Essays
Pancho and Lefty
Nature or nurture? The moms controlled half the nature, but all the nurture. Whatever the explanation, these stepbrothers of the same father but different mothers sure turned out different.
After the death of our nonagenarian cat, we decided to buy a dog. Since kittenhood, my wife had received all the cat’s love and affection. If such aloof and independent creatures are really capable of love and affection. He would only acknowledge my existence with her gone and him hungry.
I longed for a puppy. My wife’s sister had a little dog who lived happily in a Las Vegas apartment, trained to go on a puppy pad. But big city condo living with both of us working would still pose problems. Should we look into doggy day care?
After research and discussion with the vet’s staff, old friends after many visits with the cat, we decided to adopt a Mi-Ki, a mostly-Maltese toy breed created as a calm and healthy companion dog. In a life-altering moment, our breeder said, “Why not take two? They can keep each other company.” We named them Pancho and Lefty after the Willie Nelson song about the assassination of Pancho Villa by a made-up character called Lefty. Our Lefty bore no resemblance to the scoundrel of the song.
Our nicknames for them reflected their personalities. Pancho became the Terrorist, the Miscreant, the Little Criminal. We called Lefty Zen Master and Sweetheart.
Pancho, agile and athletic, raced about and leapt on and off the furniture. Lefty, overweight, a trencherman, plodded around. The sound of the refrigerator door opening would bring them both running. Precious, pleading black eyes often defeated our resolve to keep Lefty on a diet.
Pancho had a mean streak and sharp teeth he did not hesitate to use. He sometimes assaulted Lefty with no provocation, like a little black Ninja, a canine hitman. Amiable blond Lefty never bit anybody, except Pancho when defending himself.
Despite his aggressiveness, Pancho was a coward, easily spooked. Lefty knew no fear. Encountering a bigger, barking dog out on a walk, Lefty confronted, Pancho ran. At the sound of thunder, Pancho would tremble and try to hide. Lefty hardly noticed.
Pancho knew two notes: bark and growl. Lefty had a vocabulary. He would not only bark and growl, he purred when happy, whimpered when he needed something, huffed, snorted, and made a noise like an old door creaking when he was hungry.
A father of three, I used to laugh at people talking about their surrogate children, their fur babies. No more.
Several years on, as consoler-in-chief Joe Biden often said, when I walk by the mahogany boxes on the mantel containing their ashes it brings a smile to my lips rather than a tear to my eye.
Mountain School
The essay Mountain School, about my experiences as the medical support for the Vermont Army National Guard’s Mountain School, including a memorable bit about a sergeant and his goose, appeared in Military Experience and the Arts in 2023. Here is the opening paragraph and the link is provided below.
As the airliner closed in on Montpelier, a snowy landscape stretched toward the hulking silhouette of the Green Mountains in the distance, a vista radically different from the flat piney woods and marshlands of my native south Georgia. My destination lay nestled somewhere in those mountains—Camp Ethan Allen, home of the Vermont Army National Guard’s mountain warfare school, just outside the small town of Jericho. As someone who spent his youth on water skis and never put on snow skis until age thirty, I felt apprehensive about this assignment I had volunteered for. Second guessing myself. What did I know about mountains?
Flouting the Rules
There should be a rule in medicine: cardiologists shouldn’t have heart attacks, oncologists shouldn’t get cancer, lupus should spare rheumatologists. Why? Because they know too much. They can see what’s coming. They know the complications. They know the drug side effects.
Following this rule, neurologists should never develop ALS.
Unfortunately, there is no such rule.
Other rules also apply. ALS only affects nice people. The disease has a peculiar predilection to attack nice people and spare mean people. And ALS loves the ironic twist.
Another rule: when you hear hoofbeats, think horses, not zebras. This maxim reminds physicians that common things are common. A patient with fever and a cough probably has a viral upper respiratory infection, not anthrax. The aphorism is all well and good, except when the horse is an inexorably progressive and inevitably fatal disease—when the horse is ALS. Then you have to be absolutely certain this thing that appears to be a horse is not a zebra in disguise.
We know that certain diseases can mimic the clinical picture of ALS. Some of these mimickers are treatable. The evaluation of an ALS patient includes a search for conditions known to mimic it, but all of them are rare and the search is usually futile.
A new mimicker appeared on the scene in the late 1980’s, a condition called multifocal motor neuropathy (MMN). Researchers from Johns Hopkins University described two patients with a disorder involving the motor fibers of multiple peripheral nerves in the upper extremities, sparing sensory fibers.1 Both patients had presented with painless, progressive, asymmetric upper extremity weakness, and both were initially diagnosed as having ALS. But their nerve conduction studies showed striking abnormalities not typical of ALS.
