[As presented at the American College of Physicians-Alaska Chapter meeting http://www.acponline.org/meetings/chapter/ak-2008.pdf on June 26, 2008]

Richard Neubauer, MD, FACP

Mostly these talks have been well received, but last fall I faltered and my talk didn't come off very well. It's hard to believe that one could stumble speaking about something that intimate, but public speaking can be that way.  Anyway, one of the evaluations I received offered more than just suggestions for personal improvement and suggested that a further exploration of this subject -- more than could be done with just a one-hour lecture -- would be useful.

As I thought about that and searched my memory of all the medical conferences I've been to and seen advertised, I could not recall seeing a conference on Physicians as Patients.  So, while I figured it was worthwhile to try to put together such a conference, I also realized that the success or failure of the venture would be highly dependent on the quality of the speakers. So, besides reordering my own presentation, I tried to assemble a panel of speakers able and willing to give their insights about something very difficult -- their own, or their family's, experiences as patients.

This conference and the presentations we are about to hear are thus an experiment of sorts. What can we all learn from the experiences of fellow physicians who have become patients? Can these lessons make us better doctors to our physician patients and for that matter for all our patients? And, for those of us who have never experienced illness, are there ways to prepare for the inevitable time when that will change? My talk today is intended as an introduction to the conference, and to the other lectures that follow. I hope the "experiment" goes well.

Getting Diagnosed 

As I've mentioned, my own experience as a patient began when I was diagnosed with primary amyloidosis.  I'll start with a brief outline of events. Amyloidosis was discovered as I was evaluated for a progressive sensory and motor peripheral neuropathy that also had autonomic features. The diagnosis was first suggested when a serum protein electrophoresis demonstrated a monoclonal gammopathy, and amyloid was first demonstrated on a bone marrow biopsy and later on biopsies of abdominal fat and a sigmoid biopsy.  After my initial diagnosis, I had an autologous stem cell transplant, a treatment which involves a massive IV dose of melphalan followed by administration of autologous stem cells that were collected before administration of the melphalan. I had a partial remission and some subjective clinical improvement, but over time the monoclonal protein has increased again, and I've had further slow progression of the neuropathy in recent years.  I've had several trials of additional treatment with a thalidomide analog, corticosteroids, and additional oral doses of melphalan, but these don't seem to have significantly altered the course of things.

Let me make a few digressions at this point. First to return to the "bad job" I did on a prior lecture. I recall that when that happened, I was at a difficult time: knowing that I had progression of disease, having failed to improve on or poorly tolerated a couple of the medications I had been offered, and consequently being uncertain about what to do next. In my own situation, the good news (and simultaneously the bad news) is that I have a progressive disease that is doing damage very slowly. Given the slow pace of my disease, the benefit of any treatment needs to be weighed against the damage the treatment could do. That kind of uncertainty is very difficult to deal with. I know it had something to do with my "bad job" that day because I was troubled by the choices I faced and was uncertain about my future. But I've come to recognize that illness is a journey (especially a chronic illness) with many ups and downs. While I've tried hard to be a "good patient," I've also learned not to be too hard on myself for mistakes I've made along the way.

That leads me to another digression. The brief clinical outline of my illness I just presented leaves out some interesting points. While I was diagnosed in 2002, in retrospect I started having symptoms a few years earlier. In fact, I can be fairly precise and recall the first symptoms starting after a heli-ski trip to the Canadian Rockies that involved a particularly strenuous week of powder skiing. At first, I noticed that my legs "fell asleep" for a seemingly long time when sitting with my legs crossed. Subsequently I noticed sharp stabbing pains in my feet. At the time, I was working out regularly, downhill ski racing with a masters group, and working hard in my internal medicine private practice. I was in great physical shape and I thought I had plantar fasciitis. When things progressed and I started to have numbness in my feet as well as the sharp stabbing pains, I knew something else was up, and my own assessment was that I had a peripheral neuropathy. While I realized I needed to avoid being my own physician, I also knew what kind of evaluation I was in for and frankly, I did not want any of the diagnoses that might explain my symptoms.  For awhile I carried around an index card with "the work-up", telling myself that I'd have the tests done and then get an appointment with a neurologist to confirm the diagnosis and look over the test results. Ultimately, I was forced into it when I rubbed a blister on my foot related to the neuropathy, it got infected, and it got me in to see the neurologist, who confirmed my index card list of tests (including the serum protein electrophoresis that pointed to the ultimate diagnosis).

