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

Dangerous Discovery

Definitely did discover dangerous delectables during a drop into Walmart. Depressed by dint of the demise of Ding Dongs I did delve the shelves of dubious duplications. I did detain a decoction developed by diametrically dismantled engineering. My dubiosity did develop into the done deed. Dollars donated. Their designation a deceptively devilish dessert.
Then I ate it.
The distinction differed to my delight. The denoted generic 'Ding Dongs' did define themselves deluxe at a discount. My disdain dissolved; as did the devilish delight.
This is dangerous.

Kevin

Small Victories-

Eagle River, Alaska

I looked on Yahoo news, but I didn't find it. I did find out that two celebrities were wearing the same dress at the same event and that another sports figure did something stupid. But there was no mention of the woman that called me on the phone today. She wanted to tell me that she had received a new heart and that she was a changed person. This is huge. She was going to die very soon and now she will not. I did not wonder why this was not big news in all of the major venues. She is not famous. She is just like the rest of us. And what a blessing that is.
I met her and her husband in February. She was an inpatient and was placed on the transplant list that very day. I was there for my semi-annual heart biopsy. She had heard of me and wanted to ask me some questions. She has primary AL amyloidosis and was in much worse condition that I had been prior to my new heart. But, she was initially afraid and did not want a heart transplant. Finally, with her doctors' urging and her husband's support, she acquiesced and agreed to go on the list. But she was still nervous. When Barbie and I entered her room she was surprised to see how healthy I looked. We answered her questions and named her fears such that they no longer lurked in the darkness of uncertainty. When we parted she was visibly relieved and increased in hope.
I knew from what she told me of her symptoms that without a heart transplant she would not be long for this world. I silently prayed that the heart would come soon. It did. Within a month I got word from her husband that she had an uneventful surgery and recovery. She had an early heart rejection, but this was reversed with ridiculously high doses of I.V. steroids (Solumedrol: nasty stuff) and has done well since.
She called me today to ask when she should be rechecked as to the status of her amyloidosis. We discussed this and her new side-effects. She spoke of a wicked 'Prograf' tremor (Prograf is the major anti-rejection medicine that we take everyday, forever.) This causes a bad 'intention' tremor. This type of tremor gets worse as the effort at fine motor movement increases. So when the spoon begins at the bowl, it is not that bad. However, when it finally reaches the lips it is like eating soup on a roller coaster during an earthquake with a magnitude of 7.2 on the Richter scale. It is messy. I reassured her that this would greatly improve in 9 - 12 months. I gave her suggestions on managing her light-headedness after sitting for long periods. Barbie and I reminisced on how we never knew what the cause of all of my early side-effects were and how it would have been nice to have someone to call. The doctors tried, but patients understand these thing better; we live through them every day.
It was amazing to hear her describe her new life. She is no longer short of breath; no more oxygen tanks. The defibrillator vest is gone. No more pain when eating. And the nasty swelling in the legs is gone. She now walks a mile a day. What a miracle.
This should be momentous news; such an amazing event. But it happens to regular folk every day all over the world.
Fame is a funny thing. Some people actually seek it, but they are always disappointed.They often proffer some salacious tidbit that immediately vaporizes into cyberspace as they remain unsatisfied. Fame is an empty promise. I define fame as when 51% of the people who have ever heard of you have never met you. I would rather be famous among 50; within a small group whose lives you have touched while becoming better for having met them, either physically or through our ever expanding virtual world.
In this group we know each other. We share our stories and listen and understand. We give hope to each other and enlighten the path for those that follow. In this group we are each enriched as we share our small victories

Kevin

In My Father's House

We had the opportunity to purchase the house in which my father grew up. It is located two blocks from downtown Provo, Utah. Barbie and I fell in love with the house when we first saw it. It is a Craftsman Bungalow style built in 1927. But we also felt its history. My dad moved here when he was nine and lived there until he married my mom and moved to California. There is so much of the history of my ancestors that happened in this house. My first recorded memory (one attached to a fixed date) happened when we travelled to Utah for the funeral of my Grandmother in April of 1962. I was in the kitchen as I watched boiled oatmeal escape the saucepan and pour over the rim onto the stove. This frightened me.

