Happy Birthday

And Thanks for the Inspiration

It’s raining outside. I can’t remember a recent February 16 or August 12 when it hasn’t which is fine because it reflects the mood inside our home.  Yesterday it snowed here in North Carolina, or I should say the weather gave its NC rendition of solid white precipitate. That is to say a ‘dusting’ for those North of the Mason-Dixon line; but a reason to close all the schools, massive wrecks and shortages of bread and milk here in the Triangle of NC.

So last night when I walked outside, the fog of transitioning weather took me back 17 years to what had been a crisp, clean and moments of anticipatory joy. The date was February 16, 1999 and not two weeks prior our family of three was gathered in an ultrasound room looking at images of the soon to be fourth member of Team Coleman.

There we three stared at grainy gray scale images of head, belly, arms and legs wriggling in a sea of love only a mother can provide.  As the technician pointed things out there were increasing levels of joy, wonder and love – there were ten fingers and ten toes, an appropriately developed brain, a heart with four working chambers.  Our little boy was going to be born perfect and without complication.  Well we were right on one hand and so wrong on the other. 

Evan MacGilvary Jenkins Coleman joined our family at around 9:00 am on a cold, crisp and clear blue morning via emergency C-section because he got caught in the jump rope of his umbilical cord and wrapped it around his neck twice. Over the last 24 hours this moment in time has been represented by the cold unrelenting sleet and ice that signaled the impending treachery and trouble that life can throw our way.

The Giant Congenital Melanocytic Nevus that was a birthmark of biblical proportions cover his back from his neck to the cleft of his glutes, and wrapped around both sides of his body to almost meet and join over his belly button.  The colors and textures were a physical representation of every known natural color of skin possible in the human race.

He was wearing his very own coat of many colors like Joseph of early biblical fame. As we each saw this beautiful baby boy and his birthmark we were thrown into a deep and dark fog with our only beacons being ‘experts’. What had been a moment of anticipatory joy had turned cold, cruel and painful as hospital staff, doctors and nurses approached us with carrying degrees of pity, sadness, remorse, fear and the glassy eyes of the unknown.  Last night as the fog settled in and the only light was cast by sodium vapor lamps glistening in a fog so thick you could barely make out the edges of the road; it was this pea soup that threw me back seventeen years to the beginning of a personal fog.

Over the next thirteen and a half years the fog ebbed and flowed as we experienced all the events and emotions of building and being a family.  There were house moves and vacation trips of lifetimes. There were school plays and favorite teachers, and then after eight of those years were done; the fog was met with the darkness of night as the shadow of the word melanoma hung heavy over our heads and lives.

Until the rain of this morning brought back the events three and a half years ago as a sadness enveloped our lives like a cold North Carolina rain, Evan died.  No matter how hard we had tried to get over, through or around the fog we saw no light or way out.  The empty space in our hearts has not been filled, the edges are a little less sharp, almost softened by time, but the dark black hole of dread and despair looms waiting for these days of remembrance.

This year in a testament to the power of ideas and legacy I will graduate school as a Physician Assistant capable of taking histories, performing physical exams, developing differential lists of diagnosis, ordering and assessing labs; and ultimately prescribing and following patients and their plans.  This is legacy of inspiration and fulfillment of a promise made to Evan as he lay dying in our bed. That I would help as many people as I could with the breath left in my body.

The rain pours outside both literally and figuratively for the pain and sadness of loss are real I can see a glimmer of light in the future, the memories will not stop coming, but the future is a brighter place. I know that my research efforts, inspired by Evan’s perseverance through the fog, are making a difference both in the labs of the world, in the clinical lives of patients and in the power of the sun rolling back the fog that envelopes the lives of many families.

Today our little family of three will gather again, not in an ultrasound office but a restaurant; and we will look into a grainy gray scale image of the future and we will not see perfection, we will not get sucked in by a false hope.  We will instead see the joy in opportunities and hard work to be shared in building a life in this world.

Today is yet another celebration of love, life and joy.  It is a day of remembering great times and amazing people, and it is an opportunity to be inspired to do something great, like climbing Kilimanjaro and taking a T-Shirt to show the world your inspiration to do and to act was a sick little boy in a wheelchair who denied death the victory of destroying any dream while he still had breath to give.

On the eve of Evan’s death in 2012, he demanded to stay with me as much as possible and to participate in living with winners.  He saw the college and the team that I coached as winners and they saw his courage as inspirational. On his last day at practice the team took a photo, had it put on T-Shirts and wore them as a tribute to Evan after he had died.  After they had showed up to his funeral in the most respectful gesture, and wore black armbands for the season, and even worked in the Evan’s Garden, I hope they will forgive me in thinking that would be the last they would think of Evan.

In January of this year I received this photo via Facebook. A true Yorkshire lad, our Danny Stirland, had climbed Mount Kilimanjaro in support of a Hospice House in the UK.  I cannot think of a greater honor or lasting impression and inspiration than that of Evan on Danny.  If you look closely at this picture of triumph, the T-Shirt Danny is wearing is the one honoring Evan on a soccer field in North Carolina.

Do not yield to the cold. Do not get lost in the fog. Do not get drowned in the rain. Feel the light.  Be inspired by the light. Be the light.

As I finish the note, the sun is trying to break through, the birds are singing their morning songs and my heart is a little lighter as I say – Happy Birthday Evan!

The First Rule of Life

24 hours ago I had just finished tying my neck tie in preparation to go to a funeral.  My need to be distracted from the tragedy of a life cut too short lead me to FB where I read, “Paul just curious where are you getting your patient reported information? When you talk about rates are you talking about ones that are symptomatic or non-symptomatic or a combination of both?” 

Both questions need to be answered and I hope I will be able to accomplish that with this blog post.  But before I wrote this I had two very important tasks to accomplish. First, I had to attend the funeral and share some thoughts with the family who are upside down with grief; and second, I had to take Lizy out for an evening of fun and laughter with dear friends of ours who knew and loved Evan and mourned his death with us before they had children of their own.  

