Dr. Mark Lavallee: Resilience (Part One)--Flexibility

Dr. Lavallee

His “very extroverted” and kind mother studied at the University of Massachusetts and as she started her Ph.D. in marine biology at Yale she worked with Jacques Cousteau. So there were nearly yearly family vacations to Cape Cod with frequent visits to the Woods Hole Oceanographic Institution for a while. When Mark and his younger, but much taller, brothers Brian and Paul, climbed onto the Calypso, the famous research vessel, they headed straight to the galley. 

They were looking for the “funny doughnuts,” buttery flaky French croissants. Mark recalled that Jacques, (host of popular TV documentaries in the late 1960s and early 70s on ocean life, and co-inventor of the modern SCUBA gear), was “super skinny and smelled like cigarettes.”  

His Parents and Early Life

Mark’s father, Ray Lavallee, was from Northhampton, Massachusetts, before the family moved to Upstate New York just outside of Syracuse. He was an electrical engineer with GE and a defense contractor. Among other things, he developed and patented a sensitive microphone for so-called “Towed Array” sonar surveillance devices trailed behind ships. They allowed long-range detection and tracking of (quiet) Soviet nuclear submarines during the Cold War.   

The restored 43-meter Calypso in 2016 (from the Cousteau Society)

After Mom abandoned her Ph.D. aspirations she felt a calling to teach, but then gave that up temporarily to raise her three boys. One day, she took Mark to the pediatrician for an ear infection. The doctor had just read an article about a rare hereditary disorder of connective tissue called Ehlers-Danlos syndrome (EDS). The physician quickly recognized that Mark had this genetic condition of excessive joint mobility, soft stretchy skin, easy bruising, and poor wound healing with scarring. (Though it is much more than that, since the disorder may affect almost any organ.)

Anyway, a month after his fateful diagnosis, when Mark, the future Family Physician, Sports Medicine, and recognized EDS Specialist, was in the first grade, the family moved from the Syracuse suburb of DeWitt to the U.S. Naval base in Argentia in the Canadian Province of Newfoundland. Dr. Lavallee (the name is derived from the French “the valley”) told me that it was cold in the far north on the western edge of the Sea of Labrador and there were deep piles of snow for him and his brothers to play in (but it is not nearly as snowy as Syracuse, by the way).

A current view of Argentia, Newfoundland
after the Naval base closed in 1994 (from Wikipedia)

The people in the close Christian Celtic community were welcoming and deeply religious. Mark’s mother found that the women she met during her visits to town had unusually large families. And she often returned home with stories, such as this one told by Mark with a pretty good musical Irish lilt:

“Mrs. Lavallee, how are your children? And how many did God bless you with?” asked Mrs. Smith.

“I have three beautiful sons.”

“Uh, oh, problems in the bedroom Mrs. Lavallee?”

“No. Why do you say that?” 

“Well this is Mrs. O’Conner, and she has been blessed with 21 children.” 

“How many do you have?”

“I had 18, but lost two at sea.”

Small remote fishing communities, you see, often have large families for economic and cultural reasons. This allows them to survive and maintain their unique heritage and identity. 

An Adventure/Misadventure 

Well, after school was out for the year, his mother was “up for adventure.” She took her sons and one of their friends to “a park,” said Mark. She found a 1700s abandoned fort in “disarray.” Guys like that kind of stuff, right? It would be fun, right? But something awful happened.

The boys were running around, as they will do. Mark was exploring and creeping down a wooden stairway into “a basement or dungeon” (he wasn’t sure which) when the worn tread he stepped on gave way.  His right leg fell through it. As a result of his fragile connective tissue the injury to his lower leg was devastating; from his knee to his foot, the skin and underlying tissue were simply stripped off. It was “like peeling a banana,” said Dr. Lavallee. (The official medical term for the injury is “degloving.”)

Degloving injury to the lower leg (from Ben Ward)

All young Mark saw when he looked at his poor leg was “blood and bone and muscle.” His mother put down one-year-old brother Paul, quickly applied pressure to try to stop the bleeding, and sent her five-year-old son Brian back to the parking lot to look for help.

There was a white pickup truck and two men who looked official (but probably were not, thought Mark since no government employees worked on Sundays).  Brian frantically told them what had happened. They had a stretcher (and, for some unknown reason, a shovel) and would help. They took Mark to the hospital on the naval base. When the ER doctor saw the gaping wound he knew that Mark needed specialist care. He immediately sent the family to St. Johns, the provincial capital and site of the only trauma hospital, one hundred miles away.

Gangrene

At St. John’s, Mark saw a British general surgeon. By then, a portion of the torn skin flap had already died, so he was treated as a burn patient with whirlpool sessions and wrapping. But his foot soon began to “turn colors.” It went from purple, to dark purple, to black. And it started to smell. The big white-uniformed orderly who had been caring for him, carrying him to and from his daily treatments, began to sob.  

Mark had developed gangrene. His right foot was dying. So, after about a week, he was evacuated back to Syracuse and the next day he was seen at the Upstate Medical Center. The orthopedic and plastic surgeons there had to decide whether to take his leg off from below the knee or above the knee, in order to save him.

View of Upstate Medical Center, Syracuse (from SUNY Upstate)

His usually-stoic mother cried. She told her son that he needed surgery. That he would certainly lose his leg, but that he might lose his life. So he would receive the final of the seven Catholic sacraments, Last Rites (“from Father Dave of Holy Cross Parish,” as Mark was careful to recall). The first-grader thought for a minute and then said (as he was later told) that since that meant that he would go to heaven it wouldn’t be a bad thing at all, “that’s what we believe.” He knew well the children’s bedtime prayer: “Now I lay me down to sleep…”  

But as Mark’s horribly injured leg was unbandaged the next day in preparation for surgery his foot was, miraculously, pink. The leg (and his life) would be saved. But the extensive damage would be very slow to heal. He couldn’t walk for three years and had to get around in a wheelchair until fourth grade. 

Young Mark had faith then, and he still does. (His mother sent his story to “Guideposts” and it was published). And he feels that the early and accurate diagnosis of his genetic disorder of connective tissue, knowing what he had and what he needed to deal with, helped him adapt. This was, he said, his “introduction to medicine.”

Faulty Connective Tissue

What is “connective tissue,” you ask? 

From nci.org: "Connective tissue “supports, protects, and gives structure to other tissues and organs in the body. (It) also stores fat, helps move nutrients and other substances between tissues and organs, and helps repair (tissue) damage.”  Much of it consists of fibers of collagen, the main structural (and most abundant) protein in the body. Connective tissue is what holds us together. 

And in EDS, the formation and processing, or packing, of collagen fibrils may be altered by at least 19 different mutated genes. There are 13 (or more) types of EDS. Dr. Lavallee has what is called "classical" EDS, one of the most common forms. It can be due to many specific mutations in the COL5A1 or COLA5A2 genes. 

Types of connective tissue (from quizlet.com)

By the time he was in the fifth grade, the family had moved from upstate New York to Severna Park, Maryland, near Annapolis, the site of the U.S. Naval Academy. Even by then, through his experiences with doctors and hospitals as a patient, Mark felt a calling to become a physician.

An Experience at Johns Hopkins

Johns Hopkins in Baltimore was the “only place” seeing EDS patients like him, said Mark. The field of medical genetics was being single-handedly developed by the illustrious Dr. Victor McKusick at the Moore Clinic. Disorders of connective tissue were a particular interest of his. Dr. McKusick confirmed the diagnosis of EDS and told Mark's parents that he must avoid potentially dangerous activities. 

