Thursday, February 28, 2013

Thank You!!!

Yesterday,  I received the most amazing, thoughtful gift.  Becky sent me a photo of this gift while I was at work and I thought it was a cake.  I thought, awesome.  I'll stop by the grocery store on the way home and buy some ice cream.  I forgot the ice cream, but didn't forget about the gift.

Moby Dick

This is Moby Dick.  This is literally Moby Dick and the Pequod made from the pages of Moby Dick.   This is the Great White Whale and the waves of the high seas as he is swimming next to the Pequod.  This gift represents the gift we were given by TGen as I described in my post Moby Dick has been spotted...the chase is on.  The gift of a genetic diagnosis for my family.  This gift from TGen is priceless!  This gift from Karl and Chrysta Richards is priceless!  This gift will go along side our symbolic Pequod Seth made when he was in pre-school.  This was made and gifted by Jodi Harvey-Brown and shipped from Pennsylvania.  Beautiful, amazing, thoughtfull...words cannot describe what this means to me.

Our symbolic Pequod
People have asked me countless times:  "I don't know how you guys do it?"  Well, some times I don't either.  But this much I do know.  There is no way in hell we could do this alone.   Yesterday is only one example of several people who have gone out of their way to share our burden.  It's people like Chrysta who are so thoughtful.  It's people like Sharron who sent my blog to everyone in her contact list and made up flyers of Team Belnap so she can hand them to people.  It's people like my co-worker Lacey who asked me if I would help her write a research paper for her biology class on mitochondria (I am so excited to help her and educate her biology teacher).   It's people like Jodi who donated this gift to us after she read our story.

It's people like my co-workers who are all wearing the Jeans for Genes, Wear that you care ribbons today at work.  It's people like Heidi who have helped us get Team Belnap up and running.  It's people who have donated or shared Team Belnap with others.  It's people who have shared our story.  It's people like my friend Jason who sent me a thoughtful email of encouragement.  It's people who have supported us publicly and privately.

Today is Rare Disease Day and Becky are privileged to go to TGen again for some special, long-awaited events.  Rare Disease Day  will be celebrated all across the country.  Today, in honor of this day, TGen will release a video about our family highlighting the progress they have made with rare diseases and our family.  Today,  we will meet with other parents at TGen.  Becky and I have been asked to be on TGen's Parent's Advisory Council.   Today is a special day.

Today, my heart is full of love and gratitude.  Thank you Chrysta and Karl!  Thank you to everyone!

Tuesday, February 26, 2013

Team Belnap: We swim, bike, and run because they can't

In the fall of 2010, when Seth's condition started to significantly deteriorate, I was training for the Tour of the White Mountains. This is a mountain bike race that I love and have done several times. I raced the 35 mile course.  I decided I was going to dedicate this race to my son Seth.  I only told my wife my plans. I had the motto: I race, because he can't. There was something different about this race.  There was something special about this race.  This was a race not about me, it was about Seth.  I put on my headphones and road harder than I ever have before.

Exhaustion, muscle cramping, thirst, mental fatigue all were omnipresent and very frequent.  Those of you who have done endurance events have heard about "hitting a wall".  I hit this wall, but rebounded like a racquetball.  As these feelings came, I would think of my sweet little boy Seth and I would ride harder and continue on.  I took no breaks and the only time I got off my bike was when I crashed into a pile of lava rock.  Ouch!  I finished in the top 10 with a time of 03:14:23.86

Me in Tour of White Mountains 2010
The following year, Sydney was officially diagnosed with Mitochondrial Disease and I decided to change my motto from I race because HE can't to I race because THEY can't.  I told Becky and Spencer my motto and they joined me in our silent cause to race for Sydney and Seth.  The motto then became: WE race because THEY can't.   Becky ran the Moab Half Marathon, Phoenix Half Marathon, Ogden Marathon, and the St. George Marathon with this motto.  In 2011, I raced in Ironhorse, Bike the Bluff, and The Tour of the White Mountains.

Becky and her dad after Deseret News 10K
Spencer was involved in soccer and track and was an amazing athlete.  I remember telling Spencer before soccer games and track meets to play this game for your little brother.  Run this race for your sister.  Spencer was an amazing athlete.  He had the endurance of a Tarahumaran and could accelerate like a cheetah.  In 2011, Spencer finished in 3rd place with a podium finish in the 15 mile Tour of the White Mountains race with a time of 01:30:23.  He won many of his track meets in the 800 and 1600 meters.

