The causes of Jane Austen's death, which occurred on July 18, 1817 when she was 41 years old, following an undetermined illness that lasted about a year, have been discussed retrospectively by doctors whose conclusions have subsequently been taken up and analyzed by biographers of Jane Austen, one of the most widely read and acclaimed English writers.
The two main hypotheses are that of Addison's disease, put forward in 1964 by the English surgeon Zachary Cope (1881–1974), and that of Hodgkin's disease, first mentioned concisely the same year by Dr. F. A. Bevan,Dr Frank Arthur Bevan (1902–1974) was a Woodstock-based general practitioner, known during his lifetime for his boundless activity and whose interests extended far beyond medicine to many areas of culture, according to his obituary published by the BMJ () then developed and argued in 2005 by the Australian Annette Upfal, professor of British literature at the University of Queensland. In the 2010s, the British Library speculated she died of arsenic poisoning based on 3 pairs of eyeglasses owned by Austen.https://www.townandcountrymag.com/leisure/arts-and-culture/news/a9907/jane-austen-death/
The discussion is based primarily on Jane Austen's writings on her own clinical case. It does not rule out the possibility of tuberculosis, which was the usual etiology of Addison's disease in the 19th century.
Case history
The story of Jane Austen's illness has been reconstructed by Annette Upfal, who indicates at the end of her work that she has received the approval of Australian immunologist Ian Frazer.
Jane Austen was born four weeks postterm. In childhood and young adulthood, she suffered from serious infections: typhus, which it has become conventional to claim she "almost died" of at the age of seven,According to Linda Robinson Walker, the opinion that typhus nearly caused Jane's death is not expressed by any contemporary account of Jane Austen. It was first put forward in 1949 by Elizabeth Jenkins ( Jenkins, Elizabeth (1949). Jane Austen. New York: Minerva.) and subsequently repeated, uncredited and unverified, by Halperin, Le Faye, Nokes, Tucker and Tomalin. while attending Mrs Ann Cawley's school in Southampton; chronic conjunctivitis; whooping cough, which occurred at the age of 30 and was considered "unusually severe"; and otitis externa (side unspecified), in 1808, treated by applying sweet almond oil to the external auditory canal. In 1813, she repeatedly showed signs of facial neuralgia (attributed by A. Upfal to trigeminal shingles) severe enough to force her to appear in public with "a cushion pressed to her face" ("Jane was a very private person, and the pain must have been intense for her to walk out in public with a cushion pressed to her face"). Towards the end of the winter of 1815, she began to complain of generalized pruritus, without a rash.
The onset of her fatal affliction is generally placed in the early months of 1816, around her fortieth birthday, when it became clear to those around her that she was seriously ill. Her troubles consisted of insidious weakness, asthenia and progressive wasting. She had frequent "bilious attacks" (vomiting) and her face changed color, becoming "black and white". From February 1817, she was subject to regular bouts of high fever, during which she sometimes lost consciousness. She died during one of these attacks.
Symptom analysis
The retrospective diagnosis of the illness that led to the novelist's death is based on the following symptoms and clinical signs, which relate only to the last year of Jane Austen's life. This symptomological inventory is largely based on the work of Cope and Upfal.
General signs All the authors emphasize the rapid deterioration in the patient's general condition, with weight loss and weakening in the space of a few months. Asthenia was severe, and brief fainting spells occurred regularly. Febrile attacks lasting several days were observed intermittently from February 1817 onwards. On the other hand, frequent "attacks of bile" are mentioned in Jane Austen's letters, and probably refer to nausea and vomiting.
Cutaneous signs
In her letter to Fanny Knight dated March 23, 1817, Jane Austen referred to the color of her skin, which had darkened in places. There is little evidence, however, as to the exact mechanism of this color change, which may correspond either to hyperpigmentation as part of melanoderma, or to spontaneous subcutaneous hemorrhages (petechia or ecchymoses).
Speculative differential diagnosis
All authors agree that sources on Jane Austen's final illness are too limited to allow a diagnosis of certainty. Her doctors left no notes, and her family spoke only reluctantly of her illness. Moreover, her most informative letters were destroyed by her sister Cassandra after her death.In her 1995 introduction to the third edition of Jane Austen's letters, Deirdre Le Faye reports that Jane Austen's sister Cassandra had destroyed some of the novelist's letters and removed passages from others. According to Deirdre Le Faye, Cassandra did this because Jane Austen described the physical symptoms of her illness too fully: see . As a result, the information that survives today consists of "a few comments made by the patient herself in the letters that have survived". Cope asserts that Jane Austen was an accurate observer, and although to the end she downplayed her health problems, her formal statements can be trusted.Cope does not specify the origin of his reliance on Jane Austen's descriptions of his symptoms. In addition to Jane Austen's own observations, Cope consulted the recollections of one of the patient's nieces, Caroline Austen, as well as an 1817 letter written by Cassandra Austen and addressed to Fanny Knight.
