The Good News and the Bad News

Last weekend we had an instrument making workshop to combine forces to get these new inventions completed. We got one finished, two half done and one nearly started, which is pretty good going considering how much fun we had.

This post marks the commencement and completion of the Sound Swing by Claire Phillips; a simple and open-ended approach makes this a beautiful instrument. Also below is a great insight into the unpredictable nature of CMT from John Oltmann; the main cause of frustration and confusion around the disease by both people that have it, and those that don't. Thanks to you both. (Tim)


INSTRUMENTS (#8)

The Sound Swing
post by Claire Phillips

I’ve always loved the sound of wooden percussion instruments, and this love amplified when I first heard the wood blocks in Iannis Xenakis’ solo percussion piece, Rebonds. The woodblocks come in unabashedly, instantly creating an excitement in the air. Woodblocks are played very deliberately to get this confident sound, but I wanted to make an instrument that incorporated an element of the unknown in its very being.

The Sound Swing uses swinging boxes that house a golf ball each. The ball hitting the edge of the box at the end of the swinging motion creates the sound. Using a pendulum swing as the perpetrator of the attack of each note means that the next note will occur at a slightly shorter interval, decaying over time until it is still and silent. When allowed to swing, the control over the pattern of the piece is taken away from the performer and into the hands of laws of physics. When multiple swings are in motion at once a wonderful polyrhythm occurs that would be almost impossible to replicate with any traditional instrument, including the treasured woodblocks, due to the difficulty of a human being perceiving such rhythms well enough to play them accurately.

The process for making the actual sounds took much experimenting. I wanted to limit the number of variables for each sound, so I decided on using the same item in each box, in this case, the golf ball. I tried many other types of ball; bead, ping pong, marble, etc, but none made as deep and clear of a sound as the golf ball.

I prepared the bottom of each wooden box by lining it with craft foam. This dampens the sound created by the ball rolling from one end of the box to the other. I only wanted to hear the “tock” of the ball hitting the wall of the container, not the sound of it getting there. I experimented with different lining materials including feathers and felt, but the craft foam worked the best. I also considered lining only the sides and not the bottom of some boxes to isolate the roll sound as another layer to the instrument, but in the end decided to have a tighter constraint system in order to have a unified sounding instrument.

I did allow some variance by having three sets of two boxes hanging in the swing set. Each set of two hangs at a different length so there is a long, medium and short set. This allows for a more interesting rhythmic possibility as the time it takes for the ball to hit the edge of the box at the end of the long strings is much slower than that of the short. This is the same concept as the older metronomes that swing back and forth, and as you adjust the weight higher or lower the beat is measured faster or slower. I’d like to see someone keep time with the Sound Swing!

The other designed variation in sound types in the Sound Swing is that in each pair of boxes, one rectangle is facing length-ways, while the other is sideways. The ball hitting the edge of the box sounds quite different depending on the length of wood that it hits, so the lengthways sounds different to the sideways. There is also some natural fluctuation in sound depending on each individual piece of wood. This is not deliberate, however I really enjoy the result.

The actual frame construction and method of hanging the boxes also took some trial and error, as well as some help from the wonderful Tim. For example, we realized that unless we hung the boxes with the strings wide apart, the box would swing in a fluid arc that did not encourage the ball to whack the side of the box. The box needs to stay more parallel to the ground in order for a strong “hit”.

It is up to the composer or performer to come up with interesting ways to play the Sound Swing. With the limited amount of time I have spent with it (we had to deconstruct for now since it takes up the whole living room), I noticed that pulling rather than pushing is easier. Also, playing different combinations of boxes together can make an endless amount of variation: it’s fun to play just one box, or in pairs, or by box alignment, all at once, etc. There are endless possibilities, and each time it is played it will sound different! A particularly enjoyable moment occurs towards the very end of the performance when the silence is pregnant between beats, and nobody knows when the final note will be.





CHARCOT MARIE TOOTH

The Good News and the Bad News
post by John Oltmann

“The good news is it’s not going to kill you. The bad news is it’s not going to kill you.”

That’s how an e-quaintance says his neurologist gave him his diagnosis of Charcot-Marie-Tooth, a peripheral nerve disease.

My own diagnosis, which I received from two different neurologists, one more literally than the other, basically said, “Yup, you’ve got it, have a nice life.” Experience indicates that neurologists are interested in you up until they determine there is nothing they can do to help, and then it’s time for them to move on.

The first time Charcot was mentioned to me, at age 50, it was regarding my left foot, which one morning was suddenly too painful to walk on. I was told I had a Charcot foot, but nobody spelled it out for me. A web search for “sharko foot” found a site that included a pronunciation, and I learned it was spelled Charcot. I also discovered that there was even a disease called Charcot-Marie-Tooth.

The next mention was from the orthopedic surgeon working on my Charcot foot, who, based on the classic deformed shape of my other foot, made a visual diagnosis of Charcot-Marie-Tooth. Another web search took me to Charcot-Marie-Tooth.org, where a list of symptoms read like a check list for me of all the things that have ever been ‘funny’ about my physical condition.

It’s now been almost five years since that first mention of Charcot anything. At first, I thought the rate of progression was slight, and I would live the rest of my life without too much impact. Instead it has been like a switch was flipped, with the volume turned waaaaaaay up on the symptoms. At times, it seems like six months ago, and scarily, sometimes even two weeks ago, were the good old days.

So I cope, I whine, and I do what I am able to do while I still am able to do it, and try to do everything I can do to have that ‘nice life’.

Four Kinds of People in the World & The Musicycle

The completion of Instrument #7 brings speed and a sci-fi aesthetic to the project - this one will go far! Also see below a great piece from Ron Rodrigues, reflecting on being a caregiver for his wife who has CMT. Wise words, but so much easier to read than implement... thanks Ron. (Tim)

INSTRUMENTS (#7)

The Thornton OG Musicycle
post by Will Longstreth


This creation was inspired by the music box I used to play with when I was a kid in combination with my lifelong passion, cycling. The box (as seen in the video in an earlier post) is a "Thorton" metal disc music box, and was passed down from my great-grandfather. Apparently these were quite fashionable around the turn of the century. They also have sharp saw-toothed edges for torturing younger siblings, but I digress.

