If you were an M.D. practicing in Boston or New York in the '20s and '30s -- especially if you had an interest in metabolic disease and kept up to date with your field -- chances are that you would have been familiar with (astounded, perplexed, what have you) the case of Charles Martell: The Incredible Shrinking Captain. Today, his story is little more than a footnote in the annals of endocrine surgery -- someone to name-check if you, say, publish a paper on your experience in removing out-of-place parathyroid glands.
This is a pity. The story of the Merchant Navy Captain, who shrank seven inches as his bones literally turned to dust, deserves far wider recognition. First, it has all the trappings of a Shakespearean tragedy. As the medical establishment failed to make the final breakthrough, it was the captain himself who figured it out. But by the time he cracked his own case (by telling his surgeons to crack his breastbone open) it was too late. Because of this, Martell's story touches on something that is hotly debated today: patient engagement. Should we allow the patient to become a co-investigator, and grant her full and direct access to her electronic health record with test results, radiology reports, and doctors' notes?
With the passing of the Health Insurance Portability and Accountability Act (HIPAA for short) in 1996, patients were given the right to access (though, crucially, not directly) their health records. The rationale, as the Department of US Health & Human Services explains, is simple: [p]roviding individuals with easy access to their health information empowers them to be more in control of decisions regarding their health and well-being."
HIPAA was written with old-fashioned paper charts in mind -- charts that needed to be retrieved from archives, photocopied, and mailed by clerical staff. Because of this, clinics and hospitals are allowed a processing period of 30 days. Today, this provision should be moot. Electronic health records (EHRs) are ubiquitous, and with the push toward interoperability, that is, EHRs from different organizations being able to communicate with each other, the patient's complete chart could be made available with the click of a mouse. There are no technical or legal challenges.
And yet, there is push-back from some health care professionals who worry about delays and disruptions in their workflows if they give patients direct access to their notes. Instead of cutting to the chase and document their findings and assessments point blank, they fear that they will have to spend time thinking about their tone of voice and not offending the patient.
There is also a media narrative that plays into this; I am thinking of the various permutations of The Dangers of Using Google to Self-Diagnose articles. But for everyone who's convinced that the MMR jab gave their kid autism, there might be a patient who does her research not on fringe websites and support groups but in peer-reviewed publications -- someone who pores over their medical chart and picks up on something the doctor might have missed. Even if the former cohort outnumbers the latter 10:1 we still need a counter narrative, which is why I am enlisting the help of my friend Captain Martell.
His story can be read today as an appeal to open access, something that only 16.7 million patients worldwide currently enjoy, according to data from the OpenNotes project.
Not only did Martell "go online" (avant la lettre so to speak; he spent his final months in the Harvard Medical Library) he was eventually proven right and, in the process, helped advance our knowledge of parathyroid glands. So, take it away, Captain!
From Hunk to Hunchback
Born in Somerville, Massachusetts, Charles Martell entered the Massachusetts Nautical School in 1914 and graduated two years later at the top of his class. He saw action in the First World War as navigating officer on the U. S. Army Transport Shoshone (originally a German vessel, she was apparently seized in 1917; c'est la guerre!) After the Armistice, Martell served in the Merchant Marine. He had suffered from back pain for a few years, but this was hardly unusual for a sailor at a time when occupational safety and ergonomics were not top concerns. But by 1919 other worrying symptoms kicked in. "[H]is fellow officers noticed that he was growing shorter and becoming pigeon-breasted," as Dr. Eugene DuBois put it in his 1929 case report.
As Martell lost his stature, his back pain soon spread to his legs. While climbing a ladder, his knee gave way and he fractured something. When the ship came to New York a month later, he was taken to the U. S. Marine Hospital on Staten Island. "X-rays were taken. Little was accomplished." Not many weeks went by before his other knee collapsed. "One certainly gets the impression that something was fundamentally wrong with the structure of the Captains'[sic] under-pinnings," Fuller Albright, who would go on to treat him at Massachusetts General Hospital, chuckled in 1949. But perhaps not a laughing matter as far as Captain Martell was concerned.
