Traveling with Mr. ALS

I don’t go anywhere these days without Mr. ALS. Not the grocery store. Not the swimming pool or locker room at the YMCA. Not even to the mailbox at the end of our driveway. Mr. ALS is like the awkward kid at summer camp who has yet to learn how to give it a rest. 

Last week, Peggy and I traveled to Southern Arizona at the invitation of dear friends, to spend some time relaxing together, and to experience first hand the complicated issues of border security and immigration reform. More on those two thorny controversies in another post. 

Air travel with Mr. ALS is a whole new adventure. No carry-on luggage. No sprinting for a just-in-time departure. New with this trip is a welcome ride in a wheelchair to the gate and between connecting flights. Oh, and lest we forget, once in flight, there is the long, unsteady walk down the long narrow aisle to the tiny bathroom with a folding door. 

Even without turbulence, every person in every aisle-seat notices Mr. ALS.  I nod and smile, imagining what they might say when Mr. ALS and I are sitting squarely in their lap. 

And then, lucky for us, there is TSA. A supreme adventure unto itself. 

Relinquish your cane. Remove your shoes without falling down. If you’ve decided to wear your AFO (brace for foot drop), it comes off too. Then we get to negotiate the slight incline into the scanner, which I’d never noticed before traveling with Mr. ALS. Once inside the scanner, we must help dear old Lefty raise a hand overhead, which requires a bit of gymnastics. Righty grabs hold of Lefty and hoists both overhead for the 5 seconds it takes to scan my insides. The exit off the incline is the last hurdle before finding a chair to reassemble the footwear. All of it deserving a round of applause. 

In general, TSA agents seem mystified by Mr. ALS. I interpret their universal expression as doubt. I don’t often introduce Mr. ALS, which perhaps is rude of me, but based on their quizzicality, and everything else I need to do, so as not to fall down, I have chosen to let that opportunity pass by. 

Last week, a TSA agent at a busy, metropolitan airport tainted the TSA adventure of with a fetid twist of meanness unseen before, beginning at her scanner. Peggy had already helped me to get un-shoed, with cane, shoes, backpack, and vest on the belt. (There are no chairs on the entry side of the scanner.) The TSA officer barked orders like a grim-faced Victorian school marm: 

•  “Step forward!” I shuffle forward over the lip, into the scanner.

• “Get into the scanner and put your feet in position,” pointing at the yellow footprints spread apart. I adjust my feet, carefully. 

•  “Raise your hands over your head.” 

The agent’s bark must have rattled Righty, because there was no time for the usual gymnastics to help Lefty, who, upon command, barely made it shoulder high. 

This is when the barking turned into shouting which felt like screaming. 

“He can’t raise his arm,” she shouted, over and over, “He can’t raise his arm,” signaling to the other agents that assistance was needed. 

Then, shouting at me, she continued her tirade:  “Get out of the scanner.” “Move.” “Get back out of the scanner!” 

Even as I shuffled out of her scanner, she continued to yell at me, now adding a humiliating shushing-wave of her hand. “Step aside. Stand to the side.” I quietly obeyed. 

As the others behind me marched forward, I felt their sympathy. Notably, no one said a word. 

I stood to the side with Peggy who steadied me until a tall, cheerful agent took charge. He spoke calmly, and quietly. He offered me his hand as I returned to the scanner. He smiled, and gently peppered his instructions with phrases like, “ You’re good, boss.” “Take your time boss.” “ Here, have a seat boss.” What a difference. 

While Mr Smiles was assisting me, Ms. Barking Agent resumed her station at the scanner. Peggy was next in line! 

I looked back in time to see Peggy lean into Ms. Agent’s face with a stern comment or two. While Peggy’s voice was low and forceful, Ms. Agent shrieked something incomprehensible. 

“What did she say,” I asked as we re-united along the conveyor belt. 

“I told her the shouting was uncalled for, that you had ALS, and that a little kindness would be helpful.” 

“And she said…” I asked. 

She said, “I don’t feel like being kind today!’” 

This outlandish statement, and the behavior that preceded it, are examples of anti-kindness. They are unkind actions that prevent or inhibit kindness. 

Once we recovered from the effects of anti-kindness, and recounted the practical kindness of Mr. Smiles, Peggy and I wondered what might have motivated the anti-kindness in Ms. Agent. 

