Thursday, August 30, 2012

Summer of our Bliss-content

The marathon seemed to begin with 2nd of four drives through Battle Mountain, NV to bring Barbie and her patient candidate, Elaine, for her practical dental hygiene boards in Orem, Utah. On April 21st her patient was rejected and she, with the confidence of a Navy seal, kept her wits, found a new patient and passed the exam. Two days later, on her 19th birthday, Caitlin accepted Benjamin's Solari's proposal of marriage and set the wedding date for the end of July. A week later we closed on the Provo, Utah house; the only house that my grandparents, Harold and Catherine Anderson, ever owned. A house that they purchased in 1937 and was sold in 1962 when my Grandmother died. This house marked my first recorded memory connected to a fixed date at the age of two and a half.
Three weeks later, Barbie and I flew to Atlanta for the American Urologic Society's annual meeting. It was the following week that two more extraordinary things happened. Barbie turned 50 on May 27th and graduated for dental hygiene school and was chosen by her class to be their voice on this momentous occasion. Her graduation gift ended the 30 year tradition of practical family cars as this one is not kid friendly.

The following day we flew to Pennsylvania to visit Samuel and Michelle at Penn State and, while there, made side trips to Connecticut and the Pentagon for lunch. We even got to observe Samuel performing acoustical experiments measuring cavitation bubbles in a water tunnel.
A week later we flew to Alaska and spent a week visiting with my brother David and his wife Joel'lene. The week culminated in my honored opportunity to speak to the medical community there in remembrance of one of their fallen colleagues and a fellow amyloidosis patient.
Then the preparations began in earnest for Caitlin and Ben's wedding. A thousand details, all with a deadline of July 28th, miraculously flowed together to create that magical moment when your last child and youngest daughter melds her life with a new man. May he love her as I always have and always will. He married an angel.
While they honeymooned in Kauai, Barbie and I prepared for our family vacation to the Marriott Newport Coast villas. We also celebrated are 30th wedding anniversary on August 6th with a trip to Santa Clara for my four year anniversary heart biopsy and pressure studies. Not only did my heart show no rejection, additionally we found that my cardiac index is 3.0 (very good) and their is no evidence of amyloid deposition. We also celebrated by visiting the temple in Oakland where we were married 30 years before and then relaxed on the beach in Pacifica until we picked up Ben and Caitlin from the airport.
With our our four children, now all married, we met for the first time as the complete package in Southern California. It was a wonderful week as we all relaxed together before the mounting responsibilities of September began.
We spent every day in the warm southern California surf and caught some awesome and totally gnarly waves. This time I did not fracture a rib, I only tore a cartilage at the costochodral junction of my right 6th rib. It seemed to hurt less when I got back in the water to shoot some tubes on my new Boogie Board that Barbie got me for my 53rd birthday.
We returned home in time for Barbie to direct music for our Lincoln Stake Conference and celebrated a quiet birthday with an enchilada dinner ( something I have often requested since childhood.)
Two days later we drove to Utah for the fourth time to move Ben and Caitlin into our new house in Provo and fix it up for the extended Anderson family open house which we had last Saturday. It was a treat to have my Mom (age 78), my Aunt Gloria (age 88) and my Uncle Harold, my dad's brother (age 89) there with many of their children, grandchildren and great grandchildren at the home where it all began. So many stories and memories were shared. As cousins roamed through the house, Patty, Harold's second daughter shared a tender moment. She pointed to the upstairs landing and recalled that she and her older sister Karen, ages 7 and 8 respectively, were sitting there in April of 1959 when an emotional father slowly ascended to inform them that their grandfather had just died downstairs. It was their first memory of seeing him cry.
Monday we waved goodbye to Ben and Caitlin on the steps of our new home as they begin their new life together. It was bittersweet.
Today Barbie started work as a dental hygienist and I restarted chemo after a month vacation. We are back.

