Assistive Walking Devices

Several months ago, out of curiosity, I googled "exoskeleton legs." It seemed to me that surely someone out there might be working on a robotic device, like leg braces, that would allow someone like me to be able to walk farther, maybe even climb mountains.

I found a couple of things that were interesting. One is being developed by Honda, and it has a small saddle-type seat (kind of like a bike) and attaches to the feet. It runs on a battery, and apparently works by taking some of the weight off your legs and helping to lift your legs up for walking. Pretty cool. But kind of funny-looking. Still, I'm willing to look a little funny if it will allow me to walk further!

Another device I found is called HAL- Hybrid Assistive Limb. It's made by Cyberdyne, and looks a bit like a Storm Trooper outfit. But apparently they also make a half-body version for the legs only. This one attaches around the waist, and also uses a battery pack to lift the legs. They say it can increase your strength by a factor of 2 to 10, and they said it was specially designed for climbing mountains! Unfortunately it's not available in the US yet. But they say to be patient, it will come!

For now, there is a device that is a little lower tech, but may help me out. It's called Mu Smate, and it was developed by a man in England whose wife has multiple sclerosis. It straps around the back and goes down to the feet, and works by lifting the feet up. Others with MS who tried the device and were able to increase their walking speed and distance significantly. And even though I have Congenital Myasthenic Syndrome and not Multiple Sclerosis, they are both degenerative neuro-muscular disorders. So maybe, just maybe, this little device could help me too. I think it could be worth a try.

Here are links to each of these devices:

Honda device (I don't think they've named it yet!) http://www.hondanews.com/search/release/4872?q=walking&s=honda

H.A.L. by Cyberdine: http://www.hondanews.com/search/release/4872?q=walking&s=honda

Mu Smate: http://www.musmate.co.uk/index2.html

Creatine?

I remember hearing about Creatine several years ago, but I've never had a doctor recommend it for me. Last week I had a follow-up appointment with my neurologist, and I asked if there was anything to do to improve my muscle recovery time. And he suggested taking Creatine.

I'm not sure how long it will take to see if it makes a difference. It's bad enough I get weak so quickly, but then it takes so long to recover! It's really getting in the way of my 5k training! There is such a delicate balance, trying to push myself enough to improve, but not so much that I overexert myself and can't do anything for a week or two!

I've been really weak the last week or so, and there doesn't seem to be any explanation for it. But I am still determined to keep moving forward, to keep trying to figure out how to be STRONG! I already see that being more active leads me to having more energy and endurance, so that I am able to have more "strong" days. But man, on those weak days it is so hard to make myself be active. And I KNOW that even doing a little bit on those weak days is better than doing nothing. It's like I get "over-rested." So the more I rest, the longer it takes for me to recover. But if I walk, even for just a few minutes, when I'm weak, I tend to improve faster. Amazing!

Sometimes I really wish I could look inside my body and see just what is going on in there. What happens when a message is sent from my brain to the muscle? What exactly is the problem? Why is it so complicated? Once a muscle gets over stimulated, what is the best way to help that muscle recover? And there is the problem of the weakness in my mouth & eyes. That seems much more difficult to overcome than even my legs! My eyelids just do not want to open all the way! How can I give my eyes and mouth adequate rest, without sleeping? Should I become a mute and walk around with my eyes closed 80% of the time? Then the other 20% of the time maybe I could smile and my eyes would be wide open! Ugh. Wish I had the answers.

Well, if anyone has suggestions for how I might get stronger, please let me know! I am always open to suggestions! For now, I'll try the Creatine and see how it goes!

wheelchair tennis? cool!

This morning on the Today show, there was a story about people who are disabled and play tennis in their wheelchairs. I love stories like that! There are so many people with disabilities that just want to live as fully as possible. Many disabled people find ways to participate in sports, music, film, theater.

Often the world looks at those of us with disabilities and thinks that we are incapable of doing practically anything. But most of us do not desire to lie around our houses, alone, watching TV and feeling sorry for ourselves all day. We want to LIVE! We want to experience so many things. Yes, we have to be creative in finding ways to do what we want to do. But it is worth it. There are disabled people who have created a way to ski (chair skis), surf, ride horses, drive cars with hand controls. I even recently read about people who hike-mountains!!!!- in wheelchairs! It is incredible, and so inspiring!

