Last Two & a Mom's Mission

INSTRUMENTS (#6 & #9)Well here are the last two creations, as promised for the money donated, Thank you.
[For every $500 donated, we invent a new musical instrument]. Having said that, we’re only $150 off of starting the next one, so if anyone’s feeling generous… click here :)

Stringtotter:
Made by Nao Nakazawa, joined in performance by Sam Hernandez

Bartz Box:

Made by Charlie Gurke, performed by Claire Phillips

CHARCOT MARIE TOOTH
With the completion of the instruments, it is easy (and fun) to get caught up in the excitement of showing them off and exploring what they can do, hence the festivals and performances we're involved with. Thank you Jeana for writing the following post so we can keep the fundraising and awareness of CMT moving with the same enthusiasm as the instruments. (Tim)

Mom's Mission
post by Jeana Sweeney

As I tuck my two beautiful girls into bed, I give myself a little pat on the back and think, “Well I made it through another day.” Being a mom with a disability can sometimes be very challenging, so I feel much rewarded when the day ends, and I know that I gave my children everything I have.

I have been living with a disorder called Charcot Marie Tooth since the age 15. Charcot Marie Tooth Disorder, also known as CMT, is the most commonly inherited peripheral neuropathy. It affects 2.6 million people worldwide. Progression is slow, causing loss of normal function and/or sensation in the lower legs/feet and hands/arms. The symptoms and characteristics of CMT are tripping, ankle sprains and burning or pins and needles sensations in the feet or hands. They also include foot deformities such as high arches and hammertoes. CMT causes muscle wasting in the lower legs and feet and muscular atrophy in the hands, often causing people to have difficulty with tasks involving manual dexterity, such as writing and manipulating zippers and buttons.

As the alarm goes off at 7:00am, I lay in bed, and my legs are pounding in pain. I think, “Lie in bed or go in enjoy my day?” I always choose to go enjoy my day!! As soon as I hear “Mom are you up?” l put my pain aside, smile, and start my day. Sometimes it does get upsetting to me that I have a disability. The little things that people do everyday are sometimes frustrating to me, like opening a new box of cereal or cutting an apple. I have taught my “little ladies” to be very patient with me because it definitely takes me longer to button up their coats than it does for their daddy. My oldest knows that I cannot participate in a game of softball, but she knows that I will be the coach in a heartbeat. It’s not about not being able to do things, it just means I have to change the way I do them.

The day the doctor told me that my youngest daughter has CMT, I cried for days. There’s a tremendous amount of guilt that goes along with knowing you as a parent passed a disorder on to your child. Being that CMT is inherited, your children have a 50/50 chance of having it. After I wiped my tears away, I promised myself that I would try to be the best role model for her. My goal is to show her that she can live a fulfilling life even though she is living with CMT. I try to be one step ahead of her CMT progression by scheduling physical therapy sessions once a week. She also does therapeutic horseback riding once a week from spring to fall. As parents, I feel it is our job to educate ourselves and be the best advocate we can be for our child.

I have been involved with the CMT Association (CMTA) for over eight years, I started by being the co-leader for the support group in Johnstown, PA, which holds over 100 individuals with CMT (one of the largest in the country). I slowing started doing fundraisers and raising awareness. With the news of my daughter and the CMTA starting the STAR Program (which is an aggressive research program), I become a vigilant advocate for raising awareness and funds for CMT.

I call this a MOM'S MISSION!!!

I have never been ashamed or embarrassed about having a disability and neither should anyone else living with one. I truly think that one of the most interesting things about me is my CMT, because when I am telling someone that I have CMT, I am teaching them at the same time.

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.

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.