Temporal dispersion refers to the normal tendency of things moving at different velocities to spread out over distance. Runners of differing footspeed will separate further from each other the longer the race. The same phenomenon normally occurs with a compound muscle action potential (CMAP) because not all motor nerve fibers conduct at the same velocity. Conduction block refers to the failure of a nerve potential to transmit, analogous to a runner spraining an ankle and never finishing the race. Both temporal dispersion and conduction block affect the amplitude of the CMAP, and distinguishing between the two may prove difficult.
The patients in the Hopkins paper describing MMN had conduction block in the involved nerves on nerve conduction studies. Conduction block never occurs in ALS. These patients also had high titers of antibodies to GM1ganglioside.
Crucially, the Hopkins MMN patients responded to treatment with cyclophosphamide. So, the Hopkins researchers had reported patients initially thought to have ALS who had conduction block on nerve conduction studies and antibodies to GM1 ganglioside, and who responded to treatment with cyclophosphamide.
In the late 1980s and 1990s, papers appeared with titles such as: Chronic multifocal demyelinating neuropathy simulating motor neuron disease, Multifocal motor neuropathy mimicking motor neuron disease and Motor neuropathies mimicking amyotrophic lateral sclerosis/motor neuron disease.2–4 In a large Irish study, the most common ALS mimic was MMN.5
Neurologists around the world became obsessed with not missing MMN in patients who appeared to have ALS.
The American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) convened a panel to develop consensus criteria for distinguishing between temporal dispersion and true conduction block. The head of the panel and lead author of the paper that followed was Dr. Richard K. Olney, the Director of the ALS Treatment and Research Center at UCSF.6 Rick was a stellar physician and researcher and an esteemed colleague, highly regarded yet unpretentious and always amiable. He was universally recognized as a very nice guy.
The conduction block paper was published in 1999. In 2003, Rick noticed problems with his right leg. His doctors at first thought he had a lumbar disk herniation and he underwent surgery, but the weakness progressed and Rick soon knew he had ALS.
Rick was cared for in his own ALS center by physicians he had trained. Even as his personal illness progressed, he continued to study it.7 He enrolled as the first patient in a clinical trial he had designed before his diagnosis. Rick Olney died in 2012, at age sixty-four. The AANEM honored his memory by creating the Richard K. Olney Lecture, given annually at its association meeting.
Rick survived eight years. Another ALS researcher, Dr. Lisa Krivickas of Harvard, wasn’t as lucky. Lisa and I were colleagues on the Board of Directors of the American Board of Electrodiagnostic Medicine. From the time Lisa told us in a board meeting that she had ALS until she was gone was only a little over two years. She was forty-five. The disease had taken her mother when Lisa was young.
In 2017, Dr. Rahul Desikan, a prominent researcher in the field of neurodegenerative diseases, including ALS, at UCSF, found he had ALS. He died in July 2019 of a rapidly progressive form of the disease. He was forty-one.
It almost seems as if the disease is an evil, sentient entity intent on tracking down and eliminating the specific people trying to find a cure for it. It appears to have license to flagrantly flout the rules.
References
1. Pestronk A, Cornblath DR, Ilyas AA, Baba H, et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol. 1988 Jul;24(1):73-8. doi: 10.1002/ana.410240113. PMID: 2843079.
2. Di Bella P, Logullo F, Dionisi L, Danni M, et al. Chronic multifocal demyelinating neuropathy simulating motor neuron disease. Ital J Neurol Sci. 1991 Feb;12(1):113-8. doi: 10.1007/BF02337624. PMID: 2013517.
3. Bentes C, de Carvalho M, Evangelista T, Sales-Luís ML. Multifocal motor neuropathy mimicking motor neuron disease: nine cases. J Neurol Sci. 1999 Oct 31;169(1-2):76-9. doi: 10.1016/s0022-510x(99)00219-1. PMID: 10540011.
4. Evangelista T, Carvalho M, Conceição I, Pinto A, et al. Motor neuropathies mimicking amyotrophic lateral sclerosis/motor neuron disease. J Neurol Sci. 1996 Aug;139 Suppl:95-8. doi: 10.1016/0022-510x(96)00120-7. PMID: 8899666.
5. Traynor BJ, Codd MB, Corr B, Forde C, et al. Amyotrophic lateral sclerosis mimic syndromes: a population-based study. Arch Neurol. 2000 Jan;57(1):109-13. doi: 10.1001/archneur.57.1.109. PMID: 10634456.
6. American Association of Electrodiagnostic Medicine, Olney RK. Guidelines in electrodiagnostic medicine. Consensus criteria for the diagnosis of partial conduction block. Muscle Nerve Suppl. 1999;8:S225-9. PMID: 16921636.
7. Olney RK, Lomen-Hoerth C. Exit strategies in ALS: an influence of depression or despair? Neurology. 2005 Jul 12;65(1):9-10. doi: 10.1212/01.wnl.0000171741.00711.b5. PMID: 16009877.
