Hallelujah

I am a Christian. In first grade we were at recess one day. I was in line to climb the monkey bars. I had just dropped from the rings and landed wrong such that I came slamming down on the potato sized 'tan bark'; that's what we called it. The rip in the knees of my hand-me-down jeans (with double cuffs so that they fit) got even larger. No blood, so on to the next gravity testing device. The girl behind me asked what religion I was. I responded, "I am a Christian." Later, I understood that I was also a Mormon and was sub-categorized as to the type of Christian that I was. Later, in High School, I was ridiculed by teachers and students alike for being the wrong kind of Christian, or for believing in God at all. Through all this I felt in my heart that monikers don't matter. I always knew that God was there and that He loved me.
As Mormons we tend to express our faith quietly and feel our exhilaration inwardly. Our hallelujahs are in our hymns while our amens remain in our prayers. While in Connecticut, new members to our faith would often express their emotions in our meetings with the language to which they were accustom. Praise the Lord and Praise God would be loudly heard. I often wished that I had the courage to do the same. But my culture was different.
Today has been a really good day. My health score is 98.6. It started with a visit to a friend whose father miraculously recovered from a deadly brain cancer four years ago. But now it is coming back. We spoke of life and death, faith and hope, and ultimately we arrived at that unanswerable question, "why do some live while others die?" This is a hard question. Two soldiers in war: one dies one survives. Two teens in a car accident; two patients with the same disease. All were being prayed for. What does this mean to the faith of those praying? Why is it essential that we require cause and effect for all outcomes, whether good or bad? Then come the theological non sequiturs. "Why did God actively cause this death?" "Why did God passively allow this death?" and finally, "God is cruel, so I will reject Him." I truly do not understand these sentiments as they go against everything that I understand about this world and God. Bad things happen for both random and human controlled reasons. God understands and supports us thereby allowing us to then respond in a way that teaches us who we really are and what we are capable of. To do otherwise would rob us of our true purpose for existence. Yet, there are also times when we feel His influence leading us to miraculous moments. I do not now nor likely ever will understand why these outcome differences exist. I defer to His wisdom.
Then one day a few weeks ago I realized that I had it backwards. Death is not a curse, it is a blessing. Well, maybe not to the individual dying, rather to our world as a whole. Imagine, for a minute, a world in which there was no death. What would it be like? What defines a cancer cell? It is a cell that never dies; it is immortal. This would be fine if the cell was also perfect. On the contrary, these cells are corrupted. They do bad and unnatural things. It would be the same with immortal humans. They would remain selfish and vengeful. When the space and resources eventually ran low the stronger, more clever and cunning ones would subjugate and contain the rest for eternity. It would be hell on earth. Immortality only works with perfected souls.
In life we fulfill our purpose. In death we move on. It is the loved that remain who feel the pain. This may be why we choose not to talk about death; yet it is not a failure of faith rather its fulfillment.
Barbie commented this evening that she has noticed that my health has significantly improved over the last few months, even on chemotherapy. She is right. I feel wonderful most of the time. I tried to think of a reason why I am doing so well. My logic remained vacant. All I was left with was the strong feeling to consider God's continuing miracles in my life.

Praise God! Hallelujah! Amen and amen.

Kevin

NEOD001

Last Saturday I drove to the quarterly Northern California Amyloidosis support group held at the Walnut Creek Kaiser. It was wonderful to reconnect with so many friends and fellow patients. Charlesetta looks great after her bone marrow transplant. She's like me, first a heart then BMT. Dena had invited the chief science officer from Prothena to speak about their new drug in phase 1 clinical trials to target amyloidosis, NEOD001. THIS IS REALLY IMPORTANT. Never has a company targeted a drug to only treat amyloidosis. Most of our current drugs we borrow from the world of multiple myeloma. The main paradigm shift is that we can now focus, not just on the plasma cell, but rather the toxic light chain proteins themselves.