The ironic nature of death is its inevitability once life has begun.  As I sat in the church yesterday thinking about what I might say, I was drawn back to the questions on FB. By what right do I speak? By what right do I profess knowledge? And by whose authority do I act? These questions have haunted me for 17 years.  I ask those questions of myself every day, I asked them at every opportunity of all the medical providers who ever attended to Evan. But yesterday I just sat and contemplated, knowing that the wrong words or a mishandled delivery would not provide strength, help, or healing.

The woman who had passed away just a week ago was not well known to me.  Gathering from the attendance at her memorial service, it seemed that she must have been a very quiet and unassuming person.  25 of her coworkers showed up to share their loss, and perhaps another 40 or so family and friends.  But the 4 men and 3 women who sat in the front row knew her best and she had invested time, and love and caring into each of their lives so that each of them (A Husband, two sons, a daughter-in-law and 3 grandchildren) is now a living legacy of who she was in life.

Why was I there? Why did I feel the need to speak and share? Because when we were at our lowest points in the summer of 2012, when we were trying to deny death its victory, when we were planting a garden and trying to sing our way to joy – She helped us.  I think that if she could have done her work at the Miracle 2012 (the concert to help defray medical expenses and raise awareness) without any recognition she would have done so; I did not even know that she had been there to help.  This is what I knew, every time she saw either Lizy or I she would ask how we were doing.  She was the embodiment of quiet compassion without fanfare or the need for recognition. She cared.

6 months ago she had been diagnosed with pancreatic cancer, and a week ago she had died. I attended her memorial service because she cared, and I spoke to her family of her selfless care for me and my family. And I shared with all the people in attendance that for all the good that someone invests in us we are obligated to share their love with others, so that we might be ambassadors for good, carriers of compassion, and living legacies of life.

Three and a half years ago, a woman I barely knew offered her time and help to a chaotic hodgepodge weekend driven by hope but grounded in the reality of a devastating reality – a life was being ended too soon.  For as much as we know that life must end, we want to end on our terms, and it should never happen to a child.  As a race of creatures it may be evolutionary selection that older creatures will do whatever it takes to keep the young alive, but that view does not account for free will, self-interest or most importantly love.

Her loving sacrifice, along with the sacrifices of so many others, allowed me the opportunity to grieve on my terms, on my time frame, and to rehabilitate at my own pace.  Part of that process was determining how I live out the death-bed promise I had made to Evan – That I would do as much as I could with the time left in my life to help as many people as I could.

That lead me to Physicians Assistant school which would afford me the chance to become a medical provider faster than any other route.  As it happened the school I am enrolled at was undergoing some changes and a professor offered a possibility of doing an independent research project. This would be on top of the 22 graduate credit hours/semester for 4 straight semesters of the first year, and in addition to the 12 months of clinical study required to graduate.

I met with the professor and was discouraged from moving forward, but I did anyway; and then I met with the professor and their boss and was again discouraged from moving forward, “It’ll be too much to handle”, so I applied for IRB (Institutional Review Board) approval anyway.  Along the way I was in contact with Nevus Outreach about the Registry of Nevus owners, and was told by some that it was “messy” and “there may be nothing to work with.”

12 months ago my research proposal received IRB approval 3 months after submission, and soon after a data file arrived from Nevus Outreach. 4 months of work later I had my first breakthrough, only to be shot down for conducting ‘dirty’ science – I proved a negative; the medical assertions that LCMN has a higher risk for melanoma was wrong, but I couldn’t prove what was happening.

I have been told by a wise soul that I must break the narrative for a moment to explain the term ‘dirty’ science.  ‘Dirty’ science is good science – a question is formed, then it is tested against data, and conclusions are reached. It is termed ‘dirty’ by academics because it causes all the related ‘knowns’ and ‘givens’ to be questioned leaving the area of research a little muddier than before.

‘Clean’ science clarifies a subject by proving a positive assertion. The impertinence of a student to assert a new positive with no supporting laboratory findings would be seen as fraudulent at worst and looked on with disdain and derision at best.  It was essential for credibility that I initially assert the ‘given’ even if I didn’t believe the established medical understanding otherwise my work wouldn’t be given credibility.  All I wanted to do was throw the medical understanding of the past 40 years into question long enough that someone would look and find something in the lab that would be the basis for ‘clean’ science.

As a result, I lost my advisor and was floating on a sea of pain and failure until someone threw me a paddle of self-belief and I worked my way to shore. 5 months later I had a finished paper based on a reworked scientific question and submitted my work for publication and peer review.

Two weeks ago I found out that my paper had been accepted for presentation and publication, and since then I have been invited to present my findings at the Graduate Symposium of my school, and possibly to share them in other locations as my academic schedule permits.  I hope to be able to present at the Nevus Outreach Conference on my findings, but also to speak to validating the data in the Registry and how parents might contribute in many ways to the research efforts that I hope will lead to interventions and cures that will ensure that no more children die from any sequelae of being a nevus owner.

Rather than posting the entire 30-page paper here, I am posting the Abstract.  Medical legends and lore not based firmly in reproducible evidenced based science must be challenged and brought into question otherwise no one will seek out the truth.  In the instance of a cure for NCM we have been looking for a needle in a haystack; this paper is part of a larger collaborative effort lead by others that has cut the haystack in half, and allowed a more focused search by using a magnet instead of tweezers. But none of my work has been possible without the work of the giants whose shoulders I stand on, nor the caring selfless compassion of a lady who passed away 8 days ago. I am but a conduit who seeks to share a little light in the darkness.

Study of 1,160 Large Congenital Melanocytic Nevus Patients Shows No Connection to Primary Cutaneous Melanoma

Abstract

Introduction

A retrospective study on an internet based registry of patients with Large Congenital Melanocytic Nevi (LCMN) was conducted to explore possible misconceptions regarding morbidity and mortality.  Prior studies based on the Nevus Outreach, Inc., Registry (NOIR) utilized a relatively small sample size (n = 379) and may have failed to uncover significant disease processes.   The addition of >750 subjects to NOIR in the subsequent decade offers the possibility of greater insight into the underlying epidemiology of LCMN.