• Maybe Ms. Agent eschews kindness, because she equates kindness with being sweet, and she perceives her job as being tart. 

• Maybe Ms. Agent has never experienced kindness, and therefore has no reference for how to be kind.

• Maybe Ms. Agent is scared of making a mistake or being thought of as weak. 

• Maybe Ms. Agent has learned to yell at people to get things done her way. 

By the time we had reached our dear friends, and moved through the anger, humiliation, and disbelief, Peggy and I began to feel sad for Ms. Agent. Just like a shallow, scowling school marm, Ms. Agent’s anti-kindness envisaged a miserable life. 

Poor Ms. Agent. Nevertheless, the next time I encounter anti-kindness at TSA, I’ll use this TSA FORM. And, the next time kindness descends into anti-kindness, I’ll be using this TSA FORM. 

I have heard the horror stories of others who travel with disabilities. 

• Lost and broken wheelchairs. 
• Refused boarding because you must present yourself in a wheelchair long before able bodied passengers, if you hope to have your wheelchair on the flight. 
• No wheelchair waiting at the connecting gate, as promised
• Fellow passengers maligning someone who moves slowly or has slurred speech.
• Think about it. How does anyone with a wobbly gait or the inability to walk get to a toilet on an airplane? 
• And, why do trains and subways, city buses, and even the trams between airport terminals have wheelchair and handicapped seating, while the entire airline industry has gotten away with not offering these accommodations?  

Frankly, I don’t think it should be necessary for those of us traveling with a disability, like Mr. ALS, to announce and explain our unfortunate circumstance to merit a bit of practical kindness that affords us inclusion and participation in the ordinary inconvenience of present day travel.

Sacred questions

Recently I took part in a Tim Lowry ALS panel for occupational therapy (OT) students.  As panelists we answered many of the questions you might anticipate about how OT has improved our lives with ALS.  I was happy to tell the students about pencil grips, rocker knives, and splints.  The OT professor specialized in hand therapy and was eager to help with my current conundrum:  buttering toast. 

The question that most surprised me was directed to Tim Lowry who communicates via eye-gaze technologies.

"How do you prevent giving up when there is limited treatment and no cure for ALS?"

Fair enough. We tell the audience that we are an open book. Ask us anything. Still, it's not a question in the same league as how to butter toast.

Youthful students, given permission to be truly curious, will expose the elephant in the room.

"How do you face existential challenges, for real, in the day-to-day?"

Tim's poised response revealed the intentionality of a reflective life.  He has learned to nurture his mental and spiritual well-being to support the consequences of his on-going physical decline.

ALS poses its unique challenges to be sure.  Our motor neurons are dying, and it turns out that we really need healthy motor neurons to get dressed in the morning.

One option, I suppose, would have been to answer the youthful questions with a question: "How do you face the existential?  How do you keep from giving up?"  Life is chock-a-block full of existential opportunity:  our present climate crisis, raging gun deaths, a loved-one's eating disorder, a recurrent major depression, a father with ALS. How do any of us live with uncertainty and ambiguity in our lives?

I wonder, now that I have ALS, am I meant to understand more about managing existential crises?   I mean, I have stuff to do.  I have a garden to weed and water.  I have a Tim Lowry panel to prepare for.  I have asparagus to blanch for lunch with Zach and Isabelle.

Here's one thought.  What if we allowed our present reality to become sacred?  Fighting for climate justice and gun safety.  Being with a friend who is struggling.  Learning to put your socks on with one hand.  Blanching asparagus.  Living with ALS.  What if we approached all of it with reverence for the moment at hand?  

Fr. Gregory Boyle, S.J., the founder of Homeboy Industries, and author of The Whole Language, puts it this way:

"We remember the sacred by our reverence...This is the esteem we extend to the reality revealed to us. Jesus didn't abandon his reality, he lived it. He ran away from nothing and sought some wise path through everything. He engaged in it all with acceptance. He had an eye out always for cherishing reality. A homie, Leo, wrote me: 'I'm going to trust God's constancy of love to hover over my crazy ass. I'm fervent in my efforts to cultivate holy desires.' This is how we find this other kind of stride and joyful engagement in our cherished reality. The holy rests in every single thing. Yes, it hovers, over our crazy asses."

