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New evidence points to a fatal Hodgkin's disease and excludes the widely accepted Addison's

Jane Austen is typically described as having excellent health until the age of 40 and the onset of a mysterious and fatal illness, initially identified by Sir Zachary Cope in 1964 as Addison's disease. Her biographers, deceived both by Cassandra Austen's destruction of letters containing medical detail, and the cheerful high spirits of the existing letters, have seriously underestimated the extent to which illness affected Austen's life. A medical history reveals that she was particularly susceptible to infection, and suffered unusually severe infective illnesses, as well as a chronic conjunctivitis that impeded her ability to write. There is evidence that Austen was already suffering from an immune deficiency and fatal lymphoma in January 1813, when her second and most popular novel, Pride and Prejudice, was published. Four more novels would follow, written or revised in the shadow of her increasing illness and debility. The nature of her illness had baffled her medical advisors, and still remains a subject of controversy. Current medical opinion, biographers, and encyclopaedic reference all lean towards a diagnosis of Addison's disease, which involves destruction of the adrenal glands, but other medical opinion surmises that Jane may have been suffering from Hodgkin's disease, a form of cancer. Both diseases were unidentified and untreatable in Austen's lifetime, and the outcome was always fatal. Biographers generally date the onset of this fatal illness, to the beginning of 1816, and close to her 40th birthday.2 She would live just 18 months more. The mythic appeal of a 'genius whose life is cut tragically short' magnifies the contrast between the healthy, vital woman, struck down by fatal illness at the height of her creative powers, and the sad, wasted figure she became. Biographers have also tended to follow this line and ignore or trivialise previous episodes of illness that are documented in Jane's letters. There were also episodes of a chronic conjunctivitis that began in her early twenties, and became increasingly frequent in later years, impeding her ability to write. Despite traditional accounts, this was not a case of a healthy person being suddenly struck down with a fatal illness. New medical evidence suggests that Jane was already suffering from an immune deficiency and fatal lymphoma in January 1813, when her second and most popular novel, Pride and Prejudice, was published. Four more novels would follow over the next 5 years, written or revised in the shadow of her increasing illness and debility. The final novel, Persuasion, was published in January 1818. But Jane had died 6 months earlier on 18 July 1817, and a fragment of a new novel lay abandoned in her writing desk. Whilst it is impossible now to conclusively establish the cause of her death, the existing medical evidence tends to exclude Addison's disease, and suggests there is a high possibility that Jane's fatal illness van cleef and arpels bracelet imitation was Hodgkin's disease, a form of lymphoma.The importance of a close examination of Jane Austen's medical history goes far beyond the satisfaction of establishing a retrospective diagnosis for her various symptoms, and intended for an audience of medical professionals. Its significance should be seen rather against a context of the scant documentary evidence of Jane's life, and an intense public interest in everything relating to her. The first biography, a Memoir, was published in 1870. The early trickle of interest since that date has become an avalanche of titles concerning her in the past 50 years, with every known aspect of her life and work placed under scrutiny. But through it all, Jane Austen, the person, remains an enigmatic and elusive figure. There is so much in her life that is missing. She kept no diaries or personal journals, and her most revealing letters were destroyed by her sister Cassandra after her death. Other material was lost or distorted by family members half a century later, in an effort to present a sanitised version of her life, and it is very unlikely now that any new letter or personal remembrance will emerge. The biographer is left to rescan the existing inadequate evidence, and sometimes, very rarely as in this case, the letters yield up something exciting and new. These seemingly nondescript scraps of medical history lead to insights that transcend the barriers of Cassandra's ruthless culling, and at times are startling and almost voyeuristic in their intimacy. Knowledge of the illness provides the detail of symptoms that would have been excluded from the letters. Le Faye, the most recent editor of Jane Austen' Letters, notes that some letters have a line cut out, relating to "physical ailment" and considers that Cassandra simply destroyed any letter that "described physical symptoms rather too fully".4 A detailed medical history reveals the extent to which illness impacted on Austen's life, on family relationships, on her ability to work, and even left its traces on the last manuscripts. It offers a tangible impression of Jane, the woman, coping with illness whenever possible, by cheerfully dismissing it from her mind, and later struggling to maintain her own good humour and a sense of normalcy in her life, as the symptoms of disease overwhelmed her body.Illness was to dominate the first few months of her life as well as the last. Her nephew and first biographer, JE Austen Leigh, faced with an almost total lack of family letters or other evidence was forced to admit that he knew "little of Jane Austen's childhood."5 By some quirk of chance, a family letter describing