See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/5604994 Jack London's "chronic interstitial nephritis". A historical differential diagnosis. Article in The Pharos of Alpha Omega Alpha-Honor Medical Society. Alpha Omega Alpha · February 2008 Source: PubMed CITATIONS READS 2 598 2 authors, including: Philip J Klemmer University of North Carolina at Chapel Hill 47 PUBLICATIONS 2,211 CITATIONS SEE PROFILE All content following this page was uploaded by Philip J Klemmer on 13 October 2015. The user has requested enhancement of the downloaded file. Jack London’s “chronic interstitial nephritis” A historical differential diagnosis Andrew S. Bomback, MD, and Philip J. Klemmer, MD Dr. Bomback (AΩA, Columbia “a gastro-intestinal­­ type of uraemia” In 1911, Jack London was turned University, 2003) is the Doc J. that began after dinner on November down as a bad health risk by an insur- Thurson III Fellow in the Department 21, 1916, and led to “coma” and then ance company. No reason was given for of Medicine, Division of Nephrology eventual death at 7:45 pm the following his failure to pass the insurance compa- and Hypertension, at the University evening.2 ny’s evaluation, but two years later, fol- of North Carolina School of Medicine The renal failure documented on lowing an appendectomy, London was in Chapel Hill, North Carolina. Dr. London’s death certificate has never told by his surgeon—the same Dr. Porter Klemmer (AΩA, Temple University, been fully explained.3 Speculation who eventually took on London as a 1972) is professor of Medicine in that recurrent nephrolithiasis was the private patient—that his kidneys were the Division of Nephrology and source of his kidney disease is rooted failing. Porter’s assessment of London’s Hypertension at the University of North in London’s persistent renal colic, but failing kidneys was probably based on Carolina School of Medicine. the most severe scarring from kidney the combination of a normocytic ane- stones only rarely leads to end-stage mia, acidemia, and heavy albuminuria, renal disease in patients with two func- diagnostic tests readily available for a ack London, once America’s most tional kidneys. physician in 1913.4 London’s letters and famous author thanks to The Call London’s writing was intensely au- his second wife’s diary relate Porter’s of the Wild, White Fang, and over tobiographical and has helped scholars warning that he would die of kidney Jfifty other books, died at the age of unearth a detailed and fascinating his- failure if he did not stop drinking, give forty on November 22, 1916. His death tory of a writer, husband, father, sailor, up raw fish and meat, and start ex- certificate, signed by Dr. William S. rancher, gold prospector, and social ac- ercising and losing weight. By most Porter, London’s personal physician, tivist (to name a few of the hats he wore reports, the writer wasn’t a particularly pronounced “1+ days” of “uraemia to varying degrees of success). We be- compliant patient.5,6 Photographs from following renal colic” as the cause of lieve that London’s words also hold the London’s final years show him bloated death. “Chronic interstitial nephritis” key to understanding the pathophysiol- with edema. His behavior became more of three years’ duration was listed as a ogy behind his fatal kidney disease. erratic, and his writing, too, showed “contributor.” 1 A physicians’ bulletin, signs of deterioration.7 His body and signed by Porter and three other physi- 1911—Onset of mind were shutting down as uremia cians, described a day-long battle with symptomatic uremia took its toll. Jack London’s “chronic interstitial nephritis” Jack London, ca. 1900. © Bettmann/CORBIS. Jack London’s “chronic interstitial nephritis” gout likely exacerbated by the author’s steady diet of raw meat and alcohol. The author’s assertion that “All my life my skin ha[s] been famous for its healing powers” 12p196 is not consistent with the photosensitive cutaneous lupus. And while there are a number of different types of renal disease in SLE, with im- mune complex- mediated glomerular diseases being most common, there is another, more compelling, unifying di- agnosis for both London’s kidney failure and transitory dermatitis. Sick while cruising on the Snark In 1907, accompanied by his second wife, Charmian, and two crew members, Jack London set out from San Francisco aboard the Snark, a custom-made schoo- ner built to sail around the world. Their one-and-a-half-year voyage (chronicled Jack and Charmian London charting a course. in The Cruise of the Snark) instead be- The Bancroft Library, University of California, Berkeley. The Regents of the University of California. came an intense exploration of the South Pacific that ended in an Australian hos- pital where London was treated for a London’s life story provides the ba- liver failure such as encephalopathy, “mysterious malady that . extended sis for a broad differential diagnosis jaundice, pruritus, or asterixis, and the from my hands to my feet so that at of a chronic kidney disease that pro- same physicians who detected his kid- times I was as helpless as a child.” 