Holy tears

 

Tender

soothing rain

all night last night

lingers unto morning

like sweet tears

to begin this day anew

and whole.

I did not grow up in a culture of sweet tears, the kind that flow gently down the cheek, as plainly and innocently as a smirk might linger while contemplating someone's clever retort.  In truth, I learned to withhold smirks and tears at all cost, until I became a dad.

Dad tears are sweet tears. Dad tears are Holy Tears.

I rarely withhold tears anymore.

Last week I found myself in Howard Goodkin's office with Peggy and William.  Howard is the chair of neurology at the University of Virginia. He is also a child neurologist with whom I have shared many complicated patients. Howard invited us to join him and the leaders of the ALS Dart Center of Excellence to explore the possibility of a partnership with the Hummingbird Fund.

It is worth mentioning here that Howard is also the person I emailed when Peggy and I first seriously suspected a diagnosis of ALS.  We were lost, adrift, so I emailed Howard. He called my cell before I could get up from the computer.

A couple of months later, at the Hummingbird launch, Howard joined a dozen colleagues, friends and family as a docent, mingling with guests, wearing his docent's badge that read, "ASK ME ANYTHING."  He was terrific.

All of this history sat silently in the back of my mind as I calmly entered Howard's office and took a seat at the familiar, long oak table, much like I had done many times before while working at UVA.

We began with introductions and roles, going around the table, ending with me.

As I began to recount my diagnosis, and the journey which has led to the Hummingbird Fund, tears began to roll down my cheeks.  I was surprised by the tears, and I smiled at Howard who was sitting at the other end of the table.  I said, "Well Howard, this is the first time I've cried in your office."

Everyone chuckled quietly, and without missing a beat, Howard said, "Well Jim, it's not the first time someone has cried in this office.  Many people have cried in this office, including me."

More soft chuckling... followed by a brief, intimate silence... followed by me having a moment to regain my grounding as a man with ALS, in a room full of people who know Mr. ALS all too well.  The group moved on through our agenda, and we will have a meaningful partnership.

I am learning that tears and ALS are pretty much kissing cousins.  Fury, deep disappointment, grief, and heartbreak. This community also weeps for the fearlessness, dignity, and brazen honesty of our kin. The road forward is a road through tears.  Holy tears of love and courage.

ALSTLS

ALS is always a grim tale to tell, and to hear.  Sorry.  

What if we could tell a parallel ALS tale that was the opposite of grim?  I have an idea.  Read on.

ALS, Amyotrophic Lateral Sclerosis, is generally defined as a progressive, neurodegenerative illness with no cure.  Often, this straightforward definition is embellished with a clause or two: 

• eventually leading to the inability to walk, talk, swallow, or breath.  
• including complete paralysis with intact cognition.
• being 100% fatal with an average life expectancy of 2-5 years. 

ALS is commonly referred to as a brutal disease; the disease most feared by doctors.

As I mentioned, it's a grim tale.  Sorry, again. 

Did you know that the functional decline that accompanies ALS is scored as a way of tracking the progression of the illness?  There is a 12-item functional rating scale called the ALSFRS-R:  48 points = A+ = no disability.

See details here.

• Speech
• Salivation
• Swallowing
• Handwriting
• Using utensils
• Dressing and hygiene
• Turning in Bed
• Walking
• Climbing stairs
• Dyspnea (breathing hard with activity)
• Orthopnea (difficulty breathing while lying down)
• Respiratory insufficiency

I am currently a 43 or 44.  I was a 41 or 42, until I taught Righty to use a pen.  Honestly, I could probably be a solid 44, if I used a rocker-knife.  After all, if we can enhance or adapt function to increase inclusion and participation, we diminish the disability.

It is worth noting here that Mr. ALS's march toward disability does not necessarily include mental, emotional, or spiritual decline.   ALS causes motor nerves and muscles to die. The thinking-mind remains completely intact.  Spirit, drive, passion, motivation, personality, the ability to give and receive love, all remain in full force.

Let us remember that intact abilities often compensate for innate or acquired disability.  People who are blind develop an exquisite sense of touch, smell, and hearing.  Kids with spina bifida who have never had use of their legs learn to scoot around as fast as their playmates and siblings.