This is tricky. The light chain has two parts: The hypervariable region and the constant region. The hypervaiable region is like a snowflake; no two are alike. Concomitantly each patient's bad light chain is also different, likely arising from a random  mutation in this same region. As such, no one drug (antibody) could target them all. Additionally, if you designed the drug to attack the constant region, it would attack all of our antibodies, both the good, the bad and the ugly. Alas, how do we correct this conundrum? Aha! The cleverness of y'all. First you ask, "What is common to all misfolded light chains? Answer: they are misfolded. When normal good-guy antibodies correctly fold they hide, or physically cover a region that is common to all light chains. Misfolded bad light chains swing open, like a gate on a hinge and, Viola! the cryptic epitope is now exposed and visible. NEOD001 is an antibody that is designed and produced in manufactured cell cultures. It specifically targets this previously hidden amino-acid sequence, activates the immune response and the terminator arrives to clean up the mess. The toxic villains are neutralized and removed from both the serum and the tissues. The normal, good-guy antibodies are unaffected because this target sequence is still hidden deep inside; unavailable to the drug. Wow, what a concept.
One of our group members in currently in the study and shared that he seems to be doing just fine with no untoward effects. We thanked him for doing this for us.

This changes everything, if it works. 

Until now, I have always considered amyloidosis incurable. My current fight is not to be cured, but rather to shut down my plasma cells with drugs so that fewer light chains are floating around to attack my new heart. 

Now, by detoxifying the rogue light chains, it isn't exactly the definition of cure, but it feels the same. It is possible that we will stop using the term fatal with this disease. We will manage our disease and move on with our lives.

That would be nice

Kevin

P.S. I like that the Prothena logo looks like a gamma-globulin (antibody) designed to look like a "P"

The moment of ellipse

I have had more than a few people ask me recently, 'What does "The Middle of Infinity" mean?' Well, it has meant different things to me at different times. Because of this I often ask in return, "What does it mean to you? I have received many answers, most of which contain a common theme; something to do with being in the present moment. This definitely resonates with me. It is quite common to hear from almost all individuals that have experienced a 'mortality awareness event' to comment on how it changed their priorities and the perceptions of what is important in their lives. The little annoyances and pet peeves are cleared away like cobwebs and musky old drapery. The newly cleaned windows illuminate the saved soul and, for a time, they bask in the moment.
This is truly how I felt immediately when I woke up from the heart transplant. I was, and still am, a new man. The first blog that I wrote in my mind only two days later ended with the phrase, "Past regrets and future concerns have no place in the moment of presence. What I have learned from all of this is to cherish the present." Later, in a fictional story I penned called "Solids", Arthur, the protagonist, in a moment of epiphany realizes that, "The present is the gift we give ourselves."
For some reason I think elliptically and, as such, I also occasionally write in similar fashion. In our conversations, Barbie will say something which will cause me to think of something tangentially related, which will remind me of something else and so on. Five and a half thoughts and 3.791 seconds later I will begin verbalizing my new pondering; all previous frame of reference having completely evaporated. Barbie now has no idea of whom or what I am talking about. I often do this with words and phrases. Recently, at the produce market, I started bagging my groceries while the salesperson was still ringing me up. He jokingly commented that they would have to start paying me whereas I responded, "Well, it is the shortest distance between two points." It took him a second to realize that I was referring to economy of motion and economy of time. So what does any of this have to do with being present. (Now you know how Barbie feels.)
How long is a moment? Is it a zero dimensional point in the middle an infinite timeline lasting exactly 0.012 nanoseconds? For me a moment feels more like a focused connection during an event. As I recollect them I subdivide and compartmentalize them into my memory. When I remember getting married the moment that I cherished was not purely the few seconds when we were pronounced married, rather the moment began when I entered the room and ended when we left and my focus shifted. Some moments last seconds while others can go on for many minutes, even an hour. For me memories of moments occur in blocks of time. At least this is how I organize them. Somehow, now when I say cherish the moment I need to remind myself to cherish the uninterrupted focus. Then passively my mind shifts into elliptical gear and the train of thought is derailed. 
To cherish the moment means actively holding the focus on what matters most; the person present before you.
Over time it is easy to forget that epiphany manifest through salvation and fall into old habits. Then something beautiful is noticed, an amber and gold sunset, a baby grasps your finger, a hug from an old friend and the awe and wonder returns.

