The media

The Hummingbird Fund is gaining notice which makes me really happy.  Our mission is clear, and dare I say boldly stated:

Ending ALS. Starting with all of us.

The Hummingbird Fund stands on three pillars: access, innovation, and advocacy. We are on a mission to end care gaps for Virginians living with ALS, accelerate innovation to improve quality of life, and advocate for legislative action and research to end ALS. Through agile grantmaking, we work to help ALS patients and their families live full lives. Join us to help end ALS in this decade.

Hummingbird offers me the opportunity to use the experience I have accumulated from decades of work with families facing the enormous challenge of caring for a child with significant medical complexity and disability.  Moreover, I am lovingly joined by my family and hundreds of others whom I am calling the Hummingbird Champions. 

When invitations from the press started to roll in, you might imagine I would be delighted to share my passion for the vision of the Fund.  

My immediate thought was that this kind of carpe diem would be best delegated to my highly photogenic, uniquely poised, well-spoken family.

They declined, saying I was the man for the task.

So, I keep saying yes.  And you know, with preparation and some practice it gets easier.  I now see the media as a chance to share the ALS story, which has been side-lined for almost 100 years. 

Recently, Will Selden, a podcaster at the Virginia Health and Hospital Association, began his interview, asking, "So tell us, how are you doing these days." 

The question caught me off guard with its humanity. I thanked him for the question and its kindness, and then I answered as I almost always do, saying, "Oh, I'm fine." In this instance I elaborated with mention of the abundant love surrounding me.

I mean, no one wants to hear about me struggling to learn how to butter toast with my right hand, or the disappointment and fear associated with the gait-related side effects of edaravone, a medication I've been waiting to try for months, and one that required no less thank 20 hours of my time in the way of prior-auth's and payment schemes.

With the media I stay close to my talking points, allowing the daily realities to swirl like an imaginary cloud bubble above my head.

Some questions are fun.  Here's one that Will Selden used to close out our interview.  Feel free to try this at home and let me know your answers.

If you were stranded on a desert island, all alone, what one book (aside from the holy text of your choice), movie, and recording would you want to have along?

Ok, so here goes.  I will mention that I decided to go for diversity:

BOOK:   Mirabai Starr's recent translation of Julian of Norwich's The Showings

FILM:     Notting Hill

MUSIC:  Nina Simone "Pastel Blues"

Will Selden had one more question before signing off.  He asked for a bit of advice I had received that was worth passing along. My answer came immediately to mind, but I decided to place it in the context of a brief story.

When I was first diagnosed with ALS, I was at sea with knowing how to integrate ALS into my psyche, into my soul, really.  I revealed this awkwardly to a friend, who took a moment, then looked me straight in the eye, and with a gentle smile, said, "Just be yourself, Jim.  All you have to do is be yourself, and the rest will follow."

Milkweed

"Contemplation is a long loving look at the real."

                                                                --Fr. William McNamara

Most mornings, weather permitting, I begin my day sitting here in our contemplation garden.  I sit alone, or with Delta Mae, in contemplation, sipping coffee, hoping to center myself in silence, stillness, and solitude.  The intention is to sit here until I'm ready to start the day.  

How do I know when I'm ready? Not surprisingly, ready means different things day to day.  Mostly, I'm ready to stand up to start the day when I feel whole.  For some folks, I suppose whole might mean complete, or put together.  But for me, whole is when I feel more real, at least more real than when I sat down.  You might say I feel a bit less independent, judged, accomplished, or broken.  Truthfully, I feel whole when I feel more like part of the garden.  Steady, poised, rooted.

Often, the garden will offer me a gift in contemplation. 

  

 

Look at these milkweed.  Look at how they stand tall, day after day, boldly growing toward the sun.  Their brilliant flowers have passed.  The spectacular monarchs, drawn to their blossoms, have moved on.  Now, they wait for autumn.  In a month or so, each velvety pod will crack open to release silky seeds across the morning breeze.  And, as the air turns cold, their thick green leaves and sturdy stems will wither, turn a dusty brown, and fall, unceremoniously back to the earth.

This morning I see dignity, I see humility, and I see what's real.

 

The Double Life of ALS

In a recent essay published in the New York Times, Mary Pipher reveals her double life.  

Perhaps you remember Mary Pipher, PhD., as the best-selling author of Reviving Ophelia: Saving the Selves of Adolescent Girls, and Women Rowing North. Or, maybe you remember her as a provocative guest on NPRs Fresh Air with Terry Gross.  

Her latest work, A Life in Light: Meditations on Impermanence, is currently on my Kindle.

In her recent NYT piece, Pipher states boldly, "Of course, I am leading a double life.  Underneath my ordinary good life, I am in despair for the world."  She goes on, "Some days, the news is such that I need all of my inner strength to avoid exhaustion, anxiety, and depression... In times like these, we need world-class coping skills just to stay fully awake, enjoy our lives and be of service to others."

Pipher shares insights from three sources:

Her grandmother:

                   "...be the person you want to live with every day of your life."                           (Forgive yourself, be whole and grow throughout your life--jpo) 

Psychology:

 

"the best way to cope with suffering is to face it... find ways to balance [...] despair with joy" (Find balance and wisdom in a double-life--jpo)

Thich Nhat Hanh:

"His deepest teaching concerned our interconnection with all life.  We all share the same consciousness..."  (Individual action in response to despair for the world adds to the shared consciousness for positive change--jpo)

Pipher is not alone in the double life.  I imagine most of us can relate to the need for inner strength as we attempt to face a suffering world, one that includes the turmoil in our own lives.  Like Pipher, we most often keep the double-life to ourselves, and share the sunny side with others.

