Maundy Thursday, From the Silence

I was privileged to offer the following last Sunday.

Claire and Matt sit in a small office in the neurological wing at Clinic. The clinician has come to get me, just finishing up my own quarterly clinic visit, to ask if I would consider meeting them. I am so fatigued, but as she tells me that Matt is in his last weeks with ALS, that they read my blog, find my words helpful, and would like to meet, the only human choice is yes. I roll in, my daughter-in-law driving my chair, the clinician at my side. We immediately feel the desperate, resigned love, five people shaped by ALS in this moment together. Matt speaks through an iPad application, ” I’m doing as well as I can.” Claire sits slightly behind him, her hands on his shoulders willing him not to slip away just yet. She holds it together through some superhuman effort, telling us that she had to take Matt to the hospital and with a Do Not Intubate order, hospital staff were afraid they couldn’t bring him around. A chaplain had been summoned to pray over him and for some reason, when the chaplain touched Matt’s hand his eyes opened, he sat up and immediately started breathing again. Her tears belie her attempt at humor, “I have to find that chaplain to thank him, but I want him there the next time.” The next time looms over all of us in the room. “I am just not ready to let him go. Our kids are young, and when we went to the hospital, the oldest asked if his daddy was going to die tonight? I am just not ready.”

ALS crams a lot of story into short, breathless nights, minutes and hours and days and weeks of passion story.

Today is Palm/Passion Sunday. We Methodists tend to cram a lot of story into this day, partly because we don’t like to dwell too much on how dark the week feels, partly because we are so busy with lives that seem beyond the pale of such a story. If we could, we would probably compress the passion story even more, something along the lines of a tweet:

Jesus – triumph,Temple, Passover; Gethsemane – prayer, despair, arrest, denial; Pilate, Herod, trial; Golgotha –cross,cry, acceptance, death.

We Methodists cram a lot of story into this one Sunday.

In spite of its darkness, I have always loved holy week. It is the complete package, a story where each of us can find some element to which we can relate. Each of us knows what it means to succeed, perhaps even triumph. Each of us knows how passing such success can be, like turning a corner into sunlight only to become aware of the next storm on the horizon. Many of us have learned that success is nothing more than the question, “What have you done for me lately?” Indeed in my old life, no success was ever good enough because I knew that waiting just beyond the triumph, if I did not immediately move to address it, was possible and imminent disaster. Who among us has never felt betrayed or denied by friends or lovers, those we thought we could count on the most? Who among us has not perceived, even just a little bit, the lie that we are in control? Who among us has never felt so alone that we are sure even God has turned away. This is the stuff of life, blistering our emotional overlay into thick yet well-worn calluses of experience. Each of us knows how it feels to be helpless in the face of events. Each of us can point to some event where we feel like we have been figuratively, if not literally, crucified.

 

And each of us can understand viscerally, primally, the question, “Why have you forsaken me?”

You see how human the story is, this holy week? Jesus in the garden asking God to take the cup away. And here is something I believe. If he has become the human the Scriptures tell us, then he would not have said, “I will drink if this is your will.” Humans don’t start with acceptance, with “If it be thy will.” We have to hear the nothing voice on the edges of a cold wind, wrestle with God’s silence, balance in ever increasing despair and frustration between anger and sadness at the lack of perceived response. Jesus was alone in his loneliness, facing his own mortality, his own dis ease, just as we are alone in our loneliness facing our own dis ease, our own crucifixions. Christ’s loneliness screams betrayal and denial and anticipated pain. His loneliness breathes total despair. In his loneliness is his overwhelming humanity, longing to hear his father answer, entreating his father to break his heartbreaking silence. The cup of mortality will not be taken from Jesus, for now he is one of us, and mortality is our human gift.

“Will no one stay awake with me?”

When I was first diagnosed, I composed my own variations on the theme of “Take this cup from me.” The more I learned about what was coming, the more frightened and angry I became. What disease could possibly steal more completely the life that I loved, than ALS? To be stripped so naked of all the things I enjoyed – to hug, to sing, to kiss, to eat, to ride, to speak, to travel, to breathe – the cruelty was beyond my comprehension, and I could see a future where every loss would be another opportunity for anger and fear, slashing livid red streaks across my vision and into the very core of my being. No one could understand this, no one. And I would be alone. I cried aloud to God and I swear to you God did not answer.

I was so afraid.

3 1/2 years ago and dis ease has brought me to the precipice: Will I live into the life I have been given, or die in anger, frustration, grief? I don’t hear any answers from God, at least not at first. But then something happens. The answers appear, not as I saw them but in their own guise; first in a trickle of  prayers and ” I love you’s” and quiet solace as I begin to tell people, “I have ALS, we have ALS.” Then the torrent opens.

My brother tells me I can lick this, I can fight it. I want to argue, but then I realize this isn’t about me, it is about him. ALS has opened him to examining his own life, how he would react, what seems true to him – my disease and his mortality molded into deep reflection.

I don’t argue with him, I listen and open a little bit.

A healer calls me and says, “you are angry, hurt by your body. You must forgive yourself, forgive your body, it is only doing what it is meant to do. If you do not forgive yourself…” She leaves the thought unfinished, allowing my imagination, my creativity to build around it.

I don’t argue with her, I listen and open a little bit more.

