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Sep 4 11

LOOKING FOR THE ELECTRIC STARTER – MILE 5

by chuck

HOW DO YOU HOPE?

I hope I hit the lottery, I hope I feel better tomorrow, I hope I have time to take a bike ride today and I hope I can make it up that hill…

Aristotle said, “Hope is the dream of a waking man.” I like “hope” and I usually like to dream, at least the ones I can remember, but I have to think of “hope” as something that must be connected to “doing”.  If I dream about “hope” or something that makes me feel hopeful and do nothing after waking, all I have is the dream. “Hope” is not something I like to wish for or want to wait for; it’s something I need to go after. Maybe that’s what Aristotle meant by “hope” being “…the dream of a waking man.”

Our “hope” is cures for mitochondrial diseases and in order to make “hope” more than a dream we need to increase our “doers” and focus their “doing” on the right path(s).

Success in reaching our goals comes from preventing our “doing” from getting diluted while still addressing the many needs of our patient, allied health, medical and scientific communities.  I think my “hope” is also to have focused “doers.”

HOPE WITH FOCUS

“If you chase two rabbits, both will escape.” 

So once we have doers we can focus. But what is our focus?  Mission is the strategic goal, the “why” that defines the reason we exist, but mission fails to define the “how.”  We need to define the “how” by adding focus and we can do that by developing very specific ambition statements that will lay the roadmap to mission fulfillment.

HOPE WITH ACTION

“Vision without action is a daydream. Action without vision is a nightmare.”

Japanese Proverb

Strategic planning interjects action into our vision; it’s the electric charge that transforms ADP to ATP that fuels our cells.  When we lose sight of the strategic goals, if they get short-circuited and diluted, we lose power and momentum, and when we lose power and momentum we lose sight of the “why.”

Sep 4 11

Right or Left Handed Mitochondria?

by chuck

If you are right handed, you will tend to chew your food on the right side of your mouth. If you are left handed, you will tend to chew your food on the left side of your mouth.

The tooth is the only part of the human body that cannot heal itself.

And last but  not least and I don’t think this has anything to do with mitochondria:
In 2011,  July has 5 Fridays, 5 Saturdays, and 5 Sundays. This apparently happens once every 823 years!  This is called ‘money bags’.  So send this on to 5 and money will arrive in 5 days, but you must donate it to UMDF research!

Sep 4 11

Perspective

by chuck

So, my brother thinks I’m taking this biking a bit too seriously and sent me the following story of why he rides. 

Taken from the May/June 1989 Utne Reader, which took this from Shawn Gosieski, New Cyclist, Fall 1988. (and it has come in from other sources.)

A Zen teacher saw five of his students returning from the market, riding their bicycles. When they arrived at the monastery and had dismounted, the teacher asked the students, “Why are you riding your bicycles?”

The first student replied, “The bicycle is carrying the sack of potatoes. I am glad that I do not have to carry them on my back!” The teacher praised the first student, “You are a smart boy! When you grow old, you will not walk hunched over like I do.”

The second student replied, “I love to watch the trees and fields pass by as I roll down the path!” The teacher commended the second student, “Your eyes are open, and you see the world.”

The third student replied, “When I ride my bicycle, I am content to chant nam myoho renge kyo.” The teacher gave praise to the third student, “Your mind will roll with the ease of a newly trued wheel.”

The fourth student replied, “Riding my bicycle, I live in harmony with all sentient beings.” The teacher was pleased, and said to the fourth student, “You are riding on the golden path of non-harming.”

The fifth student replied, “I ride my bicycle to ride my bicycle.” The teacher sat at the feet of the fifth student and said, “I am your student!”

Most of the content present in the blog has been aggregated from various sources & may not have been penned by me. The credit and copyright for the same rests with the original owner of the article / video / photo etc. I have received these contents from various sources over the period of time and it is possible that I might have missed to quote the original source of the content in some of my blog content. If you are the original author and the credit has not been given, or you want me to delete your content from this blog, please send me a note with relevant details and I will do the same.

Aug 21 11

LOOKING FOR THE ELECTRIC STARTER – MILE 4

by chuck
 PEDDLING RUNNING TOWARD THE CURE!

 Made it to the park yesterday and was able to get 8.25 more miles under my belt.  I’m guessing that brings me to about 70 total miles to date.  Yes, I know this is minimal and many of you do that, and more, in one day. It’s taking me longer to understand how to use those 23 gears as well as get used to that skinny hard seat!  I look at my Harley next to my bike with that beautifully padded air seat and back rest and wonder…then a bit of Zen takes over and I grab the bike(cycle).