Recently, I spent an entire day lobbying congress to: (1) increase funding for ALS research, (2) create new policy to hasten distribution of safe and effective ALS medications, and (3) address the inequities in ALS care.

Our team of ALS advocates met (virtually) with legislative aides (LAs) in 8 offices:  2 senate, and 6 members of congress.  We told our stories of living with ALS, and urged the LAs to take up our cause. We also listened to each other's stories, eight times over.  Having faced this challenge before, I came prepared with a 2 minute speech to remind the LAs that ALS is like no other adult condition in its rapid decline to profound disability.  I called on the LAs to remember this fact when they heard the ALS community calling for innovative and urgent legislative action.  

As the new team members shared their experiences of managing a life of disability and loss, I could feel the emotional toll crescendo.  For some, this was the first time they had publicly revealed their double-life.  We waited solemnly, holding the silence, when they got choked up.  We "echoed points" that others had made, as a way to show support.  And, occasionally an LA acknowledged their courage.   

In the late afternoon, I kept imagining how a post-game gathering at a D.C. bar would be a welcome end to an arduous day. No such luck.  At 4:30, we waved at our screens and clicked off:  zoom world at its finest.  

I lingered in front of my computer screen, trying to take in the day.  The stories and the emotion had infused me with the interconnectedness of a shared double-life.  I wrote short emails to each person on the team.  I thanked them for their fortitude, and their honesty.  I offered to talk.  It seemed a meager attempt at an email hug.  

As is typical for me, the emotional weight came the next day.  An ill-defined, slate-gray heaviness loomed large. I could not describe it, and I could not shake it.  A dear friend named it for me, saying I had a colossal emotional hangover.  

I muddled along, weary and somewhat confused.  Contemplation helped.  Time outside with Peggy and Delta brought color into the day.  Looking back, a simple meal and a good night's sleep seemed essential to the cure.  I felt restored in the morning.

Slowly, I am learning that our interconnected double lives need time for a re-set after these really rough days, even when they are not full of ALS legislative advocacy on zoom.  I need time to regain my balance to be buoyed back from the deep.  Mainly, I need joy, wonder, beauty, laughter, and affection to balance the darkness and to remind me that we are interconnected through our positive emotions and stories as well.  

In the future, I'll plan for the time to recover.    More time with Peggy and Delta Mae.  More time in contemplation, and doing ordinary tasks like weeding the garden, or making a pot of soup. Time to simply wallow in the love that surrounds me.

   

PeggO & JimBob visit NIH: Day #2

 9 a.m. 

The day begins with Peak Flow Testing in the Respiratory Therapy Department on the 5th floor.  JimBob is an old pro at PFTs.  PFTs are a hallmark data point for every patient with ALS, and the test is repeated at every ALS appointment.  

ALS causes neuromuscular degeneration, which sadly means ALS causes the diaphragm to weaken over time.  Patients with ALS lose their speaking voice because they do not have enough strength in the diaphragm to force air across the vocal cords.  In the end, most ALS patients die of respiratory failure. 

PFTs are a high stakes test in the land of ALS.  The results are quoted like marathon times, with splits at 10K and the Half.    JimBob knows his PFT numbers.  JimBob always goes for a PR.  Always.

The testing involves wearing a blue plastic clothespin that is produced from a clean cellophane wrapper.  A disposable mouthpiece is attached to a long hose that is perched in front of JimBob who is ready to race.  The hose drapes across a short void, linking it to a large, foreboding, stainless steel machine where the respiratory therapist (RT) stands to watch the results pour in.

JimBob's challenge is to blow as much air as possible through the hose, with as much force as possible, for as long as possible. JimBob watches the face of the therapist. His secret goal is to make her eyes pop out when she sees his numbers. 

Actually, this test is less a marathon and more like a sprint.  JimBob gets three attempts to best his PR of 120% for FEV1 and FVC.  Of course, most people are happy with 100% predicted for height and age.  Not JimBob, no way.

And today is his day!

"Quite remarkable," says the RT nodding. "124% predicted."

"It was all your coaching," JimBob says smiling.

Lefty and Righty are doing a happy dance while JimBob glides out of the lab to find PeggO. 

"Excellent," says PeggO.  "So far it's a very good day."

9:35 a.m.

En route to Starbucks, before the next appointment, JimBob and PeggO find themselves in one of many long hallways, this one lined with black & white photos of mostly white men.  Anthony Fauci is among them.  The photos honor the NIH scientists who have won the prestigious Lasker Award.  The Lasker is known in academic circles as the American Noble.

As JimBob reads their names and accomplishments, he is surprised to see Bernard Beryl Brodie, the namesake of PeggO's chair in medicine. Bernard B. Brodie (August 7, 1907 - February 28, 1989) was the first scientist to determine how neurohormones, like serotonin, effect the functioning of the brain.  PeggO is a wisdom scholar at the University of Virginia, School of Medicine and this was their first meeting.

10 a.m.  Neuropsychological Evaluation

A small percentage of people living with ALS develop Frontotemporal Dementia.  The neurologists refer to it as FTD.  People with FTD have personality and behavior changes, like crying and cursing inappropriately.  They also have problems with decision making, and language.  

Tell a person with a rare disease, like ALS, that the chances are slim they will develop dementia, and they might just look at you, thinking OK, but I already have a rare disease and both my parents died with dementia.

JimBob is ready for high stakes test #2. 

The psychologist is all business.  They will have 2 hours to get through at least a dozen tests of reasoning, memory, language, logic, and depression.

Fortunately for JimBob, most of the tests are like puzzles.  And JimBob likes puzzles.  