Spencer 2011 Tour of White Mountains

Me 2011 Tour of White Mountains

Things were going well for us.  Sydney and Seth were stable and Spencer, Becky, and I were all running/racing/playing for our silent cause:  Because they can't.  Then, we were broadsided by Spencer's diagnosis.  Our lives have been chaos and unsettling.  Becky and I have not raced since Spencer's diagnosis.  I've had a hard time even getting on the bike or running on the trails.

One of the worst days on this journey is when I had to tell Spencer he could not race anymore.  I was on my way to his first track meet of the season in Winslow, AZ.  I had just gotten off the phone with Maureen at Dr. Shoffner's office.  They had discussed Spencer's case and recommended he not participate in any competitive sports until he was seen by Dr. Shoffner.  Spencer was suited up for his track meet.  I was about 30 minutes away from the track meet and his event would start in 45 minutes.

I had to tell Spencer that he could not race.  I knew the news would devastate him.  I knew how much he loved to run and compete.  Now, I had to tell him he could not race for his little brother or his big sister anymore.  Tears filled my eyes knowing what Spencer was about to go through.  As I scrambled  to find Spencer at the track meet, I pulled him to the end of the bleachers and with tears in my eyes told him that Dr. Shoffner was highly suspicious of him also having a form of Mitochondrial Disease and it was best if he did not run or compete in any sporting events at this time.  I hope I never have to ever do something like that again.  It was so painful.  Spencer was running for his brother and sister because they can't, and now he was to be included with those who can't.

Seth posing with some of Spencer's awards he received in soccer, track, and mountain biking.  Spencer would race/play in honor of Seth.  
So, for those of you who have joined Team Belnap, you are running because Spencer can't.  You are fulfilling part of his dream.  Run like hell.  Run for him.  I can guarantee you that Spencer would love to run this race because they can't.  Spencer would love to strap on his shin guards and play because they can't.  Spencer would love to clip into his mountain bike pedals and ride because they can't, but Spencer can't!

There is something special about racing for someone else.  There is something special about racing for a cause that makes the race more meaningful.  It's time to change our silent motto to a loud scream that can be heard across the country.  It's time for me to lace up my trail running shoes again.  It's time for me to clip into my mountain or road bike and race because they can't.  Thank you for all who have supported Team Belnap:  We swim, bike, and run because they can't.

Monday, February 25, 2013

Does Hope Really Fly?

I remember the first time I saw a firefly.  I was about 12 years old and was with my cousin and dear friend, Karl in Virginia.  I was fascinated by these creatures that could fly and light up the evening sky.  These creatures seemed to have a mystical almost magical power about them.  They seemed to be something more from a Star Wars movie not from the Kingdom: Animalia, Phylum:  Arthopoda,  Class:  Insecta...

Nature has some amazing fascinating processes, but an insect
that has the ability to produce light?  This bioluminescence was beyond the ability of comprehension for my young mind and seemed to be more science fiction than science.  Little did I know what the importance of this firefly would have on my future life.

Seth waiting for the bus

The biochemistry and biophysics of these beetles are truly amazing. Illumination with an incandescent light bulb produces a lot of heat and are not very efficient with 90% of the energy given off as heat.  If fireflies were this inefficient, they would self incinerate.

With fireflies, 100% of the energy is converted to produce light.  Light is a way they communicate with each other.  How does an insect bioilluminate?  It's all about the mitochondria converting energy into a usable energy source called ATP that runs an enzyme called luciferase.  This enzyme acts on luciferin to produce light.

So, why all the fuss about a flying firefly?  Well, there is some promising research going on with these pre-historic beetles and the enzyme luciferase that will potentially benefit mitochondrial disease.  This research is being conducted by Dr. John Shoffner and his group at Medical Neurogenetics.  This is our Mitochondrial Disease specialist.  He has created a foundation to help support research and increase awareness called the Foundation for Mitochondrial Medicine.  There are a lot of great organizations out there ( & who are doing research and raising awareness.  Our family has decided to devote our time and talents toward two foundations because this is where our doctors are:   Foundation for Mitochondrial Medicine and TGen's Center for Rare Childhood Disorders (C4RCD)

Sweet Tri Triathlon with TeamBelnap

So...does hope really fly?  Four months ago, the answer was NO!  Hope was a principle that seemed illusive.  It seemed we were living during the Winter of 1776 and we were a member of the Continental Army camped at Valley Forge.  A cold and lonely Winter lacking provisions, food, and shelter.  We were accustomed to defeat.

