Jane Austen's lifelong health problems and final illness: 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. 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 Austen's fatal illness was Hodgkin's disease, a form of lymphoma.

Symptom prevalence and clustering of symptoms in people living with chronic hepatitis C infection

Quality of life has been shown to be poor among people living with chronic hepatitis C However, it is not clear how this relates to the presence of symptoms and their severity. The aim of this study was to describe the typology of a broad array of symptoms that were attributed to hepatitis C virus (HCV) infection. Phase I used qualitative methods to identify symptoms. In Phase 2, 188 treatment-naive people living with HCV participated in a quantitative survey. The most prevalent symptom was physical tiredness (86%) followed by irritability (75%), depression (70%), mental tiredness (70%), and abdominal pain (68%). Temporal clustering of symptoms was reported in 62% of participants. Principal components analysis identified four symptom clusters: neuropsychiatric (mental tiredness, poor concentration, forgetfulness, depression, irritability, physical tiredness, and sleep problems); gastrointestinal (day sweats, nausea, food intolerance, night sweats, abdominal pain, poor appetite, and diarrhea); algesic (joint pain, muscle pain, and general body pain); and dysesthetic (noise sensitivity, light sensitivity, skin. problems, and headaches). These data demonstrate that symptoms are prevalent in treatment-naive people with HCV and support the hypothesis that symptom clustering occurs.

Eye movement problems and differential diagnosis of the parkinsonian syndromes

Differential clinical diagnosis of the parkinsonian syndromes, viz., Parkinson’s disease (PD), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA) can be difficult. Eye movement problems, however, are a chronic complication of many of these disorders and may be a useful aid to diagnosis. Hence, the presence in PSP of vertical supranuclear gaze palsy, fixation instability, lid retraction, blepharospasm, and apraxia of eyelid opening and closing is useful in separating PD from PSP. Moreover, atypical features of PSP include slowing of upward saccades, moderate slowing of downward saccades, the presence of a full range of voluntary vertical eye movements, a curved trajectory of oblique saccades, and absence of square-wave jerks. Downgaze palsy is probably the most useful diagnostic clinical symptom of PSP. By contrast, DLB patients are specifically impaired in both reflexive and saccadic execution and in the performance of more complex saccadic eye movement tasks. Problems in convergence in DLB are also followed by akinesia and rigidity. Abnormal ocular fixation may occur in a significant proportion of MSA patients along with excessive square-wave jerks, a mild supranuclear gaze palsy, a gaze-evoked nystagmus, a positioning down-beat nystagmus, mild-moderate saccadic hypometria, impaired smooth pursuit movements, and reduced vestibulo-ocular reflex (VOR) suppression. There may be considerable overlap between the eye movement problems characteristic of the various parkinsonian disorders, but taken together with other signs and symptoms, can be a useful aid in differential diagnosis, especially in the separation of PD and PSP.

The effects of Gilles de la Tourette syndrome and other chronic tic disorders on quality of life across the lifespan:a systematic review

Gilles de la Tourette syndrome (GTS) and other chronic tic disorders are neurodevelopmental conditions characterized by the presence of tics and associated behavioral problems. Whilst converging evidence indicates that these conditions can affect patients' quality of life (QoL), the extent of this impairment across the lifespan is not well understood. We conducted a systematic literature review of published QoL studies in GTS and other chronic tic disorders to comprehensively assess the effects of these conditions on QoL in different age groups. We found that QoL can be perceived differently by child and adult patients, especially with regard to the reciprocal contributions of tics and behavioral problems to the different domains of QoL. Specifically, QoL profiles in children often reflect the impact of co-morbid attention-deficit and hyperactivity symptoms, which tend to improve with age, whereas adults' perception of QoL seems to be more strongly affected by the presence of depression and anxiety. Management strategies should take into account differences in age-related QoL needs between children and adults with GTS or other chronic tic disorders.

Psychosocial, musculoskeletal and somatoform comorbidity in patients with chronic low back pain: original results from the Dutch Transition Project

International audience

Chronic low back pain, chronic disability at work, chronic management issues.

International audience

Ear-related problems among children attending the paediatric and otorhinolaryngology out-patients clinics of the University of Nigeria Teaching Hospital, Enugu.