One final digression is related to when I went in for my bone marrow examination to further evaluate the monoclonal gammopathy. At that point we were doing the marrow exam to exclude multiple myeloma, but we also knew that amyloidosis was a possibility. As I lay down for the marrow exam, I asked what studies were to be done on the specimen. A test (special staining of the specimen) for amyloid was not included and I asked that it be added. My oncologist and I discussed this back and forth a bit -- he felt it was not worthwhile since marrow specimens are rarely "positive" for amyloid, which is more consistently found on other types of biopsies. He added the test at my urging, however, and as I outlined, it was positive. It was a small thing, but it simplified the tests I had to go through to get a diagnosis, and that meant something to me. It is hard (and in my opinion not generally appropriate) to ignore your knowledge of medicine just because you are the patient.

I think there are several points to take away from these digressions. First, that denial is a very powerful force and may be especially prominent in physicians who "know what they are in for" and thus may be more prone to denying or minimizing symptoms and thus delaying care. In my own case, it was a great relief when I found out what was wrong with me and didn't have the burden of uncertainty that I suffered with before the diagnosis was clear. I also think the simple doctrine that we are all taught that you should not be your own doctor is a bit oversimplified. I don't think physicians can just turn off their analytical minds and fund of knowledge of medicine when they happen to be the patient. To be sure, that may make them more challenging as patients. But I think the answer is channeling that participation in their care rather than denial of it and attempting to treat the physician-patient "just like everyone else" (at least in this particular aspect of care).

About Amyloidosis 

I cannot avoid saying just a bit about amyloidosis and peripheral neuropathy, both of which I've now become intimately familiar with. While clinical aspects of disease are not the main thrusts of my talk, knowing something about the illness that confronts me has been important and has become an integral part of dealing with the illness for me. Thankfully, the sole clinical manifestation I've had of amyloidosis has been the neuropathy. While this is bad enough, amyloidosis is one of the great masqueraders of medicine and can show up in many ways, including cardiomyopathy (congestive heart failure), nephrotic syndrome, and hepatosplenomegaly. Many of these things that I have not had tend to bring patients to their knees quickly -- amyloid cardiomyopathy is particularly deadly and has a poor prognosis. Likewise, neuropathy can have many different etiologies.  Many cases of neuropathy are idiopathic, so you can go through a comprehensive set of diagnostic tests and still not find an etiology.

Many patients who are ultimately diagnosed with amyloidosis go to multiple physicians for months or even years and have many diagnostic tests done before coming to a diagnosis. I suspect there are patients with amyloidosis who are never properly diagnosed, since it is rare and requires that one think of the diagnosis and get a biopsy in order to make the diagnosis.

Amyloidosis is also interesting in that it tells something about proteins and how they behave.  A number of proteins or protein fragments can cause amyloid deposits. A significant question is: why would a variety of proteins that seem unrelated all form deposits with a specific structure (described as a beta pleated sheet due to the appearance under an electron microscope)? One way to conceptualize this is that proteins have a folded structure and that if the process of folding is disrupted by a mutation or a change in the way the protein is manufactured in the cell where it is made, the proteins can fold in abnormal ways that lead them to assemble into deposits. In fact, a new field of proteomics has recently developed to try and better understand this and other aspects of the structure and function of proteins.

My particular type of amyloidosis is related to a particular "clone" of plasma cells having lost control and overproducing one particular type of immunoglobulin -- a so-called monoclonal gammopathy. In the United States, this is the most common form of amyloidosis and is also called primary amyloidosis.  I've envisioned this as a "two hit" phenomenon. Many people develop monoclonal gammopathies, especially as they get older. Some develop a cancer called multiple myeloma, but many can have monoclonal gammopathies that remain completely asymptomatic. A small subset have proteins with the right physical-chemical properties to form amyloid deposits that may be toxic to one or another organs. So if a common thing (having a monoclonal protein) and an uncommon thing (having the monoclonal protein, or a fragment of it, to have just the right chemical properties to form deposits) happen together, you can get this rare disease.

While I'm not a research scientist, and I'm not personally contributing to figuring out the mysteries that surround this illness I suffer with, I do find it comforting to know that others are working to do so, and that it may lead to better treatments for others in the future.

