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