Last Saturday, as the my kinfolk were leaving, my cousin Doug thanked me for the wonderful day of reminiscing. I replied that, since my brush with mortality, I have become very interested in investing. The only commodity that matters are relationships; and the only investment worth making is in building memories. In our reunion we shared lost and individual memories with each other; our common home as the catalyst. The present moment captured past experiences for future generations. I believe that this summer had an incredible return on investment as we lived our lives to the fullest and reaped hundreds of moments of joy which now serve to further enrich our portfolio of cherished memories.

Let the next generation begin.


Friday, July 6, 2012

Deconstructing Amyloidosis

Each of us surviving amyloidosis, in ourselves or a loved one, can help the rest of us in often profound ways. Muriel Finkel is the maven of information. She supplies us with incredible articles and access to information that define the cutting edge progress in this disease. I say thank you to her with all of my heart. Her most recent update includes the current report by the team at the Mayo Clinic on 10 year survival data on 74 patients that underwent an autologous SCT from 1996 to 2001. The results showed that 43% of patients lived beyond 10 years. Some might scoff at that number and say, "Only 43%, that's dismal." Of course, context is everything.
Glass half empty, eh? (Actually, the glass is always full; the sum of the liquid and gas equals one.)
Ten year survival is a wonderful thing and more common than I previously thought. The article then goes on to talk about significant baseline characteristics that might predict a better prognosis. There were four, but one stood out, the number of organs involved, although heart septal wall thickness also contributed. Post therapy lowest light chain level and degree of response also correlated. I, of course, inserted my numbers, both baseline and post SCT and I clearly will not be in the long term survivor (LTS) group. Yet, for me the glass is still full. I have had a heart transplant, so it is apples to lacrosse sticks. As such, I have designated myself the current president of my own long term survivor club. With continuous chemotherapy I have only succeeded in pushing my light chains to the median level for the non-LTS group.
What was most interesting to me was what I learned from the introduction to the article. Sufficient to entice me to attempt to translate it into common American English.

I refer to the article below and reference it so that I may quote verbatim the first paragraph.

Ten-Year Survival After Autologous Stem Cell Transplantation
for Immunoglobulin Light Chain Amyloidosis
Stefan Cordes, MD, PhD1; Angela Dispenzieri, MD1,2; Martha Q. Lacy, MD1,2; Suzanne R. Hayman, MD1,2; Francis K. Buadi, MD1,2;
David Dingli, MD, PhD1,2; Shaji K. Kumar, MD1,2; William J. Hogan, MB, BCh1,2; and Morie A. Gertz, MD1,2
Cancer 2012

Systemic immunoglobulin light chain amyloidosis (AL) is a plasma cell dyscrasia in which monoclonal light chain protein aggregates and deposits in tissue as amyloid fibrils.1-3 Only a relatively small percentage of Ig light chains are amyloidogenic,as evidenced by the finding that AL amyloidosis occurs in only approximately 6% to 15% of patients with multiple myeloma. Amyloidogenicity is related to structural features such as the light chain isotype and the variable subgroup. The folding pathway of such amyloidogenic light chains is believed to pass through partially folded metastable conformations that aggregate either 1) at sufficiently high concentrations or 2) under specific environmental conditions."

Most of us know that AL or light chain amyloidosis is caused by the deposition of large quantities of abnormal proteins (light chains) that somehow band together in bundles and damage tissue. There are some pearls in the paragraph above of which I was previously unaware.

1. " Only a relatively small percentage of Ig light chains are amyloidogenic." 

It appears that a mere excess of light chains is not enough to cause the condition of amyloidosis. The evidence is in the following statement that only 6%-15% of patients with multiple myeloma show a clinical presentation of amyloidosis. The rest of the patients with multiple myeloma make massive quantities of light chains, but not the toxic type.
When a plasma cell goes rogue as either a cancer (Multiple Myeloma; the plasma cell clones don't die) or a dyscrasia (AL Amyloidosis; the cells die, but make toxic proteins before they do.), they make excess copies of a single protein. Somehow, this particular protein misfolds and combines with other proteins to create a fibril. This is a random event.

2. "Amyloidogenicity is related to structural features such as the light chain isotype and the variable subgroup."

Antibodies are incomprehensible in their awesomeness. 