One routine clinic day, Mark (probably in the sixth grade then) was enlisted (with the consent of his parents) to help teach the “next generation of doctors” (i.e., medical students). There was a child with Marfan’s syndrome (the tall one), one with brittle bones and a misshapen skull of osteogenesis imperfecta (the short one), and Mark, with Ehlers-Danlos (the one in the middle).

Young Mark waited behind the curtain for his turn. Just before he was to go out onto the stage in the auditorium he was asked to take his clothes off so the students could see all of his scars. He did as he was told and stood there completely naked in front of about “a hundred people.” They wanted to see Mark stretch his skin, to see his joint flexibility, to see his wounds. He just wanted to put on his underpants!  

Examples of skin lesions in EDS (from American Journal of Medical Genetics)

More about EDS

But EDS is much more than a medical curiosity to be shown to eager medical students. And different forms of the condition involve different tissues. For example, some people may have abnormal spinal curvature (scoliosis) and some have life-threatening heart valve or vascular problems (including aortic aneurysms). Many have chronic pain and disabling fatigue (as in fibromyalgia), sometimes along with troubling anxiety and depression. Disturbances of the autonomic nervous system (especially the POTS syndrome with lightheadedness and fatigue) and immune system (with so-called mast cell activation) are now recognized as common features of the hypermobility syndromes. The diagnosis of EDS may be missed if it is not thought about.

No Sports; More Time to Study

Moving on. In secondary school, Mark was a good student, of course. But his attempts to fit in socially through athletics fell flat. He said that he “ran like Jerry Lewis” and had trouble finding his sport. It turned out that he liked wrestling and was good at it, but he was injured over and over as a result of his faulty connective tissue and poor healing. Shoulder dislocations were so frequent that he learned to fix them himself.

And as he was followed at Hopkins he had punished his scarred and deformed body (his chest wall was sunken in, a condition called pectus excavatum) so much that he was told by the experts that he was “not allowed to exercise.”  He was to forego all physically strenuous activities, even gym class.

So, with an extra study hall, he had more time for books. And without afternoon sports, he had more time to eat. A typical comforting snack for the sedentary high-schooler could be two Big Macs, a large order of fries, two packs of Reese’s, and a large Coke (totaling about 2,310 calories!).  His weight climbed to 230 pounds. 

After high school, Mark attended Penn State as a pre-med student in the Honors program. By habit, he usually squeezed himself into the same seat in class. He said that one of his fellow students who typically sat next to him was a real “specimen.” This unusually pale classmate (looking “like Casper The Friendly Ghost’) was muscular, “he was ripped,” Mark said. They became friends and then roommates. Brett was “struggling with organic chemistry“ but Mark had “figured it out” so they made a pact; Mark would help Brett with classwork, and Brett would help Mark change his body (and his wounded psyche). 

Strength Training and on to Medical SChool

Mark learned that if he was careful, and used proper technique, he could avoid injury; he could do fairly intense body-building (and strength training) relatively safely. Oddly, the gym facilities on the big campus with the well-known successful football team were limited. So Brett and Mark (with a few other investors) opened a student-run 24-hour gym. They (and anybody else at University Park) could work out whenever they wanted to. It was a huge success, such that when the young entrepreneurs later sold the gym it paid for two years of their medical school!

The huge IM Building at Penn State Main Campus:
Open 9 a.m-9 p.m. (photo from PennState)

So, while at Penn State, Mark was “either studying or at the gym.”  And, at some point, he even lifted competitively.

He worked hard and he was accepted to the Hershey Medical School as a sophomore. But his father was leery about his son’s choice of career since Mark had fainted at the sight of blood (his grandmother’s, not his own) when he was home one summer during college (though it was a hot day and he had worked outside and he might have just been dehydrated, he thought).

The future Dr. Lavallee needed to test this fateful possibility. Could he practice medicine, could he be a doctor, if he had hemophobia, a morbid fear of blood?  

He decided to get his license to volunteer as an EMT at State College to test this. The plan worked; he proved to himself that he could stand the sight of blood without getting sick or passing out. When he wanted to continue running with an ambulance crew over the summer break back in Maryland there was a hitch. The rule was that you had to first qualify as a level 1 firefighter. He was game, but that didn’t really turn out so well.

Let's Try Firefighting!

He went to a fire-fighting school in Maryland between his junior and senior years at Penn State. One day, there was a single-family house fire. His job was to climb the hook and ladder to get to the roof. His partner was to use a circular saw to cut holes in the roof to ventilate vertically and prevent deadly back-draft. But they were given mistaken directions; instead of ventilating away from the fire, they were directly on top of it.

The roof caved in and Mark fell through onto the floor that was in flames. His heavy breathing tank was shoved into his spine. It was hazy, black, and “extremely hot,” maybe 600-700 degrees. And he was in terrific pain. Fortunately, the experienced fire suppression team was there within seconds, but as Mark struggled to crawl to safety Army-style on his forearms and knees his protective turnout gear melted. (Continue the story...)

Efforts to produce tactical ventilation through a roof (From cfitrainer.net)

By Anita Cherry 6/10/23

Dr. Carole Dorsch: She likes puzzles

Dr. Dorsch

Until she was two, Carole, her parents, and her father’s older brother lived together in her paternal grandmother’s neat row home on North Broadway in Baltimore, just up the road, the boulevard, from Johns Hopkins Hospital. After that, her parents bought a place “down the shore” in Essex-Middle River on an estuary of the Chesapeake Bay. It was just 12 miles from the city but seemed “so far away” to the little girl. So, Dr. Carole Dorsch and her younger sister Beatrice grew up playing on the water. 

Carole’s father, Oscar, did electrical work for Standard Oil during the Great Depression and was an aircraft inspector for the Navy during WWII. He was a bit of a pseudo-entrepreneur, said Carole, and after the War, he continued to work as an electrician and bought a number of rental properties. Her mother left high school to take a job in a factory and then worked as a beautician, running her own shop.

But her father had a “midlife crisis” when Dr. Dorsch was in the second grade; he wanted to enjoy life. So he stopped working, planted flowers, raised birds, and began careful investing in the stock market (and he was quite good at it). This was okay for a number of years, but when his first daughter decided on her own in the eighth or ninth grade that she wanted to go to college Oscar realized that he needed to do something else to make that happen. 

He liked working on his own, doing everything himself, and not delegating tasks to others. So he purchased one of the earliest franchised drive-in fast food and ice cream restaurants, a “Twin Kiss.” Carole said that her parents “toiled away” from morning to night running the place on Bel Air Road. She worked there when she could, even while in medical school. When the restaurant shuttered for the winter, the family sold Christmas trees. Later on, after Carole finished medical school in 1968, her father turned the restaurant into a liquor store (that was sold shortly before he died of cancer).

Vintage photo of Ken and Jean Witmer's drive-in walk-up "Twin Kiss"
ice cream and sandwich restaurant in Palmyra, PA

Her father had told her (and Carole choked up as she recalled this for me) that when he stood on the corner of Monument and Broadway, the location of the iconic Johns Hopkins Hospital, selling newspapers, he never had a thought that his daughter would one day graduate from there. 

Dentist or Doctor?

Anyway, Carole’s interest in science, in how things worked, in how they were put together, was a driving force for her as she was in school.  But being a doctor was not her initial plan. You see, she had “terrible teeth” and was “always at the dentist’s” and she thought she would be a dentist. (Her family doctor, it turns out, wasn’t really the best role model; he smoked  cigarettes while he examined her, even though he had part of a lung removed to treat tuberculosis!)