"No one is meant to get 100% on these tests," instructs the psychologist.  She smiles behind her mask, seated across from JimBob as though they are about to begin a match of chess.

This does not relax JimBob.  He is ready to roll.

Test after test, puzzle after puzzle, they zoom along.  The squiggly image of a double-decker bus is a tip-off for a test designed in Britain.  This is useful to JimBob when a teapot, that does not look much like a teapot, pops up later.  Cha Ching.

JimBob's favorite test required listing as many words as possible starting with the letter F.  It is a timed test: 60 seconds.  After F, they repeat the process with words starting with S, and again with words beginning with A.

The psychologist must write down all of the words as they are spoken.  

Stop watch in hand, the psychologist actually says, "Ready, Set, Go!"

JimBob cruises along until he slams hard against the wall of expletives.  He must not say too many expletives for fear of exposing the personality changes associated with FTD.

JimBob's mind races temporarily as he tries to get past "Fuck", and "Fucker" in the F's; "Shit" and "Shitfaced" in the S's; and "Asshole" in the A's.  Frustrated and humored at the same time, JimBob tosses off Aardvark as a final A.

Little did JimBob know at this low point in testing that his peak effort was about to transpire gloriously: name as many animals as possible in 60 seconds.

Again, aardvark made the list.

JimBob's facility for animal names drew completely from the travel journals he has kept with his family over the years, collecting a lengthy log of animals sighted on vacations.  JimBob began by continent, then zoos, then regions of the USA and finally to the common household and barnyard animals.  When the psychologist ran out of room on her paper, she asked him to stop, well before the 60 seconds had elapsed.

JimBob had been determined not to be demented, and it turns out he is not.

After lunch, the much discussed spinal tap.

1 p.m.  PeggO and JimBob enter an outpatient procedure room for a lumbar puncture, commonly known as the spinal tap.  This is a purely elective procedure to collect samples of JimBob's spinal fluid for research purposes. 

As a pediatrician, JimBob has done many LPs on babies as part of the newborn sepsis workup. "In the right hands," an LP is generally easier than drawing blood on a baby.  JimBob is not worried, although he detects a bit of angst in the room.  PeggO decides to wait outside due to the perceived angst.  

"You'll be fine.  I'll be right outside."  PeggO does not really want to leave, but she is an expert at reading the room.

JimBob sits on the edge of the bed, hunched over the bedside table.  Taryn, their NIH nurse practitioner chats about what she is doing to prepare.  Dr. Kwan offers a pillow for the bedside table.  They have reviewed the risks in two separate occasions as part of consent.  There are no real benefits to JimBob, since this is for research. 

They all chat collegially.

The first stick is a no go.  Too low.  No worries it happens.

The second stick produces a sharp pain in the spine and left flank.  

"Yeouch!" JimBob yelps, prompting Dr. Kwan to come around to the other side of the bed.

"The spinal fluid is flowing.  She got it," he says softly.

Dr. Kwan gives Righty a few gentle pats, and just before it might seem like a simple "there, there" pat, Dr. Kwan allows his hand to rest gently with Righty.

"She is almost done.  The fluid is clear.  Everything looks good."

And the LP is done.  Taryn has 4 tubes of spinal fluid on ice, and is on her way to the 2 labs who need ALS spinal fluid for their research.

PeggO returns and reads JimBob's face.  She lets him be his cheerful patient self. She knows they will talk later.

Dr. Kwan wants JimBob to lie flat for an hour.   To make use of the time, they deliver a large IPad which connects to the genetic counselor for neurology.  She uses the hour to collect a genealogical history, since 20% of ALS is genetically familial.  Gratefully, JimBob's genetics have already been tested and he is in the clear on this one.   

4 p.m.

On the way home, PeggO asks about the pain in JimBob's back.  JimBob asks about what the pain might be.  PeggO speaks in a reassuring doctor's voice.  The pain is not unusual; an auxiliary nerve likely got tweaked as the needle went in.

"You can take a couple of Advil and lie down while Delta and I go for a run.  Then, we'll find some Mexican food for dinner."

PeggO's voice reveals the weariness they are both feeling from a day of scientific inquiry.  JimBob is fairly glad that NIH Day #2 is now in the books.

PeggO & JimBob visit the NIH: Day #1

 The National Institutes of Health in Bethesda, Maryland is the world's largest center for biomedical research.  The NIH is comprised of 27 clinical centers, among them the NINDS, or the National Institute of Neurologic Disorders and Stroke.  The NINDS has an enticing tag line for anyone with ALS:  

"Solving the Mysteries of the Brain to Improve Health."

The mission of the NINDS is even more hopeful and specific:

 "...to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease for all people."

When our neurologist at UVA suggested that a visit to the NIH was an absolute possibility, and that he would make the contacts for us, send all of the records, and include a summary note of referral, we were incredibly grateful. 

JimBob and PeggO were ready to pack their bags for an adventure at the world famous NIH.

I'm sure someone mentioned this along the way, but the truth of the matter got lost in the excitement:  the NIH is purely a research institution.  Every patient is part of a research study.  Our visit to the NIH would be, first and foremost, our contribution to medical science.  Every piece of data would be used to "seek fundamental knowledge", and "reduce the burden" of ALS "for all people."

The first inkling that this was going to be an intense 3 days came with the introductory email which contained a secure link requiring the usual sign on and password.  Once inside this secure, digital location, I found 8 attachments with lengthy details about security, COVID, parking, getting around the campus, our hotel reservations, and finally the agenda.  Our days would begin at 8 am, and end around 4, with 60 minutes for lunch at noon.  They suggested in several of the documents to bring snacks.