12p208 gressed to end-stage renal disease. ney disease should have been able to Prior to contracting this illness, London The author was almost as famous for diagnose cirrhosis. had correctly self- diagnosed himself his drinking exploits as for his adven- London’s celebrated nickname, with a severe case of yaws, an infec- ture stories of the Klondike and the “Wolf,” was rooted in the love for these tious, nonvenereal disease caused by South Seas, thanks to the thinly- veiled, animals he revealed in classics such as Treponema pallidum pertenue, a sub- alcohol- soaked autobiographical pro- The Call of the Wild and White Fang. species of the spirochete responsible tagonists of novels such as Martin Eden A recent report argues that this liter- for syphilis. London believed he had and John Barleycorn. Although glom- ary “Wolf” might have suffered from caught the “vile skin disease” 12p194 from erular morphologic abnormalities with systemic lupus erythematosus (SLE),11 a French sailor he picked up in Tahiti. IgA deposition are found in more than a disease named for the facial rash of Whether true or not, the Snark’s voyage fifty percent of cirrhotic patients at ei- untreated victims suggesting the ravages certainly put him and his crew (all of ther necropsy or biopsy8,9 (presumably of a wolf bite. The argument focuses on whom contracted the disease) in an area due to defective hepatic processing or four criteria for the diagnosis of SLE where yaws was endemic. Before the portacaval shunting of circulating im- that London supposedly displayed dur- World Health Organization’s mass treat- mune complexes10), these abnormali- ing his lifetime: oral ulcerations, arthri- ment campaigns began in the 1950s, ties do not cause proteinuria or renal tis, photosensitivity, and renal disease. the worldwide prevalence of yaws was scarring. End-stage liver disease and its However, a bout of scurvy that London between fifty and one hundred million, associated hemodynamic compromise endured in the Klondike provides an with the warm and humid tropical re- can lead to renal dysfunction and, in equally strong etiology for his oral ul- gions of Southeast Asia and the Pacific some, hepatorenal syndrome. London, cerations, and the distribution of his Islands two of the rifest areas.13 however, never displayed classic signs of “arthritis” is more reflective of a case of While there are no reports of renal 28 The Pharos/Winter 2008 The Snark in the South Seas, with a visitor known as “The Nature Man.” Photographer unknown. Public domain. complications from yaws, London’s inhalation, ingestion, injection, or ab- suggests that her husband used cor- choice of treatment for the skin condi- sorption through the skin. Neurological, rosive sublimate for five months, from tion in the pre-antibiotic era clearly put gastrointestinal, and renal systems are June through November 1908, until he his kidneys at risk. the most commonly affected organ sys- returned to California with his yaws tems in mercury exposure. lesions healed.15 The heavy load of mer- Here were malignant and excessively cury London self- administered at the active ulcers that were eating me up. Heavy metal treatment—and end of his Snark voyage likely caused There was an organic and corroding an acute proximal tubular necrosis that poison at work. I decided to fight toxicity for yaws was not entirely reversible. Over the the poison with corrosive sublimate. next eight years, his gradually declin- The very name of it struck me as vi- ing kidney function, which today might cious. Talk of fighting fire with fire! I Inorganic mercury salts such as mer- be attenuated by ACE-inhibitors and was being consumed by a corrosive curic chloride are highly noxious. Their dietary protein restriction, led to his poison, and it appealed to my fancy poor lipid solubility results in a nonuni- end-stage renal disease. to fight it with another corrosive form distribution, with accumulation Mercury toxicity also explains the poison.12p196 primarily in the proximal renal tubules, “mysterious malady” that London claims where it predisposes to proteinuria, baffled a team of expert physicians in Corrosive sublimate (mercuric chlo- granular casts in the urinary sediment, Australia. ride) was then considered one of the nephrotic syndrome, and pyuria from few existing therapies for yaws, along tubular damage. In some cases, renal The mysterious malady that afflicted with arsenic and potassium iodide. failure with severe oliguria and anuria my hands was too much for the However, the correct dosing and dura- may occur.14 London, who had treated Australian specialists.