Talk:Jane Austen/Notes on final illness

This page contains notes on the question of Jane Austen's final illness. '''This is not an article. '''

Sources are keyed to the bibliography in the main article: Jane Austen
 * FYI: The French article derived from this (very interesting) talk page has just been nominated for Good article status. --Azurfrog (talk) 23:43, 27 July 2011 (UTC)

Summary
''This is the phrase originally proposed by JasonAQuest. Pointillist (talk) 01:45, 18 March 2008 (UTC)''

Most scholars believe she was ill with Addison's disease, though tuberculosis, an autoimmune disease, and Hodgkin's lymphoma have also been suggested.

Honan (1987)

 * Austen's final illness is mentioned or discussed on pages 374, 378, 379, 385-392, 394-398, 400-405 (death). Honan deals with the nature of her illness on 391-392, although he describes her symptoms in other places. He notes the symptoms consistent with a diagnosis of Addison's disease, and points out that the destruction of the adrenal glands that causes the symptoms of Addison's disease can be a result of tuberculosis, cancer of the stomach or bowels, or an autoimmune reaction. Honan cites Zachary Cope's 1964 article but is not dogmatic - he points out that Cope called his work a "surmise" and cites apparently unpublished work by pathologist Dr. P. N. Cowen, who argued that if the changes in Austen's skin color are ignored, the rest of her symptoms were consistent with a diagnosis of cancer or tuberculosis per se without adrenal involvement. Honan's descriptions of Austen's symptoms and decline are quite moving.

Tomalin (1997)

 * Tomalin mentions or discusses Austen's final illness on pages 254-255 and 258-269 (death). Tomalin discusses the nature of the illness in Appendix i, "A Note on Jane Austen's Last Illness" (282-283), written based on a consultation with Dr. Eric Beck. Tomalin points out that at this distance in time, any diagnosis must be tentative. She mentions Cope's 1964 article ("a carefully argued case"), briefly describes Addison's disease, and argues that "it has other features which do not seem to fit what we know of Jane Austen's case." Tomalin states that "a tanned, healthy appearance," and postural hypotension (faintness upon getting up) would be symptoms of Addison's disease but neither of these was noted in Jane Austen's case. She observes that Austen's recurrent fevers were not characteristic of Addison's disease. In addition she claims that "a steady progression of the disease would be expected with Addison's whereas her illness fluctuated between periods of improvement and deterioration", which contradicts Cope's statement that Addison's is "liable to intermissions during which the patient feels much better and is hopeful of recovery." Back pain is not discussed.


 * Tomalin suggests that "one possibility is that she suffered from a lymphoma such as Hodgkin's disease ... which would produce recurrent fevers and progressive weakening, leading to death." Tomalin suggests that Jane Austen's and Fanny Austen's reports of Jane's experience of severe facial pain over seven weeks in 1813 could have been an early symptom of a lymphoma, and reviews the possibility that this was caused by shingles, observing that "it seems unlikely that such a painful affliction would go unmentioned...."

Le Faye, A Family Record (2nd Ed, 2003)

 * Le Faye deals with Austen's final illness on pages 235-236, 238-240, 242-243 and 245-254 (death). Le Faye gives an explanation of Addison's disease (236) and clearly comes down on the side of Addison's disease: "in recent years medical opinion has put forward the theory, based on Jane's own description of her symptoms, that early in 1816 she fell victim to the then unrecognized Addison's Disease." Le Faye cites the 1964 Cope article and the Encyclopedia Britannica. She does not discuss Bevan's suggestion of Hogkin's disease.

Wiltshire, Jane Austen and the Body (1992)

 * Wiltshire's focus in this work is not Jane Austen's own health but rather her treatment of health and medicine in her novels. Nevertheless, he does discuss Austen's final illness (199-201) and its effects (or lack of effect) on her last work. On his last page, Wiltshire mentions one of Austen's late letters "describing the symptoms of her illness so clearly that modern doctors feel reasonably confident about making a retrospective diagnosis of Addison's disease". (221)

Smithers, "Medicine" (1986)

 * This is a brief (three page) article mainly on medicine as portrayed in Austen's novels. Smithers does discuss Austen's final illness, however. (305-306. "The well-known surgeon Sir Zachary Cope attributed it to Addison's disease, a failure of adrenal glands, making a likely guess on slender evidence. Responding in a letter, Dr. F. A. Bevan suggested that she might have had Hodgkin's disease, and the increasing fatigue, bouts of fever, and remissions during te course of a steady decline over a few months would fit his speculation well enough, although we hear nothing about lymph-node swelling." (305) Smithers notes that Cope relied heavily on Austen's description of the discoloration of her skin as evidence for the brown pigmentation of Addison's disease.