What Lefty is losing in function, Righty happily takes on with aplomb.  

Joining the ALS community through organizations like I Am ALS, I am inspired by a fierceness of spirit, a courageous drive for change, a passionate desire to leave the world a better place.  These ALS champions and their loved ones are my new team.  Everyone is welcome.  Everyone gets into the game.  Kindness is not a zero-some proposition.  Love abounds.  Courage and hope prevail.

I understand the need for the ALSFRS-R.  But, wouldn't it be great to have a partnered scale to measure positive progress?  We could call it the ALSTLS.  The ALS Turbo-charged Living Scale, measuring:  

• adaptability
• humor
• kindness
• compassion
• hopefulness
• resilience
• altruism
• passion for creating change
• fierceness and drive
• advocacy for self and others
• ingenuity
• vision and meaning

What do you think?  Great idea, right?

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.

   

My pillbox

Once a week, I fill my pillbox.  The box keeps me on track, especially since I swallow a rainbow of pills and capsules three times a day.   You might think it impossible to forget whether you have swallowed 13 or 14 pills.  Trust me, when it becomes a day-to-day routine, it's easy to forget without a pillbox that stands empty.  Crazy, right?

Before Mr. ALS arrived on the scene, I did not own a pillbox, and I did not fret about forgetting to take my vitamin D.

As for today's collection,  you might enjoy knowing that I have a giant capsule filled with golden oil, and a tiny rectangular tablet the color of a robin's egg.  Theracumin is a standout for two reasons.  It is a uniquely thin capsule, easy to swallow, and it is a strikingly beautiful butterscotch-mustard-yellow.  

A wide array of white capsules are distinguished only by size.  The six giant sodium phenylbutyrates (which I take twice a day) must be taken 2 at a time, and even then, they often stick to the back of my throat, unless, of course, I remember to tip my head forward to widen the epiglottic valleculla.

Randomly, I'll mention here that as a boy I collected marbles.  I had cat's eyes, and boulders, steelies, and solids. In all humility, my collection was the envy of Riverside Drive.  Somehow this seems full-circle.

Every pill and capsule I ingest has a clearly described scientific rationale.  Some of them come with a prescription from my neurologist.  Others I purchase on Amazon.

A major player in my arsenal is the tag team of Tudca and Sodium phenylbutyrate. This combination is also known as AMX0035, which is making its way through the regulatory approval process at the FDA.  AMX0035 is only available through a randomized controlled trial, which means some of the people are receiving the actual medication and some are receiving a placebo. 

AMX0035 has shown promising evidence in the treatment of ALS:  it appears to slow the progression to respiratory failure by 10 months.

Until recently I have been using a small compounding pharmacy in New Jersey for my monthly supply of sodium phenylbutyrate, all 360 capsules.  We purchase it off label, which also means it is not covered by insurance.

Much to my dismay, the sole manufacturer of sodium phenylbutyrate halted production in May.  I talked to them, of course, and they offered no clear explanation, nor definite time frame for when production might resume.

I ask you: what's a person to do with Mr. ALS breathing down their back?  Take the regular dose and accept a pause in a proven therapy?  Take a half-dose to make it last longer and accept the possibility of it being sub-therapeutic?  Try to find another pharmacy that compounds sodium phenylbutyrate and ships it out of state?

After consulting with three neurologists who are experts in the field of ALS, I decided on a half-dose.  

In the meantime, if I were to sense a subtle, new symptom of ALS, should I worry the new symptom is the result of the decreased dose, or just the natural progression of ALS?

Another conundrum: as new therapeutic opportunities arise, what should be the calculus for the order of operation, because certain options preclude others.

The happy ending here is that we have found another compounding pharmacy and I am back to a full dose of sodium phenylbutyrate.  The injustice and inequity of my privilege is on full display as I pay thousands of dollars each month for this opportunity.  

Yesterday, I spent the entire day on zoom, in virtual meetings lobbying members of congress with a group from the ALS Association.  As people living with ALS, we did our very best to elucidate the realities of our lives.  The legislative aides listened carefully, mostly.  They took notes and expressed dismay for our misfortune.  We asked for their support regarding appropriations for the Act For ALS, and in general they assured us they would keep it under consideration.

