Unblinded with science

I've spent a lot of time since I was diagnosed trying to understand this disease from a purely intellectual perspective.  My general interest in science, two semesters of biology, one semester of anatomy, three semesters of chemistry, three years doing media relations for a teaching hospital and 15 years in the field of data and analytics give me enough of a base that I can understand a good majority of the articles and studies that I read.  Well, good majority for someone not in the science or healthcare field.  I'm still working to understand more...I remember b cells and t cells and plasma cells and cytokines and antibodies from my biology classes but it wasn't in as much depth and/or I didn't sufficiently synthesize the information to provide antyhing more than a shaky foundation upon which I can build a real understanding of my current disease process.

As my friend Judy (PhD in Statistics, analytics professional extraordinaire and breast cancer survivor)  said to me last fall when talking about this need to understand the disease and research, "Cancer is my hobby."  I read a lot and go on internet wanders to fill in the missing gaps in my understanding.  Whenever I've had the opportunity to validate my understanding with someone more knowledgable than me, I usually get it right...conceptually at least.

Two things crossed my media stream over the last few weeks that really smacked me in the face with why this disease, and most cancers, are so freaking hard to beat.  It would be reasonable to think that this realization would upset me and perhaps make me depressed but my need to understand is greater than my fear of my disease.  I was almost giddy when I figured this out.  I'm going to talk a little about what I've figured out and whenever I make what seems like a statement of scientific fact, just know there's an asterisk after it followed by "as far as I know" or "from what I understand".  I recognize I could be off on a lot of this.

Now, some of the information is related to Multiple Myeloma, not AL Amyloidosis and those of you who have been around a while remember my struggle about whether or not I have MM or Smoldering MM.  I discussed it in "The Real Slim Shady and Multiple Myeloma".  If I don't have MM or SMM, then I don't have cancer.  AL Amyloidosis is not cancer.  Which sorta pisses me off sometimes because when you say "cancer" people almost immediately understand this could be really bad.  You don't get that reaction when you say AL Amyloidosis, or "rare, incurable blood disorder that's fatal if left untreated."  AND, the 2 diseases basically start out the same with a plasma cell clone that goes haywire and starts causing problems.

(There's a whole class of diseases from those haywire plasma cells called "plasma cell dyscrasias".  Dyscrasia is just a fancy medical word for when something in your body doesn't work right.  I think I might start using dyscrasia as a euphemism for "effed up.")

The difference between MM and AL is in the mechanism of death.  Cancer causes death by the damage from the proliferation of the cancer cells.  In AL, we start off with the same process that might eventually develop into MM, but if it doesn't develop into MM, we die from the organ failure that's caused by the misfolding of a by-product of that plasma cell clone going haywire.  A technicality, if you ask me.

This story from the NPR show RadioLab about Famous Tumors really helped me understand the progression of cancer and the challenges in developing effective long-term treatments or cures.  Here's the relevant excerpt (about 12:40-15:30 in the show)--http://youtu.be/cVrNOkKoQW0  I like to listen to RadioLab or This American Life when I'm driving solo.  I listened to this a few weeks ago as I drove for an hour to meet Alan for the TOSRV ride.  As I listened to it, I wondered if this was the same mechanism that happens in multiple myeloma.  It would seem that it would be, but I'm never sure about anything with this crazy class of diseases.  I shared this clip with my 16-year-old daughter and she said, "It's just like Darwin's theory of natural selection."  Exactly...which takes me to this article that came in my email several days later from an Amyloid listserv to which I belong.

The article, "The impact of intra-clonal heterogeneity on the treatment of multiple myeloma" starts off with this--

 It is clear that cancers comprise a mixture of clones, a feature termed intra-clonal heterogeneity, that compete for spatial and nutritional resources in a fashion that leads to disease progression and therapy resistance. This process of competition resembles the schema proposed by Darwin to explain the origin of the species, and applying these evolutionary biology concepts to cancer has the potential to improve our treatment strategies.

Exactly the process described in the Radiolab show and identified by Claire.  This picture from the article effectively demonstrates the challenge in finding effective treatments because, as they noted in the RadioLab story,  "it sounds like cancer is always evolving to be more cancerous."  And if that's true for cancer cells that originate in something like lung tissue, it blows my mind to think of how quickly cells could evolve that start in your immune system.  I mean that system is programmed to produce cell differences so it can fight never before encountered pathogens. Hijack that system for evil purposes and you've got something pretty powerful. (Since originally writing this, I've learned that the "anatomical" perspective of cancer is outdated and that the future of cancer research and treatment is to approach it from a genomic and immunologic perspective.  So, I'm striking that statement because I'm not sure if it's accurate.  Seems like bad cancer is bad cancer regardless of where it manifests itself.)

