Confirmation - Frustration

For the longest time the writing for this blog has been driven by circumstance; and for an equally long time there has been a truism – No News is Good News. But sometimes, the news we have, the information we intend to share is so gut wrenching, so utterly sickening that we feel that we have been kicked in the gut so hard as to drain the color from our faces and leave us gasping for air. We are unable to immediately share. Such is the case in this instance.

We have a holistic approach to Evan and his condition in that we can take into account his entire history, balance this with the picture of his mental and spiritual health; and from this perspective we hold fast to a faith and hope that he has/is/will continue to battle off the challenges inside his body and live a full life. But we have skipped ahead too far and too fast, let’s just slow down and start back in February of this year.

At that time, the chemotherapy regimen seemed a little lax with hit-n-miss dates, no real pressure to be on a regular schedule, staff taking chemo directions as suggestion vs. law, everything was swimmingly normal. After two previous scans showing remarkable reductions in tumor size; frankly there was more concern on our part that Evan had missed a couple of growth spurts and was dealing with some annoying side effects (The concern over side effects is directly proportional to the likelihood of imminent demise).

In comparison to constant nausea, loss of hair and lowered immune system the issues were minor; however, when they are the only indicator that you are ill and you’re having difficulty sleeping such side effects loom much larger.

We wanted a break from these annoyances, previous scans looked good, perhaps the nodules had become a more chronic situation, needing to be knocked down a peg, and then keeping them in check. So we asked about the possibility of a treatment break, the oncologist reluctantly agreed but only if Evan had a new set of scans and that after a 90 day break there would be a post break set of scans to gage what had happened.

This pre-chemo-break set of scans revealed that the nodules had not really changed since the previous set of scans. The oncologist took this to mean that the chemotherapy had run its course and that UNC was out of treatment options. The next step would be to wait and see and then make a determination for a course of action after 90 days, but that we should start the process of getting more fully connected with another treatment center, specifically NIH due to their familiarity with Evan and the underlying rarity of his disease process.

During the first part of this treatment break, and in a totally unrelated and red herring aside, Evan took an academic standardized test to see if his dad was a suitable teacher for continued home-schooling. Frankly given the amount of time that dad spent on his own schoolwork, some people may have been concerned that Evan could even spell his name correctly after a year of questionable education.

60 days later the results were in. And dad can continue being the teacher as Evan scored in the 97^th percentile for his grade; mom & dad are greatly relieved as this represents the one of two growing parts of their retirement plan (the other being Evan’s sister, Morgan).

In early April, the on again / off again / on again science conference on pigmented lesions in Germany confirmed that dad was asked to attend and to share an update on Evan, if possible in the form of a case report. Starting and ending the first weekend in May, the trip was a crazy travel affair, from last minute changes to the requested paper, a 24 hr notice for the need of a poster presentation, missed flights, lost luggage, too much German beer; all done within 96 hours.

The paper was well received and there was encouragement to submit it to peer-reviewed journals. But more importantly was information presented by other researchers on genetic pathways, triggers and chromosomal analysis related to malignant disease. One piece of information stood out in a discussion: Other patients with nevi related flank nodules have been seen elsewhere in the world, and bad outcomes had been seen where these flank nodules had been attempted to be removed. There is no data on this subset of cases yet. (and the numbers are increasingly baffling – giant nevi occur 1:500,000, but a giant nevi may be caused by any of 14 different genetic anomalies, and the trigger for these anomalies might occur at several places on the embryonic development timeline in >20 possible environmental-to-genetic links) And yet as rare as we have felt Evan to be, even possibly unique, a doctor had seen not just one patient like Evan, but several. He is not alone, (never has been, never will be) but it is strangely comforting to know that there are others struggling with the same issues.

When dad returned home, it was time to schedule the post-treatment follow-up scan - which happened 8 days ago. We did not expect miracles. In the past 3 months, Evan has grown almost 2 inches taller and gained over 5 pounds. We have witnessed over his life, that as he grew bigger – so did the nodules. So when this past Tuesday, the oncologist called to tell us that the nodules were bigger, we were not surprised, when she mentioned that they had doubled in size we did not panic; but when she asked adamantly to speak to us without Evan present we knew there was a problem.

It is time for a brief digression. Two years ago Evan began the process of confirmation in our church; a guided introspective journey of education, belief and convictions. The confirmation process will culminate today, Pentecost Sunday, with Evan’s public confirmation of his faith. Such events and cultural practices go back millennia and are considered to be the spiritual demarcation where a person moves from childhood to adulthood. We are reasonably confident that Evan has already crossed this bridge with everything he has been through, but ritual is ritual.