I have to tell my colleagues, the college that I lead, to admit my mortality and vulnerability and weakness and fatigue, I have invited them to believe that no burden would ever be too much for me, that I am strong enough to carry any load required. I must now lose that narrative and admit my humanity, and I am scared for I know that sharks circle at the smell of blood. I write them a letter. I tell them I love working on their behalf, being their Dean, that I want to continue until I cannot. And then I write the vulnerability – “… If I cannot do the job, I will step down.” Like cascades of water pouring out on a desiccated soul, they respond – notes and office stop ins and meetings in the hall – love and support that could not have been written better into a Hollywood movie script.

Their love opens me even more.

I have to tell the choir – a group for which I still carry twinges of regret, even a little guilt, for stepping away from them in order to become dean of the college. Dan Johnson brings Evelyn and me into the room, and we tell our new story, and the choir listens, quiet, respectful, eyes on us and looking away. And then they stand and surround us and cry and touch and pray over us so that the only thing we can feel is love, pure love. A year later on an Easter Sunday, in a “Hallelujah Chorus” that I can no longer climb the steps to sing, they will leave the choir loft and surround us again, lifting our voices with their strength.

What wondrous love is this…

Six weeks ago, I attended a lecture with his holiness the Dalai Lama. At the end of the question-and-answer period, he was asked to bless the over 3300 people in attendance. His answer was that he was skeptical about blessing, that blessing comes through our own individual action and motivation. It was a beautiful answer; through our actions we perpetuate blessing on and on and on, rather than waiting for blessing to happen. When the program ended, he suddenly turned toward me, walked across the stage to me, held a scarf hastily given to him up to his forehead and said, “Meanwhile, my blessing…” And he handed me the scarf. For a week I struggled in confusion as people asked me, “What was it like to be blessed by the Dalai Lama?” I tried to describe it, but I knew my frame of reference was wrong. And then it dawned on me. It wasn’t about a singular blessing, him to me. It was a charge for intentional action. It was another awakening to open even more to the love that is all around us. Not, “meanwhile my blessing.” Finished and done, but “Meanwhile, my blessing…” Unfinished, a  statement to me, to us to embrace love, for love’s action and motivation and intent can and must be lived into, breathed into until you cannot breathe any longer.

The opposite of love is not hate; it is fear.

The greatest challenge of dis ease is that the moment fear overwhelms you, the moment you are dragged into your own soul wrenching vulnerability, is precisely the moment to open yourself to love. It is fear that causes us to feel estranged and alone, apart from God and from each other. To be closed off from love is crushing, angry loneliness, whether intentional or not. To be closed is to think that God only speaks with a voice – words and sentences and phrases and paragraphs. To be closed is to be sick with the reality that impending death presents.

To be open is to embrace your own great big messy humanity, to cry in sadness but not despair, to recognize presence in the emptiness of the bitter moment of truth, to be afraid but not fearful. Dis ease presents the choice of being open or closed , and opening to her lessons, her gifts, her challenges, is not easy. But dis ease clarifies vision, bringing sight to the blindness of what you thought you knew about living, light to the darkness of cynicism that life’s grief piled upon itself can foster. I know ALS is a horror, yet when fully embraced, it has taught me, it has revealed to me pure unsullied, uncontaminated, unbelievable love.

In my heart of hearts, I know that love never dies.

We sit together in a small room in the neurological wing at Clinic. What can anyone possibly say in such a holy moment? Matt’s eyes implore me to tell what I know. I hear myself, words from another place, wrestled from Angels in long and winding dialogues between sleep and wakefulness, “You will never be alone Claire, for Matt’s love will survive this physical shell of the body. You know this is true. Close your eyes and think of how much he loves you and how much you love him. That love will always be with you. Your children will know him for his love and his bravery and his courage. And they will know his love through you. There will be sadness, at first overwhelming, but as all of you move together with that love that you have known, that sadness will become beautiful, a source of strength, a place that you can visit and be made whole again.” We cry, Claire and Matt and the clinician and me and my daughter in law. We cry together at this most holy and human and loving moment, and out of our blessed silence I begin to understand the acceptance.

“God, into your hands I commend my spirit.”

 

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Picture Perfect

Almost to the day that I turned 50, I experienced a phenomenon that many of my older and wiser friends easily recognized. I would get up in the morning, look in the mirror and wonder, “Who is that old man staring back at me?” Or I would be walking by a bank of windows or some other reflective surface, and I would catch a glimpse of myself and not recognize the person looking back, as me. As I have continued to age, this experience has only continued to heighten. You might interpret my nonrecognition as narcissistic, and I guess I wouldn’t blame you if you did. Yet, I believe something instructive exists in whether or not we fully recognize our physical selves. I had this experience recently when I downloaded pictures from a small trip we made to Chicago. There was one picture in particular that, when it came up on the computer, made me stop and wonder if that was really me.

We spent our first day at Millennium Park. Chicago has a well-developed park system along the lake, but when Millennium Park was built, it was highly controversial due to its cost and location – a park on some of the most valuable land in downtown Chicago. Now, nearly 10 years after its opening, it is a place of energy and fun and wonderful amenities enjoyed by thousands of people every day, even in the winter. We spent almost 2 hours listening to the Grant Park Orchestra rehearsing an upcoming performance of the Shostakovich Fifth Symphony, we enjoyed bizarre sculptures, and no visit is complete without hanging around the great fountain that projects pictures of faces between its two monoliths, children and adults splashing in its puddles and standing under its bubbling waters. The whole park is meant to be interactive.

