Happy New Year!  My update is long overdue. Judging by the reaction of some, to Bruce’s last blog entry, I feel I need to clarify how we are doing right now.

One of Bruce’s all-time favorite movies is MONTY PYTHON AND THE HOLY GRAIL.  There is the memorable scene in the plague-ridden town where they cry, “Bring out your dead!  Bring out your dead! ” One old man carried out on a stretcher says, “But I’m not dead yet!”  That’s Bruce.

It is hard to hear the dreaded ‘H’ word – the H, of course, being Hospice. In the United States, we don’t do hospice well.  We wait far too long to take advantage of the hospice benefit.  Sometimes, the person who is dying is not even aware they are in hospice, because hospice wasn’t called in until the patient was in a coma.  We hear “Hospice” and we all jump to the conclusion that death is imminent – a matter of a few days.

Bruce is still here and still very much alive.

Hospice is a philosophy. The main idea is that when a person is within 6 months or so of death, they ought to be made as comfortable as possible so that they can wrap up their life, say the things they want to say,do the things they most enjoy doing, and be able to say their goodbyes to the people they love in a planful way.

We began hospice in mid-September and often wonder why we didn’t start sooner.  Our lives have greatly improved since beginning hospice.

There are the little things, like not having to remember to refill prescriptions and figure out the logistics in order to pick them up. Hospice delivers all our meds and supplies to our door and pays for them as well.

No longer do I need to worry about how on earth I am going to get Bruce to a clinic to check a possible ear infection, or a skin rash or a sinus infection.  Hospice sends out a nurse practitioner to check on him in our living room.

Weekly visits with our  lovely hospice nurse allow us to ask worrisome questions, draw on a wide variety of medical expertise and specialties, and then decide on a course of action as a team.

Instead of carrying the whole heavy burden of Bruce’s ALS by myself, I suddenly have an entire caring team to help ease my load.  We have wonderful aides who come and shower Bruce, 2 fabulous masseuses who soothe his pain with healing massage, grief counselors and a social worker who check on my emotional and physical health, as well as the well-being of our sons and daughters-in-love.  We feel so supported!

And yet………. Death is here, in the corner of the room.  Death is patient; it isn’t Bruce’s time – yet.  Once in a while, Death nods his head, gives us a wink – as if to let us know, “Hey, I’m in no hurry. Take your time.  I don’t mind waiting.  I’ve got a whole briefcase full of cases I can go through while I’m waiting for you, Bruce.”

We welcome your loving messages. Better to say the things you need to say now, when Bruce can answer you back.  I am creating a scrapbook of your loving notes to share with my kids, their wives and my grand-kids (born and those not yet a twinkle in someone’s eye).  If you haven’t written a “Dear Hypatia” note yet with your memories and impressions of Grandpa Bruce, please do so now.

Bruce is still very much involved in life.  His book, WE KNOW HOW THIS ENDS:  Living while Dying is available for pre-order from the University of Minnesota Press as well as on Amazon.com.  Bruce is involved in plans for launching the book and for creating an ‘enhanced E-book” version as well.

He tires more easily these days and is entirely dependent upon his Trilogy machine to help him breathe. As his muscles waste, his joint pain increases so he is taking more medication for pain these days.  But Bruce enjoys visits with friends and colleagues. Please call and arrange a visit. Just watch for signs that he is tiring.

Thank you for all your prayers,  positive thoughts and caring messages as we walk together towards the Next Big Adventure.

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Just One More Time

Not that the phenomenon is unique, but in Minnesota you can bet that you have been fully assimilated into the culture when you participate in the “Minnesota Goodbye,”a highly ritualized, almost liturgical set of behaviors. The Minnesota Goodbye is a cultural artifact with its probable roots deeply held in the Germanic, Scandinavian, Southeast Asian and sub-Saharan African populations, not to mention the Irish and the English, that so richly spice our little corner of these United States. Combine cultural roots with a weather pattern that does not encourage going outside for at least six months of the calendar year, and you can probably begin to understand how the Minnesota Goodbye became the well-developed ritual that it is, and why it has taken on an almost religious function in our social practice here on the frozen tundra.

