Farewell Friends & Shipmates: The Pequod is Changing Course

When Abraham Lincoln left Springfield, Illinois in 1861 to start his journey for Washington, D.C. and the presidency, he paid an unforgettable and emotional tribute to his friends and neighbors in what is known today as the Lincoln's Farewell Address. 

As Lincoln boarded this special presidential train, he recognized most of the people in the huge crowd gathered outside the train depot. Ahead of him awaited unimaginable and arduous trials.  Trials in all aspects of his life--family, marriage  professional, political, and the all important task to save the union.  Behind him would be many people who helped shape his character and taught him many principles that guided his life and allowed him to be so successful.

The crew of the Pequod in front of a statue
of a great man

"My friends -- No one, not in my situation, can appreciate my feeling of sadness at this parting. To this place, and the kindness of these people, I owe every thing. Here I have lived a quarter of a century, and have passed from a young to an old man. Here my children have been born, and one is buried. I now leave, not knowing when, or whether ever, I may return, with a task before me greater than that which rested upon Washington. Without the assistance of the Divine Being who ever attended him, I cannot succeed. With that assistance I cannot fail. Trusting in Him who can go with me, and remain with you and be every where for good, let us confidently hope that all will yet be well. To His care commending you, as I hope in your prayers you will commend me, I bid you an affectionate farewell."

Becky and I made the decision a few months ago to resign from my job of 10 years and pursue other career options.  It was a difficult decision to make.  

On the steps of the Lincoln Memorial with my 
co-captain of the Pequod.  The Washington
 Monument in the background

Since January of 2003, I have worked for Blue Ridge Regional Clinic.  In many ways, this has been the ideal job for me.  I work hard and have a lot of time off to play.  This lifestyle has suited me well for a long time.  When the unexpected and uninvited guest called mitochondrial disease (that I often call Moby Dick) came into our lives in 2008, things have never been the same.  This disease has completely transformed our lives and has given us a new perspective on life.

  

Honestly, the decision to resign was unexpected, but a necessary change as we continue to search for Moby Dick.  Ahead of me and my family is the persistent war on mitochondrial disease.  Each morning when our children awaken, we give them a handful of medications and supplements.  We have no idea if these medications and supplements (often called a mito cocktail) provide any benefit.  These are extremely expensive!  This serves as an omnipresent pervading reminder that my children have a chronic disease with no cure and no effective treatment.  I would like to change that!

I have often spoken of our journey as being on a ship in the open waters of the ocean in uncharted waters.  I use analogies from the book Moby Dick, in my writing.  Moby Dick is a Great White Whale and Captain Ahab and his crew are on an expedition to find and kill this whale.  

The ship the crew are sailing on is a three-masted whaling ship called the Pequod.  I have used the story of Moby Dick and the Pequod to help describe our family's odyssey  in the unchartered waters of mitochondrial disease (see YouTube video).  Over the past several months, I have climbed the masts looking for the best course.

Our symbolic Pequod.  Seth made this boat with his
pre-school teachers shortly after he was diagnosed
 with mitochondrial disease.  This picture hangs on
the wall in his room.

The sails have been set in a completely different direction.  I plan to use my talents and professional abilities to help fight this war on mitochondrial disease.  

Where this will ultimately be, I don't know.  As we make our way to our final destination, I will continue my work at Pineview Hospital and do some part time work in the Emergency Department at Summit Regional Medical Center.

There will be no huge crowds waiting to hear a speech as I depart, but in my mind, there is an audience of people and experiences over the past 10 years that have helped shape my life that I will be leaving behind.

Some in the audience are my co-workers and colleagues that I have worked with over the past ten years.  I love these good people!  I've spent countless hours with them.  We have experienced death, life, marriage, divorce, love, disease, joy, sorrow, and hope together.

Photo taken on 2-28-13, International Rare Disease Day.  
My coworkers all wearing ribbons in honor of my 3 children
 who suffer from rare mitochondrial diseases with no cure
 and no effective treatments.  Missing from photo are Dale,
 Shawnna, and Dr Paxman

I would be ungrateful if I did not pay tribute to these co-workers and colleagues that I can honestly call my shipmates and friends. 

These shipmates have been with me as we were hit with Seth's diagnosis, his progression of disease, his uncertainty, and now his thriving.  These shipmates have been with me as we were hit with Sydney's diagnosis.  These shipmates have been with me when Spencer was diagnosed in what could be called a category 5 hurricane that did severe damage to our ship and almost caused our ship to sink.  I will never forget the love and support that I have felt from them all.