By the way, remember that hill I had to walk the bike up in “Mile 2”; no more walking! 

I am still trying to figure out whether getting into shape gets harder and slower as you age, or is it that at a certain age physical deterioration slows down as you exercise? And at what age is that tipping point?  I think the “biological clock” should be re-titled the “mitochondrial clock.” 

Only “Mile 4” and already you may think I have replaced my newly discovered mechanism of propulsion, peddling, with running.  Not so, I haven’t lost interest and I sure as heck have not decided to pound the pavement in lieu of pounding the peddles.  I have, however, befriended an outstanding runner; an athlete with impact dedicated to a cause. 

Evan was born a happy, healthy baby and by 2008 was an energetic four-year-old when out of the clear blue he began complaining of a sore stomach and was hospitalized for appendicitis. Seizures, brain impairment, and quadriplegia soon followed. About a year later, now in a wheelchair, Evan has trouble producing enough energy to hold his head up or digest food, and needs constant care. He was finally diagnosed with mitochondrial disease. 

Evan’s parents, Blaine and Sarah, had a choice; to become part of the cure and control the disease or to become another victim of mitochondrial disease and allow it to control them?

Evan pulling Dad across one of many finish lines

Evan pulling Dad across one of many finish lines

Blaine, a long time cross country skier, stepped out of his ski boots and into running shoes and now qualifies as an Ultra-Marathoner. He’s not your typical marathoner and you can usually pick him out in the pack. He’s the one running with his son, Evan, in his pushchair. 

Evan and Blaine recently competed in the “Stampede,” a 1/2 marathon in Calgary. Blaine told me he pushed Evan to a 3rd overall finish out of approximately 500 runners in a time of 1:19.  I think it was really Evan who “pulled” his Dad across the finish line! 

Some of Blaine’s accomplishments:

He was the 2010 Canadian 50 Mile Ultra Marathon Nation Champion

This past Saturday Blaine won the Iron Legs 50 Mile Ultra Marathon (2nd year in a row)

Place 9th in the Canadian Marathon Championships at Ottawa Marathon in May in a time of 2:34

Blaine also controls the disease by taking on the role of President of “Mito Canada,” UMDF’s counterpart in North America. 

Blaine says, “As you can see, running is my therapy and way cheaper than a psychologist!”  (I have a sign in my office that reads, “You Never See A Harley Parked in Front of a Psychologist’s Office.”)

Blaine’s update on Evan – “He is stable and has been for about 2 years now. (He got sick 3.5 years ago). The key things for Evan right now are on-going IVIG treatments (which really seem to help his seizures), and he is working with’eye gaze’ technology as a form of communication. He has some physical challenges as he grows combined with high tone – we are looking at installing a Baclofen pump possibly, but Evan will require surgery and botoxin his legs to prevent further dislocation. He also is going to have surgery to close off some of his saliva glands to help withhis drooling. Other than that, he has been getting out with us as a family quite a bit and enjoying the fresh summer mountain air as much as possible.”

Remember: Life isn’t a race, it’s a marathon- Keep on peddling! (and running!)

See you again at the end of mile 5.

Aug 21 11

The Worlds Tiniest Motor Making the Worlds Most Valuable Product

by chuck
  • Complex Complex 5 is the worlds tiniest motor that has a rotor that spins 10,000 revolutions/min and produces 3 ATP molecules every revolution. (And you thought what your heard in a sea shell was the ocean).
  • There are 633,000,000,000,000,000,000,000 (633 Sixtillion) molecules of ATP (633. x 1021 in a teaspoon of sugar which will keep an adult alive, at rest, for 20 minutes.
  • Divide this by 20 to get 31.6 Sixtillion (1021  ) ATP to keep the body alive at rest for one minute.
  • Divide this by 60 to get 530 Quintillin (1018 )ATP to keep the body alive at rest for one second.
  • Divide this by 10 trillion cells in the body to get 53 Million (106 ) molecules of ATP necessary to keep each cell alive for one second.

 

Worlds Tiniest Motor

Worlds Tiniest Motor

All this output from the tiniest motor in the world; the rotor in Complex V. And you wonder why a body with mitochondrial dysfunction crashes! Anybody feeling a bit tired?

As if you need any more proof of the importance of ATP – Cyanide can kill in 30 seconds by stopping ATP production, thus the importance of ATP (the body’s energy currency) in the body.