Here is a summary of the incredible story of Valley Forge in 1776:

During one of the lowest points for General Washington and his soldiers, Thomas Paine was sick in his heart over the suffering and despair he saw.  He wrote a pamphlet called the Crisis. He sent it to General Washington who read it to his troops. This is what it said:   “These are the times that try men’s souls. The summer soldier and the sunshine patriot will, in this crisis, shrink from the service of their country; but he that stands by it now, deserves the love and thanks of man and woman. Tyranny, like hell, is not easily conquered’ yet we have this consolation with us, that the harder the conflict, the more glorious the triumph. What we obtain to cheap we esteem to lightly.”

General Washington took action.  He had a bold and courageous plan. On December 25, 1776, George Washington and his army crossed the icy Delaware River at night in a snow storm, marched 9 miles in the dark during this snow storm to the city of Trenton, NJ and made a surprise attack.  With God on his side, he was victorious at the battle of Trenton, and within 2 weeks, 15,000 soldiers enlisted in the army. This was a turning point in the war

Our lives have been completely transformed by Mitochondrial Disease. We eat, drink, sleep, walk and talk this disease. I have been told we have a story to share--"a powerful story". I wish I was on the other side of the story. I wish I was the one reading the story, not telling it.

December 2012 was a fantastic and monumental month for our family.  First came OUR Thomas Paine and The Crisis moment when we threw our story to the world with the publication of OUR video called Our Mitochondrial Disease Journey.  This was followed by OUR victory at Trenton when the genetic basis of mitochondrial disease was finally found in our family by the scientist at TGen.  This is one of OUR turning points in OUR war against Mitochondrial Disease!  Now it's time to enlist more in this war.  Now it's time to give our Thomas Paine speech to the world.

So... does hope does fly!  The answer today is a resounding yes!   HOPE FLIES in many ways.  Hope Flies with Team Belnap.  Join our cause!  Join this war on Mitochondrial Disease!   

Thursday, February 21, 2013

International Rare Disease Day--"The harpoon is not yet forged that ever will do that."

International Rare Disease Day.  Seth holding Blue Denim Genes Ribbons he gave to his bus driver and "bus driver's helper"

"It was the best of times, it was the worst of times," Charles Dickens writes in the opening lines of A Tale of Two Cities as he paints a picture of life in London and Paris that led up to the American and French Revolution.  One of the goals of this blog is to paint a picture of a day in the life of a dad, mom, son, daughter, or family living life with a chronic, incurable, rare disease called Mitochondrial Disease.  This mitochondrial disease journey has been filled with duality as we have had several days that fall into either category Dickens describes in A Tale of Two Cities. Tuesday, Feb 12th was one of those days that I would consider "the best of times."

It was a meeting I hope NO parent has to have.  It was a meeting I hope ALL parents are able to have that have children with a rare genetic disease.  A meeting so many parents that have children with rare genetic diseases are longing to have.  How fortunate my wife and I feel to have met with the scientist at TGen.

Tuesday, Feb 12th, we had a highly anticipated meeting with Dr. Narayanan, Dr. Craig, and Dr. Huentelman at TGen.  We discussed the details of the genetic origin of mitochondrial disease in our family.  We discussed the Center for Rare Childhood Disorders (C4RCD).

Dr. Huentelman, Dr. Narayanan, Dr. Craig (back row right to left)

We were able to discuss in more detail the gene and where we go from here.   I am so impressed with these three scientist and this company.  Their commitment to help families like ours is extraordinary.  My children were excited to meet these scientist whom they have been praying for.  Sierra (sitting on my lap) has been the one who prays almost every night for these scientist and others across the world to be able to find a cure

It's hard not to end this post without another analogy from Moby Dick.

In the final days of the Pequod's quest to find Moby Dick, the ship, by chance, meets up with two other whaling vessels who recently had encounters with The Great White Whale that...well...turned out well for the whale.  One ship called the Rachel and the other ship "most miserably misnamed the Delight."

Our kids holding "The secret of life" 

The author, Herman Melville, describes the scene when the Pequod meets the Delight, "Upon the stranger's shears were beheld the shattered, white ribs, and some few splintered planks, of what had once been a whale-boat; but you now saw through this wreck, as plainly as you see through the peeled, half-unhinged, and bleaching skeleton of a horse."