On the steps of the Lincoln Memorial viewing the
Washington Monument

I would be ungrateful if I also did not thank my patients who put their trust in me to take care of them and their families.  I have been deeply touched by your kind goodbye/thank you notes and thoughtful words as we've said goodbye.  I have been moved by your generous donations towards mitochondrial disease research in honor of our children to The Belnap Children Mito Fund and to Team Belnap: we swim, bike, and run because they can't.

Family photo before mitochondrial disease entered our world

Spring 2013

One final thought before I end this post.  Unity and strength is the word that best describes these pictures taken by Paxman Photography.  This disease has caused our family to bond and as a result, we have become stronger.  As we continue sailing on this journey in search of a cure for mitochondrial disease, we are unified and strengthened by so many of you.  As our family looks to the horizon, uncertainty and fear are omnipresent in the way of storms, mechanical problems with our boat, illness of another shipmate, etc, etc, etc, but we have hope and will move forward with courage.

Saying goodbye is never easy for me (I think I have separation anxiety).  So...my friends, colleagues, co-workers, and patients who I can call shipmates, I say thank you and bid you an affectionate farewell until we meet again on this journey called life.

The Futile Disability Fight

"Call me [Newell]...whenever I find myself growing grim about the mouth; whenever it is a damp, drizzly November in my soul; whenever I find myself involuntarily pausing before coffin warehouses, and bringing up the rear of every funeral I meet; and especially whenever my hypos get such an upper hand of me, that it requires a strong moral principle to prevent me from deliberately stepping into the street, and methodically knocking people's hats off — then, I account it high time to [find some epic single track, strap my 29er Trek HiFi mountain bike onto my jeep and go mountain biking] as soon as I can." 1

                            

The timing of this "hypos" was perfect. My oldest daughter, Sydney, has been staying with family in Utah for the past few weeks and it was time for her to come home.  I met my mother and father in-law in Moab, Utah to pick her up.  For those of you who are familiar with Moab, Utah, you know that this town is considered a mountain biking mecca. 

It's a 6 hour drive from the White Mountains in Arizona to Moab, Utah.  I arrived late Sunday night and got up with the sunrise so I could get spend half of a day riding my mountain bike.  I have found this method of exercise a fantastic way to get the endorphin levels up to naturally increase my serotonin and dopamine levels. This is my alternative to anti-depressant medications, although there was a time in my life when I was on these.  

A joyful reunion with Sydney in Moab, Utah after she has been gone for 1 month

On the drive up here, I thought a lot about my oldest daughter as she enters a new phase of her life.  She successfully graduated from Blue Ridge High School this year.  This was no easy task for her, but she did it.  With her IEP (Individualized Education Plan) and with many wonderful special education teachers, she did it.  Sydney was graded a lot on effort.  I am so proud of Sydney for this accomplishment!  

Sydney receiving her diploma

As a father, you always want the best for your children.  You want them to be happy and successful.  It pangs you when your children are sad or when people make fun of them.  Unfortunately, because of Sydney's disabilities, she was made fun of and did not have a lot of friends.  To those people who were her friends and teachers who took a special interest in her (you know who you are), I will be forever grateful for you.  To those people who made fun of her and refused to help her, I pity you.

I will never forget the first time that someone told me of the severity of Sydney's  mental/cognitive problems.   It was a psychologist she was seeing when she was in the 3rd grade.  He told us that Sydney would be lucky if she could work at Taco Bell and wanted us to prepare her the best way we could.  I thought, how the hell can someone make this kind of assessment on a child so young.  I was in a state of disbelief and I refused to accept his assessment.  In reality, I was in a state of ignorance.

Fast forward to the 4th grade and her first science project.   I love these projects!  Those who know me know that I love science.  This was my first science project as a father and I was excited to say the least.  I (we) decided to study yeast.  Most of you know that yeast makes bread rise.   Yeast is similar to us in that we both require oxygen and sugar (glucose) to thrive and grow.  This is our main source of metabolism.

Sydney's yeast experiment

We decided to see if yeast could metabolize sugar substitutes for an energy source. We tested normal table sugar, Splenda, Equal, Aspartame, and no sugar for our control.   This was exciting.  My children saw the true nature of their father and I went from cool dad status to "geek" status.  We put warm water, yeast, and the potential energy source into a bottle and put a ballon on the top so we could catch the carbon dioxide that the yeast would produce as waste (similar to us and other multi-cellular animals).  Well, it was no surprise which balloon filled up the most and our hypothesis was supported by the data we observed.