We already know that PeggO is all about having snacks for JimBob.  In an effort to increase compliance and efficiency, PeggO has added salty, roasted pistachios to the snack pack.  Genius.

The first small glitch surfaced when we discovered that the NINDS contracts with 2 lovely Bethesda hotels, neither of which is dog friendly.   This was news to  Carol who handles all of the arrangements for patient visits at NINDS.   We suggested the Hyatt Bethesda, 1.4 miles from the NIH main gate, and fully dog friendly.

After a bit of back and forth through the secure portal, Carol agreed to allow us to submit the bill from the Hyatt for reimbursement.  Under no circumstances, however, would the NIH cover the $60 doggy surcharge.

Fair enough.  And we were off.

Day #1

As if ALS is not confusing enough, with all of its twists and turns, jargon and choice of paths to follow, the NIH is also a giant campus with a choice of no less than 5 entrances, 2 of them on Wisconsin Avenue.  

The Hyatt Regency Bethesda is also on Wisconsin, a little over a mile away from the NIH campus.  Our Day #1 schedule starts with a nurse screening, and consent signing at 8 a.m.  Peggo hates to be late so we set out promptly at 7:25 a.m.  Remember the part about PeggO hating to be late; it becomes highly relevant. 

JimBob, liking maps as he does, sits in the passenger seat with a paper map printed from one of the many informative attachments referenced above.  JimBob can store and find electronic files with the best of them, however, the maps are quite small on a phone screen, and as it happens not that big on paper.  Since they are just headed down Wisconsin, the map seems superfluous, but it adds a degree of importance to JimBob's day.

Turning into the first NIH entrance on Wisconsin at 7:37 a.m. seems an early triumph, until the handsome, tall, masked security guard waves us to stop well ahead of his guard house.  This entrance no longer admits visitors.  We will need to proceed up Wisconsin, past the second NIH entrance and turn left on West Cedar for the Main Visitor entrance.  

At 7:46 a.m. we arrive at West Cedar. The tension is palpable as we wait in the left hand turn lane.  After a full cycle of lights, with nary a left turn arrow, we spot the small white sign that says, "No left turn 7:30 to 9 a.m. weekdays". It is now 7:52 a.m.

JimBob knows better than to say anything.  Lefty and Righty sit quietly clasped and sweaty.

As the light turns green, a most unexpected and glorious event transpires.

Peggo grips the steering wheel firmly, and with a steely eye, puts the pedal to the metal like never before.  Our nondescript, 2013 Prius rockets across 4 lanes of on-coming traffic, and sails down the sedate, tree-lined West Cedar Avenue.

Lefty and Righty go wild.  Fists in the air, they dance along with JimBob's conga-line chant, "Go Peggo, Go Peggo, Go Peggo!"  It's 7:53 a.m.

Arriving at the the West Cedar entrance,  PeggO is met with yet another challenge to earnest punctuality:  TSA type security.  Everyone and everything electronic would need to come out of the car.  7:54 a.m.

JimBob is generally cheery, and given his recent brush with Indy 500 excitement, he might be feeling even cheerier, affably greeting everyone and asking about their children and grandchildren. PeggO, shall we say, is more conscious of the time.  7:55 a.m.

On to the parking garage.  More security.  Open the hatch, wipe things down with the horrible wand that always gets us pulled out the TSA line.  This time we prevail and are at the COVID-screening check point by 7:58 a.m.  Snacks and schedules at the ready, we have made it.

Top of the list is my first and only COVID test (tears and coughing for 10 long seconds), then we spend a full 90 minutes reviewing and signing consent forms.  Lots and lots of consent forms, including consent for a spinal tap, genetic testing, and many lab analyses.

Last stop before lunch: the lab.

Taryn, our nurse practitioner, accompanies us to the lab with a small insulated cooler.  She will hand carry the blood samples to their respective research laboratories on ice.

It is worth noting here that JimBob is not fond of getting blood drawn.  It hurts and the phlebotomist often needs more than one attempt.  Blood draws test JimBob's cheerfulness. 

The phlebotomy lab at the NIH is a warren of small rooms set up for taking blood.  JimBob is assigned  the "PEDS ROOM" which provides some comfort.  The Peds Room has smaller needles, and a phlebotomist with more skill at sticking smaller veins.  The Peds Room also has brightly colored balloons painted on the walls.

As the phlebotomist is setting up, JimBob eyes an entire rack of multi-colored vacutainers.  Many of these tubes have colors and speckles you would never see in a typical medical office or hospital lab.

Gazing down at the rack full of tubes, JimBob asks quizzically, "Are those all for me?"

"They certainly are," chirps the phlebotomist without looking up.

JimBob counts 15 tubes.  He counts again.  Still 15.

Truthfully, what did he expect at the World's Largest Center for Medical Research?

After lunch, JimBob and PeggO spend the afternoon with Dr. Justin Kwan, a research neurologist who specializes in ALS.  He is a tall, thin man, stylish and precise. Dr. Kwan looks too young to have accumulated all of his expertise and titles. 

Dr. Kwan (everyone calls him Justin) sits facing us, almost knee to knee, with a legal pad on his lap, and begins to take the history.  He listens carefully.  No detail is too small.  No anecdote is disregarded.  He writes it all down, pausing to think about what he hears. 

Recounting the unfolding story of ALS is a bodily experience, and it is emotionally fatiguing.  Retelling the story illuminates the early hope that thumb weakness is just a bit of arthritis.  The story recounts the early speculation that muscle weakness and atrophy in the left hand are due to a narrowing in the spine that is commonly repaired with surgery.  Telling the story from the beginning reminds JimBob and PeggO of the anxiety they carried silently.  This was always going to be ALS.

