Jane  Austen's  Illness  

    When Jane Austen died on 18 July 1817 (exactly 147 years ago) she was only 41.  Of the six novels she had written four had been published.  Though they appeared anonymously the author was known to a small circle of admirers.  But the acclaim in which she is held to-day had then hardly begun to gather.  Such is the sustained and even quality of her novels that critics have remained doubtful about the order in which some of them were composed, so that her death at so comparatively early an age surely robbed us of more at least equally delightful works.  What was the cause of this catastrophe?  Sir Zachary Cope has been studying Jane Austen's letters for clues to it, and this week he presents his findings at page 182 of the B.M.J.
    The main features of Jane Austen's last illness seem to have been its insidious onset about a year before her death, intermittently progressive weakness and languor, gastric upsets, and discoloration of the skin.  Sir Zachary's discussion of the differential diagnosis includes pernicious anaemia, myasthenia gravis, and cancer of the stomach, but many doctors will probably agree with him that the most likely condition is Addison's disease of the suprarenal glands.
    Further research may perhaps show that letters of her contemporaries provide evidence for or against the diagnosis, though Sir Zachary found nothing of help in the documents that survive from the immediate family circle or her doctors.  As he remarks, her own observations are particularly trustworthy, for her perspicacity and cool judgment of what she observed were the essence of her genius.  It is peculiarly tragic that so uncommon a disease should have struck down so rare a spirit.