Consistent with prevailing medical opinion of the past four decades, it is hypothesized that LCMN correlates to a higher risk of morbidity and mortality via primary cutaneous malignant melanoma (PCM).

Methods

To challenge this hypothesis, non-parametric models, descriptive statistics and National Cancer Institute’s database—Surveillance, Epidemiology, and End Results (SEER)—were utilized. Following IRB approval from Methodist University, NOIR data was acquired and curated according to National Institute of Health (NIH) guidelines for rare disease registries.

Results

Analysis of NOIR was first conducted in comparison with prior studies.  From this review it was determined that sample size from various geographic areas within the data were insufficient to identify a geographic bias. It was additionally concluded that previous sample size was insufficient to draw conclusions based on a single verified finding. Previous conclusions of significance of phenotype correlating to a specific comorbidity remain valid. Other conclusions regarding gender, LCMN size and LCMN location, whether implicit or subsequently inferred based on correlation to a single variable, lack statistical validity.

Conclusion

Researchers have struggled with correlating case study evidence and anecdotes of LCMN-linked melanoma to the reported patterns of morbidity and mortality within NOIR. The present study concluded that the additional data revealed a reported epidemiology of melanoma prevalence over a lifetime similar to SEER for the general population. Moreover, when prevalence of NOIR melanoma by age group was assessed for consistency with a diagnosis of primary cutaneous melanoma (PCM) in SEER, the diagnosis of PCM in the NOIR subjects was found to be overstated. The hypothesis that LCMN correlates to a higher risk of PCM was found to be false to a high degree of significance (p < 0.01), in that previously described cases of primary cutaneous melanoma arising within congenital melanocytic nevi are not melanoma.

Nous sommes tous les parisiens

To say that I begin this message with a heavy heart does not fully capture the moment.  It could be said that I always write with a heavy heart or with a sense of being oppressed by emotional, spiritual and intellectual burdens not of my own making.  Yesterday marked a new low in the story of humanity.  And yet it also marked a new high.  Yesterday a group of organized terrorists executed a series of attacks in Paris that left many people dead, more people asking serious questions, and the remainder of us with a little more to worry about.  And yesterday some child was born who will lead a nation, and another who will discover a cure to a previously incurable disease, and someone else began a career in healing with their first patient contact. And somewhere in the world yesterday a child was born with a Large Congenital Melanocytic Nevus (LCMN), and that child’s parents are just like you and I. 

These parents (maybe for the first time, maybe for the third fourth or fifth time) suddenly find themselves in a strange new world. Who is this child? What is a Nevus? Will it kill them? Can they grow up to be normal productive members of the community? Will they be allowed to grow up and become normal productive members of the community? Do you remember those feelings of inadequacy? The fear of failure, death and the piercing gaze of strangers?  There is a word for these emotions and initial understanding—Isolation.  It is the goal of all terrorists—to detach individuals from a group making them more vulnerable or to impress societal mandates upon a group creating individual panic and defensiveness.

The Societal and Political implications are irrelevant, frivolous and superficial.  All power is found in the love for one another and in the strength of relationships which are often forged in the crucible of common challenge or even suffering. All I have to do to see the truth in this statement is to observe the two strong factions within the Nevus community: those for and against nevus removal.  At extreme ends of the spectrum on this issue are people who are convinced beyond a shadow of a doubt that their position is right, unassailable and should be impressed upon the community of nevus owners.  That decision is a profoundly personal decision and can never be taken lightly given the very real risks of surgery and the unknown risks of a fatal outcome if not removed.

But all of that discussion is of no benefit, no comfort nor of any help to those families either facing a terminal diagnosis for their child or are seeking to learn to live again after they have lost a child to a disease associated with LCMN.  We engage in a vibrant community of support and encouragement and for the most part we have learned to live together in the light of LCMN without pointing out the perceived failures of decisions we did not make nor have to live with.  What I mean to say is that Nevus Outreach (NOI) has afforded us a forum to share, live and love as a community and rescued us from the terror of isolation. 

I would like to offer an assessment of accomplishments of Nevus Outreach in comparison to the timeline of the disease process we all most fear for our children: Melanoma.  Melanoma has been found in Peruvian mummies dated ~2,500 years ago. Melanoma as a cancer was not described until 1787 and not identified as metastatic until 1968, almost 50 years ago.  In comparison, LCMN were first described in 1777. A melanoma in an LCMN was written up in 1964 while Nevus Outreach wasn’t even founded until 1997 less than 20 years ago.

In less than 20 years NOI has founded, fostered, nurtured, cajoled and refereed us into a loose confederation of the people whose true strength is in our ability to truly uplift each other as we face both the disease and societies that may not understand, or may even be cruel or vindictive.  Let me share a story to illustrate.

When Evan, my nevus owning son, through a tantrum in a restaurant while we were travelling on vacation I picked him up and took him outside to cool off. He was about three years old and the threat of removal from the family at mealtimes got his attention every time.  I stood at the entrance looking out over a beautiful valley of trees and fields as the sun began to set.  In truth as I look back I may have taken Evan outside just to appreciate the beauty of the moment. Just as I am about to head back inside we were approached by a man who engaged us in conversation. He started by saying that he had noticed Evan getting upset and wanted to offer his assistance.  He stated that he was a local exterminator and that the flea bites on Evan’s legs were what was mostly likely causing his difficult behavior. 

I was then left to cool myself, explain as graciously what an LCMN was, and get Evan back in the restaurant before he figured out the total lack of civility in such a brazen assumption.  That of course was beside the inner monologue that, “It doesn’t take fleas to get my child worked up, I am quite capable of that all by myself.”

As soon as we got home I shared the experience via the Yahoo message board, the forum started by NOI.  The laughs, comfort and encouragement I received were priceless.  I have seen similar stories played out on Facebook, and it warms my heart that such an outlet for angst has been afforded while being a true source of encouragement.

