Surprises with SMA

[halsey-blocher]

Your interaction with the nurse reminds me of what used to be a common occurrence when I got my trach. Whenever I got new nursing staff, they would always tell me never to lose hope that I wouldn’t need it at some point, and they would tell me stories about people who got rid of their trachs after a few years. Like the nurse you met, they knew nothing about SMA. I would always have to explain that SMA prevents me from getting stronger and that I have never heard of anyone with SMA no longer needing a trach. Thankfully, no one tells me this anymore. I think since I’ve had it for almost ten years, people are catching on that it’s not going anywhere.

[deann-r]

After failing four swallow studies I stopped speech therapy. Someone asked why I was giving up. I had to explain I wasn’t giving up, it was just that continuing wasn’t going to be beneficial at that point.

[halsey-blocher]

It can be hard for people on the outside to tell the difference between giving up and making wise choices – and sometimes sacrifices – for our health.

[kevin-schaefer]

I guess for me, I never realized how much arm strength I would actually lose. Like I came to terms with losing the ability to feed myself (though I’m able to now with my robotic arm), but I didn’t expect to ever have so much trouble just using my iPad. I used it throughout college for all my work, but now I do most of my typing on my phone. When I’m in the pool for water therapy I can move my arms great, but it’s a real struggle to when I’m in my chair. Things are improving with Spinraza, but still this is something I didn’t prepare myself for when I was younger.

[deann-r]

That certainly can be a shock Kevin. If I look back sometimes I’m amazed what I used to be able to do as far as arm strength. Hoping over time with Spinraza I’ll get some of it back. I can definitely relate to that feeling though when you realize some movements have just become too difficult.

[ryan-berhar]

Kind of like Kevin said, the loss of arm strength blindsided me somewhat.

[adnan-hafizovic]

To me is also, the loss of power in my hands was especially hard. Particularly to me, as a teenager I had strong hands, in the third year of my life I stopped walking, but I could crawl around the house, I could cross myself from the bed to the wheelchair.I was getting weaker, although I was not so worried. I only felt that I was getting  more frightened,it was when I start to feel that I loose ability to swallow and I was a little scared, but thank God I do not know how I got the feeling that I could swallow  again without any problems. I worried about other things, like when my father was suffering from Parkinson’s disease, eventually he died of this illness six years ago. Or when my mother suffered from cancer, but thank God she had two surgeries and chemotherapy, and now she is well .And honestly, how old I have this little fear of what my life will be like if my mom dies. But in the end, there’s always some sort of solution.

[deann-r]

Thanks for sharing Adnan. Loss of ability, and the fear of the unknown can definitely be scary. As you said, there’s always some kind of solution and as individuals with SMA I think we are very resilient and can get through just about anything. Give your Mom a virtual hug, she sounds like a pretty amazing lady.

[adnan-hafizovic]

Thanks DeAnn.

[lupa-f]

How much temperature affects my strength. If my hands get cold for a while I can barely use them and it takes a really long time for them to get back to normal. Like trying to use my keys to open my door is nearly impossible since I can’t grasp the key tightly enough. And “cold” for me means about 60 degrees outside or below. I can mitigate it a bit with gloves, but then wearing gloves restricts my movement anyway so it’s not really much better. If I’m out in really cold, like below freezing, it can start affecting my shoulders, which makes it a bit interesting when I have to drive and I end up steering with T-Rex arms (this only lasts a little bit until the heat in my van warms up enough).

I used to mention this to my doctors and they’d just be “hmmm that’s interesting”, and give no advice or thoughts. Finally I had one doctor tell me it was a symptom of SMA and really common and I’ve since had a few therapists and doctors confirm it. The only solution I’ve ever been given is to wear heavier clothes or winter coats but those make things even worse so it doesn’t really work for me.

[deann-r]

Oh my gosh, exactly!  Yesterday looked nice out, so I tagged along to the store.  I neglected to grab a hand warmer or mittens and I regretted it.  Like you I find layers restrictive so opt not to if I don’t have to. It took me at least an hour for my hands to be warm enough to even function and they were still cold the rest of the day.

My docs don’t seem concerned either.  One suggested moving to a warmer climate.  Thinking he might have been on to something if it was only that easy.

[adnan-hafizovic]

I also have a problem with the cold, but since last year it seems that I have a little better circulation thanks to a device called vitaphon. It is a vibro-acoustic device and this winter was really not that cold for me, although there were a lot of cold days in Bosnia.

[kelly-miller]

Adnan, I think you are onto something about how you worried over different things. I’ve found that when I concentrate on a particular issue, get anxiety over how bad it is, things get worse, and I get weaker. When I shift my concentration to something else, the old one gets better, and the new one gets worse. I think it’s all a matter of managing expectations and setbacks.

I think the worst for me was losing my arm & hand strength. Because I had been very sick (dehydrated due to vomiting, then ER overloaded me, so I ended up on vent for a week… a story for another time) and in the hospital for 2 weeks, I lost all of my strength in 1 sitting! It was terrible. When I went into the hospital, I was able to feed myself, write letters, type on the computer; but when I came out, I couldn’t do any of these things including brushing my teeth or combing my hair. It was devastating. Since I no longer had use of my upper body, I couldn’t even drive my chair anymore with my joystick. It was at this point that I switched to a sip and puff control. I was so angry, and so depressed, that I isolated myself from friends and family for several months. Eventually, I came out of it, but it was a rough road to go. Then, when the doctors said I would need a feeding tube after a long bout with pneumonia (6 weeks in the hospital this time), I knew it was just one more thing towards the eventuality of what I was afraid would be a nursing home. Sometimes, our fears get in the way of progress. I couldn’t be happier now to have my mic-key because I haven’t had pneumonia in 5 yrs. since there are no more aspiration issues or malnutrition problems.

I guess the thing that has surprised me the most has been my inability to go to the bathroom. And by that, I mean unable to make those muscles work like they used to. I was happy to find out about SPC’s, but I still struggle to be able to poop. What used to be a 20 minute process has turned into an hour and sometimes longer. I even have a friend with SMA who decided a few months ago to get a colostomy because she was just getting to the point that she couldn’t make herself go anymore. She says it’s been wonderful, and she and her husband save a lot of time with her new bag. I’m just not sure I’m ready to go for that extreme. But then, who would’ve thought I would volunteer to have a permanent catheter into my bladder?!

[adnan-hafizovic]

Of course Kelly,I always try to satisfy with small things, but sometimes in life, we all have so many problems and temptations. And in such a situation, we can not have those little things that make us happy.