ALS is unlike any other adult infirmity in its swift path to disability.  The glacial speed of the federal government is not well suited to respond to this kind of urgency in a rare disease.  Still we must persist.

I take hope in the power of the people.  The ALS community is a feisty lot.  We will find a way. 

NIH Day #3

Day #3 finds JimBob and PeggO weary at the outset. A decent breakfast is essential.  Peggo, as we all know, comes prepared to meet that task.  

This morning they begin with Peet's House Blend pour-over coffee, followed by maple flavored Brown Cow yogurt, the kind with cream on top, homemade granola, and hand-picked, ripe, local strawberries.

Vine-ripened strawberries are a wonder of nature, no?  

"Think about it," muses JimBob, "where else in the world do you see this color of red?"

He waxes on, "Strawberries are so fun to eat... plucking their little stem hats off as you pop them into your mouth." 

JimBob demonstrates, followed by a self-satisfied smirk.

"Delicious."

Gesturing with the strawberry top, he says, "No two berries are ever the same.  Isn't that amazing?"

"Yes, truly amazing.  You realize that we still need to take Delta for a walk."  PeggO is all business this morning.

Today's snacks:  assorted charcuterie, hard cheese, artisanal crackers, roasted pistachios, dates, and ripe strawberries.  Pamplemousse La Croix to drink.

PeggO knows what JimBob likes.

9 a.m. NIH 1st Floor radiology: Fluoroscopy Suite

The official schedule begins with a swallowing study, because people with ALS eventually lose the muscular control required to eat and swallow.  The first sign of a problem can be coughing while trying to swallow.  Have you ever choked a little bit on your own saliva?  Does it make you cough?  Maybe this happens when you are laughing.  Or, maybe, like JimBob, this happens when you try to talk and eat at the same time.   Mr. ALS has this way of transforming an innocent cough into a telltale sign of decline. 

As they approach the radiology check-in desk, Monique is waiting.  She introduces herself to JimBob and PeggO, and ushers them back to her fluoroscopy suite.

Monique is the energetic Speech Language Pathologist (SLP) who will do the swallow study, followed by 2 hours of speech and language evaluation.  JimBob connects with Monique immediately.  She is direct and in charge.  JimBob especially appreciates the ways in which Monique respects his dignity. 

The study will allow Monique to visualize JimBob's swallowing in real time.  She begins with a teaspoon of radio-opaque liquid, flavored with Hershey's syrup.

Monique stands in front of JimBob as he swallows the teaspoon of liquid.  In a formal tone, she queues the radiology technician to capture the dynamic image of swallowing which is visible to her, and to JimBob, on a small screen next to the x-ray camera pointed at JimBob's neck.

"Now," she calls out.  

JimBob and Monique watch the x-ray image appear.

A flash of white liquid glides across JimBob's tongue, down his pharynx, and into his esophagus.  No aspiration.  This is good. 

"Stop," Monique commands.  The screen goes blank.

Next they repeat the test with an ounce of the same Hershey's flavored liquid.  And after that, 2 ounces of the same liquid, followed by radio-opaque pudding.  Monique is visibly relaxing as all of the studies are normal.  JimBob wonders how often Monique is the one to break the bad news about unsafe swallowing due to the progression of ALS.

They finish the study with a radio-opaque capsule, to test if it's safe for JimBob to swallow pills.  Since he swallows 27 capsules a day, JimBob is relatively confident this will make it a clean round on the swallow study circuit.

And, it does.

They move on to Monique's office for tongue twisters, reading aloud, and oral-motor gymnastics-- things they all know will one day be impossible for JimBob.

For today, JimBob is happy to repeat them faster than Monique.  

Next are the tests for language processing which include verbally interpreting a drawing of people at the beach enjoying various waterfront activities.  JimBob expresses concern for the man looking at his phone while the smiling woman on the blanket in front of him is opening a bottle of wine.  After JimBob has completed his timed description, Monique comments that she shares his concern for the man, and has never before heard anyone call attention to their bare feet.  

Monique now administers the test that asks for lists of words beginning with a, s, and f.  Then a list of animals.  JimBob has just completed these tests with the neuropsychologist yesterday. Today he does not have concerns about cursing, and he sails through the test with flying colors, and a knowing wink from PeggO.   (See NIH Day #2)

Monique's final test involves ordering a fictitious executive's schedule based on the restrictions described in an accompanying narrative.  Naturally, JimBob suggests they pass this along to an able administrative assistant.