The day, lovely and sunny and cool for July, invited us to linger in the park, enjoying its beauty, recording the occasion with lots of pictures. Toward the entrance of the park, we stopped for the picture below – Evelyn bending down to be at my height, me in the wheelchair, crooked, Buddha -bellied, hands tired from steering. I describe this in such terms because for the first time in a long time, I was surprised at my lack of recognition that it was me in the picture. Something about the picture projected what I think of as ALS posture – a picture that my subconscious has always seen in others, but not in me. It broke through my denial spilling waves of cognitive dissonance between the body I have, the person I am, and the way I see myself. Suddenly I saw myself with other’s eyes, and all of those old feelings about disability and deniability came rushing back as if I realized my disabled condition for the first time all over again.

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I guess I really am a TAB at heart. I just can’t help it.

It was the circling gyre all over again – a point on the path of dis ease that I thought I had put behind me – only to spiral around to a deeper (or perhaps more superficial) interpretation of that same event. I thought that I had reached some semblance of acceptance, where this physical body is what it is, and where my own self worth is not a byproduct of physical capacity’s superficial interpretation. You can imagine how surprised I was, not just by the picture, but by this over-the-top reaction of shock and denial.

Usually I have my head around these things, and I am able to live within my disability with a pretty healthy attitude, but seeing that picture put me right back into the denial I had experienced when my ALS first began. And associated with such denial is an unhealthy self-esteem tied up in physical projection. I questioned whether I deserved the love and attention of my family and my friends because, after all I was not whole, I was not well, I was ALS personified – scoliosis, gut protruding, wheelchair – bound, muscles deteriorating. Not a pretty sight.

All of this from one picture? Eventually, I was able to find stasis, harmony – a place where I could accept that it is just my body, and the space that I occupy is far greater than the capability and capacity this body projects.

That harmony was brought home to me this past weekend with the birth of our first granddaughter. To say that I am over the top ecstatic, in love, sappy, dewy – eyed, wowed, totally into this tiny human being would be an understatement, and I am blown away by these feelings. Hypatia, all 72 hours of her, is the mirror in which I suddenly see the real projection.

She is, in my mind, perfection.

Before her daddy came into our lives, I wondered if I would have the emotional space for a son or daughter. Would I have enough love for his mother and him? He answered that question the minute he was born, and I realized that love’s space had expanded and there was more love to go around than I knew what to do with. When her uncle was born, I suddenly realized that this loving space exponentially multiplies so that no matter how many occupy its realm, there is always more love to give. When my sons introduced me to the women that are now their wives, that space opened up again, projecting out and underscoring what I had come to learn about love in space even to this day.

And now, this tiny three-day-old beauty who follows conversations back and forth, craning her neck when her daddy speaks, contemplating with the wisdom in her face that only a newborn possesses, has completely stolen my heart, making me reconsider that man with ALS whose picture was taken in Millennium Park. Her birth was an epiphany, a realization that often the person we think we are is not reflected in the physical self we believe we project.

One of the most overused terms of leadership theory is the term “transformative.” When it was first proposed, transformative was in direct opposition to transactional, implying an experience possessing tremendous significance. Now, I have reached the point where I avoid the term as best I can, because it is applied equally to events ranging from putting up new signage in a building, to rolling out a new advertising campaign, to completely changing the culture of an institution caught in the ruts of its own history. For me, transformative has lost its significance.

Today, I must break this self – imposed rule of usage, for I have been transformed.

I now look at the picture of the man in Millennium Park, and I realize he is waiting, waiting for something that will transform his outlook, reminding him that dis ease is more than ALS. I now look at the picture of that man and I see love waiting to pour out on a tiny, helpless, long awaited babe. I now look at the picture, and I don’t see ALS at all. I just see me – heart open to the perfection and possibility of my beautiful Hypatia.

Suddenly, I believe we are both picture-perfect in our possibilities.

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On the Head of a Pin

Writing would be so much easier if I were Sherlock Holmes. I would describe this whole dis ease experience with the detachment of a forensic researcher, droning on and on about how elementary winding down with ALS is. I would seek out miniscule meanings from the clues I would offer in each and every episode of my life, moving from ultimate human engagement to the need for quiet and peace just to survive the next minute and the next. But I am not Sherlock Holmes.

Instead, I have this enormous need to describe, like an insider with a big and terrible and wonderful secret, just how the story goes. I have this enormous desire to say the real and true (at least according to me) thoughts as they circle and wheel and roll around inside my head. My needs lead to enormous assumptions. I am assuming you will let me know if my governors have come off too far. I am assuming that you will let me know when the insider knowledge is too much for you to bear. I am assuming that you will tell me when you prefer blessed ignorance to the play-by-play with color commentary that inside knowledge encourages. But most of all, I assume that we all like to be in the know, even when the informant is not as smart as Holmes, a seeming reenactor of “Six blind men and the elephant” rather than a deductive genius.