Addison's disease A diagnosis of Addison's disease was proposed in 1964 by a famous surgeon, Sir Vincent Zachary Cope, in a short two-page article published in the British Medical Journal. Addison's disease is a chronic progressive adrenal insufficiency, first described in 1855 by Thomas Addison. The cardinal signs include asthenia, low blood pressure, anorexia (with weight loss) and melanoderma, with hyperpigmentation of the skin at friction points and of the mucous membranes. Only the latter sign was specific to the disease, and Zachary Cope underlined the fact that melanoderma is not always uniform, and that "in some cases the dark patches of the skin were mingled with areas showing a lack of pigment – a true black and white appearance". Skin coloration was of particular importance, as it was the only clinical feature distinguishing Addison's disease of the adrenals from another "Addison's disease" (described by the same author six years earlier, in 1849), which corresponded to a hematological condition better known today as pernicious anemia.A more complete description of pernicious anemia was published in 1872, 23 years after Addison, by the German physician Anton Biermer. Cope concluded: "There is no disease other than Addison's disease that could present a face that was "black and white" and at the same time give rise to the other symptoms described in her letters." Cope believed that the presence of fever was due to the rapid progression of the disease, and pointed out that "back pain was noted in Addison's disease by several observers".
However, important associated signs that do not belong to the classic picture of Addison's disease remain poorly explained, as Claire Tomalin, with the medical endorsement of Dr. Eric Beck, Fellow of the UK Royal College of Physicians,Claire Tomalin addresses Jane Austen's final illness on pages 254–255 and 258–269 (death) of Jane Austen: A Life. She discussed the nature of the illness in Appendix I, "Note on Jane Austen's Final Illness". She indicated that "a healthy, bronzed appearance" and "postural hypotension" (i.e. weakness on rising) would be symptoms of Addison's disease, but neither was noted in Jane Austen's case. She observed that Austen's recurrent fevers were not characteristic of Addison's disease. She also noted that "in the case of Addison's disease, one should have expected a steady progression of the condition, whereas Jane Austen's disease oscillated between periods of improvement and deterioration", which contradicted Cope's assertion that Addison's disease was "subject to remissions during which the patient felt much better and hoped for recovery". Back pain remained unaddressed. C. Tomalin suggested that "it was possible that she had suffered from a lymphoma such as Hodgkin's disease [...] which would produce recurrent fevers and progressive weakening, leading to death". She also suggested that the severe facial pains the novelist had suffered for seven weeks in 1813 (a fact both Jane Austen and Fanny Knight had reported) could have been the first symptoms of lymphoma, and analyzed the possibility that these pains were the result of shingles, noting that "it would seem unlikely that such a painful condition could have gone unmentioned". was quick to point out.
The main Jane Austen biographers to support Cope's hypothesis are Jan FergusJane Austen's illness was mentioned by Fergus on pages 166 and 169–170 of his book: "The symptoms of Addison's disease, which probably caused her death, began to appear early in 1816", he wrote on page 163. Jan Fergus only quoted Cope's article. and Deirdre Le Faye. Australian English literature professor John Wiltshire has also advocated Addison's Disease.
Hodgkin lymphoma Hodgkin lymphoma was described by the English pathologist Thomas Hodgkin in 1832.
The possibility of Hodgkin lymphoma in Jane Austen was first raised by F.A. Bevan, in response to Sir Zachary Cope's article proposing Addison's disease. Bevan referred anecdotally to a case in which Hodgkin lymphoma, then called "lymphadenoma", had had as its initial manifestation a "pain in the back" without any superficial lymphadenopathies being noted during the course.In a short three-page article, David Waldron Smithers discusses medicine as it appears in Jane Austen's novels, as well as the disease that took her, pages 305–306: "The well-known surgeon Sir Zachary Cope identified her with Addison's disease, a failure of the adrenal glands, drawing a probable conclusion from very flimsy evidence. Dr. Bevan replied in a letter suggesting that she might have Hodgkin's disease, as the increasing fatigue, fever attacks, and remissions during a continuous weakening in a few months might fairly fit his hypothesis, although the increased size of the lymph nodes was not reported" (p. 305). Smithers noted that Cope was absolutely certain that Jane Austen's description of the discoloration of her skin corresponded to the golden-brown pigmentation caused by Addison's disease.