He was taken to the Methodist Episcopal Hospital in New York where they patched his knee up. In the next few years, as he was crisscrossing the world, Martell fractured several bones. He was diagnosed with "arthritis of the mixed perisynovial and hypertrophic type," which, at the time, was physician longhand for "we have no f*cking clue." But what the the doctors did realize was that Martell's disease, whatever it was, did not spare a single bone in his body. "Roentgenograms ... showed osteomalacia [softening of the bones] involving the whole skeletal system."
In 1923, "he stumbled, fell against a chair, and broke both bones of the left forearm." Back to the Marine Hospital on Staten Island he went. While in the hospital, he managed to break two more bones: his left radius and his right humerus. He was at first treated with "diets high in calcium." This seemed to make sense. Martell's urine contained much more calcium than he was ingesting. He was, in other words, losing calcium from his bones. Putting him on a diary diet rich in calcium might, so the doctors reasoned, correct this metabolic imbalance so that the food rather than the bones would supply the excess calcium that his body, for some reason, needed to metabolize.
When this did not improve matters, the doctors took a shotgun approach and subjected him to the full force of 1920s cutting edge medicine: "cod liver oil, calcium and phosphorus medications, thyroid extracts, epinephrin, heliotherapy, quartz lamp treatment, and irradiated milk, without noticeable improvement." This reads like Allen Ginsberg's riff on 1950s psychiatry ("insulin Metrazol electricity hydrotherapy psychotherapy occupational therapy pingpong & amnesia") and the effect was the same. Nil.
In January 1926, Martell was admitted to Bellevue Hospital in New York, under the care of Dr. Eugene DuBois. Martell's bones were crumbling and turning to cysts. Something was horribly wrong with the way his body processed calcium, but what? Debois subjected his patient to a meticulous metabolic study. Martell was given food with varying amounts of calcium while the good doctor (or the good but, sadly, unsung nurse) measured the amount of calcium in his urine and feces. The fact remained; Martell lost more calcium than he ingested, but increasing his dietary intake did not improve matters.
Para...what?
First depiction of human parathyroid glands. Sandström, Ivar, "Om en ny körtel hos menniskan och åtskilliga däggdjur." Uppsala Läkareförenings Förhandlingar, Band XV:7-8 (1879-1880)
Deciding to up the ante, DuBois prescribed his patient parathyroid extract. The role of these diminutive glands, which had only been discovered some 40 years earlier, was still not entirely established, though they seemed to have something to do with calcium and bone metabolism. DuBois had come across case reports from Germany that described a disease with severe bone deformities and cysts -- osteitis fibrosa cystica -- that seemed to fit the bill. Patients dying from this excruciating ailment sometimes had en enlarged parathyroid gland. Conventional wisdom at the time had it that this was some kind of compensatory effect. The parathyroid gland protected against these bone changes by hypertrophy -- that is, by becoming larger and (it was assumed) secreting something into the bloodstream.
DuBois tested this hypothesis. "Three hundred and eighty units of parathormone were given between February 16 and March 2 with a maximum daily dosage of 40 units," but unfortunately the patient "complained of definite increase of pain all over his body, especially on motion. There was in addition, a slight elevation in the blood calcium. The medication was then stopped."
In 1915, a German physician had pointed out that it was odd that most patients with osteitis fibrosa cystica only had one enlarged gland (out of four). If the effect was compensatory, how come all four glands were not equally enlarged? Perhaps -- banish the thought -- this tiny tumor actually caused the bone disease by interfering with the way the body regulated calcium? In the mid '20 this was still a very radical idea, but in the end DuBois got it:
"Our patient then presents a picture which agrees in its essentials with that produced by the excessive administration of parathyroid extract and opposite to that found in hypoparathyroidism. These considerations and the finding of parathyroid tumors in patients with osteomalacia and similar bone disturbances (18) led us to the condusion that the underlying basis for the osteitis fibrosa cystica in our subject was a hyperactivity of the parathyroid bodies."Martell was again referred to Massachusetts General -- this time for the surgeons to "consider the advisability of removing one or more of his parathyroid glands."