I want to be one of THOSE people. Not for glory or fame, but for my own personal satisfaction. I want my life to be full, and extraordinary. I am so thankful for the strength I have, for how much I am able to walk. So thankful that I can dance sometimes, that I can even now run- even if just a little bit! But I don't want my times of weakness to keep me from experiencing the world. I know it is not always easy to find a way to get around in a world that is built for people who can walk. But, I have been able to manage so far. And I want to do even better. I am going to keep working toward my goal of doing a 5k. I've already hiked down a mountain, and next I want to work on climbing UP!

Check out these links to some truly inspiring people:

Ester Vergeer: wheelchair tennis champ

Wheelchair hiker


Ivan Kozhouharov: paralyzed man attempting to climb Mont Blanc

Disabled Surfers

Oh yeah, I'm also an artist...

Here is a link to my art website, just in case you want to check it out: www.lizhume.com

Struggle

A couple of weeks ago I took a wonderful trip to Asheville, NC- and completely wore myself out! I went to the Biltmore and spent the whole day there- climbing all those steps and exploring the gardens. It was beautiful, but exhausting. Then the following day, we went to several of the waterfalls in the area, which required a bit of hiking. It was definitely a struggle! My boyfriend ended up giving me a piggy-back ride a couple of times! It made me pretty weak, but I still think it was worth it.

It's so hard sometimes dealing with this disease. There are days when I am so strong I almost forget that I have congenital myasthenic syndrome! Then there are days I am so weak, I wonder if I will ever be strong again.

So, here I am trying to follow this "couch-to-5k" plan, and after that weekend I was completely unable to train! I'm thinking it may take me longer than a couple of months to reach my goal, but I still think it's possible. I probably walked about that much at the Biltmore! Plus I was going up & down tons of stairs! But that was a couple of weeks ago, and I am just now starting to feel a little stronger.

Sometimes I wish I could see inside my body, and know what is really going on in there! How exactly are these nerves & muscles supposed to work together? How do muscles recover after exercise, and why does it seem to take me so much longer to recover than people without this disorder? All of the research I have read only mentions the problem of nerve transmission to the muscle. Nothing addresses muscle recovery. If I could just get my muscles to recover faster, I could do so much more!

So, I am struggling. Sometimes I get a bit discouraged, and feel like what I want is just impossible. But I reminded myself this morning that the body is incredibly complex, and no one can really fully explain it. Some people have serious illnesses and go into complete remission, while others with the same condition continue to decline. I have heard that some people with Myasthenia Gravis go into remission, but I have only heard of that in acquired cases, not congenital cases. I'd love to be in remission! I would be unstoppable! Look out, mountains, here I come!

First Run/Walk

Well, maybe I should call it a "Walk/Run." It was definitely more of a walk! But that's the way it's supposed to be. I set out on a 20 minute walk- approximately one mile, and was able to incorporate 3 bursts of running at a minute each. That third burst was pretty tough for me. I had to imagine Jillian from The Biggest Loser pushing me through- "Come on, just 30 more seconds! You can do it! Don't stop! 10 more seconds. All right! You did it!" So I raised my arms in celebration and did a cool-down walk the rest of the way home.

It feels so good to be walking. Sometimes it's hard for me to believe how much of my life I spent in a wheelchair. And I had always believed that pushing myself at all physically would only end up making me worse. If only I had known that over-resting was making me worse! Over-exertion in the short-run certainly makes me weaker. So I have to be careful not to push too much. I don't want to take a week or two to recover! I'd much rather be able to recover in a day or two. It's hard to take it slow though when I am having a "strong" day. I will have to make myself conserve so I can maybe turn one strong day into two.

I'm off to a good start. Just gotta stay consistent!

On the way to a 5k

I've been researching the last few days on how to train for a 5k. It looks like the general consensus is that it's best to train slowly and not push too hard. Good advice for everyone, but especially for those of us with myasthenia because we tend to get over-extended more quickly than everyone else. Plus it seems our recovery time is much longer.

I wonder if it's possible to minimize recovery time. There are lots of supplements on the market for body-builders, but I don't know if any of these products actually work. One that I am very curious about is Glutamine. Supposedly this is a product that helps your muscles recover. I guess I can give it a shot. Need to talk to the doc and see what he thinks about it.