We hope you can grasp that the weight of the weekend (a weight we left for another to carry) was multiplied when we added a secret meeting with his oncologist on top of the confirmation dinner, prayer-pal revealing, confirmation service, and his confirmation open-house. The insanity of the situation is that balance in our lives is directly related to the amount of other things filling our time; in such a manner we are able to balance the temptation of allowing our thoughts to delve into introspective self-pity. We also handled the pre- and post-surgical care of a top level international soccer player on this weekend just for good measure.

Going into the meeting with the oncologist we developed an idea of what we were likely to hear: the tumors are bigger, out of treatment options, does NIH have any options. We were not prepared to grasp/hear: 8 times larger than February, this will cause his death, “No, Can’t, Won’t”. It was not until we actually heard the desperation, frustration, fear and lost hope in the voice of your son’s archetypal cancer fighter; that the jack boot hits our gut and tried to suck the very life giving air from our lungs.

So what do you do after hearing those sort of things; here’s an idea, Johnny Carino’s for a light Italian lunch followed by conversation and calls to ensure that your son is ready for his piano lesson in the afternoon. We have heard doom before, we have seen people lose hope for Evan and for others; but we chose to stay calm in the moment, applying all we know and have learned to guide our little boat through the shoals of naysayers and doom mongers. If we lose our focus now, if we drop our gaze from the target, if we panic; then we do no one, especially Evan any good. In fact we will likely do him harm.

For those of you who cry for Evan, and for us; thank you. If you have read this blog in the past you know that it has been a source of raw emotion at times, a place to vent angst and spew frustration. Today we must set aside that luxury in favor of a measured evaluation of the situation to make the best decisions for Evan’s health and quality of life; a long life.

In contrast to the depths of despair that Evan’s worldy physician shared, we would like to close this post by sharing the letter we wrote to Evan for his Confirmation Sunday:

Evan;

For several years we have written about you.

We have written about your battles.

We have written about your travels,

and some parts of you that have travelled further.

We have written about your Faith.

We have written about your Love.

We have written about your Legos.

And we have written about your status in our life as our hero.

But what is a hero?

Does a hero wear a long cape?

Or perhaps a mask over their eyes?

Maybe they can be invisible,

cloaked from the ‘bad guys’.

Or maybe they wear a jester’s hat

and have a shiny mirrored disco ball spray out light rays that shatter maelstrom warriors into little, tiny bricks.

That all sounds heroic,

but it just doesn’t feel right for you

or the world.

Which brings up another question,

what exactly do hero’s do?

Do they attempt to eliminate all the evil in the world?

Or is it something more subtle,

perhaps simply providing comfort

or friendship to a suffering soul?

Perhaps shielding the innocent from the ravages

that the storms of life seem to rain down

from time to time?

And where does a hero get their strength from?

Do you eat your Wheaties?

Or maybe you are infused with power

from a sun soaked nano-suit of reactive armor?

Or do you draw upon a power from within,

from a power cell that was planted in you

with the simple pouring of water on your forehead as an infant?

And what is your real enemy?

Is it feeling weak

after holding that disco ball above your head?

Or do you feel worn down by the constant pressure

of battling invisible foes?

When you shield others do you feel chipped and broken

and not quite whole?

Or is it the tired feeling

of trying to hide from a relentless foe

that never gives up?

Or is it perhaps,

a sense of faith slipping from your grasp,

like feeling the edge of a cliff under your heals?

That just one more second of your burden,

will end in shattered shards of glass.

That the glass that shatters

might be your fragile heart.

As you choose to profess and confirm

your faith and belief in Jesus;

it should come as no surprise

that a hero cannot do

what they do,

when they do it,

or how they do it;

without having a hero themselves.

And it is this hero of yours,

Jesus Christ,

who you try to model

before those you meet every day.

With Jesus as your hero,

you do not have to be perfect,

because He is.

You do not have to be sinless,

because He is.

And when you get tired,

you know that He is tireless.

And when your heart feels empty,

you know that His is always full.

So as the journey of your life moves through this station;

we give you two blessings:

1. Preach the Good News of your Hero to all you meet in your journey;

do it with love,

compassion

and strength

words only if necessary.

2. You are our hero

as you battle against unseen enemies;

but we know that it is your Hero

that gives you

passion,

peace,

wisdom

and joy;

So remember:

Dead heroes save no lives.

And keep on living,

for today is the first day in the rest of your life

All our Love

Mom & Dad

Fish or Cut Bait

Two years ago Evan and parents went to the amazing and remarkable conference hosted by Nevus Outreach in Dallas. At that time Evan’s case was presented to the leading scientific experts in the field of pigmented lesions or nevi. The advice from that meeting have driven our path forward: keep looking, don‘t take ‘no’ for an answer, and keep on asking the hard questions.