This essay first appeared here: RRNMF Neuromuscular Journal 2023;4:8-9.
Finally Meeting David
I had been waiting most of my life for this moment, forty-seven of my sixty-five years. We were standing outside the Galleria dell'Accademia in Florence, waiting to see him, the one I had read about, whose picture I had first seen as a college freshman, the one put on a pedestal by his creator and whose creator I had put on a pedestal. David. The David. All seventeen magnificent alabaster feet of him. He did not disappoint . . .
This essay is currently under submission.
Driving in the Fog
E. L. Doctorow said, “Writing is like driving at night in the fog. You can only see as far as your headlights, but you can make the whole trip that way” . . .
This essay is currently under submission.
Clash of the Irish Brigades
Authorities argue about whether an Irish Brigade existed in the Confederate Army and whether the Union’s Irish Brigade and the Confederacy’s Irish Brigade fought each other at the Battle of Fredericksburg. The alleged fight between the two Irish Brigades played prominently in the 2003 movie Gods and Generals. Ken Burns mentioned such a meeting in his 1990 PBS series on the Civil War. Gods and Generals refers to Cobb’s Brigade as “Brigadier General Thomas R. R. Cobb’s Irish Regiment, Georgia, C.S.A.”
But was their really a clash between the two Irish Brigades . . .
This essay is currently under submission.
An Expatriate Education
I remember certain things from about the time I was in the seventh grade. Bits and pieces. I remember my teacher, Ms. Williams, a nice lady with long, dark hair. I remember learning how important coal mining was to the economy of Birmingham, England—a strange, random factoid to stick in an American boy’s brain. I remember watching Fess Parker play Davy Crockett on our black and white TV. And I remember watching Russian tanks rolling down the streets of Budapest.
Three decades later I would meet a woman whose father was caught up in the Hungarian Revolution of 1956. Sarah was one of our neurology residents at Virginia Commonwealth University and I was an assistant professor. She mentioned her Hungarian heritage from time to time and I knew she had grown up in Virginia but been educated in Hungary. She finished her residency and I eventually left the faculty.
After another thirty years, we became colleagues when I joined her private practice group as my part time retirement job. We renewed acquaintances and I finally learned the whole story—why those Russian tanks were rolling, what had happened to her father, why Sarah had gone to school in Budapest and why Sarah’s father had encouraged her to go to college in Hungary despite his horrendous experiences there.
Prior to WWII, Hungary was part of the Austro-Hungarian empire and fought as a German ally in WWI. In WWII, it again fought with Germany. After WWII, Hungary technically became a democracy, but Communist influences eventually prevailed and by 1949 a tyrannical Soviet puppet regime had seized power, brutally suppressing any opposition. In 1956, the Hungarians rebelled. Students started the revolution but it quickly spread.
Sarah had told me parts of the story before. I knew she had gone to college and medical school at Semmelweis University in Budapest, that her father had been caught up in the revolution, imprisoned and tortured, but despite this had still encouraged her to go back to Hungary to complete her education. Over lunch one day at an upscale seafood restaurant, we talked again about this fascinating story.
The waiter appeared, offering three kinds of delicious bread, a house specialty, and took our orders. As Sarah decided which slice of bread to try first, I said, “The story about 1956 and what happened to your Dad, and that despite all of that he still wanted you to go to school in Hungary is astonishing. Seems Hungary would be the last place on earth he would want you to go.”
“No,” Sarah said, “It makes perfect sense. There’s a Hungarian word called honfiság. Hungarian and English don’t translate well here because Hungarian meanings are multilayered, but in its simplest translation it means patriotism. But on a deeper level, Hungarians are Hungarian to the core. They really are. This pitiful, little country has been the doormat for everything that has ever happened in Central Europe. Always on the wrong side. The Turks were there for 300 years. It’s a very Hungarian honfiság thing for him to encourage his child to go back.”
“You were born in this country?”
“Yes, in 1958. And getting back to the whole honfiság thing, my father was imprisoned and tortured repeatedly, for love of his country. He didn’t blame Hungary. This was the regime, the circumstances of the time. By the time we became old enough to travel with my sister and my mother, we went home regularly. I say home because that was home to us. Hungary was not just some 23 and Me or Ancestry.com abstraction, this was where the rest of our family still lived. My mother’s parents, my mother’s siblings, everybody else was still back there.”
“So, you have extended family in Hungary?” I asked.
“I have a large extended family who went through all of these experiences. Now my father never went back. He felt positive that if he went back, they would throw him in jail. But this wasn’t because he didn’t love Hungary. Didn’t love this country where he grew up, where his ancestors were from. His family was landed gentry; he would have been targeted anyway. Even had he not been a revolutionary trying to overthrow the government.”