Aug 21 11

Interesting Thought

by chuck

Exxon is now advertising that their gas works on our engines molecular level.  Exxon and Mobil have engineered gasoline on the molecular level to help clean up intake valves, so it can perform its best.  So.. if Exxon Mobil can get gas to enhance work at the molecular level, could we get them to help us enhance our energy production at the mitochondrial level? 

Let’s see if we can develop a logicl path to follow… STP (Specially Treated Petroleum) treatment for the gas increasing efficiency and mileage, added to this newly engineered gasoline that works on the molecular level – if only we could re-formulate STP to be added to ATP (Adenosine Tri-Phosphate) increasing mitochondrial efficiency….

Maybe, just maybe if we had Exxon Mobil’s budget we could… Just a thought!

Aug 8 11

Looking For the Electric Starter–Mile 3

by chuck

“Unless someone like you cares a whole awful lot, nothing is going to get better. It’s not. ~Dr. Seuss

I just returned from Ukiah California so no biking this weekend. Ukiah is a small town of about 15,000 folks situated about 2.5 hours north of San Francisco and six hours south of Oregon. The City is nestled in the Yokayo valley in southern Mendocino County. This would be a great landscape to see and feel on a bike.

I meet UMDF Trustee, Linda Cooper, in San Francisco. Linda lives in LA and thought it would be a great opportunity to represent the UMDF Board of Trustees. We get the rental car and head North on 101.

2.5 hours later a wooden sign welcomes us to Ukiah. As we pull onto the pitted, sparsely paved and dusty road I feel as though I never left Western PA.

We come to a “T” in the road and are greeted by huge ornate gates and a sign declaring this the entrance to “The City of Ten Thousand Buddhas.” Yep, out of nowhere is an international Buddhist community and monastery founded by the Venerable Master Hsuan Hua, an important figure in Western Buddhism. It is one of the first Chinese Zen Buddhist temples in the United States, and one of the largest Buddhist communities in the Western Hemisphere. Talk about Zen! I wonder if there’s any motorcycle maintenance taking place on the property.

We turn left.

Through the dust I point to the left towards the 40 acres of Zinfandel grapes which are grown organically on the Gibson Ranch. The ranch is east of the city and situated between two mountain ranges. You get a beautiful view regardless of which end of the table you’re sitting.

So why Ukiah? It’s the last wine and BBQ fundraiser for UMDF in memory of Norma’s daughter Heidi, who lost her battle with mitochondrial disease ten years ago in 2001. We wanted to take this opportunity to thank Norma and her supporters for the impact they have had on our quest toward a cure. We wanted them to know that their event has helped UMDF grow from its beginning in 1995 with 85 members to what it is today.

The guests begin to arrive around 5pm. The majority represent our generation (I’ll let you decide exactly what generation that is) with one large table occupied by Norma’s children, Michele, Tamie, Devin and their friends, all in their late 20’s and 30’s.

I always wonder what really motivates people to keep coming back to support a cause, especially our cause. It could be the wine, the food, or the wine, the auction items, or the wine, and the friendships which seem to increase with the wine. All are reasons for the continued support year after year, but those are really tangential to what the basic common denominator is that brings everyone here this evening.

A very famous doctor once said, “Unless someone like you cares a whole awful lot, nothing is going to get better. It’s not. ~Dr. Seuss

They’re all here because they all care; they care a whole awful lot!

Because of the Gibson BBQ supporters and the many others who support a whole lot of BBQ’s, UMDF has grown:

  • To provide support to over 65 Chapters and Groups across the United States
  • Developed a mailing list of members and supporters of over 60k
  • Has provided support for the formation of Mito Canada and The Australian Mitochondrial Disease Foundation.
  • Is a founding member of the International Mitochondrial Patients Association – engaging the European countries of:
    • Belgium
    • Italy
    • Spain
    • Germany
    • The Netherlands
    • France
  • UMDF has been able to support over $8M of research making it the largest non-governmental supporter of primary mitochondrial research in the world.
  • And, this year, awarded its first UMDF Clinical Fellowship to support training to a physician in the diagnosis and treatment of mitochondrial disease for an additional 2 years.
  • UMDF Is the only patient advocacy member of the North American Mitochondrial Disease Consortium funded by the NIH.

All this because of a BBQ! A BBQ in memory of a young lady named Heidi.

Lance Armstrong, 7 time winner of the Tour De France, in his book, “It’s Not About the Bike” says, “Pain is temporary. It may last a minute, or an hour, or a day, or a year, but eventually it will subside and something else will take its place. If I quit, however, it lasts forever. In my most painful moments on the bike, I am at my most curious, and I wonder each and every time how I will respond. Will I discover my innermost weakness, or will I seek out my innermost strength?