JimBob is generally a sunny patient, which poses a conflict when telling the ALS story, which is generally a dark one.  The continual slow decline in function, which results from the on-going death of motor neurons, is more naturally told by JimBob from the sunny side. He acknowledges a SLOW decline, hopefully due to the POSITIVE effects of the 23 pills he takes everyday.  He often turns to PeggO to fill in the gaps or for her perspective.  They are team story-tellers.

Dr. Kwan has elicited a detailed history of ALS from hundreds of patients.  He knows how to pace the interview, creating trust with a true curiosity that never feels out of line. He creates intimacy for JimBob and PeggO to be themselves.  The three of them share an authentic mixture of laughter and silence.  They recount stories of coming up the ranks in training, and of supervising students and residents.  They commiserate about being on call and about the honor and privilege of caring for patients. Dr. Kwan knows how to be the doctor's doctor.

Next up:  the familiar neurological exam to document abnormalities in strength, coordination, and dexterity.  Dr. Kwan saves Lefty until last.  With a rare tenderness, he champions Lefty's efforts, and comments soley on Lefty's successes. Dr. Kwan's exam is thorough and dignified.

At the end of 2 1/2 hours together, Dr. Kwan summarizes his findings and invites questions.  PeggO has a detailed list of questions in her bound diary.  She goes first.  PeggO is pleased to have the time with an expert on ALS.  The conversation soon morphs into a mini-journal club, referencing and critiquing scientific studies. Dr. Kwan receives questions with genuine interest.  He is engaged in the opportunity to share the finer details of research.  JimBob especially appreciates the way Dr. Kwan can describe the limits of knowledge. As always, PeggO writes it all down.

As the mood naturally settles into closure, Dr. Kwan slides his chair even closer to share an image on his iPhone.  He has applied JimBob's data to a nomogram created from thousands of ALS patients.  JimBob's rate of decline is in fact slow, and ranks among the top 85% of slowest progression.  Neither JimBob nor PeggO are typically content with 85%, but in this moment they are elated.  Could this be true? JimBob asks for more detail about the data.  Dr. Kwan confirms the accuracy without interpreting what it might mean for JimBob and PeggO.  What it means for JimBob and PeggO is more hope for more time in each other's arms, literally.

4 p.m.

The exit from the NIH clinical center is as circuitous as the entrance, and requires driving 2 floors deeper into the garage to locate the sole exit.  The exit is no where near the previous entry, so another masked security guard offers a complicated set of directions which land us, no joke, at the Wisconsin Avenue guard station where we had earlier that morning been turned away.  This time the guards wave us through and Day #1 is in the books.

Cue the trumpets!

My wife Peggy grew up on a small lake north of Chicago.  The brick house had a long, sloping back yard bordered by giant oak trees and a split rail fence.  A slightly wobbly, wooden pier jutted out into the lake, making it the perfect launch pad for children to come careening down the backyard, across the pier and into the cool, fresh water with gleeful squeals, and splashes.  The small sandy beach, with its gentle lapping waves, and afternoon shade made for ideal imaginative play.  And as the children aged, the excitement and laughter flowed from a used motor boat that was more than adequate for all manor of water-skiing adventure.

This was Crystal Lake.  This was middle America at its summer best.

The decades of family gatherings at Crystal Lake are now part of the family lore, embedded in memory across generations.  Stories are told and retold with any number of embellishments for sure, especially when water skiing is involved. 

Yesterday was Easter Sunday for most of Christendom.  It is a Sunday of exuberant music and pageantry, with brass choirs, and soprano descants sung to familiar hymns.  It is the Grand Finale of Holy Week.  

For many, Easter is one of the two Sundays they appear in church.  Attendance is universally overflowing, and preachers everywhere are challenged to deliver a Home Run Sermon.  

Ask a preacher and they can enumerate any manor of challenges for Easter Sunday sermonizing.  Imagine having to craft a message that speaks to those who know the back story, retold throughout Lent, and those who show up for the music.  (You know who you are.)

Jumping to the end of the Easter story can lead to confusion and dismay.  How do you make sense, never mind celebrate, a prophet's gruesome, slow, death on a cross, in a trash dump, 2,000 years ago?  Is it enough to proclaim that a God/Man rose from the dead?  Resurrection from the dead is, after all, a basic tenet of the Christian faith, and in Jesus's case, it is a spectacular miracle.  

Resurrection can be understood as the end of the story... believe it, don't believe it...  it's up to you.  Either way, enjoy the music.

Those of you who were in church yesterday may have noticed, as I did, that there was plenty of talk and singing about death.  Death on the Cross.  Vanquishing Death.  Freedom from Death. Transcending Death.  No more fear of Death.

And don't forget the empty tomb, which is also really sad, until Jesus speaks to Mary.  Then we cue the trumpets for a fanfare and a final hymn.

The back story to the crucifixion and resurrection reminds believers of the intimate conversations, the shared meals, the creation of ritual, the reversal of roles (foot washing), and the withdrawal for solitary prayer and contemplation that precedes Jesus's arrest, trial, and death.

We need the back story to understand the end of the story.  We need the entire narrative to make sense of the ending.  If we jump to the end of the story, we miss the deeper meaning of the story.

For me and for many Christians, resurrection is not just an historical event.  Resurrection exists today in shared meals, rituals, reversal of roles, intimate conversations, worship, and solitary prayer and contemplation.  Resurrection is an on-going narrative of God's presence and love as a Reality in daily life.

Father Richard Rohr says, "The only way I know how to teach anyone to love God, and how I myself seek to love God, is to love what God loves, which is everything and everyone, including you and including me!"