Two weeks ago we went back to Dallas, and met new friends, gathered more knowledge, shared insights and hope and got re-energized by like-minded people to continue pushing forward. The meeting could not have come at a better time, the encouragement could not have been more timely, and the sincerity of a common faith of families affected by nevi is something that cannot be expressed in words.

This time we took our daughter who had her eyes opened to the fact that there were other people in the world just like her brother, and they had siblings just like her. And children and teens and adults talked and played freely in the comfort and knowledge that they were not alone, but members of a family. Yeah, we might be the family of 101 Dalmatians, but we love each other just the same; no matter race, creed, religion, politics, age, intellect or income. Love is a powerful, powerful weapon against darkness, aloneness and fear.

Timing is something that we do not understand but really appreciate. As we sat in a science meeting asking questions of experts; experts began to seek us out and asking us questions. And the dialogues lead to suggestions, and suggestions were relayed instantly to our team at UNC. And more conversations were started with other experts in other locations, and the entire conference was alive with citizen scientists conversing with the real scientists, each person learning from the other and building the body of knowledge that will lead to breakthroughs of peace and hope for families now and in the future.

But as we sat in that meeting, we received word that Evan’s experimental chemotherapy trial was not working. That we would have to go back to the drawing board and reevaluate all the options. This process was/is intellectually challenging, emotionally racking, physically tiring and spiritually draining. That has been the state of our house this week.

In this post we will allow you inside the process not so that you may marvel, or ask, “How do you do it?” but rather, if you see something that we have not, that you would speak up and share what you see. Your insights, thoughts and prayers are no more or less important than anyone else’s. When you have read through this post and you are willing to share, please email evan@e-colemans.com thank you.

The time to weigh options is upon all who are involved with direct care and process in the life management of Evan Coleman. These are not easy decisions to make. With each passing development and milestone (positive of negative) the pressures seem to mount. As his primary advocates we have decided to approach this recurrence with a more deliberate step by step process and we must periodically review the available information to better guide our decisions and to ensure that we are consistently acting in his best interests.

His medical team is geared to an either/or decision making process. The options are clear to this component: chemotherapy or surgery. There is at least a third option, known but not often considered, wait and see. And there may be many more options available but as yet unconsidered.

The debate: Medical options (chemo vs. surgery) vs. Parental options (action vs. inaction) the following is such a review of the situation and options.

The decision making process must be entered into with the overarching understanding that life for the sake of life is not a valid argument in and of itself. The quality (both longevity and enjoyment/pursuit of happiness) of life of the patient must be balanced against both treatments and outcomes. Fundamental ideas exist within the medical community: Hope is the objective of every meeting, every person has Evan’s best interest at heart, and no one wants to do him any unnecessary harm.

What we know:

1. The tumors are locally recurrent.
2. No known life threatening organ involvement – invasion/infiltration of solid or hollow organs
3. The tumors are not visible on Positron Imaging, but are visible on MRI and CAT.
4. The tumors are not recruiting nutrients in abnormal amounts (Nuclear Bone & Kidney/Bladder Scans)
5. That previous surgeries have failed in the attempts to address the recurrence of the tumors, and in fact may have contributed to the recurrence. This includes wide margin excisions by a highly qualified surgeon.
6. The previous presentation (from 7 months to 8.5 years of age) of the tumor process had exhibited a rapid growth curve that stabilized around 6 months from trauma. Since treatment was initiated in November 2007, there has never been a review of tumor growth rate following surgical trauma, until now.
7. The tumors have not responded to traditional or experimental chemotherapy.
8. The DNA of the tumor does not match any known melanoma DNA (Or other cancer).
9. Under the microscope the tumor cells look are consistent with nevus tissue with some mitotic and spindle elements that look like cancer if it were present in normal tissue. This is a gray area of cell science.

Medically there are several possible options although getting access to some of them might involve jumping through many hoops and cutting through a lot of red tape.

Chemotherapy

1. Taxol/Carbo FAIL – inefficacy
2. Temsirolimus/Depakote FAIL – toxicity
3. IL2 (Interleukin II/Interferon) ??? – ICU necessary due to high dose reactivity (14 doses, spaced 6-8 hours apart, 7 day rest period, and repeat for total of 28 doses over ~12 days)
4. Vaccine Trial

• Dependent of prior failure of other agent
• Late stage
• Clearance/waiver/acceptance
  

• Other Options presented via National Institutes of Health (NIH)

Surgical Intervention

1. 6^th attempt at wide margin resection with first removal of skeletal structure (Fat margin of L Kidney, Splenic border, External L Pleura, and sections of 10^th & 11^th ribs)
2. Removal of bulk of tumor in biopsy process to acquire live tissue for development of vaccine.(Done at vaccine developing facility – Bethesda. MD)

Motivations, Fears and Basis

Inaction may lead to metastasis and early death.