Today I officially started my training. I got some ankle weights and did a few sets of various leg lifts. It felt really good! I've been walking a lot, but now that I have a goal I will have to be more focused. I found a "Couch to 5k Running Plan" that could be perfect for me. It's a 3-day a week plan, starting with 20 minutes. It's a nine-week plan. So I should be doing a 5k around Oct 17. That's pretty exciting to think about! I will definitely need some encouragement along the way.

Road Block

I've been taking Fluoxetine for about 5 months now, and I seem to have hit a wall. Everything was going so great a month ago. I ran! I ran a half a mile! It was unbelievable. In fact, I did it twice! But I haven't been able to do it since then.

I have been feeling pretty weak. My mouth especially has been bothering me- trouble talking. I've lost my smile again. It is so frustrating. I had been so excited to see my smile- even if it was just for a little part of every day. Even my eyes were starting to open up. Now it's as though I'm not taking anything. I just don't understand it. How can it just stop working? Do I need to take more? Less? My doctors are just guessing.

I just want so much to be better. To be WELL. I love to travel, and it would be so great to be able to walk around the old cities without getting tired. Or to be able to hike, and see all the amazing places I can't get to in a wheelchair. I am still amazed that I was able to hike a bit in Switzerland. I think that really awakened something in me. I have seen so much beauty- even from the seat of a wheelchair. I went to Alaska and to Rocky Mountain National Park in a wheelchair. But it's a very limited way to view the world. And you have to find places that are accessible. Sometimes I just want the freedom that comes with being able to walk.

I have thought about attempting a 5k. Especially on the two days that I was able to run. There have been days when I have been able to walk as far as two miles. A 5k is slightly over 3 miles. So maybe it is possible. I just wonder how in the world I could train for that. It seems like it takes so long for my muscles to recover. If I could just figure out how to work out enough to get a benefit, without overdoing it, maybe I could really get stronger. Maybe I could really increase my endurance.

I keep wishing there was some easy solution- like some apparatus I could put on over my legs that would enable me to walk without getting tired. I've seen the exoskeletons they are developing, but they aren't available for sale yet, and they are too expensive for me any way. But I do wonder if they work on mountains! Maybe they will come down in price in the next few years so that the average handicapped person can actually be able to "walk" instead of having to use a wheelchair. How amazing that would be!

There is a woman with MS, Wendy Booker, who is so inspiring to me. I first read about her in Prevention magazine several years ago. She was able to compete in marathons even with MS. When she was diagnosed, she was determined not to let it keep her down, and worked out with a trainer to develop and maintain her muscles as much as possible. She said there were days when she was so weak she could only lift a 1 lb weight, but she did it any way. Now she is working on climbing the Seven Summits. I know that MS is a different disease than congenital myasthenic syndrome. But both are neuromuscular diseases, and neither has a cure. Both have varying degrees of weakness, and affect each person differently. My case of CMS is relatively mild. Perhaps it is possible to train my body to work more effectively and efficiently. Maybe it is possible for me to be able to climb mountains. I just have to figure out how!

4 months on Fluoxetine

Back in March my doctors and I decided to try something new. Since mestinon and other similar drugs always made me significantly weaker in just a short period of time, we decided to try Fluoxetine. Mestinon is used for several types of Congenital Myasthenic Syndrome, and works to allow more acetylcholine to reach the receptors in the muscles. Fluoxetine works in the opposite way, blocking acetylcholine from the receptors.

Generally after a week or so of treatment on mestinon, I start to notice I'm getting weaker. The longer I take it, the worse I get. So I started researching on the internet, trying to see if there was anyone else going through that. I found a story about a boy who had been taking Mestinon since he was a young child, and at age 14 was on a respirator and confined to bed. The doctors discovered he had Slow-Channel CMS and switched him to Fluoxetine. Within a month he was able to breathe on his own, and after about a year he was able to play on his high school football team! I was so inspired and hopeful that perhaps this could work for me as well.

After the first week of treatment, I could already tell I was walking more easily. By the end of the second week I was walking up stairs like they were nothing! Over the last 4 months my strength has continued to improve. I no longer have problems breathing, chewing or swallowing. My eyes are more open, my smile is bigger. I can walk farther than ever before. And recently I was able to start running! I haven't run since I was about 8 years old! That was 24 years ago! It was so exhilarating, being able to run. My first run only lasted about a minute. But recently I have been able to run up to 1/2 a mile. This is astonishing for me. I never thought I would be able to run. My hope is to be able to start hiking. I have read about a woman with Multiple Sclerosis who is hiking the 7 Summits (the highest peaks on each of the seven continents). I would love to be able to do something like that! I know it will take time, and a lot of hard work and determination. But it would be worth it.