So anyway, the theory was to mount a mechanism which is capable of producing different notes akin to the tabs on the records hitting the keys in the music box as the record spins. On the bike the best way to do this seemed to be mounting spring steel, cut to different lengths for different notes, on levers placed in the same spacing as the spokes so you can play chords (ideally).



With a little help from my more than capable machinist friend, voila! We're still working on tuning and how to mic the different tabs. Always an education.

Here is it in action...






CHARCOT MARIE TOOTH

Four Kinds of People in the World
post by Ron Rodrigues

"There are only four kinds of people in the world--those who have been caregivers, those who currently are caregivers, those who will be caregivers, and those who will need caregivers."

- Rosalynn Carter

In the beginning of my caregiving role with my wife, Donna, I wanted to somehow make her pain go away. Massaging her was with the expectation of making a difference. This expectation had a lot of side effects. For example I would deny the existence of her ailment. She'd ask me, 'do you see the atrophy in my legs'. I'd say no. It felt like if I gave it "power" CMT would overtake her. I felt I was unable to help. In some ways I felt like a failure.

Then one day I had a shift. Instead of trying to fix her I just loved her. Boundaries were set by me. In doing this I was able to say - wow sounds like you really need to see your Doctor. I'm here to love you. Technical questions and concerns need to be discussed with your doctors. I am a licensed massage therapist. However this is my wife so when Donna needed a full body massage I needed to gift it to her from a practicing massage therapist. This was probably the biggest lesson I had to learn.

I am to give love - only love and that's it. I must express compassion and forgive myself when I feel I have come up short. I am reminded that setting boundaries opens my heart.

Once I was willing to - not be enough - I was enough. This helped me care. I found relief. When I felt better everything flowed. The energy was positive and everything worked out. Then came the awareness that sometimes letting Donna figure it out for herself gave her strength and hope. It allowed me to come from love and compassion. This was rewarding.

I am now reminding myself to stay out of the outcome. Living in the moment makes it possible to see the little things that make big differences. Getting out of my ego self opens the door for true caregiving. When I am in MYSELF all is well and my inner strength connects with Donna's inner strength.

Now I give care and I receive so much. In order to be a good caregiver for Donna it is so important to respect myself. I am very hopeful we will find a way through & that's what caregiving is all about. Breathe and relax and share Love, Light & Laughter.

CMT Awareness Week marks completion of Bubble Organ

INSTRUMENTS

Completion of the Bubble Organ
post by Tim Phillips

Phew, I've done it. I've created a monster, I have a bubble organ in my midst.

I took the method for making one bubble module and did it 8 times (each time a little better so that the last bubble drone lasts about two minutes)! Each module has its own bubble tone governed by the length of its attached tube. It took a lot longer to build than I expected, but I'm more than happy with the result.

As you can see I have them all lined up from low to high, somewhat like a traditional organ. With a little more space, I'm looking forward to future variations in their positioning; perhaps scattered, in a giant circle, clusters, patterns - there is a lot of room for more exploration.

Enough with the words, I know you're really interested in how it sounds, so here are some examples of what it can do.

1. This is each of the bubbles in sequence, from high to low:

2. This is the Organ's debut as a performing instrument:

3. Just for kicks, this one shows how long a bubble module can go:

CHARCOT MARIE TOOTH

CMT Awareness Week
post by Tim Phillips

This week is the first ever National CMT awareness week in the U.S., so be aware!
Stemming from a whole stream of grassroots actions (this project being one), there has been enough momentum created to really try and raise national awareness to this disorder.

This is the website: www.wearethecmta.com

I feel confident that the CMTA have set up a very strong research program (STAR) that is making great progress, but I am convinced of this fact due to a fair amount of inside knowledge. The important accompanying task to the research is to create a public face for CMT, one that demonstrates the integrity and efficiency of the work being done, so that people unconnected with the disorder can also recognize the value of supporting this cause. (To see all I've written about the research, click HERE and scroll down).

The awareness week allows the CMTA to bring together all of the individual actions with bigger national events such as radio, television and internet segments, to voice a 'rallying cry' if you like. Hopefully it will create enough conversation to get some decent media coverage so CMT can start to become a household name.

As an interesting side note, I was contacted this week by the original maker of a bubble organ, Aaron Wendel, a stranger who by chance played a timely part in inspiring the 'CMT creates: music' project. Of his own accord, he had stumbled across my bubble organ on the internet and then wrote to offer his support for the cause. This was an exciting affirmation for the project and apt that it coincided with the completion of my bubble organ!

If you haven't told anyone else about this project or CMT, this is the week to do it. Please help us out and pass it on. In case you're wondering, I'm still very open to making more instruments if you're willing to donate more money! - DONATE HERE -

In the Pursuit of Pretty Shoes

INSTRUMENTS
Here it is, the first instrument to be finished (well as near as damn it),
#5 - The Texture Harp. (Tim)

Texture Harp
post by Meghann Welsh

I've discovered it's hard to describe the sounds of textures. "Talking about music is like dancing about architecture" as they say. So, I've used the generic "white noise", although technically incorrect, as my base sound descriptor, and applied modifiers to that. Let me know if you can hear the textures or describe the textures differently!

Currently, the strings have four different textures:

Waxed: Twine coated with bee's wax. Sound when rubbed is a sticky-smooth "white noise", or a squeal and squawk when more pressure is applied.
Grit: Twine coated with wood glue and sawdust. Sound when rubbed is a rough "white noise".
Fiber: Unaltered twine. Sound when rubbed is a soft-smooth "white noise".
Metal: Unaltered metal twine. Sound when rubbed is a hard-smooth "white noise", or a zipper-like noise if fingernails are used.