Love everyone and everything, even those places and people who have passed.  Gratefully, our narratives continue to evolve, and live on.  

Alleluia!

Cue the trumpets.

Introducing Lefty and Righty

 It's time to introduce you to the story of Lefty and Righty.

The year is 1956.  The place is the sweet womb of one Bernice Jean Galbraith Ogan, a 27 year old school teacher in Lorain, Ohio.  You'll remember Lorain, for its famous, polluted Black River that flamed its way to the national news.  I would also like you to know that Lorain is the birthplace of Nobel Laureate Tony Morrison, and actress Milica Govich.  No doubt you'll remember Milica from her roles on television and Broadway, and famously as Ansel Elgort's mom in the blockbuster movie The Fault in Our Stars.

Back to the womb.  

You see, Lefty and Righty are fraternal twins.  They've been a duo from the very beginning,   At seven months, they were passing banana bits to each other for fun.

The twins have always been happy to work and play together. Climbing trees, riding bikes, eating a field-fresh ear of hot-buttered corn at a picnic in July.  These two go way back with fun and festivity. 

Bernie, as she was known to her friends, loved Lefty and Righty equally.  She clipped their nails with great care, and inspected for cleanliness before meals. She taught them to move a chess piece, and shoot pool on a revered table in Mr. Ryan's basement. She eventually gave up on anything that involved hand-eye coordination, but to be fair, that was not the fault of either Lefty or Righty, 

Bernie, and her father before her, were known for being left-handed.  So, when Lefty preferred to hold the spoon, then the Crayola, I suspect that Bernie was silently well-pleased.

Lefty emerged as the one with notable dexterity at any keyboard.  His bass-line on the Hammond B3 could astonish, and his speed with A,S,D,F was truly remarkable.  Righty, worked to keep up with above average success, and remained content with J,K,L, semi-colon.  Lefty couldn't help feeling the tiniest bit sad for Righty, regarding the semi-colon. 

Let's keep this next part to ourselves, since now is not the time to reflect on past failures. The truth is that Lefty could be a bit of a show-off.  For example, the repeated errors in typing class were due to Lefty's competitive edge to exceed 50 wpm.  Lefty has always been the one who needs to be convinced to slow down.

As life for the twins poured forth, Lefty would emerge as the favored one.  

Lefty wears the ring.  Lefty pens the letters.  Lefty strokes the hair of children at bedtime.  Lefty uses the fork, and chops the onions.  Until, recently, Lefty's index finger pushed seeds into the fresh, cool soil each Spring.

A year ago, Lefty held Bernie's left hand as she lay dying.

Lefty has led a life of privilege.  And Righty has been, well, right there, ready to help.  Righty held the onion to be chopped.  Righty held an equal number of children's hands to cross a parking lot.  Righty happily joined in push-ups, and swimming across Walden Pond.  Righty has always been grateful to tag along with Lefty, who has always happily taken the lead.

If the symptoms of ALS had started with Righty, instead of Lefty, the diagnosis may have been longer in the making.  Slight weakness in Righty's thumb and index finger may not have manifest as a problem, like it did for Lefty trying to write clinic notes, and prepare vaccines in syringes.

Dysdiadokinesia is the medical term for the inability to perform rapid, alternating muscle movements.  It is a hallmark of ALS, along with muscle weakness and fasciculations (muscle twitching).  The neurological exam tests for dysdiadokinesia by comparing the right and left hands.  The examiner asks the patient to wiggle their fingers, and to tap the fingers against the thumb of the same hand in rapid succession.   Lefty, of course, has always excelled at this task, so much so that many a doctor has worried about Righty.  That is, until I reveal Lefty's privileged place in the world.

ALS doctors who had never met Lefty and Righty were reassured by Lefty's lack of dysdiadokinesia, but I knew.  Lefty knew.  So did Righty.  We kept quiet for awhile.  After all, what was there to do?

In an unassuming way, Righty has learned to button a shirt alone, to brush my teeth, and shave my face.  Lefty was downhearted at first, maybe still is.  But Lefty is also grateful for Righty who has never been one to brag about brushing teeth, or pushing the start button on a microwave.

What has become clear to me as ALS progresses, is that Righty and Lefty are in this together.  Righty is quite happy to hand Lefty the pen that would otherwise slip away from Lefty's loose grip.  And Lefty, God love Lefty, is honestly growing more comfortable receiving the help, because it means the two are still in this as a team, even if some of their roles are shifting.

For now, as Lefty's function declines, Righty is there for assistance and comfort.  I've noticed Lefty settling into Righty's palm, or lacing fingers as a way to settle into my lap, like the way Peggy and I spoon to sleep, night after night.  It's an act of ordinary tenderness.  It's an unconscious kindness.  It's love manifest in the reality of ALS.   

Giving up Death for Lent

This year I decided to give up death for Lent. 40 days and 40 nights to put aside any thought about death. It would be like a Death fast. If you can give up chocolate for Lent, why not death. Jesus spent 40 days and 40 nights alone in the desert, fasting and facing down the Devil. By custom, the 40 days of Lent are used for inward reflection to renew and deepen faith. Colloquially Lent means forgoing a vice, perhaps to commemorate Jesus’s time in the desert, perhaps to assuage guilt or shame. It is written that Jesus used his time in the desert to prepare for his earthly ministry. In recent years, I have chosen to alter the tradition of Lent by taking on a new challenge or discipline. I have used the 40 days to explore a practice that might add to my spiritual development. This approach has seemed more positive, and less likely to end in failure. I have convinced myself that this approach is less about symbolic atonement, and more about embracing God’s presence. Giving up coffee, red meat, or bourbon would begin as a well-intentioned sacrifice, and generally end poorly. Truth be told, these kinds of Lenten fasts never really did much for me.