Trying Fluoxetine

Several months ago I started doing some research online to see if anyone else out there was experiencing similar negative reactions to Mestinon and 3,4 DAP. What I discovered was astounding to me! There are different causes of congenital myasthenic syndrome! I couldn't believe I had never heard about these other forms of CMS.

***I started this post 2 years ago in '09, so it's hard to remember all that I was going through at the time. So instead of trying to recreate what I might have been feeling or thinking then, I'll go ahead and pick it up now with some of what I have learned.


The first thing I learned was that Congenital Myasthenic Syndrome can affect different parts of the synapse. (The synapse is the space between nerves that transmits an impulse {like a message} from one nerve to the next) CMS may be pre-synaptic, post-synaptic, or synaptic, which means the defect may occur in any of those areas. Then there are subtypes, including slow-channel (meaning the gateway to the synapse opens/closes too slowly, letting too much of the chemical in) or fast-channel (meaning it opens/closes too quickly and the chemical can't get through). Mestinon works great for fast channel CMS because it keeps the channel open longer. But this is also why it can be disastrous for people with slow-channel CMS. Too much mestinon causes extreme weakness.

Once I read this, I immediately concluded that I must have slow-channel CMS. My previous disastrous experiences with Mestinon led me to believe I needed whatever the opposite treatment would be. I read about two drugs- Quinidine and Fluoxetine.

Quinidine is a drug that is used primarily for heart patients. Because of the warnings, and because my neurologist at the time had not prescribed it before, we opted for the less dangerous Fluoxetine- better known as Prozac.

It seemed strange to be taking an anti-depressant to help me walk, but I was willing to try anything. My experiences with Fluoxetine are described in a couple of other posts.

Unfortunately, my self-diagnosis was wrong (and also unfortunately, my neurologist did not have me undergo further diagnostic tests to confirm the correct form of CMS). So I spent almost two years on Fluoxetine, and my condition continued to decline. I almost could not walk at all, and was feeling like I would never be able to walk again, when I moved to North Carolina and went to Duke Medical Center.

My new neurologist took some blood from me and sent it off for DNA testing. And what they found was a type of CMS that I was not familiar with. I had a defect in my DOK-7 gene. The good news was that they were having some success treating this form of CMS with Albuterol, which has been used for decades to treat asthma.

Throughout this ordeal, I have learned that getting the correct diagnosis is absolutely crucial. It is impossible to know based on the symptoms which type of CMS you have. Even people with the same cause may have varying degrees of severity and will react differently to the medication. I'm so happy to finally be on a medicine that works, and I am looking forward to a very active future!

Mestinon (and other unsuccessful treatments!)

Mestinon is pretty much the first line of treatment for patients with congenital myasthenic syndrome (CMS). It seems to work pretty well for most patients- enabling them to live near-normal lives. I can't tell you how many times I would read statements like that and be filled with frustration! Mestinon always made me worse! It was the first drug we tried, and I very quickly became weaker. So we decided to try another drug. My doctor prescribed Guanidine Hydrochloride, and I was on that for a few years. I was 11 when I was diagnosed, and started using a wheelchair at age 12. I'm not really sure if the Guanidine helped. It became increasingly difficult to obtain, so my doctor had me stop taking it just to see what would happen. And nothing happened. I was exactly the same. So I went through the rest of my teenage years being on no medication, and continuing to get weaker.

When I was in college, I was really having a lot of problems. My doctor suggested we try Mestinon again, thinking perhaps we just didn't get the dosage right in the beginning. So we tried again. And within a couple of weeks I was having serious problems chewing and swallowing, and was literally afraid I was going to choke to death! So, I stopped taking it.

I felt so hopeless, thinking that there was no treatment for me! How could other people have so much success with Mestinon, and it just made me worse? I really didn't understand. But I was hopeful that eventually there would be something that would work for me.