But more importantly, I observed for the first time the severe learning disabilities my daughter had and I was truly sad!  My daughter is the hardest worker I know.   She tried so hard to get these simple concepts of biology, but she could not do it.    For the first time, I realized the psychologist may have been correct in her prognosis.  For the first time, I new that some permanent cognitive problems existed and that she would need significant help, but I had no idea to what extent and what the future would hold.

Fast forward to the present.  Because of the severity of her mitochondrial disease, it was strongly recommended by her special education teachers and her doctors to obtain legal guardianship and conservatorship over Sydney.  

We sought the services of an excellent attorney, Brendon Rogers, who helped us get this done.  We were in unchartered waters with this and he helped us every step of the way.  During this process, our attorney strongly encouraged us to apply for disability.  He encouraged this for a few reasons, but ultimately, this was in Sydney's best interest so that if something ever happened to us, she would be taken care of. 

The guardianship/conservatorship process was smooth and uneventful.  The judge did not question one thing.  Sydney's court appointed attorney and the private investigator who came to our home and interviewed us all did not question anything.  It was evident that, unfortunately, this was necessary.

The next step was getting her disability.  The law states if you have a condition that prevents you from obtaining gainful employment you qualify for disability benefits.  Intuitively, one would think that if the state has found it necessary that you need a legal guardian and conservator because of your mental and physical incapacities that obtaining gainful employment is also going to be a problem.  

We filled out the paperwork and sent in the required documentation to support her case.  We fully expected to be approved based on the recommendations by several of her doctors.  Well...Sydney was denied.  We were told she did not meet the criteria and that we could appeal her case which we did only to be denied a second time.  I realized that I was David fighting Goliath.  I was standing there in my tunic fighting Goliath with my sling in hand.  I reached into my pouch for the smooth stones to throw, and realized my pouch was full of sand.  Realizing this battle would not be won with sand, I went and hired an attorney to fight and hopefully win.  We go to court next month.  

Having children with disabilities is never easy.  But there is something about these children that makes them unique and very special in my eyes and the eyes of God.   Unfortunately, there seems to always be a fight to be fought.  Unfortunately, there are those who are mean and ignorant to those with disabilities.  I love you Sydney and will spend the rest of my life fighting for you and your disease. 

1. Moby Dick by Herman Melville, Chapter 1 

Help us get a mitochondrial disease specialist in Arizona!

Becky and I have learned about the possibility of having a mitochondrial disease specialist come to Arizona on a part time basis.  Not only is there this possibility, but it just so happens that the specialist that may come is, John Shoffner, MD, the doctor we see in Atlanta, GA.  This is colosal!  This is progress and a major step for us and the many other families who suffer from this disease.  I am always amazed that so few clinicians  and non-clinical people know about these diseases.  

Seth with Becky.  One of the many of his hospital stays

In order to make this happen, we need help.  We need people to write letters to Phoenix Children's Hospital.  You don't have to have mitochondrial disease to write a letter.  You can write this letter because you are our friends, or because you have read our story, or know another family who suffers from this disease.

I am encouraging anyone and everyone to read the letter Becky and I wrote to Phoenix Children's Hospital (which I have included in this post) and write an email or a letter to the contacts I have listed below.  We have chosen to send our letter my snail mail.  I get so many junk emails that I'm not sure what to read.  Thank you for considering this request.  

Joseph P. Remitera, Director Practice Operations

Phoenix Children's Hospital

1919 E. Thomas Rd. | Phoenix, AZ 85016

p (602) 933-0060 | f (602) 546-5243

www.phoenixchildrens.com | jremitera@phoenixchildrens.com

Robert L. Meyer, President and Chief Executive Officer

Phoenix Children's Hospital

1919 E. Thomas Rd. | Phoenix, AZ 85016

July 5, 2013

Joseph P. Remitera

Director Practice Operations

Phoenix Children’s Hospital

1919 E. Thomas Road

Phoenix, AZ  85016

Dear Mr. Remitera,

The purpose of this letter is to help you understand the need for a mitochondrial disease specialist.  We have recently spoken with John Shoffner, M.D. who is a mitochondrial disease specialist in Atlanta, GA., who told us there is a possibility that he may join Phoenix Children's Hospital on a part-time basis.  We wanted to write you personally and let you know how important this would be and that there is a dire need for a specialist in mitochondrial medicine.

We would like to give you some background about our family.  Three of our four children have mitochondrial disease.  This disease is extremely complicated and requires multiple specialist with a mitochondrial disease specialist leading the team.  Unfortunately, Arizona does not have one. 