So she carefully carved a bar of soap into teeth and took the dental aptitude test. She was all set. Until her father, who rarely offered advice, said, “I'd rather you be a doctor than a dentist.” She listened, bought a small anatomy book, and shared her new excitement with her mother.

While attending Kenwood High School she studied piano at Peabody Preparatory. She recalled that on the way to her lessons the bus passed by Hopkins and she saw the medical students “floating around” in their short white coats. She said that “there were sheep” just east of the hospital since Dr. Manfred Mayer needed their red blood cells for his work on complement (an essential part of the body’s complex defense mechanism, the immune system, a system that sometimes betrays us, as we will see later). 

Postcard depicting the Peabody Institute in Baltimore 

On to College

At Kenwood, she had a “wonderful teacher of American history” who had gone to Hopkins. He told her that if she was thinking of all-male (until 1970) Hopkins she should consider Goucher College in Towson. She did, and Carole, the budding scientist, entered the all-women’s school as a biology major but later switched to chemistry. 

She did a lot of lab stuff at Goucher. She especially enjoyed genetics and embryology, and as a member of the Biology Club, she went down to Hopkins to listen to Dr. Victor McKusick (the “father of medical genetics”) as he gave brilliant evening sessions. And as a (promising) “Goucher girl” she was picked to work with the “wonderful” gastroenterologist Dr. Frank Iber in his lab for two summers (while also doing her part at the family’s busy restaurant).

“The story of my life,” said Carole, ”is that I ran into a lot of really nice people who helped me along the way.” (She really did.)

Anyway, as she liked the basic science and the lab work, she wavered between going to graduate school for a Ph.D. and a career in medicine. Her “prim” unmarried chemistry advisor at Goucher was also the medical school advisor and after Carole decided on medicine she was given advice for her critical upcoming interviews. 

So, for the University of Pennsylvania grilling, she wore (wait for this...) a Jackie Kennedy-style pillbox hat and pristine white gloves. The session went well but, in time, the interviewer “was concentrated on what would happen” if she got married, was she worth it?  That is, would she practice medicine for only a few years and thereby waste a valuable space that could have been filled by a man with a longer career? 

January 21, 1961 (from theprojectsworld.com)

The admissions interview at Johns Hopkins was different. Dr. Tommy Turner, the Dean, let Carole know that (somehow) Dr. Iber had already told him “very nice things” about her. For the illustrious Dr. Turner, intelligence, brainpower, was the most important criterion for admission, not who you were, or what race, religion, or sex. Carole was accepted.

While still at Goucher she did carbohydrate studies “hydrolyzing mucopolysaccharides” with Suzanne Brownlee from Duke for her senior thesis. And with that connection, and her “travel lust,” she spent the summer before medical school on the Durham campus working with Dr. Bob Wheat. She was able to get, in 1965, her first scientific publication: the one-page work titled (Dr. Dorsch had to think for just a second) “The occurrence of pyruvic acid in the capsular polysaccharide of Klebsiella rhinoscleromatis.”  (More than 30 papers in peer-review journals would follow.) 

Dr. Dorsch's first published paper

Medical School

So Carole immersed herself in medicine at Hopkins in East Baltimore from 1964 to 1968; there were nine women in her class (compared to only one or two in the classes just before and after hers). She recalled this was during the height of the civil rights movement and she was a witness to the terribly destructive riots that followed Rev. Martin Luther King Jr.'s shocking assassination by James Earl Ray on April 4, 1968, at the Lorraine Motel in Memphis. 

(Later on, when Carole was in Memphis during one of her travels, she visited the iconic motel, once faded, and since 1991 reborn as “The National Civil Rights Museum.”)  

During the rioting mayhem, and at the request of then-Governor Spiro Agnew, there were National Guardsmen with rifles stationed every twenty feet along Broadway, she said. Carole was visiting friends on Monument Street and  watched in horror as “North Avenue was in flames.”  More than 10,000 troops were deployed and over a thousand fires were set.

Fire on Gay Street in Baltimore on April 6, 1968 (from the AP)

While in medical school, she continued to “do a lot of lab stuff” with the “connective tissue” division. She worked with organic solvents and (“brand new”) antinuclear antibodies and their so-called staining patterns for diagnosis). She presented a paper at a rheumatology meeting in Cincinnati in the middle of winter (after her first plane flight). When the effort from a mere student was criticized, she received moral support from the chair of the session, Dr. Eugene Barnett, from Rochester. 

She didn’t wither and continued to do basic research. One summer she worked with chicken embryos at the Carnegie Embryology Lab and another one of her projects involved trying to isolate basophils, the hard-to-find white blood cells associated with allergies. 

The Johns Hopkins medical school class of 1968
 (Young Carole is third from the left in the front row.)

Internship and Residency 

When it was time to choose a career path, Dr. Dorsch decided on Internal Medicine. She applied to Vanderbilt, Hopkins, and a few other places for her internship and one-year residency through the new match program, intended to pair applicants to hospital positions equitably. But the department chairman at Hopkins, Dr. McGehee (“Mack”) Harvey, called Carole himself to offer her a spot (outside of the match, maybe not quite Kosher) on the prestigious Osler Service.  She couldn’t say no. 

On Osler, you worked hard, often through the night and the next day, with only one day off a week. She said that you took every tenth admission and followed 10-15 patients in the hospital at one time. There was no ICU (imagine that!) and very limited ancillary help, so you did almost everything yourself.  You learned medicine by taking care of patients day after day.  

Intern presenting a patient during Grand Rounds in Hurd Hall Hopkins (c. 1950s)  

She had not decided what to do after completing the two years at Hopkins when she “ran into” the masterful clinician, teacher, mentor, and diagnostician Dr. Philip Tumulty in the school cafeteria.  After they talked about her career, he told Carole that, since she liked to experience different things and had thought about UCLA for a third year of internal medicine training, he would gladly make a call to one of his colleagues there to facilitate that.

Los Angeles and Back to Baltimore

During a break in her studies, Dr. Dorsch had decided to take her mother to Europe for three weeks. While waiting in the old BWI airport (Friendship International), she opened a letter from UCLA. They offered her a third-year postgraduate position.  She quickly raced around the terminal to find stationery to send the reply. So she went to California.

Her first specialty rotation in the sun was rheumatology.  She quite enjoyed solving the “puzzles” of the patients who had “connective tissue diseases” or, more accurately, autoimmune disorders like lupus or rheumatoid arthritis. Dr. Barnett (who, you may recall, had come to her rescue at the meeting noted above) had moved to UCLA from Rochester by then. He saw her talent and wanted her for his program. (The specialty of rheumatology was relatively new as there was no board certification until 1971 and no accredited fellowships until 1987.) 

"Mary Betty" (ACP)

Carole liked L.A., took the fellowship, and stayed on the West Coast for a total of four years. She was lured back to Baltimore in 1974 by (the imposing) Dr. Mary Betty Stevens as Dr. Stevens took over the running of the Connective Tissue division.  (She was the first woman to head a division of medicine at Hopkins.)

(Speaking of strong women, here's an interesting tidbit later shared with me by Dr. Dorsch. After the hospital was built from the large estate left for this purpose by merchant and banker Johns Hopkins, there weren't enough funds to build the medical school. Four daughters of the original trustees offered to raise the needed money, but only if the school admitted women. And so it did, when it opened in 1893. In fact, writer Gertrude Stein, who studied psychology with William James, was in the class of 1901. But she didn't receive a degree, possibly due, in part,  to William Osler's known tendency to misogyny--nobody's perfect.)  