Most of our energies are expended in raising our children as best we can, but unfortunately some of us have had to tread a different path.  This alternate path is one of disease, hospitals, treatments and ultimately death.  I stood up in a general forum at a NOI convention in 2008 and stated, “My family is the one you don’t want to be, our some has been diagnosed with melanoma.”  It is no easier to write today than it was to say in 2008 or to hear it or the first time in 2007.  But NOI afforded us opportunities just as they have to you.

This confederacy of parents and nevus owners has forged paths toward research goals, built awareness and provided both first contact support and ongoing support when children have developed either Neurocutaneous Melanosis (NCM) or Melanoma.  A number of initiatives have generated results but may not have received the adulation or awareness as progress in research is often slow and the details tedious and overwhelming.

NOI has helped establish a global network of scientists and doctors with high levels of interest in LCMN.  And from my personal perspective they initiated and maintain a registry of nevus owners which became the source of material and data for an academic paper I wrote as part of my course work as I pursue my Masters in Medical Science to become a Physician’s Assistant.  This is the path I have taken in response to a promise I made my son Evan as he lay in his deathbed at age 13, “That I would help as many people as I could for as long as I could.” 

As I look back on the last year of 70 graduate level credit hours of classroom work, and look forward to the next 12 months of clinical rotations part of me knows that I didn’t have to take this path.  No more than Travis and Cassandra Bailey had to ask for a full autopsy of their son Gavin, or that Tanya Kennedy might pursue the best and brightest minds in her area to join our fight after her precious Christian passed away.  We do what we do because of two reasons: 1. there is a legacy of our children fighting for their last breaths that will haunt us until we take our own last breath. 2. Because we have been afforded to opportunity to stand on the shoulders of giants who have gone before us and given us a solid footing from which to launch our efforts that other may not have to suffer through what we have.

In a recent note I spoke of the Gavin Bailey Tissue Repository in Pittsburgh and mentioned some of the early successes and breakthroughs that have been made in just 2 short years.  Soon I hope to be able to share my findings of my research for they should offer each of you a measure of comfort and hope, but they have not yet been accepted for publication. But I would encourage each you who are reading to solidify your contribution to our cause by either completing a registry form or by updating the data you have previously entered.  That 15 minutes of your time may say another person days, months or years of worry.

As I close this note I offer up prayers of peace, comfort, hope and love to the parents and grandparents of children who were destroyed by the terror of Paris, and I hope that they will find encouragement and hope in their communities as I have found comfort in mine.  Each of you is my neighbors, each of you has a choice to uplift another, and if you are facing the terminal diagnosis of a child with LCMN please take a moment and consider what positive legacy your child may have on the world by acknowledging the gift that is afforded the world through the most caring and complete physical they will ever have. 

For questions on this essay please contact me at pjscoleman@outlook.com.  For information on the Parent-2-Parent support group for terminally ill children with LCMN please call or text 919-455-8787.  To inquire about Clinical autopsy gift for the Gavin Bailey Tissue Repository please contact Dr. Miguel Reyes-Mugica via email at miguel.reyes@chp.edu  

Awake and Ashamed

It’s 5:48 am. I lie awake staring at nothing. I am not tossing or turning. I am lost. I am lost in the memories of three years ago this very minute when the interminable hum and cycling of an oxygen condenser were no longer intermingled with the panting of my son trying to inhale and retain every last molecule of oxygen because his heart had given its last beat unable to meet the demands of life. 

Five hours before, exhausted from the challenges of trying to live life while caring for the dying, I could no longer keep my eyes open. I could not continue to hold my son’s head as he struggled to keep his airway open as big as possible, I could not stay awake to engage in the staccato conversation of the end stage lung disease. For six weeks he had labored against all odds as his oxygen saturation dropped from 98% on room air to 92% on 2 liter of oxygen till on this evening his oxygen saturation was 64% on 4.5 liters of oxygen.

But now it’s 5:48 am and I can’t sleep, I don’t want to sleep, I just want to run away from the memories of shame.  I am half a father, and today cements that fact into my brain in a manner like no other.  One of my two children died before they were close to leaving my home.  This was no car accident, no random excess of booze or drugs or suicide; He died before he was old enough to experience any of those ‘self’ induced endings.  He died of a disease of ignorance.  I did not know the answer, and no medical professional in the world knew the answer, heck only a couple of them had even thought to begin asking the right questions.

13 and a half years earlier I stood gazing at my new born son face down in a neonatal incubator.  He had been born following emergency C-section because his umbilical cord had been wrapped twice around his neck.  Only the presence and skill of the Obstetrician saw the events as they unfurled before our eyes, and all of a sudden I went from caring for my very pregnant laboring wife to being a fool alone in a birthing room with no nurses, doctors or my wife.  I wandered the halls of the hospital aimlessly until I walked into the NICU and asked if my son was there.

Surprisingly he was, the NICU nurse turned and pointed to a little mass of flesh curled up under a heat lamp.  He was small, and he had all four limbs, and a C-section newborn head (too perfect to be true), and his back was covered with a brown, black, blue, red and yellow wildly bumpy and irregular thing that looked like either afterbirth or a poor attempt at a Hobo’s blanket.  I in my paramedic wisdom and not wanting to appear like a complete idiot asked the classic newborn questions:

“What were his APGAR scores?” 6 and 9, good stuff. 

“And what is his O2 Sat?” Oxygen saturation is a good measure of health. 98% on room air.  Good stuff again. Below 94% I would start to be concerned, below 90% and I would be worried and start oxygen supplementation, below 80% and I would have been looking to intubate, so very good stuff.

“And his blood glucose levels?” 48.  Good before a first feeding, have to give the growing body and brain all the sugar we can.

“And I’m not sure of your protocols, but when are you going to wipe him off?” What do you mean that’s him? What are you telling me? Giant Birthmark? You bet I need to see the doctor, because I am such an idiot.