The strings hang from a frame that I made out of 1/2" EMT. Wye (Y) fittings at the top allow for the frame to be expanded to make room for more strings. The threaded pipe allows for the frame to be broken down and reconstructed pretty easily. I may still add a soundboard, but cans at the end of each string, acting as resonance chambers, are doing a pretty good job. Experimentation with piezo mics in process...

In addition to being rubbed, each string can be plucked.
In both rubbing and plucking techniques, the pitch of each string can be altered by changing the tension of the string (by applying pressure to the can).

Hopefully you can get an idea of the various sound possibilities with this late night demonstration:

CHARCOT MARIE TOOTH
I met Jessica through her blog and have very much enjoyed her writing, so I'm very happy that she has offered to contribute to this project! Our 1st New Yorker (though originally from California...) highlights the common fact that most people with CMT are not diagnosed for a long time, because even the medical world is not very clued in to this syndrome. (Tim)

My Story
post by Jessica Wells, a.k.a. Miss Pretty Shoes

Originally a So-Cal girl, I quit everything I knew in the Spring of 2008 to come to the Big Apple to chase my dreams! After a year of "pounding the pavement" of The City That Never Sleeps, I started experiencing unshakable pain and swelling in my left foot and ankle. I tried for 6 months to identify the cause of the pain, visiting podiatrists, chiropractors, orthopedic surgeons, and neurologists along the way--all to no avail! Finally, after a visit to an unrelenting and dedicated sports medicine doctor, we discovered a multitude of issues, including a torn peroneal ligament, severe arthritis, and a bone bruise.

After failed attempts at using natural healing methods like physical therapy and reduced strenuous activity, I was referred to a surgeon for a consultation. Immediately upon taking one look at my feet, he diagnosed me with Charcot Marie Tooth Syndrome, a progressively degenerative neuro-muscular disorder. Through my own extensive research, I learned I had most of the classic symptoms of CMT, though no other physician had ever taken notice to them (including an inexplicable propensity for broken bones, sprains, and other joint injuries; painful arches; an overwhelming dislike for running; a lifelong remorse for never fulfilling my potential as a professional ballerina; and a general sense of crabbiness without at least 8 hours of sleep every night).

Over the next few months, my surgeon (a.k.a. the mad scientist) and I formulated a treatment plan centered around progressive surgeries to strengthen my ankles, re-construct my feet, and to hopefully reverse the progression of this rare disease, beginning with the left foot first. And, in the midst of these physical challenges, I have also been forced to come to grips emotionally and mentally with the new found diagnosis of this debilitating, lifelong disease.

I am determined to overcome the challenges of CMT, all with the hope of someday again finding and putting on that perfect pair of pretty shoes . . .

Travel with the US Paralympic Cycling team

INSTRUMENTS
hj mooij has just launched a new website, it's pretty exciting...
http://hjmooijink.com/

Different Wands for Different Tones
post by hj mooij

Construction of the tinkler has begun and is progressing nicely. So far it features a side cut wheel hole for easy spinning and an open top for playing. A top cover will be created soon with a nice angled hole so one can play the tinkler with one or two hands while reaching the entire spectrum of spokes. The tinkler makes different tones with different playing wands, find your own or just use a nail, it all sounds great.

CHARCOT MARIE TOOTH

Anthony Zahn is a bike racer, a damn good one at that - He just won bronze at the Paralympic World Cycling Championships. He has CMT, and is a successful athlete with it, which is why I approached him to write a post. Anthony was very friendly and wanting to help with the project, yet this was his initial response: "I honestly don't have too much to say about CMT as I don't really dwell on it. I just try to get through my day and do what I have to do without thinking about things I can't control." I think that is inspiring in its self, but I'm very happy that he went on to write the following post describing his racing over the past few weeks to share a little of what's involved. (Tim)
[To see more pictures and news, follow this link: http://usparalympics.org/athletes/anthony-zahn/news]

Travel with the US Paralympic Cycling team
post by Anthony Zahn

Wow. What a week. Or three I think since I've been home. The summer's been a long blur. Starting in late April/ early May with 2 weeks of stage races in Spain and France, home for 2 weeks, a week in Oregon for road Nationals, home for a week, off to the Chicago area for 4 days of Superweek, straight to Colorado Springs for track camp and track Nats, home for 10 days, then back to Co for 10 days of prtp camp and off to 10 days in Baie Comeau Canada for Paralympic World Cycling Championships.

2010 Paralympic World Cycling Championships

This is a bit more travel than typical for the US Paralympic Cycling team, but it's what we do. There are about 18 American athletes here with various disabilities ranging from being born without a portion of an arm, to limbs missing from various injuries, to CP CMT (Me) and paraplegics. With all of the other countries thrown in, there are more injuries here than I think anyone can imagine. Many have full time jobs and use vacation and sick days to travel to one or two events per year, others are full time athletes, but everybody here lives and conducts themselves as a professional athlete.

The travel is a bit tough, for instance it took us four two hour flights to get here, our mechanics had to rent a truck for out bike to drive them from Montreal which took 11 hours, and our luggage didn't get here for another 3 days!

Hands down the hardest part is the 100 days or so per year away from home. That's about all there is to say about that.

The team is the greatest bunch of people though, which makes things a lot easier. Spending roughly 1/3 of a year together in 10 day to four week chunks could get pretty crazy but everyone on the team athletes and the 10 or so staffers are among the most professional and funny people I have ever met! Everybody knows when to be where, what to bring (and leave home) and what to do to make things run smoothly and win medals.

I'm done with racing now so I get to pack everything up to put on the truck for shipping, go watch the last few races and cheer for some teammates, go to the closing ceremony, find dinner somewhere in there, sleep for a few hours and hit the road to the airport for the first of another four flights home!