The expanding group of rheumatologists was based at Good Samaritan Hospital, away from the main Hopkins campus, and Dr. Dorsch was to be the lab person on the small team. Among other things, she continued her important work on autoantibodies.

Antibodies and Immunity

A few words about antibodies and immunity are needed for our review. The exceedingly complex immune system (rivaling the complexity of the nervous system) evolved to protect us from injury. To heal wounds. To repel dangerous microbial invaders. To find and eliminate cells that have mutated and might grow into widely spreading cancers. To allow us to survive long enough to reproduce. 

But sometimes things go wrong. Microbes block our defenses or even hide deep inside our cells. Cancers figure out ways to actually turn off the immune attack. And sometimes the system misfires and mistakenly rebels against our own tissues (the self) as if they are foreign (the other).  The autoantibodies so produced are the cause of many diseases. This includes, for instance,  type 1 diabetes, the commonest thyroid problems, gluten sensitivity and celiac disease, multiple sclerosis, rheumatoid arthritis, psoriasis, and many other conditions, including the prototype autoimmune disease, systemic lupus erythematosis. Dr. Dorsch often took care of people (mostly young women, by far) who had or were suspected of having this complex autoimmune disorder. 

Lupus

Systemic lupus, by the way, was first clearly described by…(any guesses?)...none other than the aforementioned towering figure Sir William Osler, the Canadian general internist who became the father of modern American medicine. As the first physician-in-chief at Hopkins, he was the originator of bedside teaching of medical students, and the inventor of the house officer model of training.    

Osler contemplating a patient at Hopkins
(from National Library of Medicine)

So, let's take a short journey into the stuff of lupus. According to an article on Medscape, systemic lupus erythematosis is “a chronic inflammatory disease that can affect almost any organ system, although it mainly involves the skin, joints, kidneys, blood cells, and nervous system. Its presentation and course are highly variable, ranging from indolent to fulminant.” 

It may be notoriously difficult to pick up in the early stages since the symptoms may be very vague. And it can mimic other diseases, diseases that are organ-specific. So Carole, using the acquired Oslerian skills of active listening, careful observation, and meticulous examination, diagnosed and treated many patients (mostly women) that confounded the non-rheumatologist specialists. (And, thereby the saying: “If you don’t know what they have, send them to a rheumatologist.”)

In lupus, there is a loss of B-cell tolerance and recognition of self-antigens. The B cells (that we learned about early in the Covid-19 pandemic) originate in the bone marrow (hence, the “B” as opposed to "T" cells related to the thymus gland in the chest). They mature into plasma cells that produce antibodies. 

There is a slew (maybe more than 100!) of such antibodies against various cellular components (especially debris from degenerated cells) found in individuals with lupus in addition to the diagnostic double-stranded DNA antinuclear antibody. And these different antibodies may affect different tissues in different ways. 

(Antibodies directed against the NMDA glutamate receptors in the brain, for example, are involved in some of the neuropsychiatric syndromes reviewed in one of Dr. Dorsch's writings with the Hopkins group.)  

Trying to limit inflammation and damage with non-steroidal drugs and antimalarials and suppressing the immune system generally with steroids or chemotherapeutic medications were the only treatment options throughout Dr. Dorsch’s career as a physician. There were often troublesome side effects and many treatment failures. 

Targeted therapy for lupus became available in 2011 with the approval of belimumab (the first drug approved for lupus since hydroxychloroquine...in 1955!). This monoclonal antibody (“-mab”)  blocks the B-lymphocyte stimulating factor (BLyS) that increases the survival and growth of autoreactive B cells and decreases self-tolerance. It is helpful when other treatments have failed, but only to a degree.

Cartoon depicting the mechanism of belimumab (from ASCPT)

Back to Maryland for a While, Then Florida  

At Hopkins, Dr. Dorsch and her colleagues were productive, producing paper after paper while seeing patients and teaching students. But things were slowly changing (as they always do).  Carole applied for research grants to support the work, but funding was often lacking. Hopkins Rheumatology had joined up with the University of Maryland group. Dr. Harvey retired and Dr. McKusick took over as department head. The teaching became routine and uninspiring. And as Carole “did a lot of work without recognition” she looked to move on. 

One of her rheumatology friends from L.A. had a private practice in Pensacola and needed help. Dr. Dorsch liked the idea of doing her own thing, of being her own boss, and she left the Mid-Atlantic for the Florida Panhandle in 1982. She was busy, but as she had to take turns admitting general medical patients from the ER she was too busy. There were no students or residents, and she found that she sorely missed teaching. And she and her friend eventually “did not get along.” (Her father had warned her to “never to go into business with a friend.”) What would she do next?

From Florida to Pennsylvania

She was at an American College of Rheumatology meeting “somewhere” and was sitting at a bar with a colleague she knew from the joint program with the University of Maryland, Dr. J. Wolfe Blotzer. It just happened that Wolfe was looking for someone to join his practice at the York Hospital. There would be residents and students to teach, and she would do only rheumatology. It sounded better than what she was doing with her friend. So Carole came to South Central Pennsylvania in 1988 for career #3.

By the early 1990s, their private practice had grown to the point that they needed another physician. But financial constraints made it difficult to recruit someone. And the business of medicine, overall, was changing, as hospitals started acquiring practices and employing physicians. 

The York Hospital’s first hired physician was Dr. Francine Camitta, an endocrinologist and diabetologist. Dr. Dorsch was the second (she was naive and simply handed the hospital her practice). They moved Carole around a few times, but she enjoyed the patients, the teaching, and the camaraderie of fellow physicians, and she was happy for a good while.      

But nothing stays the same, and as the “medical group” of primary and specialty practices became larger and larger there were more and more rules and regulations. Office managers were put in place to make sure the dictates were followed, however arbitrary. While some managers were “wonderful,” others were not so much, and could even hurt the practice. 

In addition, the hiring of hospitalists meant that Carole and her partners would stop admitting their patients to the hospital and would focus on outpatient work. Eventually, she lost contact with other doctors and felt “lonely and isolated,” she told me. On a whim, she attended a course in Philadelphia where they talked about other things doctors could do besides seeing patients.

After Years of Practice in York, A Rewarding Teaching Career

Soon after, an ad in the local paper caught her attention: the Harrisburg Area Community College (HACC) was looking for adjunct faculty to teach science to nursing students in Lancaster. She taught her first summer class in anatomy and physiology in 2002 and kept going. She was still in practice then and as she finished in the office and ran to evening sessions twice a week during the year, she “loved it.”   

Harrisburg Area Community College, York campus (from HACC)

A HACC York campus was set up in 2005 and Dr. Carole Dorsch was hired as the first faculty member. Over time, the staff grew “tremendously.” Teaching, she said, gave her a chance to “re-learn basic biology,” to, for example, learn about cell membranes and transport systems, to learn how things work. She was “quite happy” at HACC. She taught full-time for 11 years, for a total of 14 years with the community college. When she finally left the school in 2018 it, too, had changed.

In Retirement

In her retirement, as she looked forward to more travel, Dr. Dorsch has had a battle with her own immune system. Years ago, she was found to have what’s called a monoclonal gammopathy of uncertain significance (MGUS). A clone of one of her B cells was producing a single useless immune globulin in excessive amounts. There were no symptoms, but Dr. Dorsch knew that this could turn into a more serious bone marrow disorder, multiple myeloma, a cancer of the antibody-producing plasma cells. And so it did, not too long ago. The therapy for this has been complicated by neuropathy with numbness of her feet and weakness of her legs. She is improving slowly.