As I look at the clock it’s still 5:48, and Lizy rolls over in bed and asks me what time it is and I tell her.  She sighs the sigh that only the mother of a dead child can release at the moment of remembrance, an echo from the broken heart that emerges from the mouth like the distant moan of wind on a windswept and empty beach where just hours before there was the frolicking and laughter of children playing in sand and surf.

It’s 3:12 am on Black Friday 2012, I am sitting in a kitchen in upstate Tennessee sitting across from a friend, a surrogate son, a big brother of another mother to my children.  He is strong and fit, a multi-tour veteran of the US Army who has been all over the world being the sole medical provider of small groups of fighting men. And he is significantly less drunk than I am.

It has been about three and a half months since Evan died, and we couldn’t face either doing Thanksgiving at home nor with people who would have no idea what we were feeling so we hopped in a car and drove 12 hours to a farmhouse in the middle of nowhere. We walked in the pastures and played with the dogs, and horses; I can’t remember if the goats and chickens were there yet, but it didn’t matter we were in a place of love and respite.

The only thing missing was Evan, and so early on that Friday morning I let go of all pretense and machismo.  With tears streaming down my face I shared with the only person I knew would understand the fact that I had let my son down.  I had failed in my fundamental responsibility as a father, to get their child to the point of beginning his life on his own terms.  The only other person I could imagine with a similar mindset was this Man among men across the farmhouse table.  He had faced the same inner demons, he was an Army Combat Medic; a person tasked with ensuring that his entire team got home alive after each mission.

In my mind I had failed my mission. The debriefing was painful, cathartic and forgotten the next day; buried in the graveyard of ideas and concepts not fully grasped or appreciated until the years of wisdom allow a person to fully put them to rest or come to terms with.  No one knows the pain of a father who has failed to meet his own expectations; and it takes a person of similar understanding to shed light on the unreality of those expectations, and to shine light on the dimness of self-inscribed responsibility for defeat when there were so many other factors in play.

It’s 5:49 am today.  The shame I feel is self-serving and destructive.  What I now grasp is that I was ignorant. I lacked the knowledge to make the critical decisions.  In some cases the information was withheld from me (Medical Ethics conundrum Beneficence vs, Autonomy while Justice is ignored), in others ways the information did not exist in a usable form for decisions to be rendered in the full light.  It felt like the dawn right now.  We are socked in by clouds in the North Carolina Mountains having run away from the pain of home on this day.

I am no different than many fathers who have lost their children to rare diseases.  That my processing of my grief takes the form of writing and sharing makes me no better nor worse than any of them but it has given me critical insights on the grieving process and the shame that often accompanies a father’s thoughts.  But now my strengths should be more fully expressed in the realm of academic knowledge to shed a little light on our ignorance.

While I finish up work on a couple of papers I wanted to share with you this link to an article I referenced in my last post. http://www.ncbi.nlm.nih.gov/pubmed/26266759 If you recall I hinted that new material was and is being developed by an amazing group of individuals in Pittsburgh.  While this is just the abstract it is a taste of what’s coming.  If this work fascinates you or you see the common links between NRAS and BRAF and other cancers then think about lending a hand to the process.  Reach out and contact me, leave a post, take the first step in becoming a member of the Army of Citizen Scientists and see how with your help we might shed more light.

If you are unable to join the fight on the front lines but feel moved to support the fight moving fr

om shame and ignorance to enlightened empathetic knowledge we are working on direct links into the research foundation funding apparatus, please be patient.

The days like this will come and go, each one a marker of time passed or of time passing away.  Evan’s Birthday – February 16, His remembrance day – August 18, Each Father’s and Mother’s day and all the other days that can serve either as unscalable  obstacle or like a riding crop to a thoroughbred’s flank. Be it a birthday, Christmas or the 4^th of July the memories can either inhibit progress toward lasting legacy or be a terminal point of sorrowful reflection.

Everyone who knew Evan personally and all those who have come to know him vicariously through others will know that the weakness of today is just that; he would want us to rejoice in his life, love and faith.  His passion was knowledge, information, light and fun.  And so those principles are worthy pursuits for us all.

So from this dim, dreary and humid locale I implore you to find a little sunshine today and share it with all who you meet.  With Love, Light and Laughter we wish you the best of days from Blowing Rock, North Carolina to wherever you are in the world as you read this.

 

Gavin brings Hope

The death of a child wrings you out like a chamois at a high school carwash on a Saturday morning in July.  You are left damp, wrinkled, dirty and without a semblance of love discarded on a line to be dried crispier than a tortilla chip in Texas. 

You are left with a hollow heart, a little more jaded and perhaps an increasingly cynical view of what’s still remains.  Time does not heal all wounds.  But it does give a chance for seeds to sprout, rain to fall and every once in a while some sunshine to break through the clouds of heartache. 

Three years ago I stopped writing for e-nevusnotes, the blog through which I detailed my son’s journey through the last years of his life. The sign off rant was wrong. But to get to this point has been a long, twisted journey that just happened to pop out on a rain soaked garden in Pittsburgh last weekend, the weekend halfway between Father’s Day and the 4^th of July.

But before I continue, perhaps a little Donald Trump humor would lighten the moment.  An American, Brazilian, Indian, anda Mexican walked into a café. Now before you get your proverbial tighty whities in a bunched up mess, this is a true story.  It is the story of child heroes, the love of humanity, the compassion for justice and the origin of a real “Captain America”.

Over 30 years ago a doctor in another country happened upon a disease that presented such a conundrum that it wouldn’t let him sleep.  And when he studied this rarest of rare disease processes he was mortified by the deaths of children due to complications. These complications were the things only a parent could fathom, and then it was only in their worst nightmares, and only if it was their child. 

Miguel Reyes-Múgica, MD, is a pathologist, a person committed to understanding the physiology of life from the macroscale to the micro to the biochemical.  His entire life has been about autopsies, not to find out why a patient died, but to allow each patient to tell the story through the most thorough and detailed physical they have ever received.  All this is done so that someone else won’t have to die of the same cause. 