2009 Paralympic World Cycling Championships (Zahn on the right)

Weighing Up a 50/50 Chance

INSTRUMENTS (#6)

Something Dangerously Fun
post by Nao Nakazawa

Agghhh... Summer is almost over! I had three months off from school and had a plenty of time to work on the Stringtotter, but I got lazy... I blame this cold summer.

So, having a few days left until being back in grad school, I made a trip to a used lumber yard, C&K Salvage in East Oakland. The people there are extremely nice and helpful. I got 2, 4ft 6X8 and a 11ft 2X8 for only $25! If you need some lumber, this is the place to go! http://eastbay.citysearch.com/profile/1012891/oakland_ca/c_k_salvage.html

The design of the teeter totter is quite simple: drill a hole in each of the 6X8's, then slot a pipe through them both to make the pivot point, the fulcrum. Then take the long plank (in this case 11ft 2X8) and screw a couple of 2X3's in the middle of the underside with just enough gap to rest the pipe between them.




I didn't cut the 6X8's any shorter than they came because I wanted to see how high it would go and how hard it is to ride. So if you are making this for you kids, I do not recommend the center to be as high as 4 feet. It should be more like 2 to 3 feet depending on how long the main board is. But we're not kids (even if we act like them), so we get to go some 7' in the air and have something dangerously fun.




I suggested to Tim, "maybe we need to make this thing lower, so that it'll be safer and easier for the performers." But before I could finish the sentence, he said "nonsense, I won't allow it!" Then he skillfully climbed up using a chair and here we are!





Yes, it works and is a lot of fun going up and down! I guess we'll keep the height as it is.



So here are a few problems I need to fix.

Firstly, as you can tell by the video, the main board is bending. Although it is redwood and pretty strong, it is possible that it could break when someone is riding it, and that would be awkwardly embarrassing. So I'm putting a couple of 2X4's on the bottom to strengthen it.

Secondly, I realized that in order to balance out the weight differences between the two performers, they have to shift their seating positions forward and back. Currently the instrument is about 9 feet and that will only leave 1 foot on either side to sit. There are few ways to solve this problem. I can shorten the instrument, make the center of main board adjustable, or put some weight at the end for counter balance.

Another issue you can see on the video is that the angle of the teeter totter gets pretty steep and it will be hard to play the instrument when on it. I will have to install bars on which performers can rest their thighs in order to support their weight. I thought of a harness, but then it is nice for performer to abort the teeter totter in case of an emergency, whatever that could be...

Lastly, I need to make the center more stable. I like the fact this teeter totter is easy to take a part and be packed into my little Mazda, so I would have to come up with a clever way to make it sturdy and easily deconstructable.

Some issues that were addressed on my last blog still remains... I have not tested other materials for the string yet, although I have been looking around for a good materials. But, I made a new bridge which is taller and more curved on the top which made the bowing so much easier. I also embedded the pickup microphone in the bridge instead of resting between the top board and the bridge. It sounds much better and cleaner. I will work more on the other issues of the instrument.

When I get these problems fixed, next step is putting the instrument and the teeter totter together. Now, that will be exciting, so stay tuned.

I also have a questions for the readers. I will have to sand down and paint the stringtotter. What color should it be? Let me know!

CHARCOT MARIE TOOTH

Weighing Up a 50/50 Chance
post by Tim Phillips & Elizabeth Ouellette

CMT is a genetically inherited disease, it's something that get passed on... I got it by chance, a mutated gene, but now I have it there's a 50% chance I'll pass it on to any children I have.

There are 3 ways of passing it on depending on which type of CMT you have. I have the most common type, CMT 1a, which means 'autosomal dominant inheritance'. Hopefully the picture or link below will help explain that term technically, but simply it means that 50/50 chance of passing it on - that's a lot to think about.

[The image shows the mother having CMT, but it works just the same if it's the father that has it].

Other types of CMT can be passed on by both 'Autosomal Recessive Inheritance' and 'X-linked Inheritance', follow this link for more info: http://www.charcot-marie-tooth.org/about_cmt/genetics.php


I came across this video from the Hereditary Neuropathy Foundation, who (like the CMTA) are funding CMT research and I thought it interesting enough to share. It has people talking about whether they would or wouldn't have children - though it gets pretty cheesy towards the end, so watch out!



Personally, CMT doesn't put me off having children, but I'm very aware that I will bear the responsibility of any related difficulties my child could have. I think the biggest challenge will be how to manage the disease; knowing so much about it and passing that on, without making it a big deal in the child's life.

I guess I've got that to come at some point, so in place of my inexperience, Elizabeth Ouellette has kindly put forward her top ten suggestions that she would give a parent whose child has CMT (being in that position herself).

The Top Ten List for Parents of Kids with CMT
by Elizabeth Ouellette

1) Education. The new book, “My Child Has CMT”, recently published by the CMTA, is the best printed guide to-date, filled with advice, information and invaluable resources for parents whose children have CMT. Call the CMTA or order your copy on-line today. In addition, read about the fundamentals of Charcot-Marie-Tooth disorder and sign up with the CMTA to receive updates on current CMT news. Most forms of CMT can be managed with physical therapy, braces, orthotics and a fundamental understanding of what CMT is and how it affects each individual differently. The symptoms and severity of two people within the same family, with the same type of CMT, can vary significantly. So, if a parent is in a wheelchair, and disabled by the effects of CMT, this does not necessarily mean the child will have the same symptoms or outcome.

2) Attitude. The reaction and response of parents towards the diagnosis of a child's CMT will affect that child profoundly. If a parent is very distraught about the diagnosis of CMT, it is important that the parent(s) talk with a professional to eventually embrace the diagnosis. Although it is sometimes difficult to keep emotions in tact, a positive outlook and a gentle understanding toward the child, which includes empathy (not sympathy), will help a child with day-to-day struggles much more then an anxiety-ridden houseful which promotes helplessness and negativity about the CMT.