Outside of medicine, Dr. Dorsch especially enjoys travel and seeing something new. She likes to read, of course, but she said that she also likes to cook and has “more cookbooks than medical books.” A recent interest is that of piecing together jigsaw puzzles. She discovered this while sitting patiently in the courthouse waiting to be called for jury duty; she was looking for something to occupy her ever-curious mind when she found a puzzle on a small table. 

And, after four varied careers in medicine, mindfully putting the pieces of the cardboard puzzle together, and finding where they fit, where they belonged, was oddly satisfying. And it mirrored exactly what she did for her patients as a specialist in complicated immunologic rheumatologic diseases. 

And it is hoped that as her plasma cell cancer (that she strongly suspects was the result of work with organic solvents years ago) is kept in check she will be able to continue her travels, either just down the road or much farther away.

References and Readings:

1. Bartels, Christie. MD, MS. "Systemic Lupus Erythematosis." Medscape Nov 11, 2022. 

2. Fineglass, E.J., Arnett, F.C., Dorsch, C.A., Zizic, T.M., and Stevens, M.B.  "Neuropsychiatric manifestations of systemic lupus erythematosis, clinical spectrum, and relationship to other feature of the disease." Medicine (Baltimore)  1976, 55: 323-339. (My husband said he relied on this article when he was a medical resident seeing patients with lupus.)

3. Richtel, Matt. An Elegant Defense: The extraordinary new science of the immune system; A tale told in four lives. Harper Collins/William Morrow, New York, 2019. (Popular story-telling account of immunity; an enjoyable easy but informative read.)

By SC

Anita Cherry 4/30/23

Dr. Lynn Jensen: ER physician who aimed to hit the right notes

 

Dr. Lynn Jensen

Nearly two years after I asked him for his story, Dr. Lynn Jensen decided that it was time. But his wife, Dr. Leslie Robinson, who relayed the information to me, said that I had to wait until after archery season.  Archery season? I imagined him aiming at a big plump yellow, red, and blue target set up on a tripod as I recall from summer camp (where the flicked bowstring really hurt my forearm). But that was not the archery Lynn had in mind. 

You see, he was going to traipse into the woods, climb up a tree into something called a stand, and use a bow and arrow (yes, an actual bow and arrow!) to hunt for deer. If successful, he would drag the poor dead animal out of the forest and butcher it himself, to be safely stored away for the winter. Have you ever heard such mishegas, such craziness?

Anyway, the hunt being finished, Dr. Jensen finally came by to talk. He started off by telling me that he went to medical school at the University of Michigan and that this is where he and Lesie got together. (Though they were quietly attracted to each other when Lynn was funding his education by working as a painter and was doing Leslie’s parents’ house). 

Sprawling University of Michigan Medical Campus
(From The University of Michigan)

He left Ann Arbor in June of 1975 and promptly began his one-year so-called rotating internship at the York Hospital in July. His initial assignment was the emergency room. And on day one, as the seasoned staff physicians left for home at eleven, Dr. Jensen was the only doctor in the ER until the morning. Fortunately, he noted, he was supported through the night by experienced and compassionate nurses. And he was wise enough and humble enough to listen as they gently guided him. 

Before his time, he said, the nurses would see patients at night without waking, without bothering, the doctor, if they felt they could handle things on their own. They would then have the attending physician sign the stack of charts first thing in the morning.

Lynn admitted that “it was trial by fire.”  An intern in the ER by himself in the late 1970s had to be able to pick up things quickly, to be able to learn on the job, and act confidently without the help of the wide variety of rapid blood test results and advanced imaging studies now available. He said that you had to “use your brain” to make sound clinical judgments. For example, when someone came in with chest pain or belly pain you had to think systematically to generate a list of the most likely diagnoses while making sure not to miss something really serious, maybe life-threatening, even though much less probable. 

Cartoon noting the many causes of chest pain
(From artibiotics by Ciléin Kearns)

Young Lynn found it somewhat surprising that he quite liked the experience. That this type of acute medicine suited him. That it fit his learning style and personality. He said that “when you know you're going to be there (in the ER) by yourself you have a tendency to prepare that you would not have if you were being babysat.” 

In fact, there was no recognized specialty of emergency medicine itself until 1979. Any physician could work in the ER, even residents who needed extra cash.  So, after only a year of formal postgraduate training, Lynn decided to do emergency work full-time. He enjoyed the challenge and he could make enough money to allow Leslie to attend medical school herself (at Hershey, just 40 miles away) without incurring debt.  

He found the sometimes hectic ER work stimulating and rewarding, and he “sat for” and (of course) passed the oral and written Emergency Medicine Board exams. And he was active in the residency teaching program championed and developed by Dr. David Eitel, said Lynn. So, practicing acute medicine in the ER “just kind of stuck for a thirty-five-year career.” 

Dr. Jensen especially liked the demand of trying to meet each person’s specific needs even though he would likely see them only once and not have an ongoing relationship. He appreciated the importance of  “critical thinking” and informed decision-making when dealing with urgent (or even not-so-urgent) clinical problems; often with incomplete or misleading information, and the ever-constant pressure of limited time. 

According to ER physician and writer Dr. Jay Baruch, “to be an emergency room doctor is to be a professional listener to stories. Each patient presents a story; finding the heart of that story is the doctor’s most critical task. More technology, more tests, and more data won’t work if doctors get the story wrong.”

Doctor Who: "Listen" (From the BBC)
(The episode was "a creepy study of fear and loneliness")

So Lynn dutifully practiced his craft and tried to get the stories right as he took care of his patients, quickly establishing a working diagnosis while calming their anxieties and allaying their fears whenever possible. 

He told me that the first twenty years at the York Hospital were good and that he was happy. But things began to slowly change. He lamented, most especially, that the practice of medicine gradually became more and more influenced by corporate bureaucratic interests. And, importantly for the day-to-day practice of physicians like Dr. Jensen, the electronic health record (the EHR) was born.

Sure, paper charts, when they could be located in a timely manner and retrieved from the records room or one of the floors and brought to the emergency department (no longer just a “room”) had their problems. The handwritten progress notes were occasionally nearly illegible, and sometimes several thick volumes with page after page had to be combed through to find what you needed. But dealing with paper was simply the way it was, and it was okay. 

The now-obsolete but familiar paper charts
(From shorelinerecordsmanagement.com)

The first EHRs arrived with the promise of better efficiency and improved accuracy. But they were awkward and seemed to be designed by individuals not familiar with actual patient care. Oddly, the ED, where time may be critical, was chosen as the guinea pig for the new system at the hospital. Dr. Jensen found the chosen version to be needlessly cumbersome and intrusive, and frustratingly time-consuming. Over time, he began to feel that he was practicing medicine while wearing handcuffs. The electronic record was clearly built, he said, for something other than taking care of sick people.

And he gradually realized that the important diagnostic and interpersonal skills he had acquired by talking with and meticulously examining thousands of worried patients were becoming less valued than the results of increasingly sophisticated, but often superfluous, imaging and other tests. Seeing disembodied images became believing; the cold objective numbers from the lab told the tale. No need to listen to, or touch, the patient. This was very disheartening for Dr. Jensen to witness.

And as the ED became more and more crowded, more overloaded, Lynn eventually became tired of coming into work in the mornings and apologizing to patients who were being kept overnight in make-shift beds in the hallways.  Having to apologize for something that was not his doing.