The light chain is the short, lighter blue and orange protein shown on the outside of the bigger and heavier chain of amino-acids that make up this particular protein or immunoglobulin. The magic occurs in the orange part or variable subgroup. From the time we are born we have white cells that make and store millions of unique antibodies that differ in the orange tip region. A controlled fire of mutations allows for this library of antibodies, most of which may never be used. Usually, mutation is bad, however, here it is necessary for our survival. When we get an infection or any foreign protein (antigen) enters our bodies, millions of white cells, (B-lymphocytes) each presenting their own unique antibody go up and 'taste' the invader. Most don't recognize it and move on. However, one will have a unique shape that will allow it to stick, like a lock and key. An alarm goes off and the B-Cell becomes a plasma cell and dedicates it's life and the life of all of it's daughter clone cells to making that single unique antibody with it's unique light chain.
I am no expert, but it might be additional factors that cause the overproduction and misfolding of our unique light that make ours toxic and others not. This part I don't understand well. In other words, just misfolding and making fibrils out of any light chain won't cause amyloidosis. It may be a specific clone that is required. I would imagine that if you did amino-acid sequencing to get a fingerprint of each of the variable region our light chains for those of us that have this disease, they would all be unique. The commonality of the presentation is more likely related to characteristics shared by the final products; first, their preferred soil. Some fibrils have shapes, bonds and electrical polarities that favor heart muscle, while others prefer to clog up kidney tubules. Kappa light chains are monomers or single molecules, while Lambda are dimers, or double molecules. This means that they start out twice as big and this might effect there propensity for kidney damage. Regardless of the initial clonal type, the final common pathway is tissue damage.

3. "The folding pathway of such amyloidogenic light chains is believed to pass through partially folded metastable conformations that aggregate either 1) at sufficiently high concentrations or 2) under specific environmental conditions."

Here the authors teach us that it is not just the high levels of our light chains floating around, but the environment around them. Tissue types, other proteins, cellular communications: many things may impact the toxicity, independent of our light chain levels. We all know that each of us have a different range where our light chains levels live, both on and off therapy. Yet, we cannot compare numbers like PSA for prostate cancer; because we cannot compare our respective environments. We do all agree that lower is better, though.

4. The second paragraph in the introduction has a line that states, "One of the rationales for the efficacy of such treatment is the remarkable observation that it is the growth of fibrils, in contrast to existing fibril load, that inflict cellular damage through transient defects in the cell membrane."

This was a new concept for me. Essentially, I interpreted this as these nasty protein fibrils do there damage as they break through the window, not while they vegetate inside the cell as couch potatoes. Therefore, the target of treatment is to prevent new break-ins rather than remove the loafers already living there. The blood tests that measures our light chains only detect the hooligans on the prowl, not the ones already in residence. That is measured by organ health, and it's ability to heal itself once the break-ins are curtailed.
We have SCT and chemotherapy the reduce our plasma cell numbers and prevent them from making more light chains of one particular flavor.

I hope that this explanation is clear enough to add to your understanding of this article and our shared lot in life.

May you all join my LTS club.


If I have erred, please correct me so that we may all learn.

Friday, June 29, 2012

Remembrances of childhood

The Brotherhood of Art

Barbie and I just returned from a wonderful week in Alaska. I have wanted to visit my brother David and his wife Joel’lene since they moved there 19 years ago. Recently a physician from Alaska passed away from amyloidosis. We had corresponded via the blog and email for some time. In memory of Dr Neubauer, I was asked to present Grand Rounds for the department of medicine at Providence Hospital in Anchorage. I spoke on the topic of the doctor as a patient; a topic about which Dr Neubauer both wrote and spoke.
I would describe our week as a journey of both joy and discovery in which both can be applied to experiencing the beauties of Alaska and reconnecting with my brother, David. Of all my eight siblings, I am most like David who is two years my senior and two inches taller. I have always looked up to him. Of the eighteen most important decisions that I have ever made that have shaped the course of my life, David, and my oldest brother Harold, were involved with the first six. As the 4th child and 3rd son I was the great beneficiary of their experience. They forged the trail ahead that allowed me to take a more direct, pain-free path. They were my buffers. They got me all of my jobs from age twelve through college; advised me on which classes or teachers to take. We even dated sisters at the same time. (That never really worked out.) I owe my early successes in life to my brothers. But then, at age sixteen, David went off to college and we were never really together since that time. Between college, overlapping missionary service, and job moves, we haven’t seen much of each other over the last 37 years.

