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.

Peace of Wild Things

Robb Elementary School, Uvalde, Texas, May 24th, 2022...

When there are no more words to approximate the utter gut-wrenching anguish of a desperately grieving parent, whose child has been slaughtered, at school, during reading group, or a spelling test...

When the complexity of a common reality like owning and using a gun to commit mass murder on a regular Tuesday morning in May...When this complexity overwhelms the simple truth that, yet again, an angry teenager with a loaded gun is never going to end well...

When fellow citizens, who are themselves parents, grandparents, teachers, and siblings, continue to disagree vehemently on the moral response to a vast human tragedy such as this..

How do we find hope?  Without hope, how do we march on?

I have little space in my heart for hope this morning. My chest is tight with rage and sorrow.  

This morning, like most mornings, Delta Mae joins me in contemplation.  She sprawls next to me in a patch of sunlight, and waits for me to be done.  Some mornings, like this morning, Delta comes to me for a snuggle.  She senses my mood and needs to connect.  I am grateful.

Peggy has created many gardens full of perennials that bloom from Spring to Fall.  We call this particular garden, in the center of the side yard, our Contemplation Garden. A small stone patio, a single table and chair, and gentle morning sun have made it a natural spot to sit and be still.

Did you know that nuthatches favor fur for their nests?  I see them swoop in and pluck from Delta's haunches or tail.  Delta used to flinch reflexively when she felt the pluck of her fur, like a horse does when it wants to rid itself of flies in the field.  Nowadays, Delta embraces the stillness and the silence, and the nuthatch are happy with their prize.

The garden surprises me with something nearly every day. I saw diamond chips nestled amongst ferns today. Who knew that leftover raindrops on cobwebs could be so dramatic?

We have a box turtle who lives in the contemplation garden.  Invariably, this plodding reptile makes me feel happy.

Peggy has planted mostly native plants in the garden, like milkweed that will welcome monarch butterflies. I added a ceramic blue bird bath, on sale at Kroger, mostly for color, during a bland, brown period in November.  

This past fall, September thru December, I sat in contemplation and watched the garden slowly wither into winter. I knew I likely had ALS as the weakness in my left hand became more real.  Sitting in contemplation with trees losing their leaves and flowering plants sinking back into the soil, I remember feeling an odd and unexpected solace.  My reality of ALS seemed shared by a greater reality.  My truth was held up in a wider truth.

William McNamara defines contemplation as, "...a long loving look at the real."  This works for me.  The practice of contemplation reliably creates space in my heart, some might say in my soul.  The space makes room for hope.

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.