Deduction is not my forte. For me it is a question of balance, and this past week has been one where the question of balance has not only been politely raised, but shoved down my throat.

Questions of balance are not to be taken lightly. For example, the balance between foolhardy and overly cautious could be driving 5 mph over the speed limit. It could be the critical mass one reaches in deciding whether to stay or go in a relationship, or to move away from or remain steadfast in a place of employment. Most of us can point to certain experiences where our life’s stasis was disrupted, where balance became impossible, where it was as if the universe had aligned itself against us. Being homeless or losing your loved one suddenly or being diagnosed with dis ease with only one terminal ending are massively unbalancing to the overarching meaning of day-to-day existence. I used to drive 5 mph over the speed limit.

I have experienced the diagnosis, the disruption of whether to stay or go in both relationship and employment. I have experienced the sudden loss of a loved one. Such experiences require years to rediscover that center, that space where all is well, that head of a pin where the angels dance. The consequences of great disruption are easy to understand. Not so easy to get are the consequences of when the small, trivial, carefully constructed sequences that keep us in an upright space are suddenly thrown far off kilter, not by massive life experience, but by something so exceedingly small that deductive distance begins to look like the only way in which to understand their meaning. In other words, it’s the little things that really get you.

The elevator broke down in my building, and I was not in my third-floor condo at the time.

When you have very little arm extension or hand coordination, 2 inches out of reach might as well be 100 miles. When you have no leg strength, the whole idea of walking becomes fantastical. And when you have no elevator, a 400-pound wheelchair becomes an instrument totally at odds with its mobility purposes. A body that cannot walk or crawl or pull or push becomes stone that refuses to be moved, a Sisyphean feat that embraces the ludicrous, a living example of the overwhelming power of gravity.

Think of poor Ev as she sought to find one or two or three backup plans we could execute. First she called the ALS Association and located a Hoyer lift. With this at least she would be able to take me from my chair to bed and back again. Then she found a hotel that did not require the DNA of our firstborn child to hold an accessible room until 6:00 PM. Then she went online to a support group and asked for advice. And finally she turned to a number of other caregivers who might be able to offer assistance well informed by experience. I am thankful it was summer. Can you imagine such execution with a room full of kindergartners?

It soon became clear that the elevator would not be fixed this day. And as we began to plan for the move into a hotel (I won’t even describe the logistical planning and the amount of equipment in addition to the Hoyer lift required), an idea from one of the caregivers online came through. “Have you considered calling the fire department?”

As we talked, it became more and more evident that getting me into the condo, even if I could not have my powerchair, was most desirable. So, Ev called the fire department. She told them, “I don’t think this is an emergency, but I have no way to get my husband up to the third floor where our condo is. Is there any way you could help me?” The dispatcher on the other end stated, “I don’t know, sounds like an emergency to me.” And within 20 minutes three fire persons – Garrett, Lisa, and Boomer – had figured out how to slip a cloth stretcher underneath me and to carry me up to the third floor. Our lovely neighbors at the other end of the hall loaned me their powerchair for the next 24 hours. And some off-balance balance was achieved.

This took place on Wednesday, and I have to admit that it wasn’t until Saturday that I began to feel like my old self again. The ALS person I have become is so easy to disrupt, so easy to push off balance, so easy to move into painful and difficult spaces, that I hardly recognize him. Yet I know it is me. I know that the energy I expend to create a space that is calm and fulfilling and centered is much more than I realize. The experience of being carried up three flights of stairs speaks of how simply the center can be pushed to the side.

I don’t tell you this for empathy or pity. I tell you this because as cliché as it may sound, dis ease has taught me that change is the one constant on which I can depend. And what I am trying to learn is how in the face of such constant change and loss, joy and life emerge. I know that they do. Many times, even in my ALS normal, I’ve experienced joy in the face of sorrow, gain in the face of loss, constancy in the face of disruption. And yet, I also have experienced just how human and flawed I am. When will I learn that none of this is anything more than the illusion of safety? When will I learn that joy and sorrow, loss and gain, constancy and disruption are nothing more than different sides of the same human experience? When will I learn that balance requires that both the positive and negative must be present?

Sometimes, it takes the overwhelmingly physical teaching of the local fire department to remind you that spiritual space is the counterbalance of physical loss. Sometimes, it takes enormous imbalance, tipping points exponentially reached to again experience the quiet center. Sometimes, it takes dancing on the head of the pin.

It really is elementary.

July 4

July 4

It is July 4th, and in the last two weeks, maybe even in the last seven days, more than one friend has told me I seemed a little “dark.”

And I thought I was hiding it so well.

So I will admit that I have had thunderheads round my eyes, that I have felt less connection, less space, less. I admit that something I knew but couldn’t acknowledge was taking place. I admit that I just wasn’t moving through the harder times, the more challenging times, the situations that were clinging to me with all of the grasping, cloying, olfactory, primal qualities of old smoke in the morning – hard to get rid of once it is on you, yet pleasant in small amounts as memory or flavor. I did not see the same darkness as my friends, but I now realize that the last set of losses, the last paper cuts, have been particularly hard to take.

I always overestimate my readiness for such things.