Kevin

Writing is Hard

Writing is hard. Writing a book is really hard. I mentioned to Barbie the other day that I believe that everyone has at least one book in them; the story of their own life. Some would write it as an autobiography while others would fictionalize it, as did Harper Lee with "To Kill a Mockingbird". What keeps everyone from writing a book? It's not easy. Well, it wasn't easy for me. I am sure that there are many writers that can sit down every day and bang out five to eight thousand words. The thinking part is easy. I wrote the book a hundred times in my mind. Yet the application of fingers (that is two index fingers) to keyboard has always been very difficult for me.
It took three years to write. And yet if I counted the number of days that I actually wrote it would be less than 50. When I switched from Revlimid to Velcade two years ago I suffered many new side effects. One of which was a lack of motivation. However, I could be guaranteed that at least one day a week that motivation would return. This occurred on Wednesday evenings when my high dose of steroids was in full force. This is when the hypomania portion of my drug induced bipolarity bubbles to the surface. However, the motivation produced is also accompanied by a certain degree of disinhibition which allows for a more soul-baring and uncensored writing.
My two greatest challenges in the process were first: how do I truly paint a picture with words that conveys the full emotional impact of the moment? And second: how do I fuse the retrospective narrative with the prospectively written blogs to maintain continuity while avoiding redundancy. In the latter, Barbie was immensely helpful. She read and re-read the manuscript offering invaluable input.
Then one day in January it was done. Writing is just the beginning. Now you have to get it published. Ten years ago I would have been at the mercy of literary agents and the big publishing houses. We now enter the world of self-publishing. In January I knew nothing of this world. It has now become my new hobby.
First yo go to the source of all useless knowledge (and some very useful knowledge: Google.) I found a book called A.P.E and downloaded it to Barbie's Kindle. It teaches the novice author how to Author, Publish and market (Entrepreneur) a book. I followed the steps, doubling back on certain blind pathways, and ultimately decided to have the book self published through CreateSpace, a subsidiary of Amazon. They provide a publish-on-demand service such that the physical book is only printed when someone orders it through Amazon. What a concept; no inventory.
First, though it had to be edited. When my editor, Sarah Bringhurst Familia, sent me revised manuscript, it was bleeding red on every page. Yet, as I clicked and accepted her changes, it became a real book.
Next was designing a cover. This was really fun. I found a website www.99design.com that, for $299, will create a contest where designers compete to build you the perfect cover. Ultimately I had 19 designers submit 64 designs. The last day of the contest Barbie called me to say a new design came in that was awesome. I immediately knew that this as yet anonymous designer had captured my vision and that we had found our cover. In the end it was a graphic design professor from the University of Viterbo, near Rome, Italy, that one the $200 prize. The longest process was the internal book design done by CreateSpace to format the interior. And now it is done.
I have learned so much in that last six months about writing and publishing. We live in a world where so much more is available to us because of the internet. Seemingly impossible things are not just possible, but relatively easy. No one needs to forgo their dreams anymore. You just need to know what question to ask and to whom.
The book is now out there; for some I hope that they may find in it comfort and hope in their struggles. For others, I just hope it is a good story. For me it is a testament to miracles.