Coming to Grips 

I've already mentioned that I started having symptoms awhile before a diagnosis was made. While I'm not sure I can put a date to it, there was a point where I started to have inklings of impending doom. As I look back on that time, it is a little hard to put it in perspective, but I'll try. At that time, I was in my early 50's, I had a busy general internal medicine office and hospital practice, and I was governor of the Alaska chapter of the American College of Physicians. Additionally, I had a son in college and a daughter in high school. I did not want to be sick and wasn't sure how I would fit an illness into my busy schedule and future hopes and plans. But at the same time, my body was telling me things, in addition to the specific symptoms I've already described. I was getting tired a lot, and just didn't have the energy I once had. I also seemed to have to work extra hard in my ski racing just to maintain, much less improve. I've already talked about denial, but at some point in any illness that is causing symptoms, especially a chronic one, I believe that patients come to grips with the illness and prepare themselves accordingly. I prepared myself for the worst, and realized I might die from whatever it was I had. In some ways, my survival to this point has surprised and delighted me. 

I've come to look at my "time" since this all started as a bonus. So here are just a few of the things I've celebrated and delighted in during my "bonus years". My son graduated from college and went on to work in Americorps and the Student Conservation Corps as a supervisor. Currently he's working on the professional ski patrol at Vail. His goal is to ultimately work as a paramedic.  My daughter graduated from high school and went on to college and is now in her final year.

An interesting juxtaposition for me is that as my illness began and then evolved, I was becoming increasingly involved in the ACP [American College of Physicians].  It has been ironic in some ways that some of the most significant work I've done in organized medicine has occurred since I became ill. It has been my goal to make something that could be just plain bad into something that has aspects of good. 

Being a Physician with an Illness 

I believe it is impossible to separate your lifelong experiences as a physician from how you will experience a personal illness.  In my case, I've had this chronic illness, and I also have an ongoing career. In the last year, I've moved from private practice to a new position as chief of internal medicine at the Alaska Native Medical Center. I've learned some new clinical skills there, and have enjoyed the challenges of a largely administrative position. In 2005 I moved on from the ACP Board of Governors to the Board of Regents. My history of illness and my professional life have truly been a very interesting juxtaposition

I acknowledged earlier that it is very important not to treat yourself; not to be your own physician.  But I think it's also inevitable that as a physician you are going to be involved in your own care in ways that are unique and different. I believe that probably is true as well for your involvement with an ill family member or a spouse.  And I think that's one of the main difficulties that physicians treating physicians have: trying to come to grips with how to incorporate this into their notions of what's right and wrong in terms of treating patients.  However, while there are challenges, I also believe that treating another physician can be and should be one of the richest experiences that you can have as a doctor. I know that when I've had the privilege of treating other physicians or their families, those experiences have been very special.

Dr. Comenzo and Dr. Neubauer

I recently went on a trip to Italy, visiting the family of an exchange student that we had stay with us ten years ago.  Over the years, I had been reading articles written by Dr. Giampaolo Merlini, one of only a few world experts on amyloidosis who worked in Pavia -- not far from the area in northern Italy that I would be visiting. I kept in the back of my mind that it would be of great interest to meet Dr. Merlini, but it seemed farfetched.

Much to my amazement, Sylvia, the sister of our exchange student, Paolo, had become an anesthesiologist and worked at the hospital in Pavia, where Dr. Merlini worked. Sylvia had married an anesthesiologist who also worked in Pavia. Over dinner one evening I mentioned Dr. Merlini's name and Silvia's husband said, "I do all of his pain management.  I'd love to set up a meeting for you to meet with him." 

So I got to spend about an hour viewing Dr. Merlini's lab, hearing about his research, and he talked to me about my illness. It was a rich and humbling experience. I was going to take a picture, but it just seemed a bit out of place.

Three Memorable Patients

I'd like to transition to another theme related to how we as physicians learn from our patients. I'll describe three of my own patients who touched me deeply and from whom I learned things that formulated many of my ideas about how to approach my own illness.

