(1793–1882), one of Jane Austen's nieces, was the recipient of many letters in which her aunt commented on her illness.
Watercolor painted by
Cassandra Austen. 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. 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. 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. 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. 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, was quick to point out. The main Jane Austen
biographers to support Cope's hypothesis are Jan Fergus 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. from suitors. Mononucleosis is known to be sometimes associated with the later development of Hodgkin's disease. Certain of Jane's symptoms could be interpreted as consistent with Hodgkin lymphoma, such as the onset of facial neuralgia in 1813, presumably following
shingles, and the episode of intense
pruritus at the beginning of 1815. Most consistent with a diagnosis of Hodgkin lymphoma is the cyclic fever characteristic of the terminal period of Jane Austen's illness, being attributed to
Pel-Ebstein fever, a classic symptom of the advanced stages of Hodgkin lymphoma. However, Upfal was forced to appeal to a rare complication of Hodgkin's disease,
thrombocytopenic purpura, to explain such dramatic changes in Jane Austen's skin coloration.
Brill-Zinsser disease '', the body
louse, agent of
pediculosis and
vector of
typhus. Brill-Zinsser disease, a
recurrent form of typhus, was described in the 20th century by two
Americans, first clinically in 1910 by physician
Nathan Brill (1860–1925), then formally linked to its cause in 1934 by bacteriologist Hans Zinsser (1878–1940). Brill-Zinsser disease is usually mild, resembling an attenuated form of
epidemic typhus, with circulatory, hepatic, renal and central neurological disorders. The fever episode lasts 7 to 10 days. The
rash is very mild, if not completely absent. There are, however, severe forms of Brill-Zinsser disease that can lead to death, and which, according to Linda Robinson Walker, are included in the differential diagnosis of the causes of Jane Austen's death.
Tuberculosis , the causative agent of
tuberculosis, stained with the
Ziehl-Neelsen stain.
Tuberculosis can affect the
adrenals, causing Addison's disease. It commonly causes attacks of
intermittent fever, and it can also affect the digestive organs, giving rise to "
tabes mesenterica", a hypothesis briefly considered (but ultimately dismissed) by Cope to explain "gastrointestinal attacks". Annette Upfal pointed out that the
autopsy demonstrated the association of tuberculosis with Hodgkin's disease in 20% of cases. A diagnosis of tuberculosis in Jane Austen (of which Addison's disease of the adrenals would have been one of the consequences) therefore in no way precludes the coexistence of Hodgkin's disease, and would reconcile Cope's and Upfal's respective points of view. Park Honan's analysis in
Jane Austen: A Life (1987) supports this view.
Other hypotheses In addition to digestive tuberculosis, Cope's work considered various diagnostic hypotheses: •
pernicious anemia (Addison's) to explain the pallor and asthenia; •
stomach cancer, to explain the digestive disorders, deteriorating general condition and weight loss; •
myasthenia to explain the fluctuating fatigue and weakness (but he noted that there were no records of any difficulty in speaking, chewing or swallowing). These various hypotheses were refuted in the discussion as failing to explain the changes in skin coloration. Cope cited Jane Austen's letter of March 23, 1817 to Fanny Knight, in which she reported some improvement in her condition: as she put it, "I have recovered some of my appearance, which has been rather ugly, black and white and all colors askew". ==Notes==