Earlier this Spring, our daughter and son-in-law invited Peggy and me to join them for a few days in the high desert of Joshua Tree National Park. The cactus and the Joshua Trees were just coming into bloom. The days offered bright sunshine and radiant blue skies. The nights were silent and cold, with endless stars on cloudless nights. We hiked for hours during the day, awestruck, grasping for superlatives that might approximate our experience of wondrous beauty. Here are Joshua Trees in bloom:

The desert landscape is known for its allure to contemplatives and ascetics. I had always imagined this was largely due to the desert’s silence, stillness, and open space. Joshua Tree taught me about the beauty and peacefulness of the desert. I experienced it as an inherently holy place to be.

This is my first Lent in The Land of ALS. Some might suggest the desert is an apt metaphor for living with ALS, because of a perceived bleakness to both landscapes. I’ll admit, since being diagnosed with ALS, I have experienced a sense of wandering in the desert. Every person has a unique path with ALS. There is no charted course that can be mapped out, like a trail in a national park. In the desert I was surprised to note that shades of brown, beige, and gray carry their own subtle vibrance. I’m guessing part of the reason for that is the paucity of other color. Likewise, I noticed that even the smallest cactus blooms draw attention. In the desert, the blue sky seems bluer. And the sunsets are like no other.  

This brings me back to my Lenten fast. Giving up Death for Lent, seemed like it could be a meaningful way for the landscape of ALS to surprise me with rare blooms, bluer skies, and sunsets like no other. 40 days and 40 nights of wandering in a new desert without fear or thought of death. 40 days and 40 nights to experience ALS without looking too far into the future. 40 days and 40 nights to prepare for a new life.

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Pilgrimage as kindness

 

My son William experienced his first pilgrimage walking the Camino de Santiago, the summer of his freshman year at The College of William and Mary. His professor and future mentor George Greenia led the pilgrimage. George has since become a family friend, and William has become our family’s maestro of pilgrimage, beginning with his sister, and then with his parents on the Camino. We have learned from George and now from William that no pilgrimage, and no pilgrim is alike, though there are common elements that define a pilgrimage: 

•  a beginning and an end, 
• movement along the route, 
• liminality in (holiness) space and time, 
•  the experience of beauty/awe/wonder, 
• a rhythm to life, 
• and perhaps a big question. 

Pilgrimage at life’s transitions proves exceptionally meaningful. 

Such was the case for each of us on the Camino de Santiago, 

 Such is the case for William and me now, traversing the glorious California coast in my cousin’s Porche Boxster, at the launch of life and advocacy with ALS. 

William is a currently a second-year law student at Columbia. He has a full and varied life in New York City. I’m pleased for him. I’m his dad, after all, and these successes and his happiness make me proud and happy. So, his invitation to drive me along the California Coast during his spring break, on the eve of my launch into ALS, was welcomed as an offering of great kindness and love. To be clear, he gets to drive my cousin’s Porche Boxster Convertible with the top down along the Pacific Coast Highway. AND, to be clear, I’m in the seat right beside him, for holy hours, in liminal time, along the awe-inspiring coastal roadway. Any parent knows, and EVERY pediatrician knows that any hope of an intimate conversation comes during a captive car ride. This has not been lost on William, to be sure. In a profound way, our roles have been blissfully reversed, and I am a gleeful captive.

Reasonable people, who know me well-enough have asked, post-ALS diagnosis, how it is that I am taking care of myself. It’s a fair question, and one that needs asking. Having ALS requires daily and rigorous care-taking. I swallow handfuls of pills every day, and pay close attention to diet, rest, and physical activity. Trust me, I’m totally into it.

These same people, I suspect, also want to know how it is that I am caring for the deeper parts of my self.  How am I taking care of my soul, now that I am facing down the complete disability and eventual demise of ALS? Also a worthy question that bears asking.

Regarding this, I have early data to report from the pilgrimage.  

Proximity to love and kindness begets trust in love and kindness. 

The meaning of pilgrimage comes from the doing of the pilgrimage.  

Abiding in liminality, with intention and an open heart, ready to embrace awe, wonder, and beauty creates a grand opportunity for meaning-making.  It's the most natural way I know of to care for the soul.

The path of love and kindness, and this on-going pilgrimage may be our best hope to make meaning out of the tribulations of ALS.  

As William said in the first hours after we received the formal diagnosis of ALS, after the tears, and shock had settled, 

"Well then, I guess it's time to get turbo-charged."  

Amen son.  Let's go!

Launching a pilgrimage of kindness

This blog began as a way for me to write about the fruits of kindness. Patients and families would often recount the simplest acts of kindness with immense gratitude, and it seemed relevant to share their stories. Perhaps their gratitude could inspire others. Perhaps my reflections on the varied examples of kindness could create a ripple of awareness for kindness as an essential component of compassionate, quality health care. 

I retired from the practice of pediatrics last month. I can claim 40 years as a clinician, having seen patients since 1982. The last 20 years have been dedicated to solo practice in Charlottesville, Virginia. My labor of love. And for the last 10 years, I added academic medicine to the mix as an Associate Professor of Pediatrics at the University of Virginia. A separate, yet related labor of love. 

In all of these 40 years, in all of the practice settings, I have been guided by something I learned from the dean of the Lienhard School of Nursing, in Pleasantville, NY, where it all began. She taught us that our primary task was to hone the therapeutic use of self. Over time, as we matured, the therapeutic use of our whole selves would guide everything else. Certainly, our intellect, our fund of knowledge would be a mainstay of expert care, and a key component of the whole self. Achieving intellectual competence, especially in training, generally takes precedence over everything else. However, the dean reminded us that our compassion, our communication, our empathy were equal and essential aspects of the whole self and would be needed at each encounter, to be used therapeutically to ensure true quality of care. 