Background: Ear related diseases are commonly seen in clinics worldwide especially among children. They are associated with significant morbidity and frequent hospital visits. Limited data exists regarding the burden of ear disease among Nigerian children. Objective: To determine the prevalence of ear-related problems among children presenting at the Paediatric and Otorhinolaryngology clinics of the University of Nigeria Teaching Hospital, Enugu. Materials and methods: This was a cross-sectional study conducted at the Paediatric and Otorhinolaryngology Clinics of the University of Nigeria Teaching Hospital Enugu. All children aged 0 and 17 years presenting between 1st June and 31st August 2006 with ear-related problems were enrolled consecutively into the study. Data analysis was by SPSS version 11. Results: Three thousand and twenty-one children were seen during the study period. Out of these, 248 children (8.2%) presented with ear-related problems. Chronic otitis media (30.5%), acute otitis media (29.9%), cerumen auris (11.3%), otitis externa(10.1%), hearing impairment (7.3%) and foreign body in the ear (5.7%) were the most commonly diagnosed ear-related problems. Conclusion: Ear-related problems among children presenting at the UNTH Enugu were not uncommon. However, otitis media was the most commonly diagnosed ailment affecting the ears in children.

Development of the Andalusian Registry of Patients Receiving Community Case Management, for the follow-up of people with complex chronic diseases.

Background: Complex chronic diseases are a challenge for the current configuration of Health services. Case management is a service frequently provided for people with chronic conditions and despite its effectiveness in many outcomes, such as mortality or readmissions, uncertainty remains about the most effective form of team organization, structures, and the nature of the interventions. Many processes and outcomes of case management for people with complex chronic conditions cannot be addressed with the information provided by electronic clinical records. Registries are frequently used to deal with this weakness. The aim of this study was to generate a registry-based information system of patients receiving case management to identify their clinical characteristics, their context of care, events identified during their follow-up, interventions developed by case managers, and services used. Methods and design: The study was divided into three phases, covering the detection of information needs, the design and its implementation in the healthcare system, using literature review and expert consensus methods to select variables that would be included in the registry. Objective: To describe the essential characteristics of the provision of ca re lo people who receive case management (structure, process and outcomes), with special emphasis on those with complex chronic diseases. Study population: Patients from any District of Primary Care, who initiate the utilization of case management services, to avoid information bias that may occur when including subjects who have already been received the service, and whose outcomes and characteristics could not be properly collected. Results: A total of 102 variables representing structure, processes and outcomes of case management were selected for their inclusion in the registry after the consensus phase. Total sample was composed of 427 patients, of which 211 (49.4%) were women and 216 (50.6%) were men. The average functional level (Barthel lndex) was 36.18 (SD 29.02), cognitive function (Pfeiffer) showed an average of 4.37 {SD 6.57), Chat1son Comorbidity lndex, obtained a mean of 3.03 (SD 2.7) and Social Support (Duke lndex) was 34.2 % (SD 17.57). More than half of patients include in the Registry, correspond lo immobilized or transitional care for patients discharged from hospital (66.5 %). The patient's educational level was low or very low (50.4%). Caregivers overstrain (Caregiver stress index), obtained an average value of 6.09% (SD 3.53). Only 1.2 % of patients had declared their advanced directives, 58.6 had not defined the tutelage and the vast majority lived at home 98.8 %. Regarding the major events recorded at RANGE Registry, 25.8 % of the selected patients died in the first three months, 8.2 % suffered a hospital admission at least once time, 2.3%, two times, and 1.2% three times, 7.5% suffered a fall, 8.7% had pressure ulcer, 4.7% had problems with medication, and 3.3 % were institutionalized. Stroke is the more prevalent health problem recorded (25.1%), followed by hypertension (11.1%) and COPD (11.1%). Patients registered by NCMs had as main processes diabetes (16.8%) and dementia (11.3 %). The most frequent nursing diagnoses referred to the self-care deficit in various activities of daily living. Regarding to nursing interventions, described by the Nursing Intervention Classification (NIC), dementia management is the most used intervention, followed by mutual goal setting, caregiver and emotional support. Conclusions: The patient profile who receive case management services is a chronic complex patient with severe dependence, cognitive impairment, normal social support, low educational level, health problems such as stroke, hypertension or COPD, diabetes or dementia, and has an informal caregiver. At the first follow up, mortality was 19.2%, and a discrete rate of readmissions and falls.