The Minnesota Goodbye occurs when visitors indicate they are ready to go. The host carefully walks them to the door where host and guests stand and chat as if the evening is young and they have not been together for the past three or four hours. Often, the most important exchange of information at a social occasion takes place during the Minnesota Goodbye, for it is in this space where host and guest are assured of one on one time together. Frequently punctuated by the phrase, “let me say just one more thing and then we will call it a night,” the Minnesota Goodbye has been known to last for upwards and beyond an hour in length and for those who are not accustomed to its rituals, it can seem almost irritating in the breadth of its timing and depth of its sharing. Those of us who are not native to Minnesota find ourselves asking why we did not engage in such conversation during the evening’s socializing, or at least why we held such an important discussion until we were at the door. When I first came to Minnesota, I found myself asking such questions (not to my guests of course) of myself and my wife. But eventually as we shed our otherworldliness and cloaked ourselves in the cultural mores of Minnesotans, we became accustomed to the rituals associated with leaving, and now participating in the Minnesota Goodbye seems so normal that to not participate in its rituals would leave us feeling empty, as if our visits were superficial. And we have come to appreciate the Minnesota Goodbye for its utility in ensuring that what must be communicated between good friends is actually shared, that what should be said does not remain unspoken, that a silent chasm between good friends cannot exist. The Minnesota Goodbye is an assurance that just one more thing cannot be left out of the evening’s visit or the joy that comes with friendship’s deep communication.

Medical practice can be very much like the Minnesota Goodbye.

No doctor wishes to leave any stone unturned in the treatment of her patients. Good doctors especially want to make sure that they have considered all relevant variables and possibilities as they construct a recommendation for appropriate treatment. It is the medical version of the Minnesota Goodbye so that just one more thing is often the rule and not the exception. Just one more thing has tremendous ramifications for patients. In the moment of medical crisis it can offer reassurance that all possibilities will be considered. Conversely, in the moment of deep peace, just one more thing can create disruption where it may not be necessary.

I know this firsthand.

My treatment up to this point is framed by the need for managing the ever increasing demands of my symptoms, even as they become more and more difficult to manage. For example, in July, I began to experience difficulties in swallowing. With the help of my neurologist, I worked through the pros and cons of having a feeding tube placed, in case swallowing became so difficult that I would not be able to eat. At that time, I decided that the risks associated with the operation – sedation, being in a hospital, possible side effects of a surgical procedure no matter how simple a surgeon felt it to be – far outweighed the possible good of the feeding tube, and thus I determined that the placement of a feeding tube was not an appropriate treatment for me at the time.

A number of my friends and doctors, extremely well-meaning in their probity, have asked me the question, “Why did you not place a feeding tube early on in your progression?” It is a good question and probably more indicative of what we have learned about ALS in the four years since my diagnosis than on any strong personal feelings about feeding tubes. When I was diagnosed, the focus question was on whether to place a feeding tube or not. Now, many neurologists who treat ALS are more focused on the question of whether one would like to keep their nutrition up for as long as possible, citing the fact that weight loss is highly correlated with lack of survival. Upon receiving an affirmative answer to the question of nutrition, only then do they introduce the question of the feeding tube. It isn’t so much that we didn’t know the importance of nutrition, but the fact is that four years ago, the medical approach focused more on the question of a feeding tube almost as if it was separate from the question of nutrition. Since I had experienced no difficulty swallowing, even up through January of this year, I did not wish to submit to one more procedure that would require care and attention by another person when it seemed that it was unnecessary. Hence, up until July when I was able to swallow without any concern, the placement seemed superfluous, and after July when I was able to analyze the inherent risks, my decision remained firm.

I have likened ALS to a train on the tracks, seemingly in slow motion, unable to veer left or right, coming straight at me as I am stalled in this body and am unable to leave the rails.I am highly aware that with ALS, the final cause of death will probably be a combination of lack of nutrition due to eating difficulties and the buildup of carbon dioxide due to breathing difficulties, more likely the effects of the latter. At this point in time when I need to see my respiratory therapist once a month to bump up the BiPAP’s airflow and exhalation, when I need to see my hospice nurse once a week just to bump up my symptom management, when the conversations that Ev and I have are liberally salted with discussion of just how we desire the end to go, I am at peace with the life I have been given, accepting of the death with which I am gifted, and grateful for every day that I still awaken. It is a good space for a person to spend their final months. And I think that my physicians, my healers, my beloved doctors and nurses and therapists are okay with me being in this privileged space.