If you know anything about the physiology of the brain, you know that there is a great deal of motor cortex real estate devoted to the thumb, and I suppose that is why I have been able to keep some thumb function up until this point. There’s just a lot more that needs to be taken down in order for ALS to have its way. But now, my left thumb shakes, and it chooses its own direction in defiance of my desires.  And on my right hand, while my thumb is still somewhat functional, fatigue sets in quickly, so it’s functionality cannot be trusted. Add to that, the fact that my left side is always a little bit ahead, specifically that my left hand keeps informing my right of its future, and you have a recipe for a living Caravaggio* whose loss is less violent, less sudden, but no less significant.

So now here is the reckoning – no arms and no legs, no feet and for all intents and purposes, no hands. In the meantime, my neck grows weaker and and my sleep is inconsistent. I won’t even go into the challenges of ALS logistics. Each small loss must be translated into these larger, taken for granted arenas that most adults plow their way through (except for flossing) without a thought in the world except, “do I look as good as I possibly can.” At night I look up at Ev and sigh, “Sometimes, it sucks to be me.”

I’ve told you many times, I am no saint.

And yet, I cannot help but perceive a certain sense of purpose at work in all of this. It is as if a confluence of universal ethers has come together in a very personal way to teach me another lesson and another and another. My history is one where I stepped up in my youth and learned the skills required to keep a family running, and then I pushed those skills out into my own life. I realize now that many of the failures that I perceive in my first 50 years, were really my inability to translate my need to care into anything but my needs. I didn’t really learn how to care with any modicum of success until about 10 years ago, when I came to understand that human fulfillment is in the ability to translate deep care for all those with whom we cross paths, but in a way that allows such care to be reciprocated.

Please don’t read into this anymore than what was going on in my mind and my heart as I moved through the life that I knew.

Imagine that you have mastered some impossible technique, some skill set, some knowledge base, some attitude to a point where it has become like a second skin. Imagine that you are still approaching the apex of your purpose, your raison d’être, your life wonder, your grand opus, your artistic fulfillment, imagine. And then the universe writes across the sky a secret message that only you can read, “Enough! It is time to learn the real purpose.” Your head would swivel, your thoughts would lose their direction and alignment, and you might even be afraid.

I am afraid.

When my friend and teacher Matt asked me how I was doing, “not physically, but otherwise,” he stated that I seemed to be projecting darker.  I don’t like to characterize mood as dark or light, black or white, or any other arbitrary, colorful categorization. But when he said this, I realized that my grief was more present, more surface, more perceivable. It has not helped to see friends with ALS fulfill their destiny, nor to see brave caregivers in their own grief, and let me be honest, in relief that such a journey does have an ending. If you chose to perseverate on this reality, it could push your soul out of your body. But when Matt made his observation, I also realized that that while deeply challenged, I still do not despair. I have not moved through the latest losses as easily as before, yet I still see myself moving through. While the great lift that comes in the rise of the breath and the flooding of the lungs with blessed spirit has been slower to arrive, I have not lost my faith.

The fact is that none of us is truly finished until the great lessons have been learned. I learned how to push care, even for those who didn’t want it, so that my life was defined in a single dimension. Dis ease has brought me the spherical lesson over and over and over again, that care for requires care of, that caregiving requires caretaking, that caring space is not only physical but deliciously, consciously spiritual. I have faith that I will move through and become even more the person I want to be, the person I need to be. I am not finished; life is not done with me yet.

Today is July fourth, the day that our still very young and fragile and maddeningly frustrating democracy celebrates its own birth. There will be speeches and fireworks and movies and concerts and all manner of patting ourselves on the back. I will not be swept away by the speeches or music (except for Copland’s A Lincoln Portrait), but I cannot help but see the direction we are headed with the hope that at some point, our immaturity will give way to that which holds true meaning – that faith and love have far more significance than avoiding pain and suffering and death. That the remarkably gargantuan resources that we plow into death avoidance could be repurposed into life embracing, refreshingly chilled water awakening us to our possibilities, such simple love that when given, only multiplies and grows. I see this in my sons and daughters-in-law and dear friends and most of all my one true love, Ev.

It isn’t dark at all, and it isn’t light. It is faith in what will be, and the love that will come, and it isn’t as bad as I thought.

*Michael Ondaatje’s thumbless thief in The English Patient.

Letter to Cytokinetics

To: Cytokinetics, c/o Jeremy M. Shefner, MD, PhD, Principal Investigator BENEFIT – ALS

Dear Dr. Shefner,

I am a person with ALS, and I have recently completed the 13-week protocol for the study, BENEFIT – ALS, a Phase IIb clinical trial to evaluate the safety, tolerability and potential efficacy of tirasemtiv. I am writing to you to share my experience of the trial, one that required enormous commitment on my part as well as my caregivers. I am also writing you to make a request regarding how Cytokinetics, as well as other corporations engaged in drug research, might reframe their efforts as they seek novel treatments for orphan diseases such as ALS. Even though I was lucky enough to work with the Minneapolis site staff for the study, and they sought to lessen the logistical impact that the protocol required, the demands visited upon subjects such as me are significant. As of next week, I will have completed all the visits as required by the study, and I believe that my experiences might be informative on both a scientific and humanitarian level.

