Sunday, October 25, 2009


Recently I corresponded with a patient who was just diagnosed with amyloidosis. Her presentation was unusual (what amyloidosis patient isn't) But it struck me that she only had one organ involved and was not predominately kappa or lambda. Generally, primary amyloidosis is of one clone or the other. It turned out that she likely has familial amyloidosis, which is a much better prognosis. However, I was not really prepared for her response to such news; she felt guilty. She felt guilty that she may have passed the gene for amyloidosis to her children. I wondered incredulously, how can you feel guilty for something that occurred 40 years ago over which you had no control and may not even occur. To try to assuage her guilt, I reminded her of the millions of good genes that she also gave to her children. However, I also realize that any loving and responsible mother can never be completely free of her duty to her children.
The very next day I was seeing a patient of mine in the clinic who has an incredibly difficult decision to make. She has metastatic bladder cancer and has already failed one course of chemotherapy. The toxic drugs made her life a living hell and she could not complete the course of treatment. The cancer is now in her lungs and her oncologist has suggested some experimental treatments. The potential for success is unknown. The expected side-effects are definitely known.
As she shared with me the acknowledgement of her own limited lifespan, she expressed guilt. Flabbergasted, I asked how she could feel guilt about dying? Once again, her thoughts were not of herself, but rather for her children. They wanted her to do everything possible to continue her life, even if that life is, for her, miserable. She felt that to die without trying everything might let them down.
We spoke of a balance in the middle; doing just enough to prolong quality time without doing what will not work and stealing those good days that you have remaining. Unfortunately, it is impossible to know exactly where that balance is.
I do not generally feel guilt, but I do have a strong sense of duty. Maybe it is the same under a different name. When I commit to something or someone, I cannot let them down. Last June it was clear that the bone marrow transplant failed to control my amyloidosis. I was disappointed for myself, however, even more so, I was somehow disappointed that I might let down those who had gambled on putting a heart transplant into an amyloidosis patient. I was the first primary amyloidosis patient to get a heart transplant at Stanford in many decades. I was fully aware of the reason that this practice was abandoned. There were many, and still are, who question the prudence of giving a heart to someone who is incurable. This, of course, begs the question, "How long must I live to make this great effort on my behalf worth it?" I felt this even more poignantly when soon after I became very ill with cytomegalo virus. At the time, I mistakenly thought that my disease had irreversibly progressed and that I was going to die soon. The thought that crossed my mind was that if I die, they might think twice before offering another heart to an amyloidosis patient. I felt it was my duty to live long enough, as in the words of Tom Hanks in "Saving Private Ryan", to 'earn this'.
How does one measure the worth of a life? Is it in the number of days or rather what you fill those days with?
I have seen well meaning family members demand that everything be done to add more hours and days to a life that is ending. Who decides when any individual life has satisfied the purpose of its creation?
I guess this is why there are feelings of guilt surrounding the act of dying. It is because we don't know these answers and we wish that we did.
My father died well. he was surrounded by his children and my Mom and quietly passed at home. However, six weeks earlier, his oncologist offered him one last course of chemotherapy. We all knew that it would not work and that he would be miserable if he took it. The offer was more out of obligation. Initially, my dad wanted to proceed and seemed annoyed that we might question the value in the treatment. I suggested to him that since he was doing so poorly that day, the drug were certainly make him worse, but that if in a week he had improved, we could start the chemotherapy then. He understood and accepted that plan; because in it there was still hope. I certainly still had hope that he would improve. He never did. But that was OK as his last weeks were at home with his family. I know that he had fulfilled the purpose of his creation. Filling more days would not have changed that.
So what do loved ones do that have to watch with powerless guilt as a friend or family member begins down that final path? They must understand that the dying feel guilty too. They need to listen to the words to painful to utter, to be patient with decisions so irrational and difficult to comprehend, and then we can remove those shards buried along our final common pathway and allow our last goodbye to be sweet.


Wednesday, October 21, 2009

Recipe for a great day

Driving to work, I felt like it was going to be a great day. It wasn't like the stars were aligned. Actually, they were a bust. I just happened to wake up at 2:30 AM and I figured, Oh well, it is the peak of the Orionid meteor shower, let's have a look. I found a blanket and laid outside for 20 minutes. I only saw three minor meteors. I went back to bed.
Notwithstanding, I felt great, physically, when I woke up. I got to work and got my blood drawn for Prograf and Cellcept levels and noticed that they were doing Swine Flu shots. 3 minutes later, the needle was in my arm. Now I don't have to worry about that anymore. I am generally not paranoid, but I really, really, really did not want to get swine flew. After what happened with the CMV virus last June, I have been waiting for the vaccine with great anticipation.

The pig has flown.

All of my patients today were just great people; those of my old patients, additionally grateful to see me. With the feeling very much mutual. I then got to do a great surgical case, laser lithotripsy of a kidney stone.
I got home a little late (a 10 hour day) to find we were going out with our great friends, the Boatmans, to our favorite restaurant, Orchid Thai.
We ordered the usual: Crispy calamari salad, Tom kaa gai (Coconut soup), Laab (a minced chicken salad with lime, cilantro, mint and Thai chili served and scooped with cabbage leaves) and, of course, the dish to judge all Thai restaurants, pad kee mau (Drunken noodle). They were all served fresh and perfect tonight.
The Boatmans had just spent the weekend at cancer camp with their 7 year old son Tyler who has metastatic Wilms tumor, and their 3 others boys, Jack, Tanner and Robbie. It was a great experience for all of them. Later as I was describing my meds and labs to Maria, (using unfiltered medical jargon), she chuckled as she commented, "Isn't it amazing that I know exacting what you are talking about." We share a lot in common these days.
While in Raleys buying fruit, I saw Mallomars (chocolate and marshmallow cookies mad with real chocolate). These are generally sold only on the East coast during the non-Summer season. Of course, I bought a package. I love any combination of marshmallow and chocolate.