The first was a harbor master at one of the communities in the Aleutian Chain.  I had cared for him for many years when he developed a horrible gastrointestinal vasculitis with recurrent gastrointestinal bleeds.  He was in the hospital for a very long time and continued to have progressively worse bleeding. The smell of melena can be intolerable, and at times it could be a task to be in his room for any length of time. He was a very fastidious man, and I can only imagine how difficult it was for him to suffer with this illness.

Yet every time I went to see him on daily rounds, there was a book by his bedside.  The dignity and continued interest in life that he displayed in dealing with this terrible experience was extraordinary.  He finally passed away, but even at the end there was a book at the bedside. He was reading and interested in life right to the very end.  It was an incredible experience to watch that and deeply moving.

The second patient I'll describe was a lady with pancreatic cancer, and whose care I shared with an oncologist.  Unfortunately, she was not a surgical candidate and did not do well with chemotherapy. She lived alone but wanted to be at home for as long as possible, but eventually weakened to the point where she could no longer cope. While this patient had an oncologist, she had made it clear that she wanted me to be the one to care for her in her final days. So, over the lunch hour one of my nurses and I went to her house, carried her to the car and brought her to the hospital. Over the next couple of days I did my best to give her a good death, but throughout the experience felt particularly privileged to have had her specifically chosen me for the task.

The third patient and final patient I'll mention had an upper GI malignancy and was having what amounted to a very interesting death.  While she was totally awake and alert, she could not eat at all and was unable to do any of the things she enjoyed in life. She was also in constant pain that was responding poorly to even large doses of narcotics. I put her in the hospital, but the usual measures just were not working well, her cramping abdominal pain continued despite industrial doses of narcotics, and at the same time, she continued to be totally cognizant and unhappy with the situation. The discussion went something like this: "Look, I want to get this over with.  I don't want to be here for very long, and I am having a lot of pain despite all this morphine you're giving me.  What else can you do?" While there could be a number of possible answers, I called a good friend of mine, a palliative care physician in California, for advice. We devised a cocktail of medications including octreotide to decrease motility and secretions, and within a short time my patient faded into a coma and died comfortably. I felt good that I'd been able to provide this for her without calling in an army of consultants.

When I became a patient, I thought about these patients and others I'd had the privilege to treat over the years. It has been my continued goal to be a good patient and I've had many teachers over the years!

The Changing Face of Medicine 

There has been a resurgence of interest in medical professionalism lately, fueled in part by the changing face of medicine. Physicians nowadays are looking for ways to better control their lifestyle, allow for more family-friendly work situations, and avoid the workaholic habits of many physicians currently in the workforce. The Charter on Medical Professionalism, sponsored by the American College of Physicians Foundation, the American Board of Internal Medicine Foundation, and the European Federation of Internal Medicine, is one attempt to codify important concepts of professionalism and acknowledge that professionalism is being threatened by changes in the way health care is delivered in our industrialized world. The Charter sites three fundamental principles: the primacy of patient welfare, patient autonomy, and social justice.

I think that one piece that might be missing from the charter is the obligation that each generation of medical professionals has to pass on (both by example and by teaching) the fundamental concepts of professionalism to subsequent generations. Another aspect of medical professionalism that the charter skirts around is the obligation of society to create an appropriate context for professionalism to thrive within. Physician-patients are uniquely situated to test and address some of these concepts.

Paranoia Over Privacy

One of the things I became interested in early in my illness was privacy. My thesis, outlined in an article in the Annals of Internal Medicine¹, has been that an overemphasis on privacy can isolating for patients, become a problem in and of itself, and that the complicated HIPAA privacy rules can stifle dealing with illness in fundamental ways. Being a patient is a fundamentally isolating experience, and can be a very lonely one.  A commentary that accompanied my piece discussed how physicians can respond to societal rules that have legal implications but which may in one way or another hamper the effective care of their patients. I urge you to read that commentary.  It outlined the obligation that physicians have to advocate for change while continuing to follow the rules. 

One of the things that I did when I was going through my stem cell transplant was to use a web site to communicate with friends, colleagues and my patients (http://www.thestatus.com/). It kept me (and my wife) busy and was a great way to openly share my experiences.

I am struck by some extreme differences in how privacy is perceived. Another physician from a community elsewhere recently shared an experience with me that was totally counter to mine. He developed prostate cancer and was concerned that if word of this leaked to his patients that they would quickly abandon his practice. I experienced nothing of this sort, and when I returned to practice after recovering from the transplant, my patients welcomed me back graciously. They all knew about my illness and I received a huge amount of support from my patients.