Fergus, A Literary Life (1991)

 * This is a literary biography of Jane Austen. Austen's final illness is discussed on pages 166, 169-170. "Symptoms of Addison's disease, probably the condition that killed her, may have shown themselves early in 1816." (163) Fergus cites only the Cope article.

Cope (1964)

 * Cope begins by pointing out that the contemporary sources of information about Austen's final illness are very limited. Her doctors left no notes and her family was reticent about her illness. Accordingly, the remaining information consists of "the few comments made by the patient herself in the letters that have survived." (182) Cope asserts that Austen "was an accurate observer, and though she made light of her troubles until near the end one can rely on her definite statements." (182)


 * The bulk of Cope's article consists of a description of the course of Austen's final illness, based largely on her letters but also on memories of her niece Caroline and a contemporary letter from Cassandra to Fanny Knight. (182-183). Cope quotes extensively from the available written material and makes evaluative comments as he goes along. The last third of the article (183) is an attempt at differential diagnosis based on his summary. Cope feels that absence of symptoms rules out diseases of the head or chest, unless the reported faintness was a sign of cardiac disease. Only the "bilious attacks" suggest the possibility of diseases of the "abdominal viscera" and Cope notes that bilious attacks are a non-specific symptom of various diseases. He suggests that the absence of reported difficulties with speech, chewing and swallowing rules out myasthenia gravis, and the presence of gastro-intestinal attacks and fainting is inconsistent with subaccute bacterial endocarditis. He considers and rules out "tabes mesenterica and some other forms of tuberculosis". He is more persuaded by the case for cancer of the stomach, but finally concludes that pernicious anaemia and Addison's disease would account for most of the symptoms. Both result in a similar set of symptoms, including "intermissions during which the patient feels much better and is hopeful of recovery", and may be distinguished clinically because Addison's disease usually involves changes in skin color, including some areas of darker skin and some areas without pigment. Cope quotes from Austen's letter of 23 March 1817 to Fanny Knight, in which Austen reports some improvement in her condition: she was "recovering my Looks a little, which have been bad enough, black & white & every wrong color." Cope concludes: "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. Addison's disease is usually—Wilks said always—due to tuberculosis of the suprarenal capsules, and it is likely that it was so in Jane Austen's case." (183)


 * Cope observes that "The disease ran its course rapidly" and that this "might well account for any fever", and that "Pain in the back has been noted in Addison's disease by several observers."Pointillist (talk) 01:03, 18 March 2008 (UTC)

Bevan (1964)

 * F. A. Bevan wrote a short letter to the British Medical Journal in response to Cope's 1964 article. He considered Cope's "a reasonable diagnosis" but wondered if Cope had "considered sufficiently the feverish side of the illness." He says that fever is unusual in cases of Addison's disease and suggests that Hodgkin's disease is the probable diagnosis. He says "This could account for all of the symptoms mentioned [in Cope's article] including the skin pigmentation and the initial pain in the back." Bevan cited a case of lymphadenoma which he had handled "several years ago" in which the course of the disease was similar to that of Austen's final illness. (all 384)

Upfal (2005)

 * Upfal is identified in her article as a professor in the School of English, Media Studies and Art History at The University of Queensland. She acknowledges the substantive contribution of Professor Ian Frazer of The University of Queensland, Center for Immunology and Cancer Research.