    Jane Austen died at 4:30 a.m. on 18 July 1817 at the age of 41 from an ailment the nature of which has never been ascertained, or, so far as I am aware, seriously discussed.  No information was furnished by the doctors who attended her, and her relatives were reticent about her illness, so that we are compelled to rely chiefly on the few comments made by the patient herself in the letters that have survived.  Fortunately Jane Austen was an accurate observer, and though she made light of her troubles until near the end one can rely on her definite statements.
    The onset of her illness was insidious, but we know that she began to have a feeling of weakness or tiredness round about July 1816, and within a few weeks she experienced severe pain in the back, for in a letter dated 8 September she wrote to her sister saying:
    "Thank you, my back has given me scarcely any pain for many days.  I have an idea that agitation does it as much harm as fatigue, and that I was ill at the time of your going away from the very circumstance of your going."
    That comment is noteworthy.  Three months later (16 December) she refused an invitation to dinner, giving as a reason:
    "I was forced to decline it, the walk is beyond my strength (though I am otherwise very well)."
    A month later, though she told her niece Caroline that she felt stronger, yet in a letter to her friend Alethea Bigg she for the first time confesses that her illness is serious:
    "I have certainly gained strength through the winter and am not far from being well; and I think I understand my own case now so much better than I did, as to be able by care to keep off any serious return of illness.  I am more and more convinced that bile is at the bottom of all I have suffered which makes it easy to know how to treat myself."
    "Serious return" and "all I have suffered" are significant words.  The self-diagnosis of "bile" must indicate some gastro-intestinal irritation, probably nausea or vomiting or both.  Up to that time she appears to have been treating herself.
    Little information is available for the month of February 1817, though we learn that there was pain in one knee, which was therefore wrapped in flannel, but in a letter dated 23 March and written to her favourite niece Fanny we find important evidence:
    "I certainly have not been very well for many weeks, and about a week ago I was very poorly, I have had a good deal of fever at times and indifferent nights, but am considerably better now and recovering my looks a little, which have been bad enough, black and white and every wrong colour.  I must not depend upon ever being blooming again.  Sickness is a dangerous indulgence at my time of life."
    She was evidently distressed by her changing facial appearance.
    Two weeks later, on 6 April, a letter written to her brother Charles tells of severer attacks:
    "I have been really too unwell the last fortnight to write anything that was not absolutely necessary, I have been suffering from a bilious attack attended with a good deal of fever....  I was so ill on Friday and thought myself so likely to be worse that I could not but press for Cassandra's return with Frank."
    Obviously the attacks or crises were becoming more serious and she was now apprehensive. The last two available letters were written in May and show a rapid deterioration. On 22 May she wrote to her dear friend Anne Sharp a despairing letter which, however, contains several important clues.
    "In spite of my hopes and promises when I wrote to you I have since been very ill indeed.  An attack of my sad complaint seized me within a few days afterwards - the most severe I ever had and coming upon me after weeks of indisposition, it reduced me very low....  My head was always clear, & I had scarcely any pain; my chief sufferings were from feverish nights, weakness and languor."
    In the same letter she tells her friend that she had arranged to go to Winchester so as to be under the care of a well-known surgeon there, Mr. Lyford.  The other letter was to her nephew Edward and mentions that the appearance of her face was still distressing:
    "I will not boast of my handwriting; neither that nor my face have yet recovered their proper beauty, but in other respects I am gaining strength very fast."
    In this letter she also mentions that she was eating her meals in a rational way and was employing herself, though lying on the sofa most of the day.
    Two other witnesses must now be called.  First, just before the move to Winchester her niece Caroline paid her a visit and later she was able to remember that her Aunt Jane was sitting down, dressed in a dressing-gown, looking very pale, and speaking in a weak and low voice.  This testifies to her anaemia, for when in health Jane Austen had a rich colour.  The last and very important piece of evidence is to be obtained from the letter in which Cassandra Austen describes the last few hours of her sister's life in such moving words.  The letter was written to Fanny Knight on 20 July 1817.  The important passage is the following:
    "On Thursday I went into the town to do an errand your dear Aunt was anxious about.  I returned about a quarter before six & found her recovering from faintness and oppression, she got so well as to be able to give me a minute account of her seizure and when the clock struck 6 she was talking quietly to me.  I cannot say how soon afterwards she was seized again with the same faintness, which was followed by sufferings she could not describe, but Mr. Lyford had been sent for, had applied something to give her ease & she was in a state of quiet insensibility by seven at the latest.  From that time till half past four, when she ceased to breathe, she scarcely moved a limb."
    One further fact must be mentioned.  Henry Austen, Jane's favourite brother, whom she had nursed through a serious illness in 1815, who greatly encouraged her writing and helped to get her novels published, and who seemed to be very prosperous, went bankrupt in March 1816.  This was a terrible mental shock to Jane, and might well have precipitated any disease susceptible of being influenced by mental shock.
    Here then we have the story of an illness coming on soon after a severe mental shock, beginning with an insidious languor and a pain in the back, progressing steadily yet with definite periods of intermission, and attended by critical attacks of faintness and gastro-intestinal disturbance, yet unaccompanied by any noticeable pain anywhere, whether in abdomen, chest, or head.  During the intermissions, the intelligence was acute and the appetite good.  The end came in one of the crises in which faintness was a very noticeable feature.
    No doubt many of the above symptoms might be accounted for by a number of conditions, but there are very few diseases which could account for them all.  There is no symptom indicative of intracranial or intrathoracic disease, unless we regard the attacks of faintness as of cardiac origin.  Nor, apart from bilious attacks, is there any symptom that incriminates the abdominal viscera, and bilious attacks are common accompaniments of various diseases.
    The increasing lassitude and weakness might make us suspect myasthenia gravis, but in this disease we should expect some interference with speech or with chewing of food or even swallowing, and we should not expect the gastro-intestinal disturbances.  Another disease that begins insidiously and has intermissions is subacute bacterial endocarditis, but in this disease gastro-intestinal attacks are uncommon and severe fainting crises rare or unknown.
    There are indeed some abdominal diseases that give no signs and yet may progress and cause no other symptoms than great weakness and anaemia.  Tabes mesenterica and some other forms of tuberculosis should also be considered, but such other conditions are not attended by acute painless crises.  Latent cancer of the stomach might cause severe anaemia and weakness before it became obvious, but should not give rise to prolonged fainting attacks, and with cancer the course is progressively downhill.  Yet after reading all the evidence many times I had almost come to the conclusion that cancer of the stomach would most readily account for most of the symptoms when I bethought myself of two pathological conditions, either of which would account for most of them - Addison's or pernicious anaemia, and Addison's disease of the suprarenal capsules.  Neither of these diseases had at that time been recognized, and when Thomas Addison made his investigations he at first found difficulty in discriminating the one from the other. Both give rise to an insidiously developing weakness and languor, to anaemia, and to severe gastro-intestinal disturbances.  Both are liable to intermissions during which the patient feels much better and is hopeful of recovery.  Yet, in the absence of all laboratory assistance, Addison found one symptom that, in the majority of cases, enabled him to distinguish between the two conditions, and that was the appearance of the skin.  In the disease which he found constantly associated with a pathological condition (usually tuberculosis) of the suprarenal bodies he noted that the skin in certain parts changed to a darker colour, usually brown but sometimes almost black, and the face was nearly always affected. He summarized the main distinguishing features as follows:
    "The leading and characteristic features of the morbid state to which I would draw attention are: anaemia, general languor and debility, remarkable feebleness of the heart's action, irritability of the stomach and a peculiar change of colour in the skin."
    In some cases the dark patches of the skin are mingled with areas showing a lack of pigment - a true black and white appearance.
    Though I had read the letter of 23 March 1817 many times, it was long before I realized the true significance of that symptom which is almost pathognomonic of Addison's disease in Jane Austen's pathetic lament:
    "Recovering my looks a little, which have been bad enough, black and white and every wrong colour."
    Again, when she wrote to her nephew two months later she was distressed that her face had not recovered its beauty.  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.  The disease ran its course rapidly, indicating an active pathological process that might well account for any fever.  Pain in the back has been noted in Addison's disease by several observers.
    If our surmise be correct, Jane Austen did something more than write excellent novels - she also described the first recorded case of Addison's disease of the adrenal bodies.

Zachary Cope.

Note.- The extracts quoted above are taken from Jane Austen's Letters, collected and edited by R.W. Chapman. 2nd ed.

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