Guilt

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.

Kevin

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.

Kevin

Monday

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.

Kevin

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.

Barbie

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.

Kevin

On Death

How do you tell a wife that her husband has died. I truly do not know. You cannot 'break it' easily. In that millisecond between when you believe your spouse is still alive and suddenly learn that they are not, reality is suspended and rationality ceases. It is too much for the soul to accept.
I was 'on call' for the first time in 15 months today and was covering for my partner who is out of town. I saw his patient this morning who had a procedure on Friday to control bleeding from his prostate. He was fine and we discussed possibly sending him home tomorrow. At noon I got a call that he was 'coding' meaning that his heart had ceased to beat and he was receiving CPR. I arrived to find the code team fighting frantically to save him. They worked heroically, each nurse taking his or her turn giving chest compressions while the hospital physician was directing the effort. He didn't make it.
I knew that his wife and daughter were on their way and found them in the hallway. My search for a private room to speak with them came up empty as they sensed the seriousness of the situation; I could no longer delay. Their emotions mounted as I explained the events as they unfolded, ultimately, however, you cannot avoid that moment when you flatly state, "He didn't make it" or "He is dead." Euphemisms don't soften the reality. If there were a better way to say it; I wish I knew. I felt horrible. They could not handle the immense gravity of receiving the worst combination of words that can be strung together in any language. I stood there silent, feeling completely impotent in any attempt I made to try and comfort them. I did not know what to do. Finally the nurse came and had found a private room where I could take them where their uncontrolled grief could be expressed.
In every crisis there is often someone that those suffering can turn to who remains their link with reality. Soon her son arrived and he became that rock for her. I was grateful for his strength in that moment. It was as if he became my translator for the rest of the family.
As I drove home today, I wondered if I could have handled the situation better. Probably, but I still don't know how. I remember a similar situation when I was the trauma surgeon in the ER at UC Davis many years ago. A 16 year old boy was brought in with a fractured spine from an automobile accident on Interstate 5. He was the only survivor. His mother, two brothers and grandfather were killed instantly when his grandfather fell asleep and drove into the back of a semi-truck parked on the side of the freeway. It was my task to call the father in L.A. to tell him about his surviving son. He then asked about the others. My first response was to say that they were seriously injured. The nurses in the room with me were saying under their breath, "You can't tell him they are dead over the phone." But eventually he asked, "Fatally injured?" I could not lie. I only said, "Yes." I heard only sobbing for what seemed an eternity. Finally, I got the number of his father and pleaded with him to stay home until I could contact his dad to get to his house. I couldn't imagine him alone with this burden that I had laid before him.
It doesn't get any easier 20 years later.
I was once in a seminar on death and dying where they asked "How do you want to die?" Which was followed by an academic discussion on he subject. But today I learned that that is the wrong question. If someone were to ask, "How do you want your spouse to die?" I guarantee that the dialogue would not only not be academic, it would not exist. Who can fathom such a question? Yet, it is the survivors who must live through that unanswerable query. It is not a question of age or being at the end of a fulfilled life, death is death. Ultimately, our faith will give us strength. But that moment of finality can be terrifying for many. Is it better to go quickly and unexpectedly or be prepared with a slow and painful demise. I thought I knew the answer to that. But previously I only saw it from the perspective of the one dying, not the one letting go. I saw it differently today.

Kevin

Old patients

I think that most men marry above themselves. Some men marry much higher and have wives who are angels. I am certainly one of those fortunate ones. Today I looked at my clinic schedule and saw the name of one of my old patients; old in that I have taken care of him for years and, also, he is old. I previously treated his superficial bladder cancer which requires that every three months we look in the bladder to assure no recurrence. For the last year Dr Chiu, my partner, has been performing the follow up cystoscopies. I was pleased that I would see him today because he is such a pleasant man and his wife is an angel. The news was good as the bladder was clean; no cancer. They also, aware of the cause of my absence were quite content to see me as well. Sometimes the beauty in your day is reconnecting with an old friend and finding them well. (especially when you get to tell them that they are well).
I then walked over to the hospital to see a patient in whom I had removed her bladder a year and a half ago for cancer. Her disease is metastatic now and she has had a number of complications. She had to stop chemotherapy because she felt so poorly while on it. I asked about her, and her husband asked about me. We have had many shared experiences in the last year. I know how she feels. Yet, she remains hopeful, and so do I for her. I silently mused on why do these things happen to such nice people?
While conversing with her my phone rang; the area code was 408, Santa Clara. It was my biopsy result. I always know the news immediately by the tone of the greeting. This was good news. The heart biopsy showed 1R/1A, minimal rejection. I tried hard not to be emotional in front of my patient, but I was so relieved. She sensed my reaction and smiled at me in her show of understanding. The lines of doctor and patient became blurred for a moment. I reassured her that a time would come in the near future when her daily activities will not include conversations about her health. She laughed and said that she had one of those days a few weeks ago and how nice that was.
I am feeling quite well as the steroid crash on Monday is slowly resolving. I'll be back to the chain gang, breaking stones, tomorrow and Friday.

Kevin

9-9-9

I see patterns in numbers. I can't help it. It appears to be some kind of innate reflex. In second grade my principal, Mr. Moulie, would find me walking in the corridor and slip me a piece of paper with a row of numbers and blanks. I would then find the pattern, something like 2x+1 and return it to him. He never told me that I was doing Algebra; it was just a fun game for a seven year old. In every row of numbers I see I will look for arithmetic and symmetric relationships. It helps me to remember them.
Nines are really cool because any number divisible by nine, when the digits composing the number are summed, ultimately result in nine. (each multiple digit answer must also be summed).
So today is an interesting day.
In China, nines are considered lucky, however, in Japan, the opposite is true. Today in surgery I was wearing a cap made by my partner, Dr Takahashi. She made it for me when I was bald after the bone marrow transplant. The Japanese characters in the fabric represent good health and prosperity. However, the number nine in Japan is considered bad luck. Oh, those opposing forces at work on such a day. The number nine in Japanese is a homonym with the word for suffering.
Generally, I am seen as a lucky guy. Today the nurse from the cardiology clinic called to say that my heart biopsy showed moderate rejection. this would be considered unlucky and/or suffering. The pathology report listed me as a 2R/3a. The last time I showed this level of rejection was the last time I took Revlimid in October 2008. What makes this quite troubling is the realization that Revlimid is the only treatment that has actually improved my amyloidosis. Yet with it, I seem to go into heart rejection and, without it, my disease would progress and ultimately damage my heart. I am between a rock and a hard spot without a Holmium laser. Now these concerns may only be theoretical, since originally there was some doubt as to the causality of my previous rejection with respect to Revlimid. The next step will be poorly founded in real science and be based on experience, intuition and educated guessing on the part of my doctors and me. We will aspire to a new homeostasis (my word for the weak).
I am now on solumedrol (the zombie/statue drug) 500 mg.
Let's play with the numbers. Normally I take prednisone 5mg every day. this is equivalent to 25 mg of cortisone, the basic steroid dose.
This morning I took 40 mg of decadron(dexamethasone) equivalent to 267 mg of prednisone or 1333 mg of cortisone. This afternoon I received 500 mg of Solumedrol I.V. This is equivalent to 625 mg of Prednisone or 2500 mg of cortisone. For a grand total today of 892 mg of prednisone today or 178.4 times my usual daily dose. I will be wired tonight. I should see if the San Francisco Giants want to hire me. If anyone has any questions tonight at 3 AM, call me, I'll be up.

We will find a plan and a new homeostasis. That much I am sure of.

Kevin