 * The title of Upfal's article makes a large claim: that new evidence points to a fatal Hodgkin's disease and excludes Addison's disease as the cause of Austen's final illness. Her article reviews in great detail what is known about Austen's lifelong medical history and claims to refute the assertion made by previous biographers that Austen's health was fundamentally excellent. Based on her reading of the surviving letters and some additional (apparently previously known) sources, Upfal identifies (a) problems at Austen's birth due to a delivery four weeks past full term, (b) a possible lifetime immune system disorder, (c) the known serious case of typhus at age seven while Austen was away at school, (d) chronic conjunctivitis, (e) an "unusually severe" episode of whooping cough, (f) possibly chronic ear infections (otitis externa), and (g) beginning in 1813, an episode of severe neuralgia indicative of an infection with herpes zoster (shingles). Upfal says that herpes zoster infections are common in patients with Hodgkin's disease. She reviews the available evidence from Austen's letters and concludes that Austen's symptoms as described are consistent with and best described as Hodgkin's disease.
 * Upfal briefly summarizes Cope's article and disagrees with his suggestion that the onset of illness and the crisis in April 1817 are 'Addisonian crises'. She argues that the cyclical pattern of Jane's night fevers from February 1817 onwards is Pel-Ebstein fever, "a classic clinical sign in Hodgkin’s disease", citing Bevan and referring to medical textbooks. Upfal accepts Bevan's explanation of the back pain. She reviews the pigmentation symptoms that Cope considered "almost pathognomonic of Addison’s disease", but says that Jane’s letter of 25 March is consistent with symptoms of idiopathic thrombocytopenia purpura, an autoimmune disease associated with advanced Hodgkin's disease. Upfal also points out that in the 19th century tuberculosis was associated with Hodgkin's disease in about 20% of the cases examined by autopsy, speculating "it is possible that Jane had this disease as well as the fatal lymphoma."
 * I wish to read this article again more closely to be sure that the above summary does it justice. However, through two readings, it is not clear to me what the "new evidence" is that Upfal refers to in the title of her article. As far as information about Austen is concerned, she appears to base her analysis and reinterpretation on the same sources that Cope and Austen's biographers used.
 * It isn't clear to me either. I suspect the antepenultimate paragraph (on purpura) is supposed to be the "new" part, using footnote #43 (Rare Syndromes in Hodgkin’s Disease, 1998) and footnote #66 (Autoimmune Processes Terminating 24 Years in Hodgkin’s Disease, 1992) to justify challenging Cope's statement that "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."
 * I haven't been able to get either of those footnote sources online. I looked for #43 in the Annals of Oncology archive (http://annonc.oxfordjournals.org/search.dtl) but with no success: I think it is from a supplement that isn't available online (it is missing from the list at http://annonc.oxfordjournals.org/supplements.dtl). I can find the abstract of #66 at http://www3.interscience.wiley.com/cgi-bin/abstract/108061553/ABSTRACT?CRETRY=1&SRETRY=0 but prefer not to pay $30 to read the full text. I've put some misc Upfal-related notes & links into a subsection in case they are useful. Pointillist (talk) 14:59, 17 March 2008 (UTC)

Simmaren's comments
 * I've read Upfal's paper again. The underlying point, that Hodgkin's lymphoma is a possible diagnosis for Jane Austen's last illness, is unexceptionable. However, I don't think much of the article. There is too much surmise based on an absence of evidence, and there are too many uses of constructions such as "must have been" and "may have shown." Upfal suggests the possibility of a postdate (substantially overdue) pregnancy without showing that Austen actually showed any of the signs of an illness or disability resulting from such a condition. Upfal reviews the remaining evidence for Austen's health history, notes that she was ill at various points during her life and suggests this to be evidence of an immune deficiency, without discussing what a normal health history would have been for someone of Austen's time and class. This is unhistorical. Upfal's suggestion concerning conjunctivitis is interesting and appears better supported by the available evidence. Cope considered evidence of skin color changes as strong supporting evidence of Addison's disease; Upfal invokes "a rare syndrome associated with Hodgkin's disease" as the explanation for the changes in color, although the use of the adjective "rare" undercuts the logic of her argument. Simmaren (talk) 21:16, 28 March 2008 (UTC)
 * I remain neutral. Upfal isn't conclusive on the skin colour question, Cope isn't conclusive on the fevers. Painful though it may be, we should nevertheless present the current evidence in a Jane Austen's final illness article. Pointillist (talk) 01:24, 29 March 2008 (UTC)

Upfal misc notes & links

 * Re: Upfal's background. The Jane Austen Society of Australia says "Annette Upfal is a law graduate and teacher whose special area of interest is biographical research on Jane Austen. Her PhD project at the University of Queensland explores the relationship between Jane's experience of illness in her own life and depictions of illness in her writings." (http://www.jasa.net.au/sensextdec05.htm#story7). The blurb for the Jane Austen and VCE Literature Conference (La Trobe, 29-30 Nov 2007) says "Annette Upfal completed an M.Phil. in 2005, with a thesis on Jane Austen’s Juvenilia, and is currently preparing a Ph.D dissertation on 'Jane Austen’s Experience of Illness' at the University of Queensland."