3) Hope. The CMTA is now working hard on treatments and a cure for CMT through the Strategy to Accelerate Research (STAR) initiative. Learn more about STAR and just how hard our scientists are working to find a treatment and eventual cure for CMT. If there is one message to give your child, it is one of hope and promise for the future.

4) Acceptance. Parents and children will experience a wide range of emotions from sadness and despair to anger then acceptance and back to sadness again. If the parents learn to cope with the diagnosis of CMT, it will be that much easier for the child to
“own” his or her personal life process with this progressive neurological condition. In my own personal opinion, I do not believe in hiding or being ashamed of this condition, because we all have differences and CMT is yet another one of life’s challenges to manage and overcome. It is true that many parents feel guilty for having passed the disease on to the children, but feeling guilty serves no one, in the end. Every single parent genetically passes many physical, behavioral and biological traits on to his or her child, and if the CMT gene is included, then so be it. The reality is that changing the past is not an option, so accept the present and teach your kids to live life to the fullest, despite their differences.

5) Role Models. The parent who has CMT can be a wonderful role model for his or her child. Show your children how to confront conflict and adversity by role modeling coping behaviors that you would like to see him or her replicate in the future. You may also find wonderful role models from within the CMT community. Anthony Zhan is a biker who is on the US Paralympics’ team and has been very successful in his pursuits and goals, despite his CMT. Jonah Berger is a young man who wrote a book called,” Walk like a Cowboy” about his experiences with discovery and acceptance and of his own CMT. He now works with handicapped kids, plays the drums as a means of personal expression sharing his talent with others. These are just a few of the many unbelievable individuals who are winning the game with the deck of cards life has thrown their way. Find others who have CMT and share their exploits with your family.

6) Wayne State and Dr. Michael Shy. Bring your child to the Wayne State CMT Clinic, if possible. When I brought my son to Wayne State to see Dr. Shy and his team, it was as if the sun started shining in my shady neck of the woods again. We felt accepted, understood and supported by every single staff member there. We met others dealing with CMT-related issues and felt that we were not alone any longer. His website is: http://genetics.wayne.edu/faculty/shy/index.php


7) CMT Support Groups. I think it is important for parents to attend a CMT support group, if possible. I know there are not support groups in every corner of the US, but we do have quite a few (50 and counting) and the support group communities are a constant source of education and information. Parents hesitate to bring their kids to the support groups, which is understandable. However, if you find other parents at the support group with a child in the same age bracket as your own child, plan a day where the kids or teens can get together around common interests, have dinner and promote relationship-building.

8) Organizations. Make sure to join the CMTA and sign up for email updates. Our newsletter, website, informational materials provide up-to-date information on all types of CMT.
Register with the Muscular Dystrophy Association (MDA). The MDA provides services to all those who have been diagnosed with CMT. Kids grow especially fast and those who need yearly bracing and other aides or even physical therapy can provoke financial crisis within a family. The MDA also sends out a free newsletter which may or may not include CMT information, but the focus is both on kids and adults and the struggles they have to face daily. Moreover, the MDA summer camp for kids with all types of MD has been very popular among kids with CMT. Many go on to become counselors to help others with severe disabilities. It's a win-win situation.
Shriners Hospitals for Children will also accept children up to the age of 18 with CMT. Shriners provides an international, one-of-a-kind international health care system dedicated to improving the lives of children by providing specialty pediatric care, innovative research and outstanding teaching programs.

9) Medical Issues. If your child is dealing with physical pain, depression, sleep apnea, or any one of a large number of co-morbid conditions related to CMT, bring your child to your general practitioner and request specialist referrals who know about CMT. As your children grow, pay attention to their spines as scoliosis and kyphosis can creep up before you know it. The spine disorders usually worsen during puberty, so keep your eyes open and intervene quickly to avoid further complications in the future. Moreover, we found that alternative therapies like biofeedback and relaxation not only helped with pain, but also helped with the anxiety and or depression that can come with having a progressive neuromuscular disorder that is not very well understood.

10) Explaining CMT. Find easy ways for your child to explain what he or she has to playmates and peers. These explanations should be age- appropriate, so, if for example, you have a seven year old with CMT who has a hard time with PE in school, it may make your child feel better if he or she has words to explain what CMT is. For a seven-year-old, I would say something to affect that,” my legs don't work quite right and my muscles are not very strong because of CMT.” “I wear braces so that I don't trip”. It is the parents’ job to educate the teachers and your child's friends. Make sure you let schoolmates know that CMT is not contagious and it will not kill your child. These are real fears that many children have which will cause them to avoid playing or hanging around your kids. This CMTA has a DVD called, “Teaching kids about CMT” which you can use in many ways. You may want to do some of the activities on this DVD for friends and family to help them understand CMT better. Or you may want to give this to the teacher so that he or she understands your child's difficulties better. I myself made the program up for elementary school kids to foster understanding of CMT in a fun and educational way. However, make sure your child feels comfortable with the concept, before you go into the child's classroom to do this presentation.

Funky or Vanilla?

I have the pleasure of introducing instrument maker #7, Will Longstreth, who brings an expertise in bikes to the project (and all the mechanics that go along with them). Will has chosen to take the cryptic approach to blogging. An equation in pictures definitely has me intrigued as to where he's heading, all I know is that it sure looks pretty! (Tim)


INSTRUMENTS

Bicycle Music Box?
post by Will Longstreth

+

=

...?

CHARCOT MARIE TOOTH

Funky or Vanilla?
post by Nicole Burggraaf

In a few weeks, I’m being casted for my new AFOs. The docs are hoping that wearing
AFOs during the day at work will buy me a bit of extra stamina at the end of the day.
A bit of extra energy to help my husband prepare supper (much to his chagrin as my
specialty does not expand beyond Hamburger Helper), play with our son and be able to
stay awake beyond 9PM.

I have not had AFOs since the sixth grade. My experience with them was short. I did not
think they were much of help – but in hindsight I think that was the vanity of being 12
had a lot to do with my reluctance.