A few years later, my doctor mentioned a new drug that was showing a lot of promise. People who hadn't been able to walk at all were now able to take a few steps! I thought that since I could already walk a little, maybe this would help me be normal! I was so excited. The drug was 3,4 Diaminopyridine (DAP). Now, I should have had second thoughts when my doctor said you have to take it with Mestinon. But, being the optimist I am, I thought maybe Mestinon combined with 3,4 DAP would somehow end up working for me! I was wrong! I went from being able to walk around the house with no problem, to not being able to get out of bed- within 2 weeks! It was awful. I had never been so weak in my life. I was afraid I wouldn't recover. The doctor told me to stop taking it immediately. Thankfully I recovered within about a week. I was 24 at the time.

After that experience, I knew I needed to find ways to be as strong as possible, without medication. I didn't know if or when there would be a treatment for me, but I wanted to do as much as I could. I'll write more about that later!

When I was 27, my doctor suggested we could try the 3,4 DAP again. He said it had been re-formulated since my first experience with it, so there was a chance maybe it would work better for me. Ever hopeful for something that would make me stronger, I decided to give it another chance. We used a lower dose, but unfortunately I had the same result. Only this time instead of taking a couple of weeks to make me unable to walk, it took about a month. Finally my doctor said we would never try that again!

I was so discouraged by these attempts at medication that I didn't even go back to my doctor for several years. It wasn't until I read an article about Monarsen- the latest drug being tried for CMS- that I decided to go back to the doctor. My doctor was no longer working for the MDA clinic, so I got to see a new one. So, last fall I headed back to the neurologist for the first time in 4 years. I told him all about my past experiences, and asked if he was familiar with Monarsen. Unfortunately there are currently no trials in the U.S. for this drug. So that was out. He asked if I had ever been on Prostigmin, and I said no. He said it's kind of like Mestinon, only different. Well, I was apprehensive, but since it was a little different than Mestinon I was willing to give it a try.

No luck. During the last few years, my strength has improved significantly without medication. I haven't used a wheelchair in two years. But I was not able to run, or walk for long periods of time. And there were many times I had to hobble along. So of course I wanted something that could make me strong all the time. Unfortunately, Prostigmin was not the answer. Within a couple of weeks I was no longer able to take my daily walk. At first I told myself it was just a coincidence and I was just going through a weak time (it happens!) But after a couple of months, I was again completely unable to walk.

Thankfully I have again recovered and am back to my normal self. But I started doing some research to see if I could find anyone else who had such negative reactions to treatment. That was when I started reading about the different types of Congenital Myasthenia. I had had this disease all these years, and never knew that there were different forms of this disease!

I have just recently started taking Fluoxetine, which is used to treat the slow-channel CMS. I do not have a confirmed diagnosis for slow-channel CMS, but we decided to give the Fluoxetine a try since it works the complete opposite way from Mestinon. I've only been on it a month, but I definitely notice I have more endurance. I'm able to walk longer, faster, climb stairs with absolutely no problem. It's been pretty amazing. I don't see any real improvement in my facial muscles, but maybe that will come. At least now I finally feel like I could be on the road to recovery!

In the beginning...

Everything seemed fine at first. I went through the first years of my life with no major problems. I tended to hit my developmental milestones on the late side of normal, but it didn't seem to be late enough to really raise concerns. Looking back at old photos now I can see my slightly droopy eyelids and my weak smile.

I was never a fast runner, and always hated games like Duck, Duck, Goose! It was so frustrating to always get caught! But I was still pretty active as a child- jumping rope, playing on the trampoline, roller skating, riding my bike & climbing trees. I started really having problems when I was nine. I was falling down a lot- my legs would just give out. I could no longer do flips on the trampoline, and I absolutely could not run. My gym teacher told my parents there was something really wrong with me. So, the journey began....

Our first stop was an orthopedic doctor. He thought I might have arthritis in my knees, causing them to give out. I was sent to intensive physical therapy three days a week. Well, myasthenia tends to get worse with exercise. So I found myself quickly deteriorating. The doctors were stumped, but eventually someone suggested I go to a neurologist. It seems I spent the whole summer having various tests and procedures done, but finally I had a diagnosis. After about 2 years of struggling, I was finally able to put a name to my condition: Congenital Myasthenic Syndrome (CMS). My parents told me in the car on the way home from the doctor's office. The first question I asked was "Am I going to die?" They said no, so I was a little relieved by that at least. But I saw my dreams of being a cheerleader go up in smoke!

Oh well. Such is life. You never know what is going to happen. It took a long time for me to come to grips with this disease and the physical limitations I would have because of it. But with time came acceptance, and a determination to make the most of my life- regardless of my situation!