Kevin

Bashir's Invitation

Recently, Barbie and some of the family and I travelled to Southern Spain to the Costa del Sol in Andalucia. I was stunned at the beauty of the mountainous terrain as it descended into the beautiful Mediterranean Sea. The waters of 'Middle Earth' are met with numerous small towns; each with their own unique charm, Estepona, San Pedro de la Alcantara and the hermosisimo, Marbella. We thoroughly enjoyed our daily excursions as we met people who were welcoming and kind to us. I love the Spanish accent. It sounded so clear in my Argentine ears.
I had a goal to claim Africa as a continent that I had set foot in. We were close enough to see the mountains of Morocco in Northern Africa while sitting on the couch in our villa at the Marriott. To get to Morocco was actually quite simple. We drove 45 minutes to Tarifa, a small town just past the Rock of Gibraltar. Tarifa forms the corner between the Atlantic and the Mediterranean. A fast ferry then carried us to Tangiers for the day.
Our tour guide met us at the dock (If you go to Tangiers on FRS, get the tour. It's cheaper and a much better way to see the city than walking.) He introduced himself as Bashir in English that was quite understandable. He seemed quite laid-back and unaffected. I liked him. He could so easily engage in such casual banter that I could envision visiting with him over a ceviche tostada sitting on the sea wall of the Pacific Beach boardwalk in San Diego.
He described the city as we drove in and around it. Eventually he took us to the open market in Medina and finally to the Casba for lunch. While walking he made reference to the Moroccan flag with its five-pointed star, each point representing the five pillars of Islam. As he later described the minarets and the call to prayer five times a day, I asked if the frequency was also connected to the five pillars. He did not know if they were correlated.

After lunch, we were requisitely funneled through the shops of hard-sell vendors of rugs and spices. (These likely subsidised the lower cost of our ferry tariffs.) Finally, we were back on the streets of the Casba, mercilessly hounded by street vendors that would follow me for blocks.

Trying to escape, I walked down to a mosque decorated with geometric green and white tiles. Somehow I instinctively knew not to enter. I stood at the door gazing in. In a moment Bashir was next to me. As I turned toward him, his face got very close to mine and with all seriousness he said, "you seem to be a very wise man." I didn't know what to say. Then he continued, "do you want to go to hell or to heaven?" I answered, "To heaven, of course." He then proceeded to urgently invite me to study Islam. He reasoned that he could not stand before Allah at judgment day and admit that he did not share the truth with me when he had the chance. It was deja vu. This was a rationale that I had also used when teaching Christ's Gospel in Argentina. We then engaged in an absolutely lovely discussion about our beliefs in one God. He said that Muhammad's message was not just for Islam, but for the entire world. He said that Jesus was also a prophet. I said that, for, me Jesus is the Son of God and that his teachings were for all people as well.
Was I offended at his invitation? Not at all. I was honored that he saw in me someone who could listen openly to something that he cherished. I was struck that our differences paled in comparison to what we held in common. We both have the same goals. Live honorably by studying what we are taught in our scriptures that we may return to a loving God.
I thanked him as he left us at the dock to again return to Spain; to Europe. As I sat on the ferry I reflected on the courage he had to share with me his faith. And then I thought, when you value something so much, why would you not share it?
I learned much from Bashir that day.

Kevin

Hard Questions -- Good Questions

Social media allows for the interactive dissemination of information without boundaries. The possibilities then extend beyond the limits of  our personal understanding and credulity.  One must just find the right question.
For many years two questions have plagued me. Both relate to the process and results of a stem cell transplant (SCT) or to as it is sometimes referred, a bone marrow transplant. I have read numerous scientific articles describing the outcomes of SCT for AL amyloidosis but found that many of the reports did not differentiate the survival rates, or even include data regarding how many patients, and for how long, were spared the need of continued use of  chemotherapy after their treatment.
Since my SCT failed, (at least based on my blood tests that immediately showed not only no decrease in my light chain levels, but rather a doubling of those bothersome proteins,)  I was also keen to understand why at the cellular level.
I have learned that often the best source of discovery regarding a rare disease is from the people that live with it every day. So I went to that repository of combined life experience and wisdom, the patients. In the past I had joined an online amyloidosis support group and recently felt to reconnect there. As I began to read many of the comments, old personal questions began to resurface. Finally today I wrote the following.