A few months ago, our 4 year old son (and fellow CMTer) was casted for his first pair of
AFOs. The design of the AFO has not changed in the twenty years since my first pair.
They are still pretty much reverse shin pads that extend down over the foot. But what has
changed is that AFOs can be customized to become one of a kind. There is a catalogue
of funky prints that can be placed over the AFO and an assortment of colours for the
velcro straps. Gone are the days of the vanilla white AFOs and bright white velcro
straps.

Our son chose a kid-appealing print of cars and trucks over a blue background. He
also chose black straps to compliment the tires on the trucks. When we returned
to the orthotist a few weeks later to pick up his AFOs, our son was so thrilled
with them that he threw a fist pump into the air and a resounding “YES! Cool!”

I’ve spent the past few weeks debating whether or not to stick to the plain Jane vanilla
coloured splints or choose a bright colourful design. (I should also note that our son
is petrified I’ll choose the car and truck pattern. Already at 4 he recognizes having the
same taste as his Mommy is SO-NOT-COOL!)

I’ve kept the CMT part of myself pretty low-key to the public at large. I walk with a
limp that I think most people assume is just a nagging injury. My surgical scars are well
healed and not very visible. The only time people really quiz me is when I’m using my
cane in the winter months.

Its not as thought I’m ashamed of my CMT that I feel I must closet it. But I always
fear that if I go on to much about it, people will think I’m belly aching. And I always
think about the boys with Duchenne’s at my neuromuscular clinic visits when I
was a kid. CMT – at least how it affects me – is so lightweight in comparison.

And here lies my dilemma. Which is less likely to scream “Look at me y’all, I’m
DISABLED” and more likely to give off a vibe of “My legs need some extra help, but its
cool.” The funky AFO or the vanilla AFO?

Please watch this video and give it the 'thumbs up'!

INSTRUMENTS

Please watch this video and give it the 'thumbs up'!
post by Tim Phillips

The project is going so well, we've decided to try and raise it up a notch - we want more people to get involved... but they can't if they don't know it exists.

So we've decided to aim for some youtube stardom to gather momentum.
Please, please, please watch this video and give it a 'thumbs up', make it you 'favorite', tell your friends, whatever it takes to let the world know it's cool and we'll try and get it featured by the powers that be! Thanks.

If you have a quick eye, you can see my latest modifications on the bubble organ design. The cost is now minimal and I reckon I can make 5 in an hour - so I'm ready for mass production!





CHARCOT MARIE TOOTH

X-Ray Vision
post by John Berger

I got it touch with this post's CMT writer as a friend of a friend, his name is Jonah Berger. I sent him an email, explained the project and he kindly replied saying he was interested, but he also asked me to call and talk it through. I'm telling you this because when we got hold of each other, a interesting and open conversation followed, I made an inspiring friend. It was a strong reminder that although the internet is a powerful tool for connecting, it's not so hot on conversation.

One of the things I found out is that Jonah has already written a book that compiles his thoughts on living with CMT: He Walks Like A Cowboy, this is how he describes it:

"In any one lifetime, part of the work of living is to go from figuring out what you got dealt, to accepting it. Learning to wield your unique situation to best get your point across. I have found through my life lived with a physical challenge that there are two versions of how a disability is dealt with. One is the version you show to others. The other is the version you are really and truly feeling inside.

In my life, these two versions started out light-years apart. And over the span of 38 years, and the influence of many magic people, they have been growing closer all of the time. There is something incredibly free about showing the world what you have going on inside. It helps to keep the struggle of your situation confined to the actual challenge, and not intensified by shame. The pages of this book are a true marriage between my two versions. Charting the affect of a disability on the physical, emotional, and spiritual. Containing an honest view of how struggles can be beautiful, and strength can be found in the weakest of places."

I have a copy on order, but in the meantime Jonah has offered to post one of his chapters here to be part of this project.

X-Ray Vision

There is a certain mentality I have seen in those who deal with any kind of struggle in life, a tool that is used to handle and deal with this struggle. It is the attempt and eventual success of trying to see the good in the struggle. One attempts to identify the pieces or results of the struggle that are good, that are beneficial. Does it make the struggle disappear? No. Yet I have found that this mentality helps to put the struggle into a better perspective, or at least a more palatable one.

In regards to this philosophy, several positive results stand out. As a result of my disability, I am an inherently sensitive man. I had very little choice but to be concerned for the struggles of others because I have relied so much on their concern for mine. My disability has given me the desire to see others’ struggles and to help them to minimize them. I have learned to take my familiarity with pain and use it to empathize with others. My disability has taught me humility, at least in the physical sense. I have learned to appreciate strength through a perspective impossible to possess without a disability.

I must say that one of my favorite silver linings is what I call X-ray vision. Throughout my life with a disability, I have noticed a wide variety of reactions towards me. And the common denominator that ties these various reactions together is that my disability allows me to see people’s reactions. It allows me to see people’s hearts. It allows me to see people’s strengths and weakness in dealing with me. It allows me X-ray vision into one part of who they are. I have become better over the years at using this X-ray, as well. At first, it was like a new power I did not yet know how to use. For example, I used to judge a quiet response to my disclosure as a lack of concern or feeling about it. Yet, those quiet people are often the ones who later lend me assistance on a hike or similar challenge without using their words. It taught me that some people would rather show their support through action.

Enter Brandt Lewis, my shining example of the X-ray at work. In my later summers at Camp Ramblewood, when I was about seventeen or eighteen, I met Brandt Lewis. Brandt was also a counselor at Ramblewood. He was from Arizona and a truly interesting guy. Brandt was also a very good looking guy. And he was fully aware of this. As a result of being a good looking guy, Brandt was used to having his way with women. He was a player from start to finish. He was on his game all of the time, and after a few summers at Ramblewood, Brandt developed a reputation that was less than favorable. He was kind of a jerk. He very rarely spoke with sincerity, and he very rarely treated women (or men, for that matter) with a great deal of respect. Brandt was in it for Brandt, or so it would seem.