her birth at Steventon Rectory on 16 December 1775 has survived, and forms an important, first suggestion of a serious medical problem. Jane was born 4 weeks overdue, according to her parents' calculations. Her father even made a joke about it in this letter to his sister in law, Susanna Walter.Dear Sister,You have doubtless been for some time in expectation of hearing from Hampshire, and perhaps wondered a little we were in our old age grown such bad reckoners but so it was, for Cassy certainly expected to have been brought to bed a month ago.6But was it just a case of bad reckoning? It seems very unlikely considering that the Austens had six children already and from the evidence of this letter, had never previously miscalculated their dates. Birth was always a worrying and disruptive time for any family. Childbirth was managed at home without medical assistance, and even if the birth were trouble free, the new mother would be kept in bed for at least 2 weeks. Somebody else would have to run the household and arrangements were made well in advance. It was vital to these arrangements that the estimated confinement date was as accurate as possible. It was simply too important to get wrong.A normal pregnancy is calculated as 40 weeks from the last menstrual period, and over 80% of births occur between the 38th and 42nd week. Only 5% continue, as in Jane's case, after the 43rd week.7 A pregnancy that continues after 42 weeks is considered postdate, and the fetus may be severely at risk. There is a heightened risk of birth injury or death, and over 20% of postdate infants show signs of wasting of tissues a medical condition known as postmaturity, which in severe cases can be fatal. If a pregnancy is postdate, the modern obstetrician will usually order tests to monitor the fetus and to decide whether or not to intervene. In the 18th Century it was a matter of letting nature take its course. If a pregnancy is prolonged, the placenta begins to degenerate and the fetus may receive inadequate nutrients from the mother, resulting in soft tissue wasting.9According to her father's description, Jane Austen seemed normal at birth, and this is quite consistent with postmaturity:Last night the time came, and without a great deal of warning, necklace van cleef arpels imitation everything was soon happily over. We have now another girl, a present plaything for her sister Cassy and a future companion. She is to be Jenny and seems to me as if she would be as like Henry, as Cassy is to Neddy.10The fetus continues to grow in the womb and the infant is long and thin, with some tissue wasting. It is interesting that George Austen noted a close resemblance to his fourth son, Henry, born 4 years earlier. Both children had hazel eyes, but Henry was also very tall for his age.11 and the father must have noticed that this baby too, seemed quite long.A close inspection of the baby may have shown signs of tissue wasting, which caused the skin to hang in loose folds, especially on the arms and legs. There would be signs in behaviour as well, including listlessness, irritability, and feeding problems which would persist for some time, with the baby failing to thrive, and incessantly irritable. Various studies reveal the stress placed on the mother child relationship, with postmature babies described as "difficult" by their mothers. They also had more severe illnesses in the first year of life. These findings also applied to postdate babies who did not show obvious signs of tissue wasting at birth, and the problems were more severe for those born after 43 weeks (Sims, p 291).7Jane would have been frail and probably ill in the first months of her life and this seems to be confirmed by her christening in the church being delayed until she was almost four months old (Le Faye, p 27).3 According to Mrs. Austen's child rearing practice, the baby was weaned at 3 months, and then sent to a "good woman's" for at least a year, a farm worker's wife, who lived in the nearby village of Deane.12 Jane was moved there sometime after her April christening, and certainly before the first week of June, when her parents were on holiday in London.13The circumstances of Jane's birth raise the possibility of an immune deficiency, related to postmaturity, (Beers, p 1026)9 and her medical history of both unusually severe infections and chronic infection, is consistent with an immune disorder. But there is also a compelling argument against this. A child with an immune deficiency in the 18th Century would be extraordinarily lucky to survive to adult life. Most disorders that present from birth would render her susceptible to fatal chicken pox, or if she were lucky enough to survive, she would have been at the very least severely disabled by it. One factor, however, that might have preserved the young Jane's life was the isolated location of her family home, and consequent lack of exposure to childhood epidemics.Steventon, Jane's loved home for the first 25 years of her life, was a remote village in the Hampshire Downs, about 7 miles south west of Basingstoke. Access was by a narrow and winding dirt lane, full of potholes, and in wet weather became virtually impassable. The village itself was just a straggle of farm labourers' thatched cottages, each with its own small garden, and set in a pretty, wooded valley close to a stream and the village green. The rectory was a short distance from the end of the village, and stood on the corner of a 3 acre block, with its own dairy and small farm, and a tiny stone church high on the hill behind it, just glimpsed behind a screen of sycamores and elms.Reverend George Austen leased a nearby 200 acre farm as well as farming his own glebe land. Mrs Austen had her dairy, poultry yard, piggery, vegetable