And in this carefully constructed space, where we are at peace, just one more thing knocked on our door and turned our world upside down just one more time.

The best doctors leave no stone unturned. A dear friend of mine, a neurologist and expert, suggested I look at an alternative way to place a feeding tube. The logic was impeccable. Why should you have to spend so much energy and time on eating when the quality of your meal time could be focused on taste while nutrition could be taken through a feeding tube? And I had to admit that such a possibility actually sounded pretty good. We discussed a procedure that could be done with me awake and on BiPAP during the entire time. Yes, it would mean I would have to give up my Do Not Resuscitate and Do Not Intubate orders, for what surgeon would want to operate under such circumstances, but the idea of spending less physical time and especially less psychological time on eating was appealing. And, as I played out the possibilities with my family, I thoughts that I could see them lighting up with hope that such a procedure might result in longer survival, a better quality of life. But as I considered the downside to the operation, I had to be honest that with the intubation might come the need to utilize invasive ventilation, something that early on I decided I did not want to consider for my treatment. So, another way to look at this would be higher quality of life versus dying during the operation or a day or two after due to other complications.

I know that these decisions are extremely personal, and that what seems to fit the type of life that I wish to lead could be totally inappropriate for another facing the exact same decisions. I only share this to illustrate how easy just one more thing can suddenly raise itself into your world, your consciousness, your deep analysis and consideration. And that is exactly what happened. The relative peace and calm that we had worked so hard to establish was suddenly disrupted, turned on its ear. and everything that I had imagined as to what constituted a good death was thrown up into the air as I considered the procedure.

Here is the point. Whether you think such disruptions are for the better or worse, the fact is that even when you believe everything is worked out, it is not. And the process of my working through the feeding tube decision required consultation with two different neurologists, two different nurses, a trusted friend at the ALS Association, both of my sons and my beloved wife, and finally our hospice providers. And even though I perceived a hopeful outcome, the 10 days spent in attempting to make a well considered decision also raised feelings of disruption, resentment, irrational hope, and even despair for my family and friends, for me especially, as all of us considered the ramifications of the decision that had presented itself like a Minnesota Goodbye careening out of control.

And what finally led me to decide not to do the procedure? Two different pieces of information surfaced from my two dear neurologists – one was that even if the procedure worked it would not in most probability, lengthen my life; two was that this particular type of feeding tube was susceptible to needing replacement more often than I would probably find acceptable. But the piece of information that really cinched the decision was this: I would have to go off hospice. As I considered the ramifications, I realized how dependent we had become on our hospice care providers for the stability of schedule and environment that they gave us. We had established meaningful routines again, we knew who to call and for what, and we really liked the people with whom we were working as they seemed to understand our goals for peace and dignity and joy in the end. And in the end, it was the possibility of losing the human qualities that hospice had brought to this complicated process of dying, that led us to utterly and completely reject both the hopeful possibilities and the frightening counter indications of placing a feeding tube.

Please understand that there is no blame or bad feeling here. I just think it is highly illustrative of how easy the turn of one more stone can shift the foundation of the carefully constructed life. When you are dying, that careful construction is remarkably helpful. And while it is always good to consider the treatment possibilities that do exist, it is just as important to consider how such treatments can disrupt and affect quality of life. Consider how so many of my brothers and sisters in ALS reach beyond their own capacity for something, anything that might offer them a little more life. This extremely human desire makes all of us susceptible to the seduction that comes with the medical version of just one more thing. Who can blame us? So many are young with so much possibility ahead, cruelly robbed by ALS, so that the idea of just one more thing is very seductive. But if you stop and think about it, this susceptibility is more a statement of the value we place on the unknown future, than the gratitude we feel for a past well lived. I know people in their eighties who are having difficulty negotiating their impending deaths. Just one more thing appeals highly two and a irrational future when you cannot find peace with the inevitable present.