Think about it...it would be hard enough to come up with an effective treatment that wiped out all the gray cells in the diagram above. That's the single cell theory in the RadioLab show (what they really describe in the RadioLab show would be more accurately described as the single type of cell theory.)   But, the gray cells evolve into gray cells with horizontal stripes and right angle stripes and those two evolve into gray cells with boxes and gray cells with arches.  As Dr. Hofmeister noted in the Imagine Mmore Multiple Myeloma Symposium, a cure will come when they can find the "queen termite" the original plasma cell clone that started all the mess.  When they can get rid of that, then all the copying and all the errors and mayhem that follow will cease.  And that is called a cure.

Now, for those of us with AL Amyloidosis, we have hope on two fronts.  Researchers can find and eliminate the queen termite.  OR, and this is a benefit from this disease not being a cancer, researchers can figure out how to keep the plasma cell by-product (free light chains) from mis-folding and clogging up our organs.  (Although I have heard there's a toxic effect from the elevated free light chains whether or not they mis-fold so keep looking for that queen termite, guys and gals.). There's an article starting on page 11 of the Boston University Medical Center magazine about research they're doing in that area.  http://www.bumc.bu.edu/comm/files/2014/04/Spring-2014.pdf

The Amyloidosis info starts on page 19.  David Seldin, MD, PhD, professor of medicine and microbiology and director of the Amyloidosis Center said in the article that even though different disease processes can produce mis-folded proteins that turn into amyloids, the amyloids all look identical microscopically so there must be something similar in the way that they are formed.  There's a whole group of diseases caused by mis-folded proteins: Alzheimer's, Parkinson's (my maternal grandmother had both of those), type 2 diabetes and Amyloidosis.  Isn't that great--I get to be in the plasma cell dyscrasia club AND the protein misfolding disorder club.  Actually, it is good because advances in treating either class of disorder might help in treating my disease.

So, while I could look at this newfound understanding as bad news, its really not new news,  I knew it was bad, I've seen pretty scary relapse numbers (~96%), the words from Dr. Hofmeister's database documentation ("Because nearly all amyloid and myeloma patients relapse and treatment is eventually unsuccessful...") is burned into my mind.  What I've learned through this isn't new info for me.  But, I do feel I've had a breakthrough in my understanding which will help me as I continue to learn about this to satisfy my intellectual curiosity and to feed my need to understand the world around me.  And it's confirmed my sense that I don't really need to worry about what those tater tots will do to my cholesterol nor be uber-diligent about the sunscreen.

I'm sure this learning process will go on as long as I'm able to do it.  I'm still working to get through the Immunology course from UMass that I've downloaded on iTunesU.  Who knows what mind-blowing understanding that might bring into my world.

Again, just another opportunity to trust God. I know He has a plan for me and I just need to be faithful and follow it.  Regardless of how wacky my plasma cells are or how poorly my proteins are folded. So I can learn about these topics, but I don't have to worry about them.

Ends of the Earth

Today is my husband's 60th birthday and I've been thinking a lot about our journey together over the last week.   In the midst of this, I've been obsessed with a song that I listen to whenever I can.  It's called "Ends of the Earth" by Lord Huron, an indie-folk band based in LA.  The song is about someone who wants to go explore, and conquer, unknown lands and he's asking someone if they would follow him to the ends of the earth.

The description of the journey struck me as a metaphor for how I see my health journey.  The song starts out with

Oh, there's a river that winds on forever
I'm gonna see where it leads
Oh, there's a mountain that no man has mounted
I'm gonna stand on the peak 

Out there's a land that time don't command
Wanna be the first to arrive
No time for ponderin' why I'm-a wanderin'
On while we're both still alive

It closes with this

I was a-ready to die for you, baby
Doesn't mean I'm ready to stay
What good is livin' a life you've been given
If all you do is stand in one place 

I'm on a river that winds on forever
Follow 'til I get where I'm goin'
Maybe I'm headin' to die but I'm still gonna try
I guess I'm goin' alone 

Here's the video..

(and the link for you mobile folks...http://youtu.be/O42EKFw99yQ)

As I said on Facebook, regardless of what this health journey brings, I know Alan will be with me...pushing me, pulling me, carrying me as needed...while we laugh together at every step along the way.  I know he'll follow me to the ends of the earth and I know I'm not going alone.

Happy Birthday, BPK!