Cheryl Curtis

Support of Family and Friends 

Support of family, friends and pets is of huge importance when dealing with illness. My family was incredibly supportive and I know there were many moments where I could not have coped without them.

Let me just relate a story about one of my dogs, Charlie, who unfortunately died about a year ago, but was with me through my illness and I with hers. She had cancer that was discovered about 9 months before her death, and we actually treated her with chemotherapy, something I never thought I'd do with a dog.  The chemotherapy got her through an extra summer and she spent that wonderful summer with our kids who loved her greatly, so it turned out to be a worthwhile thing to do. 

Charlie had a great death and was for me a study in courage.  She had gotten to know all the people at the vet very well as she went through chemotherapy. When it was time to put her down, we bundled her up and brought her to the vet's office and all the staff -- fully 15 people -- came into the room and stayed as she got her injection. One of the staff members had saved one of Charlie's favorite toys that had been inadvertently left behind from a chemotherapy session, and she brought it in to the room to be by her side. She passed away in the presence of friends and her family.  She was a really neat dog.  She had some strange habits to be sure; one of them was collecting rocks.

Charlie

Illness and Art 

Quoting from one of Hippocrates' aphorisms: "Ars longa, vita brevis," or "Life is short, art is long." Creativity can be very therapeutic, and while turning to an artistic pursuit is certainly not something that is restricted to physician patients, physicians as a group may be perhaps a good index group in regards to this, since they are likely to have devoted themselves to a busy professional life and had to put artistic pursuits on a back burner. I'd always been interested in photography, but I often did not have the time to really pursue it. I mentioned earlier that I chronicled much of the time around my transplant in pictures, but I also vowed to myself that if I survived, I would make more time for photography. In fact, I've done so, and in the intervening years I even started a small business selling my photography. At least I've been able to support my habit for equipment! I'll just show some of my more recent pictures -- photography has become a great outlet for me and the therapeutic value of having such an outlet has been considerable. (Some of Dr. Neubauer's photographs can be seen at this website: http://web.mac.com/richard_neubauer/Site/Welcome.html)

Merging Science and Humanity 

My belief has evolved over the intervening years that going through an illness brings out a quality that for lack of a better word I'll call spirituality. For some this equates with religion, but I'm referring to something basic that we all have, whatever our religious beliefs may be. It has to do with questions like "What's the meaning of life?" "What's it all about?" "What are we here for?" and similar basic questions that may not come up in day-to-day life but that confront someone faced with illness and the possibility of death. 

I believe that we as physicians carry a great responsibility of trying to merge science and humanity.  Obviously getting the science right is important, but I think the humanistic part is perhaps arguably just as important and sometimes harder to do.  As jobs in medicine become more compartmentalized and move toward more shift work and site-specific care, this becomes more difficult.  I don't by any means think that merging these different aspects of medical care is impossible, but I think it becomes more challenging given the direction that medicine has gone recently.  I think also patients' perceptions of quality care rely quite heavily on getting this particular combination of things right. Physicians who have become patients have a special window on this aspect of care, and tapping their stories and experiences as patients may be one way to enhance our knowledge, understanding, insight and sensitivity in this area.

A Health Care Non-System 

I also think physician patients observing the medical system from their new perspective of receiving care have a unique opportunity.  Personally, I don't think we have a health care system right now.  Rather, we unfortunately have kind of a non-system with bits and pieces of functionality in a sea of confusion.  If anything, I've felt more that way after going through things as a patient than I did before that.  Maneuvering through this morass is difficult, even if you're smart about it, and for anything but simple medical encounters, medical bills can be just as much of a challenge, if not more, than obtaining care.  Furthermore, anyone who has been through a major illness is not likely going to be a very good advocate for change because they've likely been beaten down by things, and I think they're probably a little afraid to rock the boat -- especially if they have skin in the game and have ongoing problems and need ongoing care.

I had an interesting personal experience in the last year. I'd been in private practice for 26 years and had health insurance through my practice. Without warning I received notice that my health insurance was going out of business. By coincidence, this happened after I had made a decision to retire from private practice and move to a new job as chief of internal medicine at the Alaska Native Medical Center where my employer would provide insurance.  The timing was both uncanny and unplanned and so I was lucky. I continued to have uninterrupted insurance. Had things worked out differently, given my medical history I would almost surely been uninsurable and would have had to get the Alaska safety net insurance from the Alaska Comprehensive Health Insurance Association (ACHIA).