As you are aware, the protocol for the BENEFIT – ALS requires weekly visits that could range from one to over three hours. For a person with ALS, the planning it takes to get to the study site, securing a driver and a caregiver to help negotiate the way, and then the energy expended in the measurements and examinations is significant. ALS is a disease that results in great fatigue, and often, after an evaluation, I would find myself nearly catatonic with how tired I was. I tell you this not so much that you would change the protocol but so that you will understand the commitment that participation in such protocols requires. It is not as if I get in the car and drive to the site in a normal, able-bodied way. The preparation of getting dressed and cleaned up to come in to the Berman Center where the evaluations are held is only the beginning, and the actual protocol – particularly in the strength and pulmonary testing – is quite exhausting. Yet, I was happy to do it if it might push forward our understanding of a novel treatment for ALS.

My first visit for trial eligibility evaluation resulted in disqualification due to my ALS Functional Rating Scale (ALS FRS) lacking the requisite number of “2’s” and “3’s.” However, after attending the Mayo ALS Clinic, where I receive my treatment, and retaking the ALS FRS both at Mayo and at the study site, I was approved for the study.  I mention the Mayo Clinic because while I was there I was prescribed a neck brace in order to support my head and neck in the evenings. I was having great difficulty keeping my head up and the neck brace was designed to alleviate that symptom.

Since the study protocol required participants to take the drug for a week in order to blind us to the possible side effect of dizziness, I know I was on the drug for at least one week. Although I never experienced dizziness, I did experience another side effect that was actually positive. I did not need the neck brace again throughout the 13 weeks of the trial. In essence, I noticed a strengthening of my neck and shoulders so that it was unnecessary to wear the brace.

A second notable response for me was in sleeping. As you probably know, persons with ALS often suffer from different types of sleep difficulty. Some of this is due to the physical discomfort we experience. Some of this is due to breathing difficulty.  For some, anxiety interferes with sleep. Until the trial, I was rarely able to sleep more than two hours at a time, and I required constant readjustment due to physical discomfort. Unlike an able-bodied person, I have no ability to turn or change my posture, and my experience of sleeping was uncomfortable at the least and sometimes quite painful. My inability to sleep had other side effects, most notably on my principal caregiver and wife Evelyn. Not only is she my principal caregiver, but she also supports us by teaching in an elementary school. Trying to teach during the day after night upon night of interrupted sleep is very difficult for her.

Within the first week of being in the BENEFIT – ALS study, my sleep became much less interrupted and much more comfortable. I was able to put together 7 to 8 hours of sleep in three and four hour chunks. This was a remarkable development, and it resulted in less fatigue, a better mental outlook, and most importantly a rested wife.

On May 30th, I took my last dose of tirasemativ. The following day, I awoke feeling as though I had recently been in a street fight, receiving much more punishment than I meted out. I ached from head to toe, and it is only in the last two days (today is June 18th) that I have begun to lose a headache and bodily aches and pains that started over two weeks ago. In the meantime, I have consulted with an ENT over heightened ringing in my ears and perceived loss of hearing. Of course, I have also documented all of this with the research center, and they have moved to support me through prescriptions of potassium and magnesium and well–considered advice about water intake.

The bottom line is that where I felt the greatest effects of tirasemativ, study protocols did not measure. The measurement of sleep quality for persons with ALS is significant, and protocols need to take sleep into account. In addition, it would not be difficult to measure strength in other places besides pulmonary, leg, arm and hand. As I can no longer walk, you have received no measurements of my lower body strength. As I can barely hold up my arms, you have received less consistency, particularly from my left side. But were there to be some way in the protocol to measure an area where strength is noted when strength did not exist before, you would have data supporting muscle strengthening that could be analyzed through statistical meta-aggregation.

Please understand that I share with you these observations not just to tweak the science, but because I am a human being. I am not number XYZ out of the 30,000 or less people who currently have ALS in this country. I continue to hope for effective treatments, even though I know that drug trials will not result in treatments quickly enough that I might enjoy their benefit. I continue to hope that somewhere the human factors of how we do scientific research will be given as much consideration as the actual double-blind, placebo-controlled protocols that have resulted in one approved drug for ALS in the last 160 years.

I continue to hope that people like you will begin to recognize the futility of gold standard drug trials and push for more creative approaches that control independent variables, and minimize the objectification of subjects.

You have received a great service from the nearly 300 subjects who have enrolled in BENEFIT – ALS thus far. To cut even one of us off from the drug at completion of protocol – especially when some clear benefit has been experienced – saying that you have met your legal obligations and scientific responsibilities, is inhuman and a squandered opportunity. The humanity is in offering something that might make us feel a little bit better. The opportunity is in a readily available and committed population in which you could continue to study drug effect.

I do not know for a fact that I was on the drug, but the effects of going on and going off were certainly profound. If the drug is found to be well tolerated, why would we not offer it to those who have given their bodies for scientific research if they want it? Were you to offer tirasemtiv to me today, I would happily go back on the drug. Did it create strength in my legs or arms? I don’t think so. But a good night’s sleep and a stronger neck were meaningful results of my participation in the protocol.

Therefore I am requesting access to the drug tirasemtiv. I request this for myself and for others who find similar experiences within the trial. I offer to remain a study subject if that will advance the science forward.

Thank you for your kind consideration, and I look forward to hearing from you very soon.