Barbie and I traveled to Provo last week to see the basement that we had remodeled so that Caitlin and Ben could live downstairs while Samuel and Michelle reside in the main house. This was a long long-distant process and had its share of both minor bumps and major issues. We had excellent help through our friend and interior designer, Wendy Ormsby, and our contractor, Jeremy Brown of AllElectric Construction. We did our best to maintain a virtual presence through smart phones as we texted, sent photos and face-timed to oversee and hopefully not overlook the many details involved The result created one of the nicest basement apartments that I have seen in Provo. (A university town with its share of basements dungeons. I lived in one in 1980.)

I decided to include some before and after photos.

(Click to enlarge)

Bathroom

Before, Note the painted ducts

After

Two areas of the old basement not seen here in photos are the cinderblock coal room which was behind the water heater and originally stored coal for the first furnace in the house. This was removed and made room for the bathroom above. The old coal shoot was converted into the bathroom window. Additionally, there was a root cellar that ran the width of the back of the house behind the basement kitchen. This was excavated and finished to become the pantry off the kitchen, the laundry room and a back exit to the internal staircase to the main house. This staircase was required by Provo City in order to get a building permit and caused a large delay and a large cash infusion.

 Front Room

Before

After

View from door

                                                                       

Before

After

Bedroom

Before

After

Kitchen 

Before

After, with view into pantry

We decided to create the basement that we would like to live in if we were young married BYU students.
I'm ready to go back to school.

Kevin

All Things Being Equal

I am fascinated with words, even more so with phrases. I wonder how they get crafted and adopted. All things are rarely equal. But it helps to attempt equality when deciding between things. Currently all things are going well, and yet they aren't. I am reminded of this in the blog post that Rebecca so courageously shared yesterday about dealing with a miscarriage. a-wretched-life
I am proud of Rebecca and Jason for their faith and perseverance. We so love our children and want them to be happy. But there will always be days that are sad.
I continue to do extremely well with my health. No changes of note. I still get Velcade weekly along with my Decadron. There are, however, some promising new drugs on the horizon that may change the course of my disease. I will keep you updated. Work is going well and I will be working with Kaiser to develop a video for patients newly diagnosed with prostate cancer. I wrote the script and will be in front of the camera. I'm trying to lose 10 lbs so that I look better on computer screens around Northern California.
Barbie has been working consistently in temporary positions as an RDH and is considering some more permanent options. She is a great hygienist.
We recently returned from a very romantic cruise to the eastern Caribbean. We visited Turks and Caicos, San Juan, Puerto Rico, St Thomas and a private island that Holland America owns called Half Moon Cay. We had such a relaxing week with 7 days of no cell phones, no Internet and no schedule. The sea was a beautiful clear turquoise, the water was warm and the few fish that we saw enough to say we snorkeled.

Half Moon Cay

The basement of the Provo house was finally finished and Caitlin and  Ben moved in on Monday. It is not a typical BYU basement apartment. Barbie's friend, Wendy Ormsby is an interior designer and, working with her contractor, Jeremy Brown, were able to take Barbie's vision and create a wonderful 'space'. Caitlin is delighted to have a little more room for her womb as she is now in her 17th week and wants to nest. Once I get the before and after shots of the basement, I'll post them.
With the book done and a grandchild on the way, my previous bucket list had officially expired. While watching the ocean from our stateroom balcony I penned a new one. It has some fun stuff like reading Dickens, learning French, doing a culinary experience in St Helena, CA and riding the Orient Express from Paris to Istanbul.
I hope to continue writing, both here on our blog and other places as well. Maybe if the prostate cancer video plays well, I might get a call from Kaiser Hollywood.