"I rather assumed you might say that," chuckles Monique, with a nod toward the test that means: "get to work."

Right.

JimBob buckles down and solves the tedious puzzle that includes when to order flowers for the wife and still see his most important customer, while making all of the day's deadlines.

Now it's time for a real lunch in the lobby.

1 p.m. Electrophysiology Lab.   

There is a solid rationale for saving the EMG and nerve conduction studies until last.  They are generally presented to patients as "somewhat uncomfortable."

JimBob and PeggO asked about the need to repeat these studies at the NIH, since they were completed at UVA as part of the diagnostic work up for ALS.  The recommendation from everyone was to repeat the EMG, since it had been done at such an early point in the disease progression.  Repeating the test now should be helpful in a number of ways.  The nerve conduction study, on the other hand, was comprehensive the first time and would unlikely need to be redone. 

Entering the electrophysiology lab, JimBob and PeggO are introduced to a short, gray-haired woman in a long white lab coat.  She speaks sternly with a thick Eastern European accent.  Her face shows intense concentration.  She does not smile.  She gestures to two chairs by the wall and instructs JimBob and PeggO to have a seat.  Next, she hands JimBob a blue paper gown that she has produced from a gray, metal drawer.  As she leaves the room, presumably to allow JimBob some privacy, she chuckles to herself, saying the teenagers always want to take the blue paper gown home.  Huh?

PeggO and JimBob look at each other and need no words to express their hesitation for how this is likely to unfold.

The exam starts with an ultrasound exam of various muscles looking for fasciculations, or tiny muscles twitches.  Along with progressive weakness, fasciculations are the hallmark of ALS.  Clinicians debate whether fasciculations precede weakness.  If they do, then they may be able to predict the spread of the disease throughout the body.  

JimBob's fasciculations started in his left arm.  Currently, he feels fasciculations all day long in his arms, chest, back and abdomen.  The twitching is exacerbated by activity.  This means he has more fasciculations after many ordinary tasks like making the bed, working in the garden, typing, or standing for more than 10 minutes.  With a bit of rest, the twitching quiets down, until the next wave of activity.

Today's ultrasound and EMG will provide objective, scientific evidence of JimBob's everyday experience of fasciculations.

As she finishes with the ultrasound exam, the neuroelectrophysiologist (the white-haired woman in the long white coat) moves to the other side of the room and begins to pull the nerve conduction device closer to JimBob.  

JimBob starts to get nervous.  He remembers this experience all too well from the first time. 

A nerve conduction study measures the speed of electrical current running along a nerve.  To do this a receptor tab is placed near the end of the nerve, say at the wrist, and an electric probe is placed firmly against that same nerve, say in the elbow.  The neuroelectrophysiologist shocks the nerve repeatedly, with increasing voltage, sending a wave of electricity down the nerve.  The speed is recorded at different voltages, and the probes are placed along many nerves throughout the body to complete the test.

The first shock in each series feels like a little tingle and is easily tolerated.  As the voltage increases, the shock feels like an electric fence at the farm, and then like a shock from an electric outlet, and finally, the shock causes the limb to involuntarily jump off the table. The maximum shock is then repeated 3 times.

I'm not kidding.  This really happens.

The study begins and JimBob immediately remembers the "discomfort".  As they approach the maximal voltage, he instinctively clutches the edge of the exam table, bracing himself for the jolt of electric current.  

PeggO sits behind JimBob as the nerve conduction study progresses.  She is out of his sight line, but he senses her rise out of her chair with each maximal voltage. On the third sequence of  shocks, PeggO asks forcefully, "Is this really necessary?"

An awkward silence hovers in the room.

"This is the way I like to do these studies," responds White Coat, without turning away from her instruments.

"I knew we needed to repeat part of the EMG, but it was my understanding that the nerve conduction would not be required.  Has something changed?" asks JimBob.

No response.

With the next series of shocks, as JimBob's left foot and lower leg come jumping off the exam table, PeggO moves to get out of her chair, and White Coat calls it quits.