Captain Ahab yells out: "Hast seen the White Whale?"  The captain of the other ship cries out: "Look!, replied the hollow-cheeked captain from his taffrail; and with his trumpet he pointed to the wreck."  The Delight had seen the White Whale and suffered the loss of 6 men and catastrophic damage to the ship.

Ahab responds: "Hast killed him?"

The captain of The Delight responds most eloquently: "The harpoon is not yet forged that ever will do that."

Ahab replies:  "Not forged! and snatching Perth's levelled iron from the crotch, Ahab held it out, exclaiming--Look ye, Nantucketer; here in this hand I hold his death!  Tempered in blood, and tempered by lightning are these barbs; and I swear to temper them triply in that hot place behind the find, where the White Whale most feels his accursed life!"

The Rachel's encounter with Moby Dick also had fatal carnage as the outcome as the captain of the ship lost his son during the pursuit.  What a beautiful biblical metaphor the author uses here to name the ship--The Rachel.  This biblical reference is "Rachel weeping for her children" (Jeremiah 31: 15 and Matthew 2: 18).

February 28th is the 6th international Rare Disease Day.  Our family will be participating in this special day as we join with thousands of other families around the world to help find cures and raise awareness.  It is true that "The harpoon is not yet forged" to cure mitochondrial disease or other rare diseases, but significant progress is being made.  Our family's story is an example of the progress.

I see too many families who are like The Rachel or Delight and have been broken to pieces financially pursuing a diagnostic odyssey.   Death and/or divorce is all too common because of this disease.  I see too many green candles on Facebook which represents another child who has passed away from mitochondrial disease.

It will take more than a harpoon forged from iron to kill the Moby Dicks that plaque us families that have children who suffer with rare disorders.  It will take more than just one whaling boat named the Pequod.  It will take an army of fighting families motivated and dedicated to raise awareness.  It will take a navy with the greatest technology using sonar and GPS.

It will take cooperation and collaboration amongst the scientist.  It will take philanthropist and others to help raise funds for research.

Our family has pledged to do our part.  In the coming days, more will be announced of our specific plans.  I hope I can look back and say, we did our part.

James Watson and Francis Crick discovered the
structure of DNA (deoxyribonucleic acid) in 1953
Francis Crick declared: "We have discovered the
secret of life."
Sydney and Spencer standing in front of this original
DNA molecule in the London Science Museum.

Sydney and Spencer standing in front of this original DNA molecule in the London Science Museum.   Since that time, our genome has been put on one of these chips that a computer can read and sequence.  Absolutely amazing to me.

As I look closer at the above photo, I notice that everyone has small bites from their fingernails, except Seth.  I wonder if there is a gene for this behavior.  Thank you TGen.  Thank you all the scientist who are studying rare diseases.  Thank you to those who help us raise awareness.  Thank you to those who help us raise money to find a cure.

Saturday, February 2, 2013

Today I will eat Moby Dick

I have decided to post something a little different today.  I have a dear friend who has been with me on this journey from the beginning.  He owns a company here in the White Mountains called Alpha Oxy Med.  Each year, he has a weight loss competition.  It is a motivating force for many people to change their lives.  I have included a link to his blog below.  Check out his blog.  He writes some great stuff.  His latest post was the following:

Just saw this:
According to a recent study, 42% of Americans are doomed to become obese by 2030
I would love to hear your views as to why we, as a country are becoming fatter.

Email me, comment, let's hear it.

Alpha Oxy Med Blog and Weight Loss Competition

I used to have the web address of this blog  I chose that name because shame has been such a big part of my life for many years.  I recently changed the address as this blog has grown.  I have always wanted to post some thoughts on shame and fear.  This was the perfect opportunity.  The following is my response:

My Dear Friend Pedaldork, 

I've got a little to say on your post regarding why Americans are fat?  Ok, I have a lot to say.

I remember during the presidential debates in 2004 when George Bush was debating John Kerry, the moderator posed a question that went something like this:  What is more harmful to Americans, CO2 emissions from fossil fuels or high fructose corn syrup?

I thought to myself, ok Mr. President or Mr. Kerry, here's your chance to knock this one out of the park.  Here's your chance to wake up America and give a remark that will go down in the ages and be remembered for generations.  Here's your chance to have a Ronald Reagan moment when he responded to a question with the question: "Are you better off now than you were four years ago."