garden, and beehives to supply the house. Bread was baked, and beer was brewed and stored in the cellar, together with van cleef and arpels pendant replica a large supply of Mrs Austen's honey mead and some homemade wine. The rectory's self sufficiency extended to the education of the children. The Austen boys were educated at home by their father, until entering university or the navy. Apart from two brief periods, he supervised the formal education of his daughters as well. The Austen biographer Claire Tomalin notes that in an age when "few families were spared the deaths of several children," the Austens did not lose a single one of their eight children (Tomalin, p 6).2According to family tradition, Jane did become dangerously ill however, within a few months of being sent away to school. In April 1783, Jane, aged 7 and Cassandra, 10, were sent with their 12 year old cousin Jane Cooper to Oxford, to board with Mrs Ann Cawley, the widowed sister of Dr Cooper. Sometime later in the year Mrs Cawley moved her few pupil boarders to Southampton and was soon in the midst of an epidemic of "putrid fever", which was raging in this town by late August. Jane and Cassandra were both infected, but Mrs Cawley did not inform their parents (Le Faye, pp 47 49).3 Fortunately, Jane Cooper wrote to her own mother, who came immediately in her carriage to rescue all three and move them to her home in Bath.14 In some accounts both Mrs Austen and Mrs Cooper arrived to take their daughters home, but all agree that Jane Austen nearly died of this illness. In one version her life was saved by a remedy that the mothers brought with them.15The "putrid fever" was in fact epidemic typhus, brought to Southampton that August by troops returning from service in Gibraltar (Le Faye, p 48).3 It is associated with cramped, insanitary conditions, and transmitted to humans in faeces of the human body louse. The symptoms include a constant headache and high temperature. About the fourth day the characteristic pink spots appear and rapidly darken and cover the whole body, only excluding the face, palms and soles of the feet. After 14 days, in uncomplicated cases, the temperature drops and the patient spontaneously recovers. The disease can be extremely serious in adults, and mortality increases with age, and is as high as 60% in persons aged 50 years or more (Beers, p 1228).9 Mrs Cooper was 47 years of age, and became fatally infected and died at her home in Bath on October 25th. In these fatal cases, death occurs from heart failure about day 14 of the illness.16Mrs Cawley may have been negligent in not contacting the Austens, but perhaps she was aware that this disease is characteristically mild in children up to the age of ten.17 In any case, it would have been out of the question to take the girls home to the rectory, where they might introduce the infection to their brothers, or worse still, Mr Austen's 3 or 4 pupil boarders. The fear of infection in an age when no adequate treatment was available, is reflected in Sense and Sensibility in the Palmer family's immediate abandonment of their home when Marianne Dashwood's illness is described by the apothecary as "having a putrid tendency" and he allows "the word 'infection' to pass his lips."18 Mrs Palmer had left with her baby within the same hour.No other scrap of medical evidence remains from Jane's childhood. The major resource for a medical history consists of her own surviving letters, including 97 to her sister Cassandra, written over a 20 year period. Unfortunately, these only date to 1796, when Jane was already aged 20. Despite Cassandra's censorship, these letters, supplemented by other evidence, do provide medical insights, and reveal for example that Jane suffered from chronic conjunctivitis, with the first episode occurring in January 1799, when she was aged 23:Wednesday I have had a cold and weakness in one of my eyes for some days, which makes writing neither very pleasant nor very profitable, and will probably prevent my finishing this letter myself. My Mother has undertaken to do it for me. . The eyelids would look sore and swollen. This condition is often called "red eye" because of bleeding under the conjunctiva, and the white of the eye becomes bright red either in part, or totally (Macpherson, p 214).16The discomfort of her eye did not prevent her from attending a ball a few nights later, but her account of this ball contains the rueful comment "I do not think I was very much in request People were rather apt not to ask me till they could not help it; One's consequence you know varies so much at times without any particular reason."22Biographers have seized upon this statement as evidence that Jane, unlike the heroines of her novels, was physically unattractive. But a young lady at a ball who was suffering from the 'red eye' was unlikely to be 'very much in request'. It not only affected her appearance. Her partners would be concerned about catching a possible infection. No wonder gentlemen were 'apt not to ask till they could not help it.' Her last comment gives an insight into the way she viewed her own illness. She had dismissed the problem of her sore eye so effectively from her mind, she was unconscious of its effect on others.She attended another ball the following week, with a predictable aggravation of her symptoms.My eyes have been very indifferent. . keeping them so many hours open on Thursday night, as well as the dust of the ball room, injured them a good deal. I use them as little as I can, but you know and Elizabeth knows, and everybody who ever had weak eyes knows, how delightful it is to hurt them by employment, against the advice and entreaty of all one's friends.23

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