As David and Joel’lene gave us the grand tour of south central Alaska I could sense how who you marry and where you live truly can shape who you become. They love it there, now I understand why. And yet, after 37 years, you can’t shake genetics and early childhood influences. Barbie also discovered how similar David and I are in our sense of humor and the sub-conscious tendency to correct factual deviations on diverse topics. Despite our common upbringing, I learned that we have very different memories of our early childhood. I have always felt, and still do, that I was blessed with wonderful parents. However, my father did have a temper. What very young children most crave in life is consistency and predictability. It is easier to understand now the pressures and stress that my parents had in raising nine children, but not when you are six. And yet, why are my memories different? I believe that painful memories are punctuated and easier to retain while many of our happy memories relate to the mundane activities of play and exploring. It may be that we choose which memories we will use to build the story of our childhood based on the value systems that we formed and locked in by age ten. In any painful encounter we subconsciously or consciously decide to avoid that behavior in our own future or to adopt it. This is also true for the positive and constructive lessons that our parents teach us. I decided very early that I wanted to trust my children to make their own decisions the way my parents trusted me. I valued my parents’ trust and the freedom that it gave me.
Yet I also must admit the fact that all parents make mistakes. Parenting is on the job training and the steep learning curve is more evident to the oldest children. I know that my older siblings suffered more than I did. By the 4th child parents have learned a few things, and as the 4th child it is easier to hide from both a parent’s anger and my responsibilities. I became very adept at avoiding both work and pain; something that my older siblings are happy to remind me.
David shared a memory that is still difficult for both of us. At age 9 I asked for a trumpet to play in the school band. After certain coaxing, my parents relented and rented to own a trumpet. What I did not know until years later was that David, two years earlier, had made the same request and was denied. He felt that this was unfair; and it was. I have always felt guilt since I learned this. However, a child of nine cannot comprehend the complexities of context and timing in adult decisions. To the adult it is a simple pragmatic decision. To a small child, a seemingly small decision is huge and can greatly impact their developing value systems. This scared me as a parent wondering how many absent-minded decisions that I might have made in the moment that had life-long impact on my kids. Yet as a whole, I have many more happy memories of my childhood than painful ones. I have always seen myself as blessed, never as a victim; because not a day has gone by since my memory began that I have not felt loved.
It was so wonderful to reconnect with the brother that I looked up to and still do. He is a really good man. The last day we were there he was constantly watching the water on the lake, waiting for a good wind so that we could jump onto the small catamaran he has and serenely sail across the lake. It just felt good to hang out with him again; no cares in the world except for naming the birds that we saw. It was peace; it was joy.
As we flew home another memory presented itself. There was a moment when our band of brothers was briefly reunited in the same city during my last year of college. David had an art class that required him to create an original work in mixed media. David cut a 2 x 8 foot piece of wood, coated it with a white sticky coat of enamel and leaned it against a fence. Harold, David and I then took turns throwing clumps of dirt and snow, sticks, trigs and tiny pine cones at the upright board. We even drove his car over it to leave tire tracks. In the end we reveled in our collective creation as we thumbed our noses at the ‘art’ in the work that stood before us. We laughed and had such a good time and bemused ourselves as to how it would be received by his professor. A few days later we were all shocked to hear that David had scored an “A” on his installation.
Childhood can be very messy. Too often adults expect us to think like them. They have forgotten that before the age of ten we have yet to fix our sense of shame and inhibition, while this is important to function in adult society, it also ultimately dampens our creativity. The world of the child remains wondrous and our adventures endless. We look up in confusion at cynicism and despair. However, we also sense, at times, great beauty and safety in the love we feel.
Maybe David’s professor saw something that we did not; something lost, again found. Reflected in the mess of mixed messages that stuck to the board of our forgotten youth was a moment of our innocent past as we had played together so many years before. We have since cherished that moment. From the mud and dirt, sticks and stones of our childhood we ultimately create a life of lasting beauty; of course, art, like beauty, is in the eye of the beholder.