Continued chemotherapy is causing neurological & liver damage and is leading to increased risks of illnesses later in life .

Continued surgical therapy is validation of Einstein’s assertion regarding insanity, and will likely lead to early death.

Failure to comply with medical advice may lead to removal of valued counselors

Psychologically the entire process has worn increasingly upon Evan’s psyche and his hope is dampened by every failure.

NEXT

When reached this point in our discussions it was time to call upon more objective minds with the only bias being for Evan’s well-being.

First was asking Uncle Jon, the sustainability expert (sustaining Evan is right up his alley) and he pointed us in the direction of deep ambiguous questions of qualitative thought. Messy, deep, introspective, intangible questions of gut feelings based on information at hand without trying to match the quantitative information to our hopes or desires. He posed 4 thought provoking questions as a starting point to our investigation. They were: what are the stressors on our family, if you could remove one stressor, what would it be and why? Who would benefit from this action? Would this resolution allow other stressors to be removed?

Second on our list were Nana and Poppa, the medically experienced and smartest people we know to ask about science and medical processes. After they looked over the information you have now read, they directed us to start asking all the “What If” questions. At the top of our list is “What if Evan dies?” This question should be relegated to the bottom after all other questions have been asked and answered. So now we have Chemotherapy, Benign, Malignant, Discovery, Surgery, Vaccine, Interferon, and many others. Help us push that first question down to the bottom, send us you thoughts on “What if” Let us work through the possibilities and know that no question is too small or assumed to have been asked by someone else.

We meet with Evan’s medical team leader tomorrow to start the review process, but the real deadline for our review will be July 28, 2010 at 7:30 when Evan starts a series of MRI and CAT scans and when done with that we will gather for a team review.

Please keep us in your thoughts and prayers, and if you have the smallest idea of a question, please email us at evan@e-colemans.com Thank you and God’s many blessings to you and your family.

We're Back!!!!

The potential for the length of this blogpost might use astronomical units for accuracy of length. If we thought the previous weeks were full of information, they could not hold a candle to the amount of knowledge we gathered in 3 days in Dallas, and 1 day in Houston. I will for the sake of the reading publichit the high points here, share some pictures, and most importantly move Evan's email address to the top.

Evan can be reached at Evan@e-nevusnotes.com.

1. The conference was awesome. Tons of information, coupled with new friends, old friends and the laughter of children.

2. The Nevus Science Group had some answers. The literature we brought forward was not discounted, but embraced. Evan's case grabbed their attention, and they advised slowing down and getting the CGH testing done to find out what the mass is/was. PS it can be done on older fixed tissue, not just live tissue as we had been lead to believe. (The article in question presents a process for determining whether a growth in a GCN is either a benign nodule or malignant melanoma at the only differential level, the genetic level)

3. Statistcally speaking, in an amusing misapplication of math, If Evan had a dollar for every likelihood that he would not have his 2 conditions (MM and GCN) he would have $1,500,000,000,000 or slightly less than the US national debt. The risk being ~1 : 1.5 trillion!!!

4. The geneticist at UCSF confirmed that the CGH testing can be done from fixed tissue (already taken out). It takes 4-6 weeks. It can 100% confirm MM, or 85% R.O. MM.

5. The compassion, support, caring and love we felt in Houston was amazing. There are no quick answers to Evan's situation, and the rush to do something needed to give way to a slower more methodical and 21st century approach. It was given in a 19th century soothing bedside manner. We saw 3 specialists in 5 hours. Our question, "Do we have 8 weeks to get the CGH testing, or do we need to operate NOW because this has life and death implications in that time?" The answers: Oncology - take the time; Dermatology - take the time; Surgery - take the time, but if I see something that needs immediate attention, I'll tell you immediately.

In the coming days we will expand on each of the previous items, but for now you know the gist. In the meantime, we'll leave you with the advice of Matt Luke, a retired MLB player who has a nevus on his face. Survival and thriving in the world is based on a 4 level life. One level is family that loves you, encourages you and shares this with you every day. An second level is a close group of friends who accept you for who you are, or who you are not. The third level is finding the thing you are most gifted or talented and accept this calling with a passion. But the most important is the base level found in one's faith. While we hope that you have each found your faith base, we know that you are part of our extended famly lifting us up in thought, word and prayer.

Thank you