There are two questions to which I wish I knew the answers. First, does a SCT fail because the Melphalan fails to kill all of the plasma cell clones in the bone marrow? Or is it that survivable clones are re-implanted with the stem cells? Second, my goal for stem cell transplant was to be free of chemo for at least two years. (I don't like taking dexamethasone.) What percentage of patients that have a SCT remain off chemo for at least two years? 

I was encouraged at the number and caring quality of the responses that I received. But three in particular had important insights that taught me things that I needed to learn.
First was from a man whose experience was  a major reason that I wrote the questions. I had met some amyloidosis patients that had complete responses from their SCT, they were successes, yet their oncologists still kept them on chemotherapy, just in case. Tim wrote the following.

I had an SCT in July of 2011 at xxxxxxxx xxxxxxxxx (had consults at mayo as well). I had VelDex as a front line treatment prior to  transplant and achieved near complete response after transplant. My doc wanted to see if we could get to complete response so we resumed VelDex in November of 2011 and continued treatment until June of 2012. I was getting really run down from chemo so voluntarily stopped after a consult at mayo where the specialist there said that they would not have treated at all and would have just waited to see. Flash forward nearly a year and my numbers remain stable and I'm hoping to be chemo free for at least a year. I feel good off the chemo teat and will be wary of getting pushed back on in the future.

Another woman described her story with the preface that she generally did not respond on the site because her story is 'discouraging'. Yet her story was my story. Her SCT failed and she remains on chemotherapy.  I felt connected with her and shared my hope that new treatments are already available and on the horizon. It is a good time to be alive.

But the one that affected me the most was from woman who also failed the SCT and her light chain levels remain elevated. But her doctors are not treating her with chemo. This is the question that Barbie and I have been asking for four years. Is the chemo adding days to my life? What would happen if I stopped it?
This woman, named Jan, offered the following after describing her numbers, which were my numbers; except that she is not on chemo.

But as someone said, we are all different. And for us as individuals, the
statistics are meaningless. We either had a response or not. We either need more
treatment or not.

I totally agree with you. I really would like to avoid chemo if I can. So far I
can...I live from 3 month check up to 3 month check up, always knowing it could
change. Hoping the best for you.

Ultimately, Muriel Finkel, the site administrator found the answers through her connections at the Mayo clinic.

To question 1: The SCT doesn't always kill all of the bad clones in the bone marrow.

To question 2: 80% of patients with a successful SCT remain off chemotherapy for at least two years.

This news was very encouraging to me. Generally, scientific studies speak of success in terms of overall survival (who's still alive at 5 years) and disease free progression (who has no evidence of disease progression at 5 years) These numbers refer to quantitative success. They often don't report on whether those patients were on adjuvant (or continued) chemotherapy, (qualitative success). This is what I want to know as this is what makes our days miserable while we wait to add to the survival statistics. Living a long time is great. Living a long time off chemo is outstanding.
So what's next? For now I will remain on chemo. But I am encouraged by those that have chosen a different path having asked these hard questions. Not with just their words, but with their minds and bodies.

Kevin

Where do we go from here?