To this list of therapeutic virtues, I add kindness, because it has a simplicity that is easily understood by any reasonable person. As an example, before leaving the bedside, a doctor asks simply, “Is there anything I can help you with before I leave?” Or the nurse arrives with a cup of cool water for the family member who has been up all night with an aging parent who has been admitted with delirium. Or, the clinician stops in the middle of the routine mechanics of patient care to respond with kindness and empathy, when hearing parenthetically about the unexpected death of a grandparent, or pet. Or, how about a simple, genuine greeting and introduction when entering the exam room. I’ve been warned that therapeutic boundaries become tricky when we introduce hugs and tears to the list, but they can also be part of the whole self. 

I am writing this while on a pilgrimage with my son, traveling the California Coast. It is a liminal time for me to explore the transition from doctor to patient. In December I was diagnosed with ALS, amyotrophic lateral sclerosis. ALS is a tragic diagnosis which portends a limited lifespan, and certain progress toward complete disability with the need for total care. There is no cure. The underlying biological cause is unknown, and the few available treatments have limited effect in altering the course of the illness. It’s a harsh reality. 

Many questions arise. What is the role of the self as a doctor-patient? How do I reflect or offer kindness in this new role? How might I engender kindness in the health care setting now that I am no longer the clinician? What are the gaps in ALS care? How can the gaps be assuaged or eliminated, especially those that limit the opportunities for acts of kindness? How does kindness inform advocacy? What are the fruits of kindness as experienced by me, the patient with ALS. And lest we forget, how is kindness germane to those who are walking the lonely road with me, my dear ALS family and friends? https://www.als.org/ https://iamals.org/

The blog returns to reflect on these questions, and to chronicle my on-going pilgrimage of ALS.  

Expanding the Frontiers of Hope

With the advancements of science and technology, it is now possible to reliably diagnose rare conditions early in pregnancy.  Likewise with the explosion in genetic analysis, we can now identify the cause of previously uncharacterized syndromes.  Whereas these diagnostic capabilities offer the certainty of a diagnosis, they also have the potential to launch families into new, and often uncharted waters filled with an array of questions about the future. 

www.trisomy18.org

Many rare conditions carry risks of future cancers, intellectual disability, autism, psychiatric co-morbidities, or seizures.  The risks of future diagnoses are based on small numbers of patients with conditions that have variable presentations and outcomes.  Parents are faced with statistics that we might try to represent as a slim possibility, but we must remember that they are seeing this slim possibility through the lens of a parent whose child has a rare condition.  

Receiving a diagnosis of a rare condition is a random and unimaginable event for any parent.  Making the leap to seeing your child with a whole series of future random and unimaginable events is actually quite natural.   If we then add to this the anachronistic label of “lethal diagnosis”, our offers of the potential for ever-expanding technological boundaries may not seem so comforting. 

My experience has been that most parents will do anything within their means to help their children.  Parents of children with disabilities or medical complexity are no different and are frequently quite innovative in their attempts to create opportunities for participation and inclusion.  When the status quo is not enough, they advocate for something different or they figure out a way to make it work. 

Statistics also provide a window into survivability.  We know with certainty that many conditions carry the prognosis of a limited lifespan.  Since it is nearly impossible to predict the length of a lifespan, especially as the boundaries of science and technology continue to expand, we land, once again, in the arena of uncertainty.  Parents and clinicians alike must learn to live with uncertainty.

So, where does hope fit into this equation?  We all need hope to continue in the day-to-day care of our children and patients.  We certainly need hope to support one another.  We also need hope to innovate, to advocate, to make meaning and to risk love.

And what about the notion of false hope?  If we know that an outcome is unlikely, is it wrong or misleading to encourage abundant hope?  Is it truly in the best interest of the patient, family or clinician to protect each other from the disappointment of a sad outcome, even if it is anticipated?  Is it any less sad if it is unexpected?   Is it false hope, to imagine with a parent that their child will walk, or talk, or make it to Disneyworld with a ventilator in tow?  I don’t think so.

Encouraging hope in a parent or caregiver is an act of kindness.

Recently I was invited to take part in an annual round table dinner-discussion hosted by the Anne L. Brodie Fund for medical education.  The fund also supports students as they learn the "skill and heart to care for patients" with the aim of becoming The Brodie Renaissance Physician.

The Brodie Renaissance Physician: Is called to artful, compassionate, 

patient centered care. Takes responsibility for the whole patient / person. 

Is adept with the newest technology but also possesses 

finely honed listening and physical exam skills 

to limit dependence on technology. 

Strives to improve on each individual patient encounter, 

and also on systems of practice. 

Is a scholar, teacher and role model.

In preparation for our dinner discussion, each guest was asked to reflect on the intersection of technology and caring, and to give an example of a situation where technology could never replace the physician.  Offering hope, and expanding the frontier of hope seemed an apt example from my work with families of children with medical complexity.

As I have gotten to know families whose children carry a “lethal diagnoses” or live with significant, life-limiting disability and medical complexity, I have seen magnificent hope blossom amidst slim possibility for success.   I have experienced the frontier of hope expand as parents and clinicians join together with proximity to the challenges and the risk of disappointment. 

 

Extending hope across the chasm of feared disappointment is a great kindness.  Learning to expand the frontier of hope is as significant as extending the boundaries of science and technology.   We need to challenge ourselves with equal energy and determination on both fronts.