The Maimonides ER after Hurricane Sandy 
(From The New York Times)

Regarding this very common scenario, Dr. Jay Baruch notes that:

Crowding and prolonged waits in the ER are more than an inconvenience; they’re linked to grave medical consequences, including higher inpatient mortality, longer length of stay in the hospital, increased medical errors, more harmful cardiac outcomes, and delayed treatment for pain. (p. 108)

So, at some point, Lynn decided that he had enough. And Dr.  Baruch has this to say about "burnout," or moral injury and disengagement: 

Many influences contribute to burnout in medicine. They include lack of control over the work environment, a disparity between personal values and those of the system, more time spent with electronic health records then with patients, and a sense of not making a difference. (p. 102)

We can readily see some of these factors playing a role in Lynn’s story and his decision to retire early. So he left clinical medicine at 62. Since then, he has had dreams of being back at the hospital. In some of these dreams, he’s frustrated as he deals with the cumbersome EHR. In others, everything ”goes to hell” and he has to reassure his beleaguered coworkers and nursing staff that they are just in his dream and that things will be okay. 

And in these lucid or sentient dreams, as he is aware that he is dreaming, he has sometimes wondered why he even agreed to take the shift in the first place, knowing that he no longer had privileges at the hospital and was not allowed to see patients at all! Lynn said that, ironically, he did not recall dreaming about the ED when he was actually working there.

The pattern of 40 Hz brain activity in lucid dreaming 
(From "New Scientist)"

Dr. Jensen told me that he would have probably continued to practice to age 70 if he felt he was still able to deliver what he considered to be high-level care. And he said that as he got older he cared more. Anyway, he did a few years of didactic teaching after retiring from hands-on work but decided to give that up as well when he sensed that he no longer had “credibility,” when nobody knew him anymore. 

Reflecting on his experience, he said that while manning the ED with talented colleagues suited him well overall he would not make the same career choice now. What would he have done instead? He thought a moment and offered that he might have gone into ophthalmology.

This brings up the question of how Lynn decided to go into the medical field in the first place. Nobody in his family was in medicine. His father was a Presbyterian minister in Valley Forge and his mother had a degree in home economics. Both of his parents were strong advocates for social justice and international peace. So Lynn was raised in a culture in which getting an education was important, and providing service to people, providing something of real value to others, was expected. And Lynn thought that he should also be a good provider for his family.  

Valley Forge Presbyterian Church, King of Prussia in 1956
(From the Presbyterian Historical Society)

He was certainly smart enough, as he was the first student from his high school, Upper Merion, to attend Harvard, and a career in medicine as a way to help people was an obvious good choice in the idealistic late 1960s. Becoming a professional poker player, to take a trivial example of another option, would not be an acceptable career choice (though he admitted that enjoys the game) since it gives nothing to others, he said, apart from a bit of light entertainment. 

(Lynn comes from a family of four and his older brother chose the same path and is a primary care doctor.)

So Lynn and his wife, OB/GYN-turned-urogynecologist Leslie Robinson (her story can be found here), were able to successfully navigate two active medical careers while raising their two children (Annie is a pediatric ophthalmologist and Dane is a clinical psychologist). And they brought two complementary styles to the task, noted Lynn. How so? For example, take the simple task of packing a dishwasher as an insight into people. Dr. Jensen said that he will carefully read the directions and make sure that everything is fitted into its proper place to avoid “shadowing.”  Leslie, on the other hand, will just cram in everything she can, filling every possible crevice until there’s no room for anything else. (What does your dishwasher style say about you?)

"First and foremost, load the dishes with the dirty side down and
at an angle toward the center of the dishwasher; don't overload it."
(From WikiHow)

And in retirement, Leslie (in line with the above) fits more into a day than anyone Lynn knows; he can’t keep up with her, he said, though he is busy, too.  He hunts for deer (when in season), plays pickleball (having given up the more strenuous racket sports), does close sleight-of-hand card magic (no self-working tricks for him), reads critically (non-fiction), and keeps up a daily routine of practicing the piano (especially enjoying the classical works of Schubert and the modern difficult 12-tone compositions by Samuel Barber). Yes, Dr. Jensen still seems to like doing stuff that requires lots of patience and focused hard work.

Well, after the pleasant ninety-minute interview came to a close my husband walked Lynn back to his car, at which point Dr. Jensen admitted that maybe he was getting a bit too old to be deep in the Pennsylvania woods by himself strapped high in a tree in chilly November waiting for a suitable target for his sharpened arrows. But maybe not.

Archer Dr. Lynn Jensen perched securely and scanning for prey.
(How did he get up there? And how will he get down?
And who took this photo?)

Readings and references:

1. Baruch, Jay. Tornado of Life: A Doctor's Journey Through Constraints and Creativity in the ER. MIT Press. Cambridge, Massachusetts, 2022. (A very candid series of short takes on ER work.) 

2. Ceresi, David. "Aim for safety in your tree stand." Mayo Clinic Health System Oct 7, 2022 (3,000-4,000 hunters are injured each year in the US falling from tree stands; Dr. Cresi explains how to prevent this.)

3. Talbot, Simon G. and Dean, Wendy. "Physicians aren't 'burning out.' They're suffering from moral injury." STAT+. July 26, 2018. (Moral injury involves knowing what care patients need but being unable to provide it due to constraints beyond one's control.) 

One of my paintings from the 90s

By Anita Cherry 2/17/23

Dr. Kenneth Brein: Seeing More Clearly

Kenneth Brein, M.D.

Ken’s mother helped support the family with a thrift shop in Germantown. Her youngest son had an ear for music, so when she acquired a slightly-cracked Gibson guitar for twenty-five dollars one day, she lovingly handed it to him. He was ten years old. He practiced. And practiced. And by the time he was in the seventh grade his band, “Celestial Fire,” was performing somewhere every weekend. It was the early ‘70s and they played “everything you heard at Woodstock,” said Ken. It was his life. That is, his life apart from his early interest in science, math, and physics. Yes, Dr. Kenneth Brein confided to me that he was “the nerd” in the family. But he was a nerd with a guitar.

He did well at Harriton High in Lower Merion just outside of Philadelphia, and he thought he’d be a scientist. His father (who, said Ken, joined the Army after graduating from Overbrook High but then struggled in a variety of businesses) recognized his third son’s potential. His often-whispered advice to Ken? “Be a doctor, be a doctor...”

But when Ken finished high school in 1974 near the top of his class of 250 his plan was to study science and engineering, not medicine. He applied to four elite schools and when he visited Princeton’s 500-acre bucolic campus it was “a gorgeous day in April.” When he toured Penn in West Philadelphia, it was a gloomy rainy day. So, Princeton it would be. 

Princeton University in the spring (from the Fulbright Commission)

PRINCETON AND PENN

While in central New Jersey, Ken refined his interests and decided to study biomedical engineering. But the course offerings were limited. He took whatever he could, and for his senior thesis he wrote the complex math for a theoretical model of blood flow  (a fluid, but with cells floating in it) through the small tubes of an artificial lung. Ken was (once again) advised to go to medical school. 

So he applied to a number of medical programs, received acceptances, and chose Penn (despite his weak first impression of the area). He enjoyed learning biology and doing medicine but seemed to lose interest in research. And as he progressed through the various rotations, he didn’t develop a feeling for a particular specialty. But one day, he was given a nudge. This happened when he was visiting his mother and her second husband George at their apartment on leafy Rittenhouse Square in downtown Philadelphia.

WHY OPHTHALMOLOGY

Dr. Zubrow

His mother’s personal physician and good friend, the highly-esteemed cardiologist Dr. Sidney Zubrow (who lived in her building) called her. He said that he wanted to see Ken. Dr. Zubrow, said Dr. Brein, was “the old-fashioned internist” who knew everything about his patients. The wise older clinician gently asked the young student how things were going in medical school. He asked him what he was considering doing in his career.