"He is so hyper-reflexic; I'm not sure this is worthwhile.  And it is obviously not his favorite, " says White Coat to her assistant.  She says this sitting next to JimBob...her hand still resting on his leg.

Not worthwhile?  Not his favorite?

What is going on here?  Who is this for?

Next test: the EMG.

An EMG measures the electrical activity in muscles, especially as the muscles are stimulated by nerves.  To do this, small needles are placed in muscles all over the body, one at a time.  The needles are connected via a wire to the EMG unit which measures the electrical activity in that particular muscle.

White Coat begins by placing the EMG needle into JimBobs left shin.  She observes the readings on the EMG unit, and adjusts the needle by wiggling it around in the muscle or pushing it deeper into the muscle.  Once she has the reading she needs, she asks JimBob to flex the muscle as hard as he can with the needle in place.

White Coat repeats this in JimBob's left thumb, both biceps, and finally in JimBob's neck and back.  She decides against the tongue since that was tested the last time JimBob had an EMG.  

Wrapping up the consultation, White Coat sits facing JimBob and PeggO and delivers the news. The EMG confirms that JimBob has fasciculations in his arms, chest, back, and abdomen.

I'm not kidding, this is a true story.

3:30 Final Wrap-Up with Dr. Kwan

JimBob, PeggO and Dr. Kwan sit alone in a small, quiet exam room.  Pale yellow weariness hangs like a fog as Dr. Kwan begins to speak.  

"Without patients who are willing to participate in research, we can make no progress in understanding ALS," he says.   "I am so grateful for your participation in my research."

Together, the three doctors summarize the positive outcomes of the three days.

• Blood samples may detect HERV-K leading to an anti-retroviral treatment trial
• 85th percentile for rate of decline in people living with ALS
• Excellent results from neuropsychological testing
• Excellent forced vital capacity
• Excellent swallow study and speech
• Electrophysiological results that support a diagnosis of ALS
• No further need for EMG or nerve conduction studies
• No further risk for Frontal-Temporal Dementia
• A connection to the NIH and to Dr. Kwan for future consultations.

When it's time for closing questions, PeggO begins.  "Does the NIH have plans to expand this study to become longitudinal, tracking patients' progress over time?"

"Wouldn't it be more helpful to understand how and why patients progress over time, rather than just having data from one point in time?" she asks.

"Absolutely.  We would love to do that; however, at this time we do not have the bandwidth for that kind of study," responds Dr. Kwan gently.

JimBob clarifies, "Will every piece of data collected in the three days be used for research?"  

Dr. Kwan assures him it will.  

As they stand to say goodbye, Dr. Kwan thanks JimBob and Peggo once again.   It feels to JimBob like Dr. Kwan would welcome an embrace.   More than likely, ALS takes its toll on the doctor's heart too.

The three doctors linger, bowing slightly, tipping their heads, smiling and saying thanks and goodbye a few more times.

4 p.m.  

On the way back to the hotel, JimBob and Peggo begin to realize their cumulative fatigue from three full days of highs and lows at the NIH. 

Each evaluation held the power for good news or disappointment.  Each test brought the possibility for increased hope or further evidence of functional decline.  Each encounter carried its own risk, and required JimBob and PeggO to muster steady courage, stamina and good humor.  

5 p.m.on the road back to Charlottesville

"Well, are you glad we came," asks PeggO from the driver's seat.

"I am," responds JImBob, "I'm grateful for the opportunity." 

"I hope it helps someone down the line.," he continues.

"You never know,,, you might be HERV-K positive and then you could try ARVs" says Peggo with a hopeful tone.

"That would be so cool..." nods JimBob, "I would really like the chance to try ARVs."

'I want you to take ARVs too... it would be amazing if ALS could be the next HIV story," adds PeggO.

"Totally," says JimBob, looking out at the tree-lined streets of the passing Bethesda neighborhoods.

"It's kind of unbelievable that no one at NIH is running longitudinal studies for ALS," reflects PeggO.

"And there is no central coordination, like in the HIV days," adds JimBob. "There really doesn't seem to be anyone in charge, just a bunch of silo'd labs doing their own things."

"It's enough to be a patient, and now we're supposed to figure out how to get the NIH organized," grumbles PeggO.

