life who don't understand FSH and what it means, those who don't believe me when I tell them what I am going through, and those who just tell me to shut up about it. It is sometimes those people I value (mostly in hindsight) because they make me fight for the life I want to lead.

Ironic, when you defend something it ends up pushing away your own doubt. The words that sting the most aren't new. I say them to myself a million times. But when I hear them, I am driven to defend myself.

"No, I don't want to tell people I have a back problem. I want to tell them the truth."

"No, I am not lazy. I am really just tired."

"No, I am not vain. I really needed that operation."

"No, I am not taking this too seriously. I am trying to learn, teach and inspire something positive out of the situation."

"Don't tell me I can't."

Some of these thoughts came from the people closest to me. Some meant well, some were just thoughtless and some still live in a reality where having a disability means game over. As my sweet nephew likes to say, "No, game NOT over".

It has taken me a long time to get "through the muck" and realize how strong I am. Not just because I fight the ideas I don't like (whether they come from me or someone else), but because the majority of my circle consists of strong, caring, compassionate people who care about me and see past my disability. They don't all have to be experts in FSH. Some of them don't even know what it is. But it doesn't matter. They see me for who I am, listen to my dreams, give me a hand or a kick in the butt when I need it. Most of all, they make me laugh. With laughter you can get through just about anything.

To my circle of influence, thank you.

The Fire Drill

At some point in the life of an FSHer will come the inevitable moment when the cat jumps out of the bag. The quiet and mysterious condition will slowly start to make itself known and the journey takes a sharp turn.

The closer the people are, the quicker the transition comes. But often, it is when those not as close to you find out that it becomes much more difficult.

For me, a surprise fire drill at work seems to be the game changer.

It happened a couple of days ago around 10 am. I used to get a heads up from the Emergency Operations Coordinator who would quietly come to my cubicle and suggest I go for coffee. His wife was one of my colleagues and the six people in my department knew about my "secret". They retired last year and the HR Manager and Operations Manager, who also knew about my condition for safety reasons, forgot. Guess that's the thing with FSH, it doesn't show until the condition has progressed significantly and then it seems to catch people off guard. But as the person feeling each strain, each burn of the muscle as it deteriorates, it feels like you are slipping off a mountain and that point when others find out is when you land on your butt.

The shrieking sound of the bell meant I needed to walk down the three flights of stairs to the plaza. Three long flights on concrete steps with people rushing behind me, and me side-stepping down the stairs with my legs starting to feel like jello. The president of the company walked behind me and asked what was wrong with my leg. I brought out my usual humour all of us seem to have and made some comment about my job being hazardous to my health. He laughed, but you could see the concern in his eyes. He is a lovely man and I am sure would only be empathetic to the situation. But we are in the midst of budget cuts and restructuring and who wants to put their hand up and say, "hey over here, I am a liability to your bottom line."

My co-workers flanked me as they always do, such lovely people. But I guess this is going to have to come out to the masses sooner rather than later. Now I am on the radar and questions are being asked, "What's wrong with her?" Being in a high profile position doesn't help. Although I am fortunate to work with people who are very understanding and compassionate, it's my clients I worry about. How do you stand up with conviction and argue a point when you struggle to stand up?

The president's on vacation now, so I have a few weeks to gather my wits and find a clever way to reveal the truth without all the scary words that go along with this explanation. Wouldn't you know it, just as my star is on the rise here, a sobering dose of reality hits me. But, I am not ready to fall just yet...

I am grateful I only have FSH

An odd thing to say, I know. But I just received an email from a close high school friend of mine updating me on another friend who is dying of cancer. She is 40, just like me.

We all went to school together and I can still remember her face in my head. Not having seen her since our reunion, in my mind she remains healthy and glowing as she always was.

She describes her experience as an unreal hell and can't grasp what is happening to her and how quickly her breast cancer spread to her liver, lungs and brain. She is undergoing painful radiation treatment to buy her some time, but spends that precious time going from one hospital to the next for treatment.

She remarked on how quickly life changed - one minute in her husband's arms, the next on a hospital bed. A good reminder of how absolutely out of control our lives are.

One way I tried to find the silver lining in my prognosis was to say everyone will get some kind of illness in their lives. It is inevitable. If this is all I get dealt, then my thanks to God.

I know that is a tough pill to swallow for many. FSH robs us of so many things in life and at times feels like a struggle that just gets you to next struggle. But have faith as the saying goes, if God get's to to it, he will get you through it. And for those of you who may not believe in God or have faith, don't worry, there are people around you who believe for you.

My heart goes out to my friend and her family. They will be in my prayers tonight.