Yet, as right and good as I feel with the decisions I have made, with the space I am given as I wend my way toward death, I can still feel the pull of the Minnesota Goodbye. To remain in this peaceful, beautiful space, requires a concentrated effort, a projection of gratitude and acceptance, and the conscious receipt of a peace that passes all understanding. I have come to the point where just one more thing is fine for executing the ritual of the Minnesota Goodbye, but for realizing a life well lived,  just one more thing interrupts the stream of conscious love I am becoming, as I tumble into the river where all life finally ends and continues to its source.

And if I change my mind, I’ll make sure I tell you as we walk toward the door and bid each other adieu.

Grudge Match

I haven’t spoken much about the drug research that is taking place in ALS Land, not because I don’t have skin in the game.  I am in the open label phase with the Biogen Idec dexpramipexole trial, and although we haven’t been unblinded yet, the rumors are rampant. There are other trials as well, one in Phase 2 that looks pretty interesting, and a seriously hyped stem cell trial in Phase 1.  Before ALS, I never thought about drug research.  That has changed in the last two years, although I don’t claim deep knowledge. In terms of drug research, I think exploration of intents and unintended consequences is instructive.  More specifically, how the larger interests of dis ease research, oversight, investment and profit affect me is something that I take very personally.

In ALS Land, the race is on for anything that will supplement the only drug currently approved for treatment.  That drug, Riluzole, was approved in the 90’s with less than stellar effects, but enough to throw us PALS a bone on the drug front.  Our reality is that in the 73 years since Lou Gehrig was diagnosed, no other drug has been developed.  There is a clear sense that this reality could change.  The three specific drugs or treatments mentioned above have us buzzing, and one of the drugs, NP001, has really caused a stir among the PALS community to the point of developing self-treatments.

The methodology adopted by the FDA in coordination with the scientific community for approval of a new drug is designed to keep the public safe and to definitively show that any positive effects are both significant and resultant of the drug.  The development of any drug requires hundreds of millions of dollars in resources—gargantuan infrastructures that can only be supplied by Big Pharma or the federal government.  This means that the entrepreneurial researcher-developer can only take a new idea so far, usually through Phase 1—determining safety and possible dosage, and perhaps to Phase 2—establishing whether a belief in the drug’s efficacy is well founded.  Phase 3—the gold standard randomly assigned, double blind, placebo controlled (meaning neither the researcher nor the participant know who is on the drug and removing the possibility of placebo effect), is almost always beyond the resources of start up or small operation entrepreneurs.  It is in Phase 3 that all promising ALS drugs have not been just downed, but crushed.  Thus, any drug that makes it to Phase 3 now attracts high interest, even though Phase 3 has not been kind to possible ALS drug treatments.

As an example, Biogen Idec paid 80 millions for the rights to dexpramipexole and took on the cost of a Phase 3 study with 900 international participants at 82 domestic and 10 international sites.  I don’t know the cost of the dex study, but I do know that Research and Development costs for Biogen were reported at over 1.25 billion in 2011 for all the trials they were running.  That represented over 26% of their revenue.  Biogen Idec is into Knopp, the drug’s developer, for up to 265 millions if the drug makes it through the regulatory gauntlet. All these figures illustrate the point that it costs a pile of money to develop treatments; thus introducing two specific considerations to the process–profitability and perseverance.

Clearly, there are two sets of desired outcomes.  The first is that we need more research and drug development in ALS.  This is the outcome I care most about.  It is literally killing me that we have nothing beyond Rilutek, the drug name for the Riluzole compound.  And Rilutek is expensive.  If it was not covered by insurance, I know I would probably not take on the 4300 dollar per month retail cost.  So that brings me to the second outcome that must align for a drug to be made available.  It has to show potential for profit.  Biogen estimates that their return on dexpramipexole could be as high as a billion dollars per year, representing an additional 20% in revenue over 2011 figures.  No wonder they are willing to put out such huge amounts of money.  And you can only imagine the costs and returns when you get out of the orphan diseases like ALS and into something like diabetes or breast cancer.

Stay with me; there is a point to all of this.