Norma and her family have chosen to seek out their inner most strength. Everyone attending this BBQ is helping turn a tragedy into a tribute and a tribute into a triumph!

We never know what direction our lives will ultimately take. John Lennon said, “Life is what happens when were busy making other plans.”

My wife, Adrienne, and I are normal people. We met when we were both teaching at a local high school. I quit teaching and went into the restaurant business when one day, in the middle of my 80hr workweek expanding my business, Adrienne called and said, “you better come home, Gina is very sick.” Sometimes a lightbulb goes off and somtimes you need hit in the head with a 2×4! The call was the 2×4.

Gina made the honor role in 7th grade, was too sick to finish 8th grade and died before having a chance to enter 9th grade. But man oh man those short 15 years have had a greater impact than many who live to a ripe old age. You know those folks – the ones that coast through life not wanting to get “involved.” The ones that don’t register to vote because it could get them called for jury duty. The ones that could care less about what happens “over there,” until, of course, when “over there” creeps into ”here” and “here” gets “near” their backyard. They’re the ones who tip toe through the years and in the end leave little if any print on humanity or a sign that they ever existed.

So, how do we respond to these unexpected roadblocks that life sends us when we’re busy planning other things? Do they cause us to stop dead in our tracks, or do they encourage and motivate us to continue forward – and in what direction do they cause us to go? Do the roadblocks cause us to discover our innermost weakness, or do they help us seek out our innermost strength?

I first met Heidi in 1997 and from that point probably talked with her 2 to 3 times a week. Each time Heidi would call she would always ask, “How are you doing?” At that moment I would be thinking, “Girl if you only knew.” “I have a pile of work on my desk that I am already a week behind and it will take me another week to get completed which will leave me two weeks behind, I had to fire another manager last week, my back is killing me and Adrienne wants the bedroom painted…”

I would catch myself because in that moment I realized who was calling me. It was Heidi. Heidi, who was diagnosed with MELAS, a terrible mitochondrial disease. Heidi, who suffered from terrible migraines, poor vision, ataxia, poor speech and muscle coordination and needed a walker to get around. And Heidi knew she was not going to get better and understood she would get worse; asking me how I was doing!

In that moment, because of Heidi, I was doing great! I didn’t have a problem in the world. It’s the Heidi’s and the Gina’s that are responsible for getting us up an hour earlier when we know we could sleep and hour longer, helping us keep our problems, aches and pains, in perspective. Heidi and Gina encourages me to go fishing with my son every Sunday and when he reaches out to shake my hand they convince me to grab his hand, pull him in and give him a hug and kiss. Not an easy thing to do with a 28 year old Rugby player! They teach thousands how to be part of the cure and not a victim of the disease. They teach us that it is our ATTITUDE toward a disease or problem that is stronger than the facts of the disease or problem.

Even though mitochondrial disease took their bodies it did not take their attitude, and their attitude would not let it take their spirit and who they were; they were stronger and bigger than the disease and because of that they still “are.”

Writer, Ben Holden, tells the following story about a weightlifter -

When asked the secret of his winning ways, a champion weight lifter replied, “I try not to think of the weight, just the lift. Once you start thinking about the weight, you won’t get it.”

What an insight into life’s problems, its perplexities, and its burdens! We can worry about the problem, or we can focus on possible solutions. We can dwell on our difficulties, or we can zero in on ways to overcome them. We can see only the lack in our lives, or we can open our eyes to the abundance and to the source of our supply.

The successful weight lifter realizes that there is something more powerful than the dead weights; there is his lifting power readily available within him. He knows that there is something far more important than the facts; there is his attitude toward the facts.

Whatever problem confronts you, whatever burden threatens to overpower you, whatever temptation seems to have domination over you, whatever crises presently engulfs you, remember this: Get your mind off the weight, and turn your attention to the lift!

How do we respond to these unexpected roadblocks that life sends us while we’re busy planning other things? Do they cause us to stop dead in our tracks, or do they encourage and motivate us to continue forward – and in what direction do they cause us to go?

Well Heidi, Gina and many other children, teenagers and adults are turning into tremendous weightlifters!

The proof is in the thousands of people attending 100’s of BBQ’s. And the fact that the reason this is Norma’s last BBQ is because she and her family has decided to turn their attention to leading a UMDF Energy For Life Walk (EFL) in San Francisco. By joining many others across the United States, Norma, along with her family and friends, will continue to promote Heidi’s positive attitude by looking forward and not backwards. They are part of the cure.