Thursday, June 14, 2012

It's all about Barbie

Can I just say, "I am so proud of Barbie." Most people don't realize how hard she worked over the past three years to accomplish her goal of becoming a dental hygienist. I do. Nobody in the history of thinking spent more effort to learn such things as she thought were foreign to her nature. Barbie's gifts lie in the area of music, not the metric system. However, she was determined not only to pass her classes, but to do well; and she did. I have always felt that nothing make you feel more alive than taking risks. She dove into the unknown in the fall of 2008 as she struggled with the decision to begin a new career path as a dental hygienist.
Now, at this end, her success brings her new confidence. Clearly, her fellow students recognized her tenacity in choosing her as the class graduation speaker.
In her own words:

Graduation Speech

If asked 16 months ago what a dental hygienist did, we might have said something like, “They clean teeth, take x-rays, and sometimes give anesthesia.” Now if asked that question, we take a big breath and say, “How much time do you have?” Looking back at that first day we arrived for orientation, we left with a suitcase full of books, an armful of instruments and a laundry list of odds & ends you would never find in a scavenger hunt. As we hauled them to our cars we may have had the thought, “What have we gotten ourselves into?”  Little did we know that the months ahead would require a huge amount of sacrifice: lack of sleep, lack of family time, lack of entertainment and lack of everything fun. Our dental hygiene school became our new home and at times we felt as though we never left. We stopped exercising regularly, stopped eating well, and frankly worried that our friends would abandon us since we had no time for them.

Sacrifice is the act of giving up something valued for the sake of something else regarded as more important. Surviving and graduating from this program became our utmost valued and important act.

Almost 4 years ago my husband was diagnosed with a disease that had damaged his heart. At the time, I was working in a dental office as a dental assistant. I had wanted to go to dental hygiene school at some point but never found the opportunity.  With his diagnosis we both needed to quit our jobs. I became a full-time caregiver and he became a full time patient. He received a heart transplant, bone marrow transplant and many chemotherapy treatments as well as illness that come with a low white blood cell count. His life expectancy was less than desirable such that he usually wouldn’t buy green bananas.  It became clear that I might spend a significant portion of my life without him. He strongly encouraged me to follow through on my desire to become a dental hygienist as that would ultimately give me both purpose and security in whatever future we found ourselves. Initially I feared that such an effort would require me to sacrifice what I valued most; time with him while we still had it. Yet, the answer was clear and I immediately enrolled in school to begin my pre-requisites.

School took time away from my husband. I could no longer accompany him to doctor appointments, care for him when he was sick or overall just spend time with him. And I worried that the time I spent away from him could take away from the time I might have left with him. As he fought to stay alive, I fought to graduate. Now I might not be able to give him a heart transplant but I can sure clean his teeth!

C. S. Lewis once said, “No great wisdom can be reached without sacrifice.” 

In these last 16 months, we have grown abundantly to become who we are now. What was a suitcase full of books became a brain full of knowledge. An armful of unfamiliar instruments became necessary tools. And those laundry lists of odds and ends were the finishing touches. And with all of that, we have proven to ourselves, our instructors, our friends and our family that the sacrifice was worth it. As Joe would say, “You can do it.” And we did. Congratulations class of 2012!
Thank you.

She has secured a future of service to others as her new skills will be used to help patients and allow for financial independence. But for me, I also am happy that she is done because I get her back. Now it's time to begin making new memories together in earnest. In this month alone we went to Atlanta, Pennsylvania, Connecticut and the Pentagon and this Sunday we fly to Alaska. 
This is just the beginning.