 As they move into rush hour traffic, the mood in the car goes blue-gray. 

"I thought you were going to jump across the room when she kept zapping me for the nerve conduction," interjects JimBob, knowingly changing the subject.

"I was ready to,,,"  says PeggO without missing a beat. 

"I could tell.  I could totally feel your energy in the room.  It was great," smiles JimBob.

"We're a team," responds PeggO.

"Yep," sighs JimBob quietly looking away, "We're a team."

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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.

Proximity

Yesterday, May 12, 2022, my mom would have been 93 years old.  Peggy and I were on the National Mall with Lefty and Righty planting 6,000 flags to honor people living with ALS, and people who have passed away from ALS. Each flag bore a name and a date of diagnosis. 

A strong breeze caused the flags to flicker and buzz across the hillside, like thousands of plastic whirligigs on sticks.  To make it a bit easier to locate a name, the flags were loosely organized, alphabetically, in rows by first name.   Honestly, I was hesitant to look for my flag.  I was not at all sure how it would feel to see my name among all the others with ALS. 

 

I walked the rows like a gardener inspecting Spring seedlings.  I saw Jims, and Jimmys, and Jimmies, and Jameses.  Row upon row upon row, I walked silently, sometimes kneeling for a closer look.

When I came upon my flag, with my name, and my age, I felt a surprising  affirmation in my chest.  I felt solemn, and connected.  I sensed that I was among new kin.  I felt whole and at peace.

An hour later, my son William and I addressed the assembled gathering.  We chose a call to action as our offering. "The voices and stories of people effected by ALS will be the driving force of progress.  Our activism, our 'good trouble' will be our hope together."

Many of yesterday's speakers had very little voice left due to the advancing muscle weakness from ALS.  We leaned in to decipher their words which were often too soft or garbled to fully understand.  As you might imagine, wheel chairs of all shapes and sizes cruised among the ambulatory.  Children darted in and out, and our dog Delta Mae stole the show, making her way into dozens of pictures.  Everyone wanted a selfie with Delta.

The day was also full of tears.  ALS, after all, is steeped in loss.  Loss of function.  Loss of dignity and autonomy. Loss of futures. And, eventually loss of loved ones.  People wept for themselves, for their families, and for those they have lost.  People cried tears of rage and frustration.  They cried for each other.

And, in the space of hours, Peggy and I connected with people from all over the country who are at every stage along the ALS journey.  We hugged people whom we have only known via zoom.  We thanked people for their inspiration and their courage.  They hugged us back and they meant it. 

This event was equal parts rally and reunion.  Speaker after speaker remarked that  this small group, who shared an intimate knowledge of the ALS journey, had become a kind of family for them.   Folks who were farther along the path welcomed those of us who were new to the journey.  In that way, it felt like an equal part church.

Bryan Stevenson often sums up his public lectures with a call for greater proximity to injustice.  He rightly surmises that those with proximity to the problem have the best hope for an authentic, meaningful solution.  By analogy, the closer the proximity, the truer the response.

Tears, it occurs to me, are a true manifestation of authentic proximity.  So are the spontaneous hugs that hold you close, belying social custom. 

The Launch

"I hope you know how loved you are!"  "You are so loved."  "People love you."

Over and over and over again I have received these sincere exclamations that are meant to remind me of the bountiful love that surrounds me.  I am most often without adequate words in response.  

Usually, I begin by looking down at my feet.

Then, I say something like, "the feeling is mutual."  Or, I talk about the relationship that has fostered the love.  While all of this is certainly true, the words do not match the magnitude of the experience, no matter how earnestly I try to make them sound.

Love en masse can be overwhelming. . .

. . . which is no excuse for minimizing the response.

Overwhelming love calls forth gratitude beyond words. 

For this crowd, overwhelming love definitely means showing up. 

Abundant love is meant to be thoroughly enjoyed.  Don't you agree?

Enjoyed with great good humor. . .

...and wordless, heartfelt embraces.

This kind of love is meant to be shared.

The Hummingbird Fund is now a dream come true.  My family and I have a new labor of love.

Through the Hummingbird Fund we will be able to dramatically improve the quality of life for families living with ALS.  

(contact us: thehummingbirdfund@gmail.com)

We are grateful beyond words, and we have meaningful work ahead.