 * Dr Bill Coote, reviewing Upfal's article in Sensibilities Volume 32, June 2006 (the journal of the Jane Austen Society of Australia) says "We will probably never know whether Jane Austen died of Addison’s disease, Hodgkin’s disease or some other disease, and Upfal does add a new idea with her conclusion that Jane Austen suffered chronic ill-health throughout her life. However, her arguments are not persuasive." (abstract at http://www.jasa.net.au/sens/sensextjun06.htm#story12 - I haven't seen the full article as it requires JASA membership).


 * At the 2005 JASNA Annual General Meeting (Milwaukee October 7-9, 2005) two physicians, Cheryl Kinney, M.D. and Cynthia Lopez, M.D. held a breakout session on Jane Austen's Illness (see http://www.jasna.org/agms/milwaukee/Breakout.html). Unfortunately notes from this session don't seem to have been published in Persuasions (http://www.jasna.org/persuasions/printed/pers27.html). However, the article "James Stanier Clarke's Portrait of Jane Austen" (Joan Klingel Ray and Richard James Wheeler, Persuasions, No. 27, pp116-7, http://www.jasna.org/persuasions/printed/number27/ray-clarke.pdf) says that they "confirmed Dr. Zachary Cope’s 1964 diagnosis of Addison’s disease, using not only the symptoms described in her letters, but also Regency and modern medical knowledge. In so doing, Dr. Lopez discounted Hodgkins Disease as the cause of Austen’s death (Upfal)."


 * It would be interesting to get Prof Wiltshire's feedback. Wiltshire is on the editorial board of JASNA's Sensibilities (where Bill Coote's article was published) and was one of the hosts of the Jane Austen and VCE Literature Conference where Annette Upfal and Jon Spence were speakers. If only they had taken time out to discuss Upfal's paper....

Medical commentators and their qualifications
(Simmaren, I've added this so you have the various references in one place for convenience. Pointillist (talk) 01:56, 16 March 2008 (UTC))

Sir Vincent Zachary Cope (1964)
Source for statements by Honan et al.
 * Author of article "Jane Austen's Last Illness" in British Medical Journal (18 July 1964, p182ff, http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1816080&blobtype=pdf).
 * Curriculum Vitae at http://www.aim25.ac.uk/cgi-bin/search2?coll_id=3291&inst_id=8.
 * Additional biographical material is available in volume 6, page 84 of Plarr's Lives of the [Royal College of Surgeons] Fellows (source: http://livesonline.rcseng.ac.uk/lives_index.pdf) but this is not yet available online.
 * Author of "The Royal College of Surgeons of England: A history" (see http://www.rcseng.ac.uk/library/helpsupport/faqs.html/?searchterm=Zachary%20Cope).

Dr F A Bevan (1964)

 * Author of letter "Jane Austen's Last Illness" in British Medical Journal proposing Hodgkin's lymphoma as more likely diagnosis (8 August 1964, p384, http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1816385&blobtype=pdf).
 * Apparently in 1953 a general practitioner (source: "Marital Tensions: Clinical Studies Towards a Psychological Theory of Interaction" (1967) by Henry V. Dicks, page 18 and footnote on page 27, http://books.google.co.uk/books?id=mHA9AAAAIAAJ&printsec=frontcover&dq=Marital+Tensions:+Clinical+Studies+Towards+a+Psychological+Theory+of+Interaction&source=gbs_summary_r)

Dr Eric Beck (1997)
Consulted by Tomalin for her Appendix I.
 * Former chief examiner of the Royal College of Physicians (see http://www.rsmpress.co.uk/bkdias.htm).
 * Author of "Tutorials in Differential Diagnosis" (4th Edition, July 2003; ISBN 0443061572, see http://www.amazon.co.uk/Tutorials-Differential-Diagnosis-Beck/dp/0443061572).
 * Chairman of the Royal College of Physicians committee "Practical Assessment of Clinical Examination Skills" (see http://www.rcplondon.ac.uk/pubs/exam_mrcp_pacebooklet.htm).

Professor Ian Frazer (2005)
Consulted by Upfal for her paper "...New evidence points to a fatal Hodgkin’s disease and excludes the widely accepted Addison’s" (J Med Ethics; Medical Humanities 2005;31:3-11 (2005), http://mh.bmj.com/cgi/reprint/31/1/3).
 * Founder of University of Queensland’s Centre for Immunology and Cancer Research
 * Australian of the Year 2006 for his development of Human Papilloma virus vaccine, to prevent and treat cervical cancer (http://www.australianoftheyear.gov.au/pages/page58.asp)
 * Curriculum vitae at http://www.acrf.com.au/page/bio_prof_ian_frazer.html