“So, was he involved in the revolution?”
“Oh, yeah,” Sarah said. “That’s why they ultimately fled, because he’d gotten word they had another warrant out for his arrest because of his activities with the revolution.”
“Was he one of the students?”
“No, he was not a student. He was in his late 20’s by then and already working but he was one of the organizers of the student movement. I can’t tell you a lot of specifics, because he would shut down every single time.”
“He never talked about what he went through?”
“He never discussed any details. He found it too painful, even years later. Visiting Hungary once, I went to a place called the Terror House. The bottom level was the interrogation rooms. Floor slanted, drain in the middle. I don’t believe in ju-ju and ghosts and such, but I have to tell you, a feeling came over me when I walked through there. I am positive he was tortured in that room.”
Selecting another slice of bread, she continued, “But, ultimately, he loved his country. This was why he risked the things he did, to do what he could to free it and ensure a better future for everybody.”
“So,” I asked, “How did he talk you into going back there to college? I’m envisioning a headstrong American teenager being told she needs to go to Hungary to college.”
“I wasn’t told,” Sarah said.
“How did you make that decision?”
“It was my idea. It was important for my parents that we maintain our Hungarian identity. We spoke Hungarian at home. We attended a refugee group meeting once a month. My father would stand up and recite from famous Hungarian poets. They would sing and dance. The wives would make Hungarian food. And we went home throughout my childhood. I would go every couple of years and stay all summer long. I had people there I cared a lot about.
“You also have to understand, I went to high school at St. Anne’s, an all girl’s Catholic school. The girls were all the same. They all came from upper middle-class backgrounds; they were all Catholic; they all thought the same things; they all walked the walk; they all talked the talk and I stood out like six sore thumbs. I was not the typical Virginia girl. Most of these people had no idea Hungary was even a country, much less in what part of the globe.”
“l can imagine.”
“Oh, yeah,” Sarah said. “I got used to morning announcements consisting of ‘In the name of the Father and the Son and the Holy Spirit and Sarah go to the office please’. Because I was a bit of a hell raiser. I did not fit that mold. So, it came to: am I going to have this life forced on me?”
“You thought college would just be more of St. Anne’s?”
“Well, pretty much. I played piano and my teacher encouraged me to apply for a music scholarship to Catholic University. So, I did and they offered me a scholarship. And I thought, damn I don’t want to do this. I also applied to the University of Virginia. And damn, UVA accepted me too. And then it’s the whole jeans and pearls thing and they’re talking about the sororities and what they’re gonna rush and everything and I’m feeling viscerally sick. You’ve got to understand, I don’t fit that mold. No. I had enough self-realization. Not a society girl.”
“So, you looked at other places. You didn’t just pick Semmelweis, you considered alternatives.”
“I did,” Sarah said. “I had friends in high school, but their dreams were like everybody else’s dreams. My dreams were so alien I didn’t even share them.”
“What do you mean, your dreams were so alien?”
“What I wanted to do with my life was so alien.”
“You had dreams of a career, something more than domestic life?”
“Yes, exactly. They dreamed so little. They were going to stay in Virginia. They thought I was totally weird that I wasn’t playing sports year-round, being on travel teams. ‘You’re never around in the summer. You’re not on summer swim team.’ Well, that’s because I’m in Europe. I’m thinking, you haven’t even crossed state lines, man. You’ve never read Tolstoy.”
Sarah’s father never would talk about his experiences in 1956. He told his wife early in their marriage that when it was time for him to go, he would tell her about it. Then when he was in hospice, on his deathbed, Sarah’s mother finally brought it up and he looked up and said “Please don’t torture me. Let me go in peace.”
Amazing Grace and the Grand Tetons
I remember looking at the picture of the Grand Tetons on the church fan and trying to go there in my mind as my father’s sermon went on and on and on. The snow-capped peaks looked cool and inviting inside the stultifying sanctuary of one of the many white clapboard Methodist churches far from town down a red dirt Georgia road. After exploring the mountain, I would flip the fan over and read the funeral home ad on the back, again. I had heard all my father’s sermons many times. I spent more time in church growing up than was good for me.
Around age fourteen, some of the proselytizing had sunk in and my fevered adolescent brain became obsessed with the idea of eternity and driven mad by Amazing Grace. I remember lying in bed, trying to go to sleep, not able to stop thinking about eternity, not able to stop thinking about the last verse of Amazing Grace.
When we’ve been there ten thousand years
Bright shining as the sun
We’ve no less days to sing God’s praise
Than when we’ve first begun
To me, this meant heaven would be church. All day. Every day. Singing. Bored out of my mind. For ten thousand years. Then another ten thousand years. And on and on. It made me crazy. I decided hell might be preferable because at least it would be more interesting.
They say recovering Catholics have nothing on recovering preacher’s kids.