Bring out the Scalpel
In Boston, Martell went under the knife, courtesy of Dr E. P. Richardson, who made a cervical incision and went looking for a rogue gland. He found a "small vascular area 6 mm in diameter, deeper red than the remainder of the thyroid lobe" and removed it. Histological examination revealed that it was indeed a parathyroid gland. Sadly, this made little difference in Martell's clinical course. Not to be deterred, Richardson went at it again. He found a nodule on the other side of the neck. The pathologist once again confirmed that it was a parathyroid gland, but once again, it made little difference.
Chart showing little difference in blood calcium after removal of two parathyroid glands.
"A Case of Osteitis Fibrosa Cystica (Osteomalacia?) with Evidence of Hyperactivity of the Parathyroid Bodies. Metabolic Study II," Bauer, Albright and Aub, 1929.
Although stumped, the doctors noticed a slight improvement -- both clinically and physiologically -- and Martell was promptly discharged. He was able to hold down a job as a maritime insurance clerk for a few years, but in 1932 things took a turn for the worse. His kidneys were failing and he was once again admitted to Massachusetts General Hospital. Dr. Pattersson and and a colleague, Dr. Oliver Cope, operated four more times, but did not find any enlarged glands. This is where things get interesting. Cope would later recall how Martell:
"took a scientific interest in his own case and became an investigator as well as an investigatee; he was often found in his room poring over an anatomy text, he demanded that the surgical search should be continued until it succeeded, even when the next step was a sternotomy"At Martell's insistance, the surgeons cracked open his breastbone and found the culprit: a 30 mm encapsulated parathyroid tumor (adenoma) in his mediastinum. But the disease had taken its toll on the former sea captain, and Charles Martell died six weeks after the operation -- after an emergency attempt to remove a kidney stone (the result of his long-standing parathyroid disease that caused calcium to be excreted through his kidneys) that was stuck in his ureter.
What Martell realized -- albeit too late -- by poring over anatomy texts was that parathyroid glands are not always found adjacent to the thyroid. As a result of how the gland develops and migrates (or fails to migrate) in the fetus, it can end up high in the neck or in the area in the chest known as the mediastinum. How did Martell come to suspect this? Did he find an obscure reference to a mediastinal gland in the medical literature, or did he form the hypothesis himself by synthesizing what he had discovered by studying embryology and anatomy? Cope's description is elusive. On the one hand, it suggests someone who is barely literate (Martell was of course highly intelligent) and is clutching his book, like the pious peasant his bible. But it also conjures up the image of the lone genius: an Einstein waiting for a Millikan to confirm empirically what he had intuited.
If there is a moral to this Most Lamentable Tragedie, it would be: so much for the dangers of self-diagnosis.
Sources:
Albright, "A Page out of the History of Hyperparathyroidism," J Clin Endocrinol Metab, 1948;8(8):637-657.
Bauer, Albright, Aub, "A Case of Osteitis Fibrosa Cystica (Osteomalacia?) with Evidence of Hyperactivity of the Parathyroid Bodies. Metabolic Study II," J Clin. Invest. 1930;8(2):229-248.
Cope, "The Story of Hyperparathyroidism at the Massachusetts General Hospital," N Engl J Med. 1966;274:1174-1182.
Hannon, Shorr, McClellan, DuBois, "A Case of Osteitis Fibrosa Cystica (Osteomalacia?) with Evidence of Hyperactivity of the Parathyroid Bodies. Metabolic Study I," J Clin. Invest. 1930;8(2):215-227.
McLellan, Hannon, "A Case of Osteitis Fibrosa Cystica (Osteomalacia?) with Evidence of Hyperactivity of the Parathyroid Bodies. Metabolic Study III," J Clin. Invest. 1930;8(2):249-258.
Sandström, "Om en ny körtel hos menniskan och åtskilliga däggdjur" Uppsala Läkareförenings Förhandlingar, Band XV:7-8 (1879-1880).