Ken thought for a bit. Maybe he’d go into Hematology-Oncology, he said, having recently done an intense rotation in that.

“Try something else,” said Dr. Zubrow flatly.

“Maybe Cardiology?” (Surely, Dr. Zubrow would be happy with that answer, thought Ken.)

“Try something else,” was the cryptic response.

“Maybe Infectious Disease?”

“Try something else,” came the reply once more.

By that point, the perceptive medical student began to catch on.

“Oh. Well…ophthalmology looks pretty good,” offered Ken.

“That’s a good one,” said Dr. Zubrow.

“Maybe Dermatology?“ Ken guessed as he was guided along.

“That’s a good one.”

The attentive pupil was “getting the message, loud and clear.” And when the time came to decide on the next phase of his training, a residency, Ken chose to study the many diseases of the eye. He would have loved to have gone to the Will's Eye in Philly but was content to be accepted into the ophthalmology program at the University of Pittsburgh. So, after medical school, and a year of internship at Pennsylvania Hospital (where a portrait of Dr. Zubrow is prominently displayed) he and Jessica took the turnpike (called “America’s First Superhighway” when it opened in 1940) 300 miles west. Wait a minute! Did I forget something? Who’s this Jessica? 

UPMC Presbyterian Complex in Pittsburgh (from UPMC)

HOW KEN MET HIS FUTURE MATE

Well, during the Christmas break the first year of medical school Ken took the train to Manhattan to visit one of his college roommates. When it was time to head back to Philadelphia he boarded at the busy Penn Station. He realized that he forgot to bring a book or a newspaper to keep him occupied for the hour-plus trip. What was he going to do? As he made his way down the aisle of the crowded car he spotted an empty seat next to a pretty girl. Somehow, he had the intuition that she was from Long Island and returning to Penn (no, she was not wearing the U of P red and blue). He thought they might hit it off.

So he sat down next to her, but she made an expression like ”you're invading my space.” He would have to play his cards right. So Ken, trying to be optimistic and trying to impress, reeled off his credentials (you see, he didn’t have a guitar with him). After chatting for a while, he asked her to dinner. But Amy (that was her name) was on her way to Penn to see her (quick stab in the heart) boyfriend. However, “I have a sister you might like,” she said. So she gave Ken Jessica’s phone number. What did he have to lose? He decided to give her a cautious call and soon wined and dined her, taking her to the fancy “La Terrasse” French bistro on the Penn campus. I guess it worked out, as they married and have three adult children and six grandchildren. 

Watercolor of the well-known Locust Walk at Penn (by elliemoniz.com)

AFTER RESIDENCY, WHERE TO PRACTICE?

Though Jessica would have been happy to stay in Pittsburgh after Ken finished his three-year residency, and he was offered an opportunity there, he wanted to be closer to the Jersey shore, to tiny Ventnor City, a two-square-mile vacation spot of many soothing childhood memories. So as Ken searched for a place to practice through print ads in the journals, he learned that Dr. Charles Letocha (also a product of Princeton and Penn) in York was looking for a partner. After a series of letters (yes, actual handwritten letters) back and forth, careful Chuck finally decided to meet Ken (and Jessica). They clicked right away, and Ken moved to York and joined the private practice in 1986. 

As a clinical ophthalmologist, Dr. Brein focused his energies on perfecting his skill in cataract surgery, addressing the clouding of the lens in the eye that is the cause of half of the world’s blindness. But a type of blindness that can almost always be cured.  

CATARACTS

Let’s talk about the cataract. The clear crystalline lens focuses incoming light to the fovea at the back of the eye, the area of the retina with the highest density of light-sensitive cones serving color vision. With age, (and UV light exposure) the proteins in the lens change and coalesce and the lens becomes progressively opacified, letting less and less light pass through. Though attempts to stick a sharp needle into the eye (ouch!) to push the clouded lens out of way go back as far as the fifth century BCE in India, the first “modern” cataract surgery was performed by a French surgeon in 1747. Serious complications were not unusual, and vision after the procedure was poor; thick heavy “Coke bottle” glasses were needed to see clearly in the absence of a lens. 

So physicians used to wait until there was a nearly complete loss of vision before removing the severely opaque lens. But advances in surgical techniques eventually made the procedure much safer. And Dr. Brein said that cataract surgery was revolutionized by Dr. Charles Kelman in 1967.  He devised a procedure termed phacoemulsification. With this, a thin ultrasound-driven needle inserted through a tiny incision breaks up then and then sucks it out. This is a very safe technique. It is the standard now, though it took a while to catch on (like most bold innovations in medicine). 

The development of the plastic artificial lens in 1974 by Dr. Harold Ridley in London to replace the worn-out body part provided a much more satisfying vision outcome for patients. And when the foldable plastic lens was invented in 1980, the incision required to insert it became even smaller and less traumatic.

Phacoemulsification and intraocular lens implant (artwork by Christine Cote)

For experienced surgeons, like Dr. Brein and his partners, cataract surgery became extremely controlled and consistent. And it is claimed that nearly 98% of patients can expect a successful outcome, a truly remarkable statistic for any surgical procedure. Complex lenses can now correct a variety of refractive errors, like astigmatism, etc. Over his satisfying 31 years in practice, Dr. Brein was able to deliver better sight to many thousands of individuals in York. 

HE HAD TO STOP DOING SURGERY

But Dr. Brein had to stop performing the extremely delicate surgery after he developed nerve injuries affecting the strength and coordination of his right hand. He underwent surgery for median and ulnar entrapment, but recovery of dexterity was slow and he did not want to expose his patients to the risk of a slip-up (fear of such things during his practice would sometimes keep him up at night) so he put away his scalpel. He still saw patients for refractions and for the diagnosis of the many ailments that can affect the eye, but he could no longer offer them his surgical expertise. He was okay with that, but then something else happened.       

ANOTHER BLOW

This next part of Ken’s story is particularly difficult to take in. And it was painful for me to hear. It happened five years ago. 

It was a routine day at his office. For a few weeks, Ken had been experiencing what he thought was right-sided tooth pain spreading into his cheek. He did what many doctors do (and shouldn’t): he diagnosed himself. He assumed that he had developed maxillary sinusitis. But when Ken spoke with his wife from the office he sounded confused. It was clear to her that something serious was wrong. Jessica told him in no uncertain terms to stay put. That she was calling for emergency medical services. She then spoke with one of his partners, Dr. Matt Bilder, and said, “Make sure he gets into the ambulance.”

A head CT scan in the York Hospital ER showed that Ken had a right frontal lobe mass, an angry-looking mass compressing and distorting the brain. There was swelling and an ominous “midline shift.” He had to be admitted to the ICU to be monitored and watched closely. Later that night, he was wheeled away for an MRI.

Well, it turns out that Dr. Brein had created his Wellspan Health account a week before. With this part of the electronic health record, a patient can use their laptop or their phone to view their test results, any time of the day or night, wherever they are, even when they are in the hospital. So, lying in the ICU, with several IVs dripping and devices beeping and flashing and keeping time, alone in the darkness, he picked up his iPhone and cautiously opened his portal. There was a message: “You have a new test result.” The report of the MRI was already posted!  Ken paused briefly, then opened the document. The radiologist felt that the mass was a tumor, “most likely a glioblastoma.” Ken, was very aware of the terribly grim statistics for survival after the diagnosis of a GBM and thought, “I’m dead.”

Images of a frontal GBM with a midline shift (from David C. Preston)

“How were you able to deal with this?” I asked.