In an effort to help you understand the importance and need for this type of a specialist, we would like to give you a summary of our experience at Phoenix Children's Hospital.  In December 2008, our two year old son, Seth, started to develop multiple problems in a different body systems.  He had developmental delays, chronic metabolic acidosis, severe problems with ambulating and coordination, cardiac problems, speech delay, vision problems, and "cysts" on his brain MRI.  It was thought that Seth would benefit from being admitted to a multidisciplinary hospital to try and find out what was wrong and to get a diagnosis.

Seth was admitted to PCH in the winter of 2009.  We will not go into the details of every problem we had (we sent Mr. Meyer, CEO of PCH, a letter a few years ago about the details of our experience), but Seth's brain MRI was misread and he was misdiagnosed.  The outward appearance of Seth was a happy two-year-old little boy.  The image of a happy little boy was the picture the doctors appeared to only see, but inside, a terrible, fatal, neurodegenerative disease process called Leigh’s syndrome was beginning.   We were delayed over 2 years in getting a diagnosis and treatment for our son because of the unacceptable experience we had at PCH.

I am a physician assistant and have worked and trained in hospitals, including children's hospitals.  I was amazed how he was treated and must say that the attention given to his case by the doctors was not what I expected and was sub-standard. 

Seth's condition continued to deteriorate and we were referred to the Neurogenetics Clinic at St. Joseph's Hospital where we met Vinodh Narayanan, MD.  Dr. Narayanan saved Seth's life!  He told us that there was (and always was) a serious neurodegenerative condition causing Seth's problems.  Dr. Narayanan was highly suspicious of mitochondrial disease and referred us to Dr. John Shoffner, in Atlanta, GA.   We travel to Atlanta, GA  two times a year to see Dr. Shoffner. 

Some facts about mitochondrial disease from umdf.org and foundmm.org:

• One in 2,500 people have mitochondrial disease (children and adults)
• As many as 2 million people in the United States suffer from mitochondrial disease
• About one in 4,000 children in the United States will develop mitochondrial disease by the age of 10 years.
• 1,000 to 4,000 children per year in the United Sates are born with a type of mitochondrial disease.
• Mitochondrial disease has no cure and no effective treatment.  
• Mitochondrial dysfunction is related to other diseases such as Autism, Parkinson's, Alzheimer's, Lou Gehrig's, muscular dystrophy, type II diabetes, and many others

Some facts and statements about PCH as listed on phoenixchildrens.com:

• In 2012  "Phoenix Children’s had 19,286 inpatient admissions; 78,360 visits to the Emergency Department; 197,280 outpatient visits; and 15,424 surgical procedures.”
• "Phoenix Children's offers world-class care in over 70 sub-specialty fields of pediatric medicine."
• "Phoenix Children's is one of the ten largest children's hospitals in the country and provides specialty and sub-specialty inpatient, outpatient, trauma, and emergency care to patients throughout Arizona and other Southwestern states."
• "Phoenix Children’s Hospital is Arizona’s only licensed children’s hospital and among the largest freestanding facilities of its kind in the country."

You can do the math and realize that there are a lot of patients that are admitted to PCH that have mitochondrial disease.   There are a lot of patients in this state who suffer from this disease and are either misdiagnosed or haven't been diagnosed yet.  It has been my experience both professionally as a physician assistant and our experiences as parents of children with mitochondrial disease that the majority of health care providers have never heard of mitochondrial disease let alone know how to diagnose and treat this complex disease.  

Given the prevalence of this disease, taking into consideration Arizona's population in 2012 was 6.5 million, and considering all the above statements and facts the need for a mitochondrial disease specialist in a hospital as big as PCH is not only important, but greatly needed.  We have NO where to go but to other hospitals that have a mitochondrial disease specialist on staff.  Having Dr. Shoffner on staff at PCH would be a significant way to change this.  

We want to use our unacceptable experience with PCH not for spite or to point fingers, but as a learning experience, where all can grow and help provide the needed change so it doesn't happen again.  Unfortunately, our family’s story of being mis-diagnosed is not the only one.  Our hope is that PCH will see our experience as a way to better serve the children and families who suffer from this disease.

It is our hope that Phoenix Children's Hospital will see the importance and need for a mitochondrial disease specialist and will continue to move forward as expeditiously as possible the credentialing process and any other logistical matters so Dr. John Shoffner can come to PCH and help families like ours and hundreds of others who lack access to care and have to travel out of state for services.

Sincerely,

Newell & Rebecca Belnap

cc:  Robert L. Meyer

President and Chief Executive Officer, Phoenix Children's Hospital