Kevin

Looking for help

It's been over a year now since I effectively stopped writing regularly in our blog. The reason I perportively gave at that time was because I was going to work on writing a book about this experience of a patient as a doctor. I haven't done a very effective job at doing either over the last year. As I reflect on my life at that time, I had just switched from Revlimid to Velcade to treat my disease. While Revlimid had more physical side effects, Velcade has produced more insidious psychological side effects. As a result, my motivation suffered. It is very difficult to write without motivation. It would briefly return on Wednesday evenings when the morning dose of Decadron produced a state of hypo-mania coupled with insomnia and mild dis-inhibition. (This is where I am tonight.) All of which can be useful for writing.

Yet I would occasionally force myself to sit and write in fits and spurts. As such, the book is now essentially written. I am now ready to free it from the solitary confinement of my mind (and a file on my desktop) to let others read it, and critique it. This is actually a scary proposition. That is why Barbie will read it first. I trust her. Over the last week I have been quite anxious about publishing it. What if people don't like it? What if they can't relate? Millions of books are written. Why does the world need another one? I tell myself that my only audience is that of my future grandchildren, and that helps me to keep going. Sometimes I think that I will share great, previously un-thought truths, some enlightened revelation to benefit humanity only to realize that there is nothing that I have ever thought, said or written that hasn't already occurred to some millions of other observant humans so many times over the last 10,000 years; and then I realize, "It's new to me, and is therefore of great value." "These are the lessons that I needed to learn."

Do we really need books anymore? Books take too long to read when we have social media. Of course there are blogs which represent the cyberspace union of narcissism and voyeurism. But given their wordiness, they might require a longer attention span. For those with medium attention spans there is Facebook. While those with limited attention, and prone to quick boredom, can opt for Twitter. For pre-schoolers there is Pinterest. (I like to look at the pictures.)

There are times when trying to describe the scope of a life requires more, and therefore more investment from the reader. I once heard, while attending a lecture on chaos theory, that in New York City there is only sufficient food on the island of Manhattan to last for three days. And yet, there is no master plan on how to get this food resupplied on a daily basis. It just happens. Any regular mathematical model would fail in trying to describe this so the problem is given to those who can speak in the terms of chaos theory.

Imagine that Manhattan is a book. Chapters would be found in SoHo, the Village, The Upper East Side, Time Square. The pages are made up of the restaurants in China Town, the penthouses near Columbus Circle and the Galleries in Chelsea. The words are us. We build the stories of the book everyday of our lives. But you couldn't truly read the Book of Manhattan if you never left Wall Street. You would have to take the Subway for a day at the Met; a stroll in Central Park. You would need to stand in Battery Park and gaze off at the Statue of Liberty. However, while you may get a feel for Manhattan, you could ever comprehend it all.

A single human life is more complex than all of Manhattan. Are lives are not as ordered and planned as we think they could be. Chaos is all around us and in us. It has been difficult for me to assemble even a year and a half of my life so that I can make literative sense to those that may read it. I struggle to tie strings of relevance with a knot at each critical turning point to guide the unfamiliar along this foreign path. My only tools are memories and words; woefully inadequate. There are passages of my own prose that are torture for me to read as they rekindle painful memories that are immediately real. And yet I know that my failing as a writer can never convey that same visual memory burned in my brain. So why bother.

My hope comes from the reader. They will subconsciously recognize my gaps in narrative and fill them seamlessly with their own imagination, experiences and intuition. Our common human wisdom may serve to save this endeavor. This gives me some hope. My story, in a way, becomes their story in the sharing.

Once Barbie, and a few others, read this and it is finally done. I have no idea how to publish it. Or how to even start. I have heard that I could put it as an E-Book on Amazon. I don't know. If anyone has any good suggestions. Please let me know.

Thanks,

Kevin

kray0819@gmail.com