He is our, “Mexican,” he is also a student of Congenital Melanocytic Nevi, and as a student he has always craved having a living library of nevi tissue to examine, study and learn from. Dr. Reyes is the Chief of Pathology at Children’s Hospital of Pittsburgh which is part of The University of Pittsburgh Medical Center (UPMC).  I have personally known him since 2001 when I met him at Snow Mountain Ranch, Colorado, speaking at a Nevus Outreach conference; he was at Yale at that time. This past weekend he invited me to his laboratory which is committed to pigment diseases of the neural crest.

To learn more about Dr. Reyes this links to a in depth 2007 interview http://www.pathsoc.org/conversations/index.php?view=article&catid=58%3Amiguel-reyes-mugica&id=82%3Amiguel-reyes-mugica-full-transcript&option=com_content

The, “Brazilian,” in our story is Claudia Maria Salgado, MD PhD, originally from Federal University of Goias in Brazil.  She is a pediatrician and has been a student of pigmented disease for many years. She has dual roles in our narrative as she functions in both a clinical role with patients and parents; and as a researcher of nevi tissue.

Dipanjan Basu, PhD, who fulfills the role of the, “Indian,” in my story. Dipanjan aka ‘Deep’ is a microbiologist whose love of his laboratory is only rivalled by the love of his children and wife who see him intermittently as he delves into the cells that represent the ‘enemy’.

For all the medicine and academic prowess of the first three parties the bond I have with the, “American.” is one I wish we didn’t share.  Travis Bailey lost his son Gavin at 17 months of age.  If Gavin is another child hero robbed from his parents by an insidious disease, then Travis is my vision of, ‘Captain America.’  I never saw a cross word come from his mouth and he is always trying to keep the greater good central in his role as son, husband, and father.

In a moment of serendipity, Travis and his family met Miguel and Claudia seven days after Gavin was born. Everyone became fast friends to the point of family.  Time went by with the friendship growing and blossoming in the light of a common goal of living life to the fullest when a child has CMN.

For the overwhelming number of people with CMN there are no repercussions associated with either melanoma or neurocutaneous melanocytosis(NCM), but for a select few the worst case scenarios are visited upon us.  For the Bailey family their world received a devastating blow around the time Gavin turned 6 months old.  The melanocytes visible on his body and invisible in his brain and spinal cord conspired and became a cancerous tumor within his central nervous system.

According to Travis, and confirmed with photographs, Gavin was an electrically engaging child.  The sort of curiosity of life and people that was evident in the near mischievous twinkle in his eyes as he grew towards his child hood.  A childhood that would be cut short because at that six month diagnosis his family was told that his condition was terminal.  There were no cures, there were no medicines, only trials of this drug or that.  There just hadn’t been enough research.

For those of you who knew of Evan or followed his story on this blog, that is an all too familiar story.  So you are left scrambling to try and find some way to save your child.  You will learn the medicine, you will travel the globe, you will spend all your money and much more.  Anything in the world will be done to have a doctor take back the words, “I’m sorry”.

I am sorry. What does that mean?   I was told repeatedly as a child that if you say those words you are promising on your honor that you will never commit such an act ever again.  I know that Doctors, Nurses, PAs are all trying to show compassion. I also know that their intentions are well placed, but with a dying child in your arms these words are cheap, empty and without true intention.  They actually hurt more than, “Well that sucks,” because they convey pity and disconsolation in an attempt to disconnect from an awkward situation.  But as usual, I digress.

Gavin lived his short life to the fullest.  The tumor at one point rendered him paraplegic but a motorized wheelchair allowed him to keep up with his older sister and he would play tag with her and a number of other children from his hometown near, Sommersville, WV, on the big open floor of a local gym.

But cancer is unrelenting. Because there were no answers, his decline was predictable.  For Miguel this was the antithesis of his very being – he would have given anything to have prolonged Gavin’s life. He already had set up a tissue repository at UPMC, a place to study this disease that was robbing the Bailey family of joy while stealing Gavin away from them.  But there wasn’t enough tissue, nor was it alive. There just wasn’t enough time.  It was all just so unfair.

Four months before Gavin passed away, Miguel and Claudia took Travis and his wife aside and talked in more detail about what they did professionally, and how, the Baileys, through Gavin might extend his legacy of smiles by helping get a truer understanding of neural crest fatal disease processes.  They proposed that Gavin’s tissue be the first living tissue in the repository, that if the tumor cells could be kept alive they would offer an invaluable insight into a disease that has never been studied in this manner.

Tired of their pain and seeing an opportunity to truly help others the Baileys consented that Gavin should be autopsied at his death, but that all measures to prolong his life should be exhausted up to that point.  Gavin received radiation therapy in the 2 days prior to his death, and in the hours after he died, Travis carried him in his arms and handed him to Miguel and Claudia in a private moment and private space that was filled with love, grief, profound sadness and a glimmer of potential that someone in the future may be helped. 

Travis told me that only good could come from his actions.  He had reasoned that Gavin wouldn’t need those tissues when he was buried; and he knew Miguel would do all he could to ensure that parents and children of the future would have better options for care and life. No one had done this before, no one had seen the path to doing the right thing, this was a job that only a, “Captain America,” could get done.  And no matter the personal pain suffered to this moment, it was being done for all the right reasons.

And so was born the Gavin Bailey Tissue Repository for Neural Crest Disorders.  But three things were missing: Money, Tissue and Life.  Enter, ‘Deep.’  Dr. Basu began to tend the tissues.  By his own admission he is a lab rat, rarely coming out into the sunshine and never meeting patients or their families.  He just wanted to get to know the, ‘enemy.’  The good cells gone bad were to become his life –  to find them, separate them, to grow them, to study them and to KILL them.

A week last Friday I took the day off and flew to Pittsburgh; Miguel wanted me to see something.  Well to get the whole story correct, Mark Beckwith of Nevus Outreach told Miguel that I should see what he was doing.  So after a long afternoon of travel, I walked into a Café in Pittsburgh and had dinner with Miguel, Claudia, Dipanjan, Travis along with Tanya Kennedy and Taylor Scott.  Tanya lost her son to this disease nine months ago after he had lived and died a short but valiant battle; and Taylor is the Executive Assistant at Nevus Outreach.