Being a "Good Patient"

A blog post about the pressure to be a "good patient" came across my Twitter feed last week and I can relate to many of the scenarios the writer discusses.  The author, a fourth year medical student at Harvard, describes the characteristics of a "good patient" in her post "Good patients cover their emotional cracks"--

"There’s a lot of pressure to be the stereotypical “good patient”.  It doesn’t necessarily mean inviting the medical team to your house on Cape Cod for a traditional Russian dinner.  But there are certain characteristics I’ve noticed that my medical teams like: a “good attitude”, politeness, agreeability, compliance with treatment, and the ability to understand without asking too many questions.  Fall down on one or more of these and during a busy morning we may round on you last, or we won’t round on you as a team at all.  (Instead, you will have one-on-one conversations with your physicians throughout the day, so as to spare the members of the team not directly involved in your care the inconvenience of meeting you.)"

Now, I've never experienced the "medical shunning" she describes and I don't know if that's due to me, my team or some combination.  I do know that I've felt a pressure to fit into the system and manage my behavior accordingly.  I'm not sure if that pressure comes from observation or intuition but I certainly feel it.  Those who know me and have seen my usual attitude of "this is who I am and I don't really care what you think" would be surprised at the deference I show my medical team.

Don't get the idea that there's this huge observable difference between how I usually behave and how I behave with the medical team.  It's not like I'm generally a completely self-absorbed, inconsiderate a-hole (unless we start talking college sports and you're ragging on the Gators) and become the epitome of niceness with my medical team.   Most people generally consider me a pretty likable and caring person.  But within the medical system, there is an underlying tension to be a "good patient".  It's not overwhelming and definitely doesn't interfere with developing strong relationships but I know I feel it.

When diagnosed with an incurable disorder that will require intense management for the rest of your life, the need to have a strong relationship with your health care team increases exponentially.  Multiply that when it's a rare disease with few experts and the patient is a bit of a victim of supply and demand.   Again, whether real or perceived, I have that sense. Back to my Managerial Economics class, the exit costs in this patient-provider relationship are exceptionally high.  There's also the element of knowing that your life depends on the full engagement of your medical team.  Back to Managerial Economics, the incentive to have the team, especially the physicians, really, really, really like you is also really high.

As a patient, you hear this drumbeat about asking questions, understanding your disease and treatments, and being an active partner.  What's not understood is where the line is drawn between engaged patient and problem patient.  When do questions move from accepted need to know into the realm of questioning an expert's professional competence?  How is that "engaged patient" behavior practiced appropriately?  It's like you're traveling in a foreign land that you know has mores and behavioral standards but there's no tour book to tell you the words and gestures that will offend the locals.

The inextricableness of the patient-provider relationship found in my situation does have an up side.  The providers seem to have a real sense of caring that transcends what I would normally expect.  I understand this could happen routinely in situations with serious diseases.  I recognize that my sample size of one has limitations on how broadly it can be generalized.  But this sense has been confirmed by my husband who has spent the last 35 years of his life participating in and observing patient-provider relationships.

As we discussed this dynamic the other morning, he talked about a patient he had cared for the night before.  This gentleman had Castleman's Disease and had undergone a stem cell transplant seven years ago at the University of Arkansas and was still being followed by a hematology oncologist there.  Alan said that the cell phone number for that hem/onc was in the chart.

He talked about how impressed he's been with the strength of the patient-provider relationships that he's observed...not just with me but in general...within the Multiple Myeloma team at The James.  He said, "If you went up to the average Cardiothoracic Surgeon a month after he operated on you and said, "Hello" he'd give you a sideways glance that says, 'Should I KNOW you?!?!?'"

Granted, my time working at the teaching hospital that had 13 residency programs showed me that certain specialties had a higher ego quotient.  I can imagine that with the diseases as poorly understood, and with the high morbidity and relapse rates of plasma cell dyscrasias, the ego folks may shy away from this field.  If a 0.300 batting average gets you into the Baseball Hall of Fame, I'm thinking something similar will get you into the Plasma Cell Dyscrasia Hall of Fame, if one were to exist...which would be pretty weird.  (I mean, really, purple blobs and exposed epitopes are not all that interesting to the general public.  But, it wouldn't be any more weird than the Strollin' Colon, the giant inflatable colon you can walk through at the Mayo Clinic.)

I'd love to hear from other patients with serious, incurable diseases to see if they feel the same pressure.  I recognize that my ENTP personality type may play into this.  This description of the ENTP may describe some of what's happening--

As an ENTP, your primary mode of living is focused externally, where you take things in primarily via your intuition. Your secondary mode is internal, where you deal with things rationally and logically. 