The first week after receiving the diagnosis of amyloidosis is the most confusing. Everyone that knows you wants to help. Suddenly dozens of voices are offering advice and it is difficult to see which way to turn. These are good voices of people you trust and who care about you; but you just don't have all of the facts yet. Not to mention, you feel like crap.
The internet only makes it worse. Initially, you find sites that only speak in vague generalities and appear to be copy and pasted from some unknown literary progenitor. Finally, you find and abstract written in medical jargon that offers more details, but you cannot read the whole article unless you pay for it.
Your primary care doctor make an appointment with a hematologist/oncologist, but they have only had one prior amyloidosis patient in the last 18 years. Besides, how do you know what questions to ask them? It is a very confusing week. It was for me and I am a doctor.
My sister works with cancer patients and has vast experience with chemotherapy and bone marrow transplants. I told her that my Kaiser doctors had suggested that I go to the Mayo Clinic for my work-up. Her colleague, a professor at the University of Washington said, "No, he needs to go to Boston University, they are the best at treating amyloidosis." I felt torn. Do I go back and tell my doctors that they are wrong in their recommendation? Or do I trust them implicitly?
Finally, around week two - three a groundwork is laid. The smoke clears and you find your advocates. The doctors, nurses, social workers and caregivers who light the first part of the path so that you can move forward. A plan is proposed and it feels right. You take the first step.
Every amyloidosis patient has a story of their delay-in-diagnosis. Unfortunately, this is the rule, not the exception. It is an uncommon disease with common symptoms. It is not easy to diagnose. Someone has to think about it. Notwithstanding, once diagnosed, and properly verified. We need to forget past delays, miscommunications and annoyances and move forward. This need no longer be a fatal diagnosis, and to the doctors that recommend getting our affairs in order we could respond, "with all do respect, I suggest the same for you, doctor." Doctors are terrible at predicting when someone will die if it longer than a month.
Where you receive your care is an emotional decision affected by many factors: the burden of travel, family issues, work issues, money, cost of care, insurance coverage. These limitations are set against the understandable desire to get the best care that we can. It truly can be a matter of life and death. What I have learned as a physician, and now as a patient, is the power of the team. No one person can be at the top of their game 100% of the time. With a team, each person specializes in certain aspects of the process so fewer things are missed, Teams tend to use protocols and 'best practices'; learning from the successes of others. Teams are not necessary for all diagnoses, just the really complicated ones. Often these teams are referred to as centers of excellence. They are everywhere.
Amyloidosis centers of excellence are found throughout the country. Although, we all owe a great debt of gratitude to the pioneers in our diagnosis that have laid the groundwork, done the research and written the papers so that all can learn from their collective experience. The Mayo Clinic and Boston University and others, stand out as giants in this area. We owe them our lives. Those who find new treatments for multiple myeloma also help immensely as so many drugs that start there transfer to us.
All of this knowledge, available to all and administered through centers of excellence can truly assuage our anxiety such that we know that the place that we are at is the right one for us. This is not a time for doubt, but for trust.
I no longer consider my diagnosis fatal. Yes, I will die and yes, it will likely be from complications of amyloidosis. But I am still alive and I shouldn't be. Since each day is, for me, a gift, the need to live a long life is no longer the goal. Rather, the goal now is to learn every day and to give love through service every day. This is where we go from here and the journey continues.

Kevin

Fruit Flies

Yesterday the operating room where I work suffered a four hour delay. Someone had left a bag of fruit in the break room over the weekend. A few fruit flies were seen nearby. However, when the bag was finally found and opened thousands of fruitflies escaped. (I see a metaphor here to gossip, but with a pillow and feathers; but I digress.) It was clear that it would take a few hours to clear out and sterilize the area. We were scheduled to do three radioactive seed implant cases for the treatment of prostate cancer.
Wanting to ascertain the status of our day, I hovered near the main office where a high level pow-wow was in progress. The head anesthesiologist was commenting on how cases using implants would need to be rescheduled because of their heightened need for absolute sterility. Still just outside the door, I commented to him, as an aside, that there existed no bacteria in creation  that could survive on our radioactive implants.

A moment later, finally inside the office and the conversation, the OR director looked at me and queried, "Now, Kevin, you guys are using implants, correct?" Clearly, wanting to make the distinction that we were safe I answered. "Yes, but bugs don't like radioactivity.
Without missing a beat he threw up his hands in a feigned sense of importance and exclaimed, "but what about Spiderman?!"

Kevin