I need to establish a context for the way things are and why it would be so difficult to construct new ways of seeking effective treatments.  The current system is a Holy Alliance of government oversight, scientific rigor and capitalism.  Each fulfills a role that, when it works, can be very powerful.  We have seen great results of this triad in such areas as space exploration, food production, technology, and healthcare.  But as so often can happen, it is easy to emphasize one side over the other two.  And right now, it is hard to tell which–science, government or capitalism–has the upper hand.  If we overplay the science, then research for the sake of knowledge; brittle in its method, religious in its commitment, closed to further development, oblivious of the human costs its liturgies exact, is the result.  Overemphasis on regulation unnecessarily drives up costs and delimits access.  Too much attention to the bottom line means that the goal of new and more effective treatments can be lost to the call of profit.  When you consider the enormous resources brought to bear, the delicate dance that must be affected in order to bring a new drug to market is breathtaking.

See anything missing?

The Holy Alliance makes it easy to lose sight of the lives most affected by dis ease.  What happens if a startup has a good idea, gets it into phase 2, shows some efficacy, not a home run, not enough to attract the deep pockets of Big Pharma, but some?  Probably nothing, and that is the point.

An orphan disease like ALS, with the complexity of its manifestations that we are just beginning to understand, is not susceptible to a knockout drug.  More likely it will be some drug combination, each compound fulfilling a specific role in a mighty cocktail targeting everything from genetic propensities to the macrophages that are eating axons to the energy needed to keep motor neurons alive. The current model does not encourage the kind of iterative approach that complex dis ease requires.  There isn’t enough profit, science does not like so many variables, government is uncomfortable with the regulatory complications a cocktail will require.  The current model engages three partners, none of who make dis ease management their primary concern.

Please recognize that for each of us already diagnosed, it is too late.  Even though hope is always present, it is not really possible for a drug to develop and then be brought to market in time for us unless we are in a trial. The realities of a system designed for blockbusters combined with the cross purposes of profit, regulation and science, plus the fact that those of us who live in the dis ease milieu have very little voice in actual research decisions, points to the surety that our fates are sealed by such a lack of creativity.  It is fundamentalism at its best–the religions of science and regulation and profit unable to conceive of new liturgies through novel methodologies or yet conceived ways to analyze and reanalyze data, because there is no reason except the dis ease to do so.

Which brings me to the title of this essay–“Grudge Match.”

The Google definition of “grudge match” is “A contest or other competitive situation involving personal antipathy between the participants.”  I admit my own bad feelings, dare I say grudges, for systems that have calcified with the inevitable drift toward self-service that all bureaucracies experience.  And I freely admit that my understanding is incomplete.  But I would point to two salient facts that the system is not as robust as the billions of dollars it costs.  First, there exists a Phase 4 in the drug approval process in which follow up takes place, and there have been some major clunkers revealed by Phase 4 analysis—hormone replacement therapy comes to mind.  Secondly, a system that slaves to market forces will always yield drugs for male erectile dysfunction over orphan disease management.  Neither of these bodes well for complex human dis ease conditions.

As a choral artist, I am well aware of the creative possibilities humans can forge together.  Perhaps we need to place as much emphasis on researching new, more robust and creative methodologies, achievable with the resources of an entrepreneur.  Perhaps we should make it the rule rather than the exception that members of different disease communities have much more to say about the direction of research.

Or the system could work and I swear, all grudges would be forgiven.

Measuring Up

I am on my way to Mayo for a quarterly ALS management consultation. This is my fourth consultation since being diagnosed, and my second at Mayo, where I have decided to go for my ALS Clinics. As early as I am in the “progression” of my dis ease, I am getting into a familiar routine with its management. There will be lots of observations, pushing and pulling by neurologists and physiatrists, blood drawn, pulmonary tests, OT and PT info, speech and diet evaluations, and an incredible amount of information offered (I might have a couple of questions not easily answered myself). In short, it is a time of measurement and prognostication, and I have to be sharp, even though the ALS Clinic is incredibly exhausting. The greatest challenge of this experience is to remain a whole being, in spite of the systemic reduction of me from human, to a series of measurements and symptoms indicative of dis eases’ control and promise.

I am going to share with you one of my earliest experiences with dis ease, as it is instructive. When Ev and I received my diagnosis, it was an assault on everything we hold as human. It was like a psycho-emotional cyclone, obliterating self, family, even our friends. My ALS diagnosis was delivered in a way that reduced me to my basest and most inhuman self. The brutality of the delivery inspired PTSD moments (and flashbacks). The way that the neurologist forced us into his measured reduction consisting of a series of medical tests and a presentation of symptomology, stripped away any delusions of humanness we might have held. Then to follow it up, he removed all hope through his masterful use of a psychic probe of suicidal tendencies (“I must ask you,” he said. “Do you feel suicidal?” “No,” I answered as I wondered if homicide was justifiable in this circumstance).