We laughed, we cried, we shared, and we prepared. We prepared to tour the UPMC Children’s Hospital specifically the clinical laboratory and then the research laboratory and finally the tissue repository.  We were also scheduled to hear from a doctor about end of life issues related to children.

Saturday was quite possibly the hardest day I have been through in the past 24 months.  The first 12 months after Evan died were a blur of tears, anger, frustration and an overwhelming sense of failure.  That this weekend was a week removed from one of my personally most difficult days, Father’s Day, seemed almost fatalistically cruel.

The Diagnostic Lab was fascinating and I would be lying if I said I wasn’t paying slightly more attention at this juncture because I had a Lab Medicine Exam on Monday.  As we crossed the literal bridge from the clinical hospital to the research labs the group got noticeably quieter and a little more subdued.  I suppose the researchers were anxious that their work would be appreciated, and the parents perhaps being overwhelmed.

When we reached the Rangos Research Center which houses the research labs, we were privileged to receive a fantastic guest presentation by Dr. Scott Maurer about the clinician’s side of end of life processes. Then things got surreal.  If Dipanjan had been quiet and reserved at dinner he was active and engaging, a ball of electric energy as he showed us this and that.  His work obviously brings him tremendous joy and the opportunity to share this with lay people who would be fascinated brought a palpable energy to his demeanor, and then the first bombshell hit.

“These are Gavin’s living tumor cells,” he shared with reverence as he brought a specimen jar into focus on a video monitor attached to his microscope.  There they were in two dimensional imagery.  They had done it.  They had discovered the right conditions and media to allow these cells’ lines to grow and replicate.  They had enough cells to run serial trials to generate research, they had enough success that other institutions were coming to see how they had done it, and other institutions are asking for cell lines so that they might study them.  This was a “WOW!” moment like no other. I will never forget the manner in which Travis crouched down to get a closer look at the screen, there was a moment of slack jawed awe, appreciation and love that only a parent could fathom in that instant.

As we gathered in the conference room preparing to hear some of the details of Dipanjan’s work, Miguel took the time to share his personal history with CMN and why this project was so special.  But Travis, a quiet and reserved man by nature shared his appreciation with a gentle smile and a wrinkle at the corner of his eyes as the story of Gavin was told.  Miguel called us brave and courageous, even as we feel anything but those things.  He spoke of other parents 50 years ago who made similar contributions to research so that there are now cures for many childhood leukemias.

When Dipanjan took the floor we didn’t know that he had been preparing for over 6 weeks for our visit. There are interpersonal skillsets that clinicians hone over years of practice that researchers do not.  One only has to watch an episode or two of the Big Bang Theory to appreciate a caricature of this truth. And then before he began he confessed that dinner the previous night had driven a personal note home to him.  Real people, patients and parents, will forever look to the lab for answers that only non-clinical bench scientists can provide to fundamental questions.

His work is due to be published soon, and I will not steal his thunder here, but it was mind blowing what has been made possible through Gavin.  Not the least was the addition of 126 other patient tissue contributors, 20+ with symptomatic NCM.

Since I left Pittsburgh on that Sunday morning, my head has been spinning.  I went there with trepidation, fear and no small amount of dread.  I left inspired, educated, and encouraged.  Thank you Captain America for your humble courage and pragmatic understanding of what needed to happen.  And thank you Gavin for living your life to the fullest and leaving a living legacy that may yield curative results in my lifetime.  

If any of this sounds in even the remotest part worthy or honorable please feel free to contact me at paul@e-colemans.com regarding how to contact Dr. Reyes at UPMC regarding tissue or monetary donation.  As for the Life part of Gavin Bailey Tissue Repository: A Mexican, Brazilian and an Indian walk into a lab every day and give their hearts and souls that life will be long and joyful for CMN children of the future.

Travis and Gavin Bailey

Moving Forward

I will never do justice to the memory of my son, nor replicate the emotions or events that have been encompassed in the posts to date.  That said, I have begun a new blog that is part of my ongoing process of trying to come to terms with the loss of such am amazing spirit with whom we were charged to watch over for the past 13 years.

You can reach it by going to Observations

We thank you for coming here and taking the time to feel some of the emotions associated with dealing with a chronic fatal disease in a child, and we pray that you will never have to experience this yourself.

A Year of Firsts

Jesus said, "Let the little children come to me, and do not hinder them, for the kingdom of heaven belongs to such as these."

This is not the end. It is also not the beginning. It can at best be described at half-time. For those who follow Evan and his story through this portal, there is no small amount of voyeuristic horror, fear and revulsion of a story that holds only one central theme of the pain and suffering of a child become man and now a memory to those left behind. Evan’s story has at times reached out and grabbed readers by their throats and shaken them from the calm reverie of their lives.

There can be no apologies for those words and the emotions that conveyed a tale that so long as it was being written was in itself confirmation that it was not happening to the typical reader. For those who read with intimate knowledge of the agonies of this journey a large debt of sorrow and regret is extended for the pain and memories that Evan’s story may have dredged up.

At 5:45 am on Saturday, August 18, 2012 Evan MacGilvary Jenkins Coleman breathed his last breath. The details of that moment are so intense, personal and overwhelming that they will not be recounted here. What can be shared is that the previous day Evan had begun to decompensate (A medical term indicating that his body was no longer able to maintain the necessary balances required for his life to continue) As the evening approached he fought to find a position of comfort that ended with him doubled over in his bean bag while watching ‘Pirates of the Caribbean’ by way of a mirror on his knee.

As the evening wore on it was clear that he was going to be unable to sleep and the only thing any of his medications was accomplishing was to take the mental edge off the distress of breathing 80 times a minute, unable to cough, heart rate over 160 and a steadily decreasing oxygen saturation level. And as Evan came to an understanding that his fight would soon end, he leaned back into the arms of his mother and allowed her to cool his brow. The five plus hours of this physical ordeal is akin to running over two marathons.