Tomorrow, we again step into the unknown. I will start Revlimid again, even though I know it puts me at greater risk of heart rejection. Eight years ago I remember saying to a medical student at Yale, "It is the risks we take that truly make us feel alive." Somehow that feels hauntingly more apropos today. Occasionally we pass through an experience that exists in a heightened sense of reality. This is one of them. We are making some changes in our new and made-up protocol. I will only take 5 mg daily for 21 days. And instead of one week off, I will restart the next course 5 weeks later to give my heart time to recover. Again, a guinea pig ( I sense a pork theme today).

I have had many good days recently, but today just felt great. You can't beat that.


Monday, October 12, 2009


It is always nice when I am in the OR. I did two surgeries today with our new Chief Resident. They both went well. I do a lot of stone procedures using a lithotripter to non-invasively break kidney stones. The chief of orthopedic surgery found out that I also have experience using this technology to treat calicifications in tendons, specifically calcified tendonitits of the shoulder. Since then I have had numerous patients referred to me to get this procedure. It is still considered experimental, but has shown some promise in alleviating pain in these patients. I have always seen my role in life as alleviating pain, whether it be physical, emotional or spiritual. That may be why I am still here.
For 2-3 weeks I have been feeling weak. This was why I ended up getting a heart biopsy last week to assure that it was not from rejection. Fortunately, it was not. I have improved in the past few days. It may have been a virus. I am now ready to begin Revlimid again, at a lower dose, to keep my amyloisosis controlled. I will probably be on one month then off one month. Hopefully, this will not lead to heart rejection. My light chains only increased a little since I have been off Revlimid for over a month. I was happy to see that. I am content and at peace because nothing has been left undone.


Wednesday, October 7, 2009

good news

Kevin hasn't felt well the past few weeks: extra tired, week, dizzy, strange heart beats etc. The worry was that he was in heart rejection again. Yesterday he drove to Santa Clara for a biopsy. I just receive a call that he IS NOT IN HEART REJECTION. Great news!!

The question now is, "what is causing all these uncomfortable symptoms?"

Don't know yet - but gratetful his new heart is strong.


Kent from New York

My life is a river. The river does not know that the ocean lies hundreds of miles ahead. It continues on. On my journey I have met and come to know thousands of individuals. Some flow with me as part of my life. Others I meet, share a moment and never see again. But their influence stays with me, changes me as I move on. But the world has changed. Previously, these encounters were face to face, person to person. Now I have come to know individuals through the world of the internet. They are no less real, nor do they impact me any less having never grasped their hand in a physical greeting.
Some time ago, Kent from New York commented on his progress with amyloidosis. We conversed over time as he prepared for the possibility of a heart transplant. I was glad to share my experience and give him hope. He was still working, but could feel himself slowing down. His close friend, Elise, informed me last week that he has died. This is hard for me. I feel like amyloidosis is this enemy that we fight every day. It is not like fighting a conventional army that you can see and plan for. Rather, it remains insidious, like a terrorist, attacking unexpectedly.
I know that Kent from New York fought a good fight. He did not give up. But I can't shake this sense that he was somehow blindsided by this nasty deposition of unconscionable warped proteins. How they move within us without regard to the damage they leave in their wake. They infiltrate not only the heart muscle, which can lead to slow death, but also attack the wiring at the center of the heart leading to a sudden fatal arrhythmia. Is there no defense against this?

We who share this diagnosis, and even more so, those who care for us with this disease, keep this fear silent within us so as not to tempt fate. Amyloidosis is an orphan diagnosis relegated to a dark corner of medicine; mentioned in medical school and promptly forgotten by most doctors. Where is the celebrity that champions our cause? It is not only rare, but affects each patient so differently that any attempt at discovering its modus operandi is hampered by varied responses in rare patients allowing for little statistical power in planning a unified attack.
Sometimes people ask me what is my prognosis. I honestly don't know. Does that somehow make me like everyone else; blindly living every day with sublime ignorance of their own mortality?
Sometimes I wish that I could respond, when asked, that I have cancer. At least with many cancers, there is a cure; or it only affects one organ in your body. People understand cancer. They don't know where to catalogue amyloidosis; and describing it becomes cumbersome.

Just because I share these thoughts does not mean that I am ever without hope, nor am I sad for myself. I still find incredible joy in every day. I am however, sad for Kent from New York and for his friend Elise. Because I will move on toward the unseen ocean and leave him behind. And yet, it may be that my sadness for him only stems from the briefness of my encounter with him. He may not have shared my sadness, because, like me, he lived a full and happy life of no regrets. If we truly could know the complete essence of any one person, would we mark their absence differently?

My hope remains strong; if I gave Kent hope it was not in vain. But he will be missed as are all of those whose battle against amyloidosis has ended. My heart goes out to all of them and to those who continue to remember and to love them.