Well, neither men gave a good answer and as expected, Mr, Kerry went off on his hypocritical environmental views (as he's flown around on private jets).  Mr. President gave an answer that's not worthy of doing a Google search for.

The bottom line:  American are fat because of the quality and quantity of food we eat.  There is a deeper question that needs to be asked here:  Why do we eat so much and so poorly?

I believe the answer to this question is food can be our Moby Dick.   Let me explain.  We use food as a coping mechanism.  We use food to hide deeper problems.  We use food to comfort us.  We use food to run from our fear and shame.   Let me expound on this metaphor.

Moby Dick has become one of my traveling partners through this journey we call life.  For those of you not familiar with this great American novel, let me give a brief outline.

The book is narrated by Ishmael who decides to go on a whaling expedition.   "Call me Ishmael" is the famous opening line as Ishmael introduces himself to the reader.  Ishmael then gives the reasons he has decided to go on this voyage.  I believe Ishmael is despondent and wants to deal with the underlying reasons for his depression which I believe some are shame and fear.

The voyage turns out to be Ahab's (the captain of the ship) personal vendetta to find and kill Moby Dick, a large sperm whale.  On a previous whaling expedition, Moby Dick permanently disfigured Ahab and bit off his leg.  Ahab is a controversial character who is  "grand, ungodly, god-like," egocentric, persuasive, motivated, and full of fear and shame.   Captain Ahab's monomaniacal pursuit of Moby Dick eventually becomes the goal of the entire crew.   (Lance Armstong's monomaniacal pursuit of the Tour de France is a classic modern day example of Captian Ahab).

 The Great White Whale can represent many things to many people.  If you read my blog, you know I have compared Moby Dick to many things.  For this response, to my good friend Pedaldork's question, Moby Dick represents our shame and our fears.  Shame is toxic.  Shame is powerful.  Shame is painful.  Shame tells us we are unlovable and worthless.

Shame is an acquired emotion as we learn the expectations imposed upon us by others.  Shame is magnified as we develop expectations of ourselves.  When these expectations are not met, the emotion of shame is felt.

Shame pops its ugly head up in many different forms many times during the day.  Most of the time, shame is unrecognizable and is just below the level of consciousness.  When the expectations are not met, the powerful emotion is felt.  You feel worthless and unlovable.  You feel like...crap.  No one likes to feel this way, so you develop coping mechanisms in order to avoid the pain.  One of the ways people deal with this toxic emotion is by changing the way that you feel.  This can be done by a variety of ways, and eating is one of the most common toxic ways.

The reason eating changes the way you feel has to with the brain's reward system.  Activities like eating, drinking, and sex are examples of rewarding activities.  When these activities happen, they cause a neurotransmitter to be released in the brain called dopamine.  Dopamine is called the pleasure neurotransmitter.

When someone eats for comfort, the brain is flooded with this neurotransmitter and the feeling of comfort, well being, or euphoria is experienced.   Drugs, alcohol, gambling, and other addictions are other ways dopamine is released.  When someone takes methamphetamines, heroine, or prescription drugs the brain is flooded with dopamine just like it is when sex or eating occurs.  Think about this the next time you see someone who is obese.  Think about this the next time you see the alcoholic, drug addict, or sex addict.  Hopefully, you will have a little more compassion and understanding.  It's deeper than saying, just quit.

The challenge for us all is to deal with your shame and fear in healthy ways.  Don't be like Captain Ahab or Lance Armstrong (I admire Lance Armstrong for finally dealing with this toxic emotion).  Deal with your shame and fear.  Don't run from it.

Recently, Moby Dick has taken on a literal physical representation.  A sandwich.  There is a new Bakery in Show Low, AZ called the Sunshine Bakery & Deli.  This bakery has the slogan: "All natural.  All from scratch."  My wife showed me the menu this morning, and on the menu is Moby Dick.  I couldn't believe it.  I knew this must be a classy bakery and deli.  Albacore Tuna, carrots, celery, onions, pickles, mayo, lettuce, tomato, and sprouts.  I'll take that on the 9 grain bread.

Today I will deal with my fear and shame in healthy ways.  Today, I will not be like Jonah and get swallowed by a whale as I run from my fear and shame.      Today, I will swallow the whale.  Today, I will eat Moby Dick.