It was an odd and disquieting feeling to be a physician, having spent my life taking care of patients, facing the prospect of having to rely on a safety net for insurance just because I had the misfortune to have an illness.  It didn't feel good, I'll say that, and it reinforced my feelings that our system is terribly unfair and cold-hearted toward the needs of our people.

There have been a number of things in the newspaper that I'm sure you've all read about patients' perceptions about understanding health insurance, rate adjustments that are skyrocketing, costs of the insurance.  When my insurance company went out of business, my health insurance for myself, my wife, and my one child was costing $24,000 a year, and that's for insurance that certainly was not luxury insurance by any means.

In my view, physicians have an obligation to advocate for change when they see injustices and inadequacies, and these abound in our medical non-system. Physician patients are in a special position to experience some of this firsthand and I hope can play a special role in advocating for change.

Advocacy and System Reform 

I think that changing our health care system is only going to happen through the political system.  I think physicians need to play an active role in that process.  I also think advocacy is going to be a slow process.  I don't think we're going to see results instantaneously, and that's especially frustrating to physicians who are used to controlling their environment perhaps more than others.  We are gathered today at a chapter meeting of the American College of Physicians, an organization that has chosen to present positive ideas for change rather than just grinding in our heels to protect a status quo. I'm very proud of ACP for being an organization that represents that pathway.  I'm sure David Dale and Bob Doherty, who are going to speak later, are going to talk about that.

ACP is really uniquely positioned to have a big part in comprehensive system reform based on policies that have been carefully put together by the organization, and also the careful approach that the organization has taken to present these ideas. I've enjoyed the rare privilege to be a small part of ACP governance, and the experiences I've been through as a patient have certainly played a role in how I've viewed the importance of advocating intelligently and carefully for change focused on the primacy of patient welfare.

Summary

Illness is fascinating in many ways.  We as doctors are presented with fascinating medical issues, but also fascinating human issues. I've tried to summarize a few of the themes that I've thought about over the last six years since I became a patient. I believe we need to continue in a quest to merge science and humanity in a system that tends to dehumanize patients.

My own involvement in the ACP first on the Board of Governors and now on the Board of Regents has corresponded with a time of personal illness. In some ways my involvement in the college is reinforced by my own experiences as a patient.  I've certainly found such involvement a wonderful way to get through difficult things and find higher meaning.  I think physicians are uniquely positioned to see the weaknesses in our system, and I would urge that as we see those things, we need to advocate for change.  We need to do it in effective ways that really work.

I look forward to hearing the other lectures that will follow and I'd be happy to take a few questions.

1) Neubauer R, Paranoia Over Privacy, Annals of Internal Medicine: 2006 August 1; 145; 228-229

Question-and-Answer Session 

Q:     Rich ... your life advancement chart, you go through a series of different stages.  It seems to me as physicians we have a harder time mustering the denial to help us through something like this.  Could you tell us what your anxiety level was like at those different stages?  Was it constant, or did you find it waxing and waning depending on what was happening?

A:     Waxing and waning.  But my own personal thing was trying to analyze and figure out the right pathway.  My anxiety level was highest when I couldn't figure out what the right answer was.

Q:     Have you figured out what the mechanism is of why amyloid causes peripheral neuropathy?

A:     Interesting question, and the answer is I don't think anybody knows.  Amyloid is deposited everywhere.  There are even cases where by accident somebody's diagnosed with amyloid on a biopsy specimen and they have no clinical manifestations, so there's essentially no toxicity of the deposits.  There's a series of those cases that were followed by Dr. Kyle at the Mayo Clinic over the years. 

Amyloidosis can cause nephrotic syndrome, hepatosplenomegaly and hepatic failure, cardiomyopathy and neuropathy amongst other clinical menifestations. The reason why the deposits are toxic in any given tissue is not currently understood.  It may be direct toxicity, in the case of primary amyloid, the light chains that are deposited.  It might be something about the deposits themselves.  It might be an ancillary component of the amyloid, because there are some other proteins that get incorporated.  Nobody knows and it currently remains an active area of research.