With Extraverted Intuition dominating their personality, the ENTP's primary interest in life is understanding the world that they live in. They are constantly absorbing ideas and images about the situations they are presented in their lives. Using their intuition to process this information, they are usually extremely quick and accurate in their ability to size up a situation. With the exception of their ENFP cousin, the ENTP has a deeper understanding of their environment than any of the other types. 

So, maybe some of this is just me trying to understand this new world that I will live in for the rest of my life.  I'm guessing neither Fodor's nor Lonely Planet has published a tour book.  Maybe I should.

Inpatient Retrospective

This past Saturday was the first anniversary of my discharge from the hospital.  I was in The James Cancer Center on the Bone Marrow Transplant Unit for 3 weeks and in the Doan Hall Rehab Hospital for 1 week and 2 days.  I needed to be able to walk 500 feet and climb 6 stairs for discharge from the rehab hospital.  This was due to the deconditioning from the transplant and the fluid issues that I had.

The day I got home from the hospital last year, my good friends Lori and Dave came over and planted a bunch of spring plants in my front yard and put some potted and hanging plants on my porch.  Dave's family owns a commercial greenhouse, Cuthbert Greenhouse that supplies annuals to local grocery and discount stores.  This is his BUSIEST  time of the year yet he and Lori took time to spend hours traveling to my house and putting in plants to brighten up my return home.  It was beautiful and I sat there in my wheelchair watching them do this overwhelmed by their love (and feeling a bit surreal that I actually had to sit in a wheelchair.)


Fast forward one year later.  I spent Saturday looking for spring plants to put in my front yard, photographing my daughter and her friends before their prom, and doing a bit of shopping.  Here's a picture of Claire and her date before prom.

On Sunday, Mother's Day, my husband was on the return leg of a 2-day, 200-mile bike ride, the Tour of Scioto River Valley or TOSRV as its known.  He asked me to ride with him for the last 50 miles and I was excited to do it.  In the past, there were times when he wanted me to ride with him and it felt a bit like a chore.  Cycling is his passion, not mine.  My passion is doubles beach volleyball but my doubles beach volleyball playing time has reduced proportionally with the reduction in the number of beaches between Ft. Lauderdale and Ohio.

But, like so many things now, I'm just thankful that I have the opportunity to go for a ride.  So, I got up Sunday morning and drove the 50 miles to the town of Chillicothe and met Al at the park that hosts the TOSRV stop.  We met up and were on the road at a little before 11.  We had a great ride back and made pretty good time.  The 10 mph tailwind didn't hurt but I rode really strong and pulled my husband along the last 6 miles or so.  Of course, it was mile 194 for him and mile 44 for me but I still felt good about it. The stats on the ride were--51.4 miles, avg speed 16 mph, max speed 28.2, time 3:11'18.  Even though it involved 200 miles of travel between Columbus and Chillicothe (50 to drive down, 50 to ride back, 50 to drive down to get my car, 50 to drive back home), it was a great way to spend Mother's Day one year after my hospital discharge.

And it was quite fitting to spend the day with Al.  He's been the foundation that has supported me throughout this entire illness and recovery.  I talked a little about that here--World's Luckiest Guy-- early on into the journey--less than one week after I was diagnosed.  His persistence (also known as nagging) is one reason I've been able to recover so quickly.  "OK, Kat, get off the couch and do your physical therapy (simple things like walking toe to heel or walking sideways.) I'll put the gait belt on you and catch you if, or rather when, you fall."  "OK, Kat, you've been able to walk 3 houses down the street, tonight you're going to walk 5."  And on and on and on.  He said that his fear that I'd never get back to my former physical condition was what drove him to push me and he's gotten me most of the way there.

Being able to spend the day with him, doing something physically challenging that he loves was a way I could honor his love and support over the last year.  So, on the first anniversary of my hospital discharge, here is a dramatic interpretation of me and Al during my hospitalization and recovery.  Playing the part of me is Dory and playing the part of Al is Marlin. Kat/Dory and Al/Marlin

Maybe he's not the only lucky one.

Finding Nemo: Jellyfish Sequence from Twenty One Inc on Vimeo.

Record, supposition or SWAG

rec·ord
noun, ˈrekər/
a thing constituting a piece of evidence about the past, especially an account of an act or occurrence kept in writing or some other permanent form 

sup·po·si·tion
noun, səpəˈziSHən/
an uncertain belief 

SWAG
acronym, swag
scientific wild ass guess

As many of you know, I had to get a copy of my medical record when I went to Mayo at the end of 2013 for my second opinion.  And, as many of you might presume, I spent some time reading it.  Granted, the Ohio State University Medical Center has a pretty handy patient portal online.  It shows all my past appointments with basic information about the visit, past test results with handy graphing option (nerd alert!) and the ability to request medication refills and send messages to my providers.  I've learned that key info can be buried within appointment summaries...like the results of an ultrasound or my bone marrow biopsy.  I've mined that thing like a prospector in the Klondike Gold Rush.