Fighting to the end he continued to sip water through a straw and at 4:45 even asked for a medium Coke. At around 5:30 his final words to his parents were, “I love you.” As the fight and life left his body fifteen minutes later, a calm serene peace descended on his body and he breathed his last. No one will ever be able to say that he did not fight the good fight far longer than any other we have known. Many medical providers have commented along Evan’s journey that his lack of complaining put many adults to shame and set a high standard and wonderful example for his fellow patients.

And so began the Year of Firsts. There was no fanfare of Angels trumpets; there was near silence due to the absence of ragged breathing and an oxygen concentration machine turned off. For those who with the morbid fascination of an entranced audience who have been watching and praying for a different outcome, the moment of collision is but the beginning of the dissipation of energy that had been brought into the impact equation. (Impact equations are a fundamental topic of Physics: The Law of the Conservation of Energy states that energy cannot be created or destroyed only transformed. All the energy going into the initiation of an event must be accounted for at the conclusion of the event.)

The external image of a slow motion train wreck serves the purpose of creating a picture of an unstoppable event resulting in cataclysmic damage that resolves itself not in the moment of impact but through the derailment of cars, collisions with unmoving adjacent objects and structures and general chaos and mayhem. If this verbal description has not grabbed your imagination, please consider watching the beginning of the Steven Spielberg movie “Super 8”.

The internal image for those closest to the event the image is that of a nuclear bomb disposal unit that has suddenly realized its failure as the white hot searing light of nuclear detonation announces the unit’s failure to contain and render the problem inert. The resulting crater lies across all life paths of the unit’s members and the crystallized glassy debris is left behind to explode on contact or cooling at unannounced times and circumstances that leave the impression that one is walking in a minefield where every step, every turn, every attempt at avoidance merely sets off another explosion of a glassy radioactive mine.

The hole left behind is an immense crater of unexploded ordinance that reveals itself in sudden overwhelming waves of intense painful sadness. There is no way to cross this hole quickly without imminent self-destruction, as every turn reveals a trip wire of a memory that explodes into an instant full realization that there will be no more trips to McDonalds for snacks or trips out to relieve anxiety. There will be no more need for Evan’s laundry to be done, or finding out that the last load in the dryer was his. The discovery of a casually discarded jacket over the backseat of the car is enough to initiate total meltdown.

Let there be a necessary intervention of tangential information. Evan is in a better place – whether your belief system runs toward Heaven as does ours, or you are agnostic or atheist, there is no denying that he is no longer suffering the agonies of suffocating to death as a disease process robbed him of the ability to exchange oxygen and carbon dioxide in his lungs. Therefore this writing and the services of this weekend serve a singular purpose of providing those who mourn Evan’s loss a place to jump back into life as we begin the process of coming to terms with our loss.

The metaphorical reference now returns to the smoking radioactive crater that lies in front of all paths of future travel. A look behind shows that the last five years have been littered with wonderful memories through a wasteland of the battles fought, some won some lost, in a war that seems to have been forfeit to an enemy known only as cancer. And as we look at this hole in the ground pockmarked with mines and triggers a salt rain of tears falls in an almost continuous downpour. There is a fear that the sun will never shine again, and any laughter is forced, inappropriate and cut short. Life seems both very wet and very over because we cannot step into the hole. The hole is deep, treacherous and functionally impassable. We are stuck.

As we peer into the future we see that the salt water rain will fill the hole, and around us are growing trees and plants that over time will be fashioned into boats for travel across the salt water lake to the other side. The hole will never go away, but it will become passable and a beautiful place of remembrance.

But today the future does not take away the Year of Firsts. Today it was the first time to request a table for 3 instead of 4. Three days ago it was a first visit to a funeral director and tomorrow it will be a first visitation. There has been the return of now unnecessary medical equipment; and then there was the gathering of pictures and things that defined Evan’s life which is not easy for a young man who lived such a rich life in just 13 short years. In the next twelve months among the known Firsts will be birthdays and Christmas, Halloween and Easter; and these can be accounted for, anticipated and supported. What we fear are the unknown Firsts. These are so numerous at the moment due to proximity of people, places, stories and events. But what will we do in 3 months’ time when we get an invitation for Evan to attend some event that he attended so joyfully in the past, or turning up a lost sock, shoe or mitten when cleaning a closet. Those are the times when the black sheet of overwhelming grief will only be lifted by the joyful sharing of the best moments of his life with people such as you.

Final notes: A Memorial Webpage has been set up for Evan, where you can leave memories about Evan, pictures can be posted, and you can read how Evan impacted the lives of so many others. It also contains the details on services and connections to the ongoing Miracle 2012 legacy. It can be reached at http://www.cremationsocietync.com/sitemaker/sites/Cremat2/obit.cgi?user=688738Coleman This site will remain in place in perpetuity.

Lil’ Duck: Lil’ Duck was born out of a legacy from another victim of cancer and her friends extension of vicarious living through another duck, Squeaky Edgar who travelled the world capturing events, stories and memories that were shared through a public blog so as to maintain an ongoing interaction with the world. And so our Lil’ Duck is a visual representation of the remaining spiritual presence of Evan in our lives. As Lil’ Duck goes with us, he provides a viable cover for those moments that we forget that Evan is not with us as we ask for tables for four instead of three, or we do activities that we know that he loved to do. We do not plan a blog for Lil’ Duck but rather convey the presence of his memory through pictures on facebook in those places that he loved to be. In the past day Lil’ Duck has been spotted in Evan’s Garden, at the Adidas Outlet store where he got his hallmark shoes, and at the Cracker Barrel where he loved to order pecan pancakes.

And so this piece is done, the story will continue and the writings will be given life and venue in so other arena. In parting and in some ways hinting at the future; children no matter how unique should never be used as Guinea Pigs. For all the failures of medicine, morals, and people – guilt and regrets will never change the outcome of events in the past. And while we have traversed some rough days in the near past and have some rougher ones in the next week; we hold onto Peace, Hope, Joy and Love – knowing that the greatest of these has been, is and will continue to be Love.