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?