Sunday, August 18, 2013

Between Birthdays

Thank you, Shane

I get to celebrate two birthdays. Friday, August 16, was my heart's 5th birthday while tomorrow, August 19, the rest of me will be 54. I feel great. No, I actually feel better than great, but I would need to invent a new word to describe that; .... I feel euphorolicious. ?? Maybe not, how about wonderpendous. Recently Barbie commented that for the last many months I haven't been sick. Reading between the lines I also understood her to say, "You are not as annoying as usual two days after Decadron." I took this not only as a complement, but also as an affirmation that things are working well. When asked by a friend why am I doing so well five years later I responded that I don't have any medical or logical explanation. I thank God for this.
Last week I went to Santa Clara for my biennial left heart catheterization and my semiannual right heart cath. (how are semi and bi different?) My coronary arteries are wide open. My cardiac index is 2.3 (normal), my right heart pressures are normal. My echocardiogram looks great with an E.F. of 65% (Very Good to Excellent). The heart biopsy showed No Evidence of Rejection (NER) and, the envelope please, no amyloid in my 5 year-old heart.
Barbie and I are so pleased at this news. We have incrementally increased our 10-year marital bond yield to a 40th anniversary. There is a lot of interest in that.
Today Barbie directed the choir for stake conference. She was magnicredible. The best two choral directors that I have ever sung for are Barbie and John Bringhurst; although I like Barbie a little better and she is nicer to look at. She has this gift of pulling beautiful music out of a choir of mixed experience and we all feel better having sung together under her direction.
I love how music expresses what words cannot convey. Maybe I should sing a song about how I feel now. But since I sing Tenor eleven notes off, I shall sing solo that no one hears me. Groan.

You've been a great audience

(No, this was not written under the influence of steroids; this is really me.)

Thursday, August 8, 2013


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.


Thursday, August 1, 2013


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

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