For whatever combination of reasons, I always got along with him. I liked Brandt. He made me laugh and I did the same in return, and I wasn’t a woman, so he had very little reason to piss me off. By the last summer that Brandt and I were at camp together, he and I had talked a small amount about my disability. Nothing of major substance, but it is fair to say that he was aware of my condition.

Each night of camp, after the kids had gone to sleep, a quarter of the staff would stay back to look after them. That left three quarters of the staff free to go out to the local town bar and get drunk enough to forget we were childcare workers. When the kids were asleep, those who were bar-bound would gather and walk up to the parking lot together. It was dark at this point and to get to the parking lot, we had to walk up a long hill and cross a small field. The small field was no more than about 200 yards across, but it was made of uneven ground, filled with craters and dips. And was completely overgrown with tall grass, allowing no vision as to where the dips and holes were located. It was a disabled person’s minefield. For the most part, I was still in the phase of my journey where I kept my fear and discomfort to myself. I didn’t want to be seen as different. I would dread that field as it approached, and when the group would start walking across it, I would simply slow down a bit and take it at the most normal pace that my fear and feet could muster.

Except for the nights when Brandt and I were both part of the bar-bound crew. On those nights, it looked something like this: the group of about 25 staff would be walking up the hill and laughing, and when we got to the edge of the field, the group would keep walking at their pace, barely noticing that they had entered the pothole field of doom. Except that Brandt, each night that he was with the group, would always slow down a bit at the edge of the field, and subtly position himself about one foot ahead of me. He would never look back or speak a word, but he just walked ahead of me with his hand held out behind him. This guy, this player who had little respect for women, a guy most people had written off as a total asshole, was the one member of that whole group who never missed a chance to be my guide through that field. Most nights, we would walk across that field just fine. Me, uneasy, and Brandt with his hand behind him. But when I would stumble or my foot would land in one of those invisible holes, I would grab his hand to keep me from falling, and he would be right there for me. I don’t think anyone else in the group ever noticed that this went on, but I did. I have never forgotten it. It is a glowing example of X-ray vision.

As a result of my disability, I was able to see a side of Brandt, a side of his truest heart that most people never would have seen and probably wouldn’t have thought existed. I was fortunate to have that vision of him. He is a good guy who made a lifelong impression on me. There have been countless others who have helped me in this way over the years, countless others who have lended me a hand or an ear without me ever having to ask for it. I use my X-ray vision in all of those moments to see into the true heart of people. And more times than not, I see only good things.

____________________________
Jonah's a 38 year old native of Rockville, Maryland who has spent the last 10 years enjoying life in Denver, Colorado. He founded and runs a business called The Rhythm Within; a therapeutic mentoring service for children and young adults with special needs. He can be contacted at: bluewoodfire@hotmail.com

Music Drives...

INSTRUMENTS (#6)
Nao Nakazawa is an extraordinary film maker who has agreed to lend his imagination towards creating an instrument! Here is a link (http://vimeo.com/user2696976) to a little of his work - I'm a big fan of {X}.

Stringtotter?
post by Nao Nakazawa

To be honest, I'm not much of a carpenter and I'm a bit scared of power tools. So when I volunteered to make an instrument for Tim's CMT creates: music project, I wanted to make something simple that does not require too much sawing and nailing. This is no longer an option, because as I was brainstorming the instrument got more and more complicated and interesting.

The original idea was to make a ridiculously big string instrument. My concern with this is that although it would be big and ridiculous, it wouldn't be adding anything to the world of experimenting. So I thought about it for a little longer, like a month or so. One day, it came to me: since the instrument would be too big for just one person to play, it requires to be performed by at least two people. I thought, "Why not make this giant string instrument into a teeter totter!"

I researched online to see if it is possible for me to build a teeter totter string instrument. It seems like quite a lot of people have made their own teeter totters and blogged about it. I didn't know there were so many teeter totter enthusiasts out there. This guy, Homeless Dave in Ann Arbor, has an excellent website about his teeter totters and other cool stuff such as a bike powered washing machine: http://homelessdave.com/totterhome.htm.

The more I surfed the net, I realized it is not that hard to build, so it is time to get over my fear of carpentry and do this thing. After getting a little instruction from my housemate Sam on how to use power tools, I was ready. My first prototype is just the instrument part as I didn't want to build a whole teeter totter and then find out the instrument sounded horrible. So here it is:


And this is how it sounds:

This instrument part will be set on top of the teeter totter. One of the players will be at one end strumming, picking and bowing the strings and the other-end player picks the pitch and cords all the while going up and down on the teeter totter. It takes two to tango and to teeter totter. Playing this instrument is like having musical conversations and it requires you to pay attention to what your partner is doing in order to play good music. If you are a geeky couple, this could be the most romantic musical teeter totter experience you'll ever have.

Anyway, here are some parts that need improvements.

The box or the resonant chamber needs to be better. It has too much space between the walls. The top board is too thick - if I replace it with thinner one, I think it will sound better. I also feel like I could build something that's more aesthetically pleasing.

Here's the bridge. It needs to be higher so that when bowed, the bow doesn't hit the corner of the box. You can see the pickup microphone is between the bridge and the top board which works well, but picks up a lot of clunking sounds from the body of the instrument. I'm guessing that if instead I placed the mic on or in the bridge it would sound the strings more clearly. Maybe I should pick up a bridge for cello or double bass, but I'm afraid that will be expensive.

The tuning peg needs some better ideas too, I need to experiment more with this. Any suggestions? I also need to try other materials for the string. Right now, it's 40lb tested fishing line. I wanted something like 80lb tested line, but that's also expensive. Other ideas for the string are steel wire and tennis racket string. If you have any ideas for the string, please comment to let me know.