Sincerely,

Bruce H. Kramer, PhD and person with ALS

Just Delivery

If you are paying attention, recall that I have written of how ALS circles around, how just when a suitable strategy for dealing with the current reality emerges, how just when the day to day living becomes something routine enough to handle, how just when comfort in the new normal is almost old normal; one more piece falls away, and the circle morphs into spirals down and down. I have written of circles delivered in packages sparkling with new knowledge or laden heavy with despair, packages complete, with no room for negotiation, no space for discussion, no rejection, no acceptance. I have written about how circling back is not for the faint of heart, for even today, five days after the event, with visits and dinners with dear friends and time with family in between, with the opportunity to physically process and psychically work through the latest loss, I am still amazed at how insidious my dis ease teacher can be.

It was the smallest of things.

I just finished a phone conversation with the editor for the MDA ALS newsletter. She had suggested that an occasional sampling of this blog might be good for persons and caregivers in the ALS arena. You may or may not realize this, but I am sensitive to anything that might be perceived as me speaking for more than me. It is not my intent for this blog to represent anyone’s experience but my own, for that is the only experience for which I can claim any deep knowledge. So I was concerned that in sampling the blog, I might be seen as overstepping the boundaries that I have set for myself. Such boundaries weigh heavily, and I take them very seriously. I am also very aware that for a number of people who read this blog, these descriptions of my experiences are close enough to their own experiences that voice is given where voice might have been silent. In the end, I agreed to the sampling due to her understanding and sensitivity to my concerns. It was a nice conversation.

And then…

My phone is on a piece of Velcro that sits on the right arm of my power wheel chair. It is not the most ideal placement for a phone. Sometimes when it rings, I have great difficulty getting my arm twisted around to slide the unlocking mechanism and answer it. Other times my arm is resting over the phone so that it is totally inaccessible. Usually I try to hold the phone so that I can point the microphone toward my face, as I lack the arm strength to actually bring the phone up to my ear. All of this is to say that were you in the field of risk management, you probably would have easily predicted some minor disaster with the way that I do the phone. Five days ago, I was just weak enough after the aforementioned telephone conversation, that as I tried to place the phone back in its right arm position, it flew over the side of the chair. In my effort to keep it from falling to the floor, my right arm became wedged behind the chair’s arm.

And there I sat.

This has happened to me before, so I didn’t panic. Instead, I tried all manner of ways to get my arm back on the chair. I tried turning my upper body, grabbing the leg guides with my left hand and pulling myself forward, flipping my right arm out and forward, even quasi-fishing my hand up with the seatbelt in order to get it back on the arm of the chair. After about 10 minutes, I realized that I was actually in real trouble. My arm position meant that I could not change the position of my body, which was leaned in just the right way as to constrict my breathing, and with it being my right arm, I could not reach the controls for the chair. I could feel my hand and forearm swelling with the gravitational pooling of fluids so that my fingers would not bend, and awareness slowly bloomed into the full consciousness that I was caught, trapped, unable to breathe deeply, unable to move, unable to perform the simplest of acts.

I watched the time tick forward, one minute, five minutes, one hour. Every once in a while I thought I heard someone walking in our building, and at that point I would yell as loudly as I could, “Help, help, help, help!” I soon realized that this yelling was pointless. It was just tiring me out, and making it more difficult to yell when the time might be right.

So I waited.

An hour and one half after my arm’s tumble, I heard the UPS truck pull up, I heard the rolling door clatter open, I heard the deliveryman come into the building, and I held my breath. Would he bring the package up to our condo? Would he ring the bell and dash off as he often does? Would he come to my floor, to my side of the building? I knew that I couldn’t allow him to get away. I started yelling, and when the doorbell rang, I redoubled my efforts, yelling at the top of my voice, “Help me, help me, please open the door and help me!” And this very kind man came rushing in, “I’m here, I’m here. Tell me what to do.”

I’m sure he was puzzled that all I needed was my arm lifted back onto the chair. I’m sure it seemed like such a tiny thing to him. I’m sure he had no realization of the relief that he offered, breathing and mobility and comfort. But he did it, and I spent the rest of the day seeking some equilibrium. My hand was quite swollen and did not want to operate the wheelchair controls. My body overheated with the exertion of trying to free myself, yet once I was free, sweat evaporated into shivering, teeth rattling, frozen to the core coldness. And when Ev came home, I was so relieved to see her that I burst into tears.

Welcome to the new normal.

Today, I know that our plan, our strategy to try to get me through to the summer at the level of care I currently receive, is not going to work. I am just too helpless, and I hate it. From now on, I need to make sure that somebody is around, at least checking in, just in case. In essence I have turned a corner into a new level of ALS. It seems like just yesterday I had chosen to begin walking with a cane. It seems like just yesterday that I gave up driving. It seems like just yesterday that I accepted that I needed homecare assistance. And today, I have to accept that the assistance that I need is far more significant than the assistance that I want.

I have no words of wisdom or philosophy, no frameworks from which I can turn these cold truths, no spaces of healing or warmth or acceptance from which I can approach this new reality. It will come, at some point it will have to come. But today, I am just shaken up by how easy, how fragile, how fleeting this gift of living is.

And that is quite a package to have delivered.