Because of our UMDF supporters, Heidi, Gina, other children, teenagers and adults battling daily against mitochondrial disease continues to have a positive impact on tens of thousands from all over the world. So think of them next time the alarm goes off and you want to sneak another hour of sleep or when you find yourself complaining about the amount of work you have to do. Think of them when you begin to complain about the aches and pains you have or when the kids are consuming your time with their needs and wants and driving you crazy. Think of those who will never have a family of their own and the great opportunity to complain about them, and the next time your son or daughter, mother or father reach for your hand to shake it, grab it, pull them in and give them a hug and a kiss. If they ask what’s up, tell them Heidi or Gina made you do it!

And, of course, think of them the next time your wife asks you to paint the bedroom.

Well, these trips continue to help me make necessary adjustments to my bike that I know will help me peddle up the next hill. I wonder if my travels near “The City of Ten Thousand Buddhas” had something to do with…hmmm, I wonder if being that close could I have absorb enough Zen to “be more of the bike.” Can the Zen force be that strong?

Keep it up on two wheels and remember; you’ll only fall over if you stop peddling.

Aug 8 11

Get Your Mind Off the Weight!

by chuck

When asked the secret of his winning ways, a champion weight lifter replied, “I try not to think of the weight, just the lift.  Once you start thinking about the weight, you won’t get it.”

What an insight into life’s problems, its perplexities, and its burdens! We can worry about the problem, or we can focus on possible solutions.  We can dwell on our difficulties, or we can zero in on ways to overcome them.  We can see only the lack in our lives, or we can open our eyes to the abundance and to the source of our supply.

The successful weight lifter realizes that there is something more powerful than the dead weights; there is his lifting power readily available within him.  He knows that there is something far more important than the facts; there is his attitude toward the facts.

Whatever problem confronts you, whatever burden threatens to overpower you, whatever temptation seems to have domination over you, whatever crises presently engulfs you, remember this:  Get your mind off the weight, and turn your attention to the lift!

Ben Holden

Aug 2 11

Looking For the Electric Starter–Mile 2

by chuck
“The study of the art of motorcycle maintenance is really a miniature study of the art of rationality itself. Working on a motorcycle, working well, caring, is to become part of a process, to achieve an inner peace of mind. The motorcycle is primarily a mental phenomenon.”      – Robert M. Pirsig

 

So, as I grow older with my motorcycles I find myself substituting hard shocks and chrome with gel pads under my seat, a back rest and soft hand grips.  Doesn’t the ride always seem a bit longer and harder even though age provides you with a bit more “natural” padding?

I finally have the seat height adjusted properly so my legs don’t lock on the down stroke.  Peddling is now easier and more efficient.  Getting on and off will take a few more miles of getting use to before it feels, and more importantly looks, graceful and natural.

11.3 miles yesterday at 6.13 minutes/mile, (so says my iMapMyWalk app on my iPhone fastened on my handlebars – no Zen here) and that included one very slow peddle and two walks up the same hill.  I told my brother Bob, a long time avid cyclist, how pathetically out of shape I am to have to walk the bike up the long but low grade. What I didn’t tell him was how embarrassing it was. Thank goodness for helmets that can help protect your pride as well as your body.  Bob told me that he and his wife, Mary Beth, just finished Bike Virginia; 5 days of riding in the mountains of Virginia.  He said a fellow rider told him, as they were walking their bikes up a mountain during Bike Virginia, “I’ve never met a hill I couldn’t walk up.”

I’ll remember that the next time I’m facing a hill. My mitochondria can produce enough ATP enabling me to peddle or walk, and one way or the other I can get to the top.

Bob also told me to always remember; “you really don’t “ride” the bike, your “are” the bike and together become the most efficient machine created my man.”  I guess he also read Robert Persig’s book and peddles with a bit of Zen.

Keep it up on both wheels and remember to keep peddling, but if the hill gets a bit too steep or long, get off and walk, the top is only a few more steps away.

Jul 29 11

ARE YOU RELATED MORE TO YOUR MOTHER OR YOUR FATHER?

by chuck
  • Most healthy people have homoplasmic cells — Each cell has normal mitochondrial DNA.
  • People with mitochondrial DNA mutations have heteroplasmic cells — Each cell has a mixture of good and bad mitochondria

     

     

A biologist’s song to his mother – http://youtu.be/osWuWjbeO-Y

MATERNAL INHERITANCE OF MITOCHONDRIAL DNA MUTATIONS

MATERNAL INHERITANCE OF MITOCHONDRIAL DNA MUTATIONS