Lastly, as we were playing this instrument we came across a plastic ball with a bunch of holes and when we placed it on the string, it produced awesome sounds. There are so many ways to make sound out of this instrument and I certainly need to play more to find out other fun ways.



Oh, there's one more thing which is probably the most important issue. This little puppy needs an awesome name. Please let me know if you got one.
Thanks for reading!



CHARCOT MARIE TOOTH
Through this project I have recently come into contact with a great catalyst for CMT awareness, Gretchen Glick. I'll let her introduce herself through her post but can assure you she is someone who makes things happen. She has very kindly agreed for me to post two pieces of writing about her. The first was written by Susan Wheeler and describes a brief history of Gretchen's life with CMT. The second is by Gretchen herself and is an example of her current strength of mind. This is a link to a great online CMT resource that she both founded and runs: www.cmtushope.info (Tim)

Gretchen & CMTUS
post by Susan Wheeler with quotations by Gretchen Glick

CMT is the most commonly inherited form of peripheral neuropathy. Often referred to as a "family disease," CMT provides an element of emotional comfort within families that share affected genes. An unspoken connection exists, even if family members with CMT have died or are merely distant relatives. Since it's rare to meet strangers with CMT by chance, family ties may be the first and frequently--the only--hedge against isolation.

There is a small percentage of individuals with CMT where absolutely no genetic link can be found within their biological families; some of these individuals are adopted and are not privy to medical records. Others simply have no medical links. Though the percentages of such cases may be small, feelings of isolation are overwhelming for people in this rare category. Take it from Gretchen Glick someone who knows: "In some cases, individuals develop life-long addictions as a means to escape feelings of isolation or inner turmoil. It can be extremely difficult to sort through the haze of addiction and emotional darkness to re-create a life in which there is hope."

Now 57 (58 next month), Glick, has traveled an unusual path since her diagnosis of CMT at age nine. Clumsier than her friends, she recalls, "My feet always felt like they were burning, especially after hiking at summer camp. I'd be the first kid to eagerly jump into an icy cold stream, or should I say, fall into the stream." At that time, Glick walked outwardly on the left side of her left foot, and her right foot rolled inward, also to the left. This was corrected a year with surgical tendon transplants. Although surgery improved things physically, the real mystery was the origin of her CMT. Within her immediate family there was no genetic link. Neither parent had the disease, nor did her only brother, who was adopted and therefore excluded from the equation. Upon examination of her extended family, once again there were no apparent signs of CMT. The conclusion left Glick feeling like an outcast.

As a young woman she traveled the path of alcoholism until the age of twenty-seven. Now celebrating 31 years of sobriety she reflects, "I drank to ease the pain involved with CMT and my feelings of being alone and uncertain. After all, this was before computers, and finding information about CMT, or other people with the disease was almost impossible." This was something Glick would remember, and as fate would have it, she came full circle. Eventually, she created a Web site that informs and offers positive support for people with CMT.

Glick is outgoing by nature, and likes socializing with friends and having fun. Despite her ongoing inner struggle, she achieved a Master's Degree in Art History and went on to pursue an eclectic range of interests. She has worked as a Museum Curator, Public Relations Director, Nonprofit Fundraiser and in 1998, an independent travel agent specializing in eco-tourism. Glick has traveled all over the world, exploring destinations such as Australia, Central America and Europe, and readily offers firsthand advice to her clients. She lives in California, and enjoys what she calls "a balance of activity and couch time" as general fatigue is a daily battle. With her dog in tow, Glick likes to hike in the hills nearby and spends a fair bit of time in the garden.

To accommodate high arches and hammer toes, Glick wears custom orthotics to help stabilize her balance and ensure that her walking gait is heel-toe. "I'm a big fan of New Balance Shoes, even though I have to change my shoes three times a day." "Despite some muscle atrophy, my legs are pretty strong and I enjoy swimming, biking and snorkeling. I also have hand tremors which, thankfully, are very well controlled since I started first started taking the medication Elavin-Amitripytyline and now Symmetrel."

In 1999, Glick started CMTUS, an e-mail forum for people with CMT to share experiences and offer positive support to one another. Now in its fourth year, the membership is 600 strong, spanning 33 countries. She says proudly, "CMTUS has evolved to stand for Charcot-Marie-Tooth Universal Services, and our upbeat slogan for CMT is we 'Can Manage This!' Our goal is to increase awareness about CMT and offer support." Her organization has created informative Fact Sheets for both the medical profession and the general public. It has also linked with several neurology sites and to offer hundreds of useful links for people with CMT. "Through discussions we all learn a great deal from one another," she says.

Glick's goal for CMTUS is to move forward with upbeat and balanced steps, just as she has done with her CMT. "This is not a forum about being right or wrong," she says, "but instead a comfortable meeting place where like others can share and explore new and different ideas, one day at a time."



Music drives the devil away. (Martin Luther)
post by Gretchen Glick

How many times have we seen a movie and liked the music so much we went out and bought the sound track? It may have been classical music in the background, or country, western, jazz, or even rock music. Perhaps the music was soothing, or fun and spirited. It made us want to sing or dance. It was so good to listen to it made us feel good all over!

Music of many kinds can enrich the spirit, drive away our worries, and soothe tension. It's a gift we all can have as long as we can hear. And many who are hearing-impaired may still benefit from the rhythmic vibrations of music that are felt more than heard, but which can still be soothing.

My own experience with music helps me to meditate and focus on the NOW of the 'moment'. In doing so, I strengthen my emotional and spiritual life that sustain me as I continue living positive with CMT. The best music for this practice I have found is by Australian Richard Goldsworthy". (www.richardgoldsworthy.net)

When we take some time each day to stop and listen to music, we contribute to our physical well-being and our spiritual health. And we appreciate it when those who have the gift of making music share that gift with others. We may even find a music-making gift in ourselves that we can share and enjoy.

Affirmation: Today help me take time from the hectic part of my day and allow music to heal and refresh me.