Ken waited for a moment, and then replied: “I have not said this before, but I think I went into denial.” 

You see, when he was an intern in Center City Philadelphia he had taken care of a woman dying of this rapidly progressive untreatable tumor. So while he knew “intellectually” that he had received a death sentence he quickly determined that this harsh decree would not cripple him emotionally. 

“Do you think you should have been allowed to view that MRI report, to be faced with such a grim diagnosis when you were alone?” I asked (but already knew the answer).

“No. I don’t think I should have been put in the position to see the test results at that point,” he answered. Sure, one of Ken’s doctors did come in the following morning to talk about his MRI and to explain things, but the psychic punch of facing imminent mortality had already landed; he said his “whole world was turned upside down” in that instant. 

He recalled that early in the AIDS pandemic when there was no cure (there still isn't), and no effective treatment, the results of the testing for the HIV virus were to be given to the patient only with the help of a counselor. “That would have been helpful for me,” he said.

So, as a student of science and mathematics, Ken looked carefully at the dismal survival curves. While almost all patients died within two years (many in the first year), there was a tail of the curve to the right, an exceedingly small tail, but a tail nonetheless. In fact, it has been reported that about 5% of adults with a GBM survive five years and nearly 2% make it to ten years. He could, statistically, find himself in those tiny groups (higher physics and mathematics informed him that even exceedingly unlikely things do eventually happen).  

GBM survival curves with different dosing of Temodar
(from Bin Huang 2021)

FOLLOWING THE DIAGNOSIS OF THE BRAIN TUMOR

After standard treatment with radiation and chemotherapy with Temodar, Ken’s oncologist in York found an ongoing trial of immunotherapy at Duke that appeared promising. Fortunately, Ken met the very strict criteria for the study. In that, they produced a personalized vaccine by harvesting some of his own white blood cells and exposing them to a specific antigen found in GBM tumors but not in normal brain tissue. They then injected these reactive cells into Ken’s thigh so they could find their way to the lymph nodes in his groin. Once there, they could generate T-cells to attack and destroy tumor cells carrying the targeted antigen. It seemed like a long shot, but it made sense to Ken to try; he had nothing to lose and everything to gain.  (Two reported studies from Duke were very small, but 4 of 11 patients in one group and 2 of 6 in the other were alive at five years compared to 0 of 23 historical controls.)

Five years on, and after regular trips to Durham, and a bunch of noisy MRIs Dr. Brein, remarkably, has no evidence of recurrent disease. There is no talk of cure or even remission, he said, just no visible disease at this time. The “sword of Damocles” is still dangling precariously over his head, he noted. 

Dr. Ken Brein chanting Torah on Yom Kippur 2022
 By Jewish tradition, it is on this holiest of days that God decides
 each person's fate for the coming year, who will live and who will die.
(Rabbi Marshall Klaven, Jessica, and daughter Courtney are looking on.)

WISDOM GLEANED

“Have you changed over the past five years?” I asked (again, knowing the answer).

“ How can you not?” he replied.

Early on in his journey, he was given the simple advice to “make every day count.” At the end of each day, he and Jessica ask each other these few questions: Did we do something good today? Did we have fun today? So Ken tries to do what he enjoys. And he believes that his positive, attitude allows those around him to feel better, as well. For example, Ken has had the opportunity to meet with others coping with brain tumors like his. He discovered, somewhat to his surprise, that he could connect easily with them and that he could be compassionate in light of their shared experience. And he often imparts the wise counsel he received after his diagnosis. This has been rewarding. (Ken’s father, having lost a leg in an auto accident, helped console Veterans who had similar traumas.)

THE MUSIC

As Ken tries to follow his own guidance, (forced) retirement has had a “silver lining,” he said. It has allowed him to pursue his life-long interest in music in more depth. When Ken began playing the guitar he followed the popular rock and roll track. This served him well for many years. But when he, by chance, listened to a jazz album one day not too long ago he was struck by the colorful sound. There were different chords and chord progressions and complex harmonies. And there was an emotional element that intrigued him.

So he studied music theory; that there are specific patterns of sound frequency and rhythm that are especially pleasing to us, and moving. He studied the mathematics and the physics that result in, literally, “music to our ears.” 

(My husband reminded me that there is no music or sound "out there," and no color. These are the private experiences the brain produces as it processes different wavelengths of energy detected by various sense organs. The tree that falls in the forest does not make a sound if is not perceived, it just disturbs the air.  And he reminded me of Duke Ellington’s famous comment about music; "If it sounds good, it is good.") 

Ken became serious about this and studied jazz guitar technique and theory (mostly online) with master guitarist and teacher Martin Taylor.

Music theory: The complicated (mathematical) circle of Fifths

Jazz music originated in the African-American communities of New Orleans in the late 19th and early 20th centuries. It evolved from Blues and Ragtime.  Jazz (according to masterclass.com)  “rarely uses three-note triads that define pop, country, and folk music. Nearly all jazz chords feature the seventh chord tone, and many include tensions like ninths, elevenths, and thirteenths.” And (importantly) improvisation (promoting creativity and flexibility) is an...element that “unites nearly all forms of jazz.”  

Ken is grateful to have been part of York’s “Unforgettable Big Band” for the past five years as they perform swing and classic big band music for appreciative audiences. He said that he has learned a lot from these talented and  “professional musicians.”  But he has been toying with the idea of playing solo jazz guitar in coffee house-type venues. And he might even try his hand at the intricate fingerwork of classical guitar.

AND MORE

Not content with sixteenth notes and syncopated rhythms and the physics of scales and harmonics, another one of Dr. Brein’s current interests is (wait for this…) cosmology, the study of the origin and development (and eventual running down) of the entire universe. Modern cosmology builds on insights about the curved fabric of space-time and elusive gravitational waves envisioned by Albert Einstein (a resident of Princeton in his later years). Remarkable insights that revolutionized our concepts about the nature of reality. (This, alone, should be enough to keep Ken plenty busy.)

And when he’s not with Jessica, or the rest of his family, or with his bands, or playing golf (where, he noted, you “keep your own score”), or fooling with one of his many guitars (including his first one), or reading stuff by Brian Greene or other cosmologists, you might find Dr. Kenneth Brein carefully covered up on the beach in Ventnor enjoying the peacefulness of the rolling ocean waves and the gently setting sun, the celestial fire sustaining life on Earth.

The quiet, soft, Ventnor City beach (from Shawn R. Smith)

References and Suggested Readings:

1.   MasterClass. "What is Jazz: A Guide to the History and Sound of Jazz." accessed at https://www.masterclass.com/articles/what-is-jazz (A succinct overview; but a start.)

 2.  Batich, Kristen, et. al. “Once, Twice, Three Times a Finding: Reproducibility of Dendritic Cell Vaccine Trials Targeting Cytomegalovirus in Glioblastoma." Clinical Cancer Research 2020 Oct 15;26(20):5297-530. (A report of the encouraging, though limited, experience at Duke, one of a number of centers looking for a way to shift the survival curve in GBM to the right.)

3.  Greene, Brian. Until the End of Time: Mind, Matter, and Our Search for Meaning in an Evolving Universe. Alfred A. Knopf. New York, 2020. (Not just physics and math, but a far-ranging erudite exploration of what it means to be human and concluding that "in our quest to fathom the human condition, the only direction to look is inward." p. 326)

4.  Davis, Geetha. "The Evolution of Cataract Surgery." Missouri Medicine 113(1): 58-62 2016.

By Anita Cherry 12/16/22

 volume21, Article number: 424 (2021)