Don’t feel sorry for us. But understand that measurement has consequences when we reduce humans to merely the measure. I am thankful to have found healing in the love and caring of my partner in crime, my sons as they deal with their father’s mortality as imperfectly and perfectly as they can, and of course in the myriad of friends and colleagues who have reached out in so many significant ways. But measuring up to dis ease still remains. It is seared in my psyche as I move into this quarterly liturgy of managing the symptoms.

Thus, even in the supportive environment of an ALS Clinic, I find myself fighting through the reduction of me into a progression of symptoms, constantly telling myself that these folks have my best interest at heart, while carrying the fresh memory of diagnosis delivered even though it is yesterday’s news. I have to admit to you that I am so tempted to lie to them, to tell them nothing about this symptom or that. I know this is a delusion to maintain my own sense of control, but it would allow me to say, “I am in charge of this person dammit, and y’all can go play your measurement games with some other patsy!” I know I shouldn’t feel so defensive about something that is actually beyond my control, but I have this old normal need to measure up to the old ways, to behave as if everything, every measure taken, was normal. And herein lies the insidiousness of dis ease.

We really do lack control. All of us. To make life somewhat predictable, we have devised multiple sets of measurements by which we judge our circumstances and the people within them. And when those measurements fall outside the range of normal, a range of which we are not even aware most of the time, we create a new normal that allows us to control these seeming freaks of nature, as if we had control. Bigotry works this way. The big institutions–government, religion–work this way. And this is the way that dis ease management seeks to work–taking the remarkable, the extraordinary, the frightening and yes the supernatural–and reducing these to their simplest forms for management’s sake. It is classic. It is so typical for us as humans to reduce our experiences into these easily normed categories, that we have made the experience of reduction unconscious. Professional dis ease management takes this unconscious reduction, and makes it a conscious act, assuming that in consciousness lies truth.

There is a scene in the movie Apollo 13 where the astronauts rip off all their health monitoring equipment in defiance of the flight surgeons. Suddenly, they take back their whole humanness, by refusing to participate in the vitals and symptoms reduction that the monitors foster. As irrational as their actions may seem, I understand the way they feel. Are they really in control? Nope. They are still in deep manure with a spacecraft that won’t behave. But it feels like they are more in control, because they don’t have to measure up to the assumptions of the (so called) mission controllers. The scene speaks enormously to me.

If you knew me pre-ALS, you knew an active, healthy guy with all the unconscious assumptions by which he was measured. Now, with dis ease, I am framed by the symptoms and measurements held within ALS. To get the most out of my dis ease management, I must consciously tell myself not to lie to the team, that maybe some reduction isn’t a bad thing. Because deep down, even though my spacecraft is also not behaving as planned, I need to believe that I too exercise some semblance of control. In order to take this on in a meaningful way, I have to feel like I’m steering the ship. It is the human dichotomy of knowing that ultimately the ship will go where it goes, because of or in spite of the exercise of my will in a way that makes me feel I have influenced its trajectory.

ALS, dis ease has changed me. I am both more open, and more measured in my approach to life. I am more demanding of control, and I have less faith in control’s delusions. I am much more conscious of others’ perceptions as to whether I am measuring up, and I am much less worried about what others think those measures mean. Dis ease picks off all of those scabs, exposes all of those fallacies, and it reduces the superhuman medical establishment and the ordinary human day-to-day experience to the realm of merely mortal. Measuring up takes on a whole new meaning, and it is way beyond the meaning of the measure. In the end, the well meaning clinic, and the psyche battering neurologist will both have to get it. In spite of everything, we humans are basically in the hands of something much bigger than ourselves. Any control we might exercise, is only because our existence aligns with its ultimate purpose. I find comfort in this statement of faith, and as I get ready to start the Mayo run, it allows me my own measured reduction–my symptoms into the wholeness of being in this world.

It really is the only way to measure up.