One thing the online portal doesn't contain that the medical record does is the physician's progress notes.  According the the Wikipedia definition, "They are the repository of medical facts and clinical thinking, and are intended to be a concise vehicle of communication about a patient’s condition to those who access the health record."  There's that word record that I defined above.  Reading some of the progress notes in my health record, there are some suppositions and swags in there.

During my stem cell transplant, I developed a pretty common heart arrhythmia called Long QT syndrome.  It was pretty minor but was noted in my record.  What was also noted was that this was "most likely due to cardiac involvement from amyloidosis."  NO!  None of the tests (echocardiogram, cardiac MRI) showed cardiac involvement from amyloidosis.  There are many medications that can cause this arrhythmia and the opinion of those in the know...my oncologist and her team...is that it was due to medication.  The fact that it is now resolved seems to support that.  But some well intentioned practitioner supposed or SWAGed that since this patient has amyloidosis and it is known to effect the heart therefore this arrhythmia must be due to cardiac involvement from amyloidosis.

Reading through the progress notes from my primary care physician while she was looking for a diagnosis also shows some suppositions and SWAGs.  When I came in with the edema in my legs she noted that it could not be due to chronic kidney disease or congestive heart failure, the two primary causes of unexplained extremity edema.  What "evidence" did she have to support this?  None...she never game me a urinalysis or an EKG.  The record said there was no blood in my urine.  How did she know that?  She asked me.  No tests to confirm...no evidence...just supposition or SWAG based only on what could be seen, not what was proven.

She diagnosed me with fatty liver even though that is primarily due to obesity or alcoholism.  We know the obesity option is out and she never talked to me or Al about whether I had a drinking problem.  But it was a convenient supposition that explained away some of the symptoms with a somewhat benign, or at least not urgent, condition.  When I asked Dr. Levin about how he went about diagnosing a hematologic condition when he's a gastroenterologist, he said, "The fatty liver diagnosis just didn't make sense."

Thankfully, Dr. Levin recognized the supposition or SWAG and did not build his diagnostic approach on her perspective.  I'm glad he didn't fall victim to the problem of confirmation bias -- once a finding is recorded, human nature seeks to confirm rather than refute it.  It's hard for an unsupported and inaccurate supposition or SWAG to be forgotten.  And, early on in my diagnostic process, it seems like a lot of diagnoses were "thrown against the wall to see what sticks."  Knowing that confirmation bias exists, it seems like it would help for practitioners to share the evidence that supports their statements and the level of certainty they have in the statement so others don't try to build on something that's a pretty shaky foundation.

No, this isn't another rant about my delayed diagnosis.  But it is a rant about the evidentiary standard that exists to record information in a patient's health record.  Although I'm not sure if you can rant about something that doesn't seem to exist.  Back to the definition of a progress note, I see periods short on facts and clinical thinking, and heavy on unsupported guesses.

As I look at my medical record, there's no way for a practitioner to link what appears to be a suspected diagnosis with the evidence that backs up the statement and/or the practitioners level of surety that the statement is accurate, ie is it a record (evidence), supposition (uncertain belief) or SWAG (just a wild ass guess)?  In the grand scheme it may seem minor but I hear so many horror stories of Amyloidosis patients who go years before being diagnosed.  I know how frustrated I was with my four month process.  I often wonder about the impact that medical record as information dumping ground has on introducing confusion or interfering with the clarity that's needed with a disease as difficult to diagnose as this.

I spoke at the INFORMS Business Analytics and Operations Research Converence in late March and Tom Davenport, de facto king of business analytics, spoke about the power of unstructured data in the world of Analytics 3.0 and the increasing role of data in medical decision making.  He quoted the CEO of Novartis as saying that informatics is just as important as basic biology and chemistry in the development of new drugs.  Davenport went on to say that "50% of data in an electronic medical record is unstructured physician notes."  Looking at my record, it worries me to think that this unstructured data would be used for evidence-based decision making since so much of the physician notes in my record aren't really based on evidence. 

It's too bad that anyone with access to record a progress note can enter something that has been definitively diagnosed or something that is just a supposition and, to my untrained eye, there is no clear way to tell which is which.

I'm guessing labels of record, supposition or SWAG are out of the question.