Thursday, May 17, 2012

Too much for one blog

Ben and Caitlin
I increased my dose of steroids today and had the epiphany that I need post some blogs. Yet, so much has happened in the last month that I can't fit it all into one post. Suffice it to say that someone up there must like us as we have seen much joy recently.
First, Caitlin is in love with a wonderful young man named Benjamin Solari.They met (for the second time) on the day he gave his homecoming talk from his mission. He served in Spain (habla espaƱol). They began dating at BYU and got engaged on Caitlin's 19th birthday. We are so excited. Ben is a wonderful guy and is perfect for Caitlin. I know what you are think. How is it possible that our baby is getting married? Yes, Barbie and I find it hard to fathom such an idea, but love does not happen on a schedule.
To make things better, Barbie is done with school so she and Caitlin can plan her dream wedding together without the stress of classwork and studying. They are having a blast. The wedding is planned for July 28, 2012, in the Sacramento Temple. This will occur in the middle of a very busy summer. Our traveling schedule begins this Saturday when we fly to Atlanta for the American Urological Association annual meeting and will end when we move Ben and Caitlin into the house we bought in Provo. (No, we are not moving, we just had an offer that we could not refuse.)
I had an interesting call last Friday from Dr Schrier. He is my oncologist at Stanford and he never calls. I had sent him a copy of my most recent Kappa light chains level which was the lowest that it had ever been; 39 mg/l. (I parenthetically added that the extra steroids I was taking to prevent post-herpetic neuralgia from my recent episode of shingles may have further kicked down the number.) He completely surprised me with the call as he proceeded straight to the point. "What is your treatment protocol?" he asked. "I am on weekly Velcade; four weeks on, one week off and dexamethasone." I said. "What dose of dexamethasone." then followed. I was caught. I had once been told that 20 mg was as good as 40 mg and opted for the lower dose to avoid side effects, but it had become clear recently that I am very dose sensitive to steroids regarding the lowering of my numbers. "I taking 24 mg." "You need to increase it to 40mg." He said without hesitation. "I am very concerned about you." he added. Wait, my numbers are good, I thought. Then I realized what had happened. "This is because of another patient that showed amyloidosis in their new heart." "Yes," he responded, "I'd like to get your numbers as low as possible; even start you on a 3rd line chemotherapy." I wasn't really ready for this. But I said that I would push up my steroid dose (beginning today) and that we could go from there.

Obviously, I will need to expand on some stuff I alluded to above. I promise I will promptly post again to do due justice to those events.

I had a thought last week driving home. Not a new thought, rather an idea that occasionally inserts itself as I consider my current station in life. I can't imagine a happier, more blessed man than I am. My life is so good in so many ways. When I try to discover why this might be, I cannot not escape the realization that the bulk of my daily dose of joy comes from my relationships with others; friends, family and God. I could not be happy alone.
So I thank you, all of you.

P.S. The book is close to being done. I just need to figure out how to get it edited and published.

Thursday, February 16, 2012

I was there first; I was their 1st

Heart transplantation and cardiac amyloidosis: Approach to screening and novel management strategies
The Journal of Heart and Lung TransplantationVolume 31, Issue 3March 2012Pages 325-331
Brandon C. Varr, Michaela Liedtke, Sally Arai, Richard A. Lafayette, Stanley L. Schrier, Ronald M. Witteles
Limited data exist regarding screening methods and outcomes for orthotopic heart transplantation (OHT) in cardiac amyloidosis. As a result, uncertainty exists over the best approach to OHT for cardiac amyloidosis and for the timing of critical post-transplant therapies. This article reviews 6 patients who underwent OHT for cardiac amyloidosis at the Stanford University Amyloid Center from 2008 to present. All patients with light-chainamyloidosis received chemotherapy in the interval between OHT and autologous hematopoietic stem cell transplant. Five patients remain alive up to 25 months after OHT, without evidence of recurrent cardiac amyloid deposition. A novel strategy of OHT, followed by light-chain suppressive chemotherapy before autologous hematopoietic stem cell transplant, is feasible for patients with light-chain amyloidosis.

This was very cool to read a scientific article where I was one of the subjects. I am celebrating 3 1/2 years today (42 month follow up); so I rode my bike to work.
The Velcade is working great.
Life is good, really good.