Arcs and Vectors

I’ve been pretty quiet about an upcoming event, but I think I can now announce that my son and daughter-in-law are expecting their first child, Ev’s and my first grandchild in August. As you can imagine, we are really excited, and every once in a while I catch Ev tapping her toes looking forward to “getting my hands on that baby.” We anticipate birth with such excitement, superimposing hopeful fantasies and imagined perfection on these tiny humans yet to be born. At the same time, we call into question our own childhoods, exploring our parents and their parents, seeking understanding and wisdom before the experience. I can’t help but be comforted by the fact that as I wind down my time in this space, a new human being poised for birth finds his own way, perfect in possibility, not yet shaped into the joys and sorrows of life as we know them. Long explored by poets and philosophers and scientists and clergy, one cannot help but marvel at the contrast – beginning and ending, alpha and omega, birth and death, baby and grandfather yet to be.

Life is up and down.

A new baby and a man with dis ease are at different points on the same trajectory. A baby, like a sunrise reveals the hope of a new day, dew on the grass, birdsong, buds opening into new life. While she must acquire every single behavior associated with a fully functioning human – speaking, bathing, toileting, feeding, dressing, schooling – for just a brief moment between birth and breath there is nothing but pure potential. And over time, she will learn to make responsible decisions leading to the  independence in living that we so value as a culture. Our new grandchild, still in utero, is now nothing but a hopeful point, barely perceptible on the life arc that we all experience.

On the other hand, my trajectory is at the end of its curve, an Apollonian finale hissing into the ocean’s bubbling cauldron of the life that was. All of those human basics, pounded into me in a lifetime celebrating independence, indicative of adulthood’s responsibilities, are shedding like so many feathered layers, melted from the wax wing bindings of life’s earlier flight. Where our grandchild will acquire the intellectual and physical capacities necessary to independent life, I now lose these very same capabilities. While I still take responsibility over my own body, I can only do so through the help of others.

The arc, birth to life to death, is a story of acquisition and loss.

We accept the lack of ability in an infant, hoping and expecting that capacities will develop and capabilities will be achieved. It is far more difficult with our elders. In conversations with people my age, “Mom just doesn’t feel like mom anymore,” has become a mantra. If you think about it, the idea that “mom” would remain evermore the “mom” of memory is illogical. There is not a person on earth who has the same capabilities today as they had yesterday. We age, and our physical capacity wanes, trickling out in dribs and drabs of lost elasticity and flexibility and strength and eyesight and hearing, or worse our mental faculties fail us until we feel our youth as some distant fantasy of another person beyond our memory. The dependent needs of a baby are framed in hope while the dependent needs of mom and dad foment despair, yet the expectation that our moms and dads would be like they were when we were young is just as strong as our expectation that a baby will grow up.

The arced trajectory is a story of upward mobility and precipitous fall. Its narrative is one where youth is celebrated, envied, and ironically disposed of in adult expectations that are unattainable and unreasonable. In our culture we superimpose the avoidance of dependency at all costs on to the expectation. Thus, when our lives reach their independent apex, old age looms as a tragedy to be avoided and put off. We are born, we live and if we are so fated, we age until we die.

In anticipating the birth of our first grandchild, in anticipating my own death, suddenly I am hyper-aware that independence from others, this most desirable state, implicitly means that independence gained must never be lost. It is an illogical belief, fraught with mythos and irrational assumption. Our bodies are designed to gain capacity and then, in what Steve Jobs once called “… the single best invention of Life, … [death] clears out the old to make way for the new.” Our minds are designed for greater and greater analysis and efficiency at the cost of less and less plasticity. Our souls are designed to cling to this physical existence as if our very lives depended on it, and they do. In anticipating the birth of our first grandchild, the grand design, the arc of life, the trajectory of birth to death, soaring to its apex and tumbling freefall into death, provides me little comfort.

A number of years ago, I was preparing to do Brahms’ A German Requiem with my small but mighty church choir. Since it was for church and the German would be problematic to teach, I was working with all manner of translation software and biblical renderings to try to truly understand the biblical texts that Brahms had selected, rendering them into a more meaningful English translation than the Victorian English provided in the score. In the second movement, the chorus begins with the line, “Denn alles fleisch es ist wie Gras.” I have always translated this line as “then all flesh is like the grass,” but one of the translation engines I was using at the time came up with the following: “we are like meat.” I had to laugh, partly because of how far this translation was from the original text, and partly because of its accuracy in capturing the human condition.

Having just heard a lovely performance of A German Requiem last weekend (auf Deutsch), I am reminded with a smile of both the sentiment and the ultimate reality. Rather than a trajectory that implies upward hope and downward despair with all of the crazy energy we put into denying the fall, the birth of a first grandchild causes me to think of life as a vector, pointing up and forward, acquiring all manner of joy and sorrow until, weighted down by life’s cumulative experience, our only chance to break free is to shed our fear and sadness, our hurt and tragedy, our triumph and success, the very things we strove for with such energy, with such purpose, until we are only love and empathy and pure collective humanity. In the arc, we flourish and we fade away, and the gymnastics we perform to maintain the charade of physical independence will ultimately fail. But the vector is a story of comfort, for there is great hope that babies bring and great truth that aging teaches, leaving us pure spiritual connection within ourselves, with others, and with God, even into death.

No wonder we cannot wait to get our hands on those babies.