Chronic disease self-management in Aboriginal communities : towards a sustainable program of care in rural communities

The Chronic Disease Self-Management (CDSM) strategy for Aboriginal patients on Eyre Peninsula, South Australia, was designed to develop and trial new program tools and processes for goal setting, behaviour change and self-management for Aboriginal people with diabetes. The project was established as a one-year demonstration project to test and trial a range of CDSM processes and procedures within Aboriginal communities and not as a formal research project. Over a one-year period, 60 Aboriginal people with type-2 diabetes in two remote regional centres participated in the pilot program. This represents around 25% of the known Aboriginal diabetic population in these sites. The project included training for four Aboriginal Health Workers in goal setting and self-management strategies in preparation for them to run the program. Patients completed a Diabetes Assessment Tool, a Quality of Life Questionnaire (SF12), the Work and Social Adjustment Scale (WASAS) at 0, 6 and 12 months. The evaluation tools were assessed and revised by consumers and health professionals during the trial to determine the most functional and acceptable processes for Aboriginal patients. Some limited biomedical data were also recorded although this was not the principal purpose of the project. Initial results from the COAG coordinated care trial in Eyre suggest that goal setting and monitoring processes, when modified to be culturally inclusive of Aboriginal people, can be effective strategies for improving self-management skills and health-related behaviours of patients with chronic illness. The CDSM pilot study in Aboriginal communities has led to further refinement of the tools and processes used in chronic illness self-management programs for Aboriginal people and to greater acceptance of these processes in the communities involved. Participation in a diabetes self-management program run by Aboriginal Health Workers assists patients to identify and understand their health problems and develop condition management goals and patient-centred solutions that can lead to improved health and wellbeing for participants. While the development of self-management tools and strategies led to some early indications of improvements in patient participation and resultant health outcomes, the pilot program and the refinement of new assessment tools used to assist this process has been the significant outcome of the project. The CDSM process described here is a valuable strategy for educating and supporting people with chronic conditions and in gaining their participation in programs designed to improve the way they manage their illness. Such work, and the subsequent health outcome research planned for rural regions, will contribute to the development of more comprehensive CDSM programs for Aboriginal communities generally.

Sisyphus and self-management : the chronic condition self-management paradox

Chronic condition self-management is promoted internationally as not only a possible solution to the health problems of our increasingly chronically ill and ageing population, but as part of a new wave of consumer-led and volunteer-managed health care initiatives. Consumers are now indicating that they want to be more involved in the management of their lives and their health care options, while, especially in rural and smaller communities in Australia, a shortage of clinicians means that health care is rapidly changing. This emphasis on self-management raises crucial questions about where consumer action and control in health care should end and where clinical and medical intervention might begin. Hence, as in the case of Sisyphus and his rock, the self-management process is a difficult and demanding one that poses major challenges and loads for health system reformers and represents a struggle in which new difficulties are constantly emerging. This paper examines some implications of new self-management approaches to chronic illness from an ideological perspective and highlights key elements that underpin the effort to promote health-related lifestyle change. While peer-led self-management programs may assist certain individuals to live engaged and meaningful lives, the essential social and economic determinants of health and wellbeing mean that these programs are not the answer to our urgent need for major reform in the health care arena. Rather, self-management, from an ideological perspective, represents a minor adjustment to the fabric of our health system.

An intervention program for children with school failure problems.

School failure is a chronic problem in many developing countries and in some developed as Portugal due to factors like cognitive deficiencies of children, an inadequate family environment, low SES and bad teaching methods or school organization. It is important to develop effective and simple intervention strategies able to cope with the problem at an individual level independently of the cause. The general objective of the following study is to develop behavioral training techniques to help children with problems of school failure. 6 - 12 years old children attending ISCED 1, with academic difficulties because of family problems, social exclusion (living in poverty or belonging to an ethnic minority) or poor schooling were trained. Cognitive-behavioral techniques that have been widely used to train persons with intellectual, sensorial, physical or social deficiencies were applied. Results are evaluated in terms of % of attained objectives, time, and (subjective) teacher satisfaction. Some results of individual children are shown. Training programs seem to be successful independently of the (mostly unknown) cause of failure of the trained subject.

Health Problems of Institutionalized Elderly

Introduction: human aging is marked by a decrease in the performance of some daily tasks, some even considered banal and imperceptibly when this limitation is followed by chronic diseases, the elderly becomes a source of concern for the family. Objective: identifying the health problems of the elderly living in long-stay institutions from self-reported diseases. This is a descriptive and quantitative study, conducted in northeastern Brazil capital, involving 138 elderly. For data collection we used a questionnaire containing demographic variables, institutional and related to self-reported health problems. Data were evaluated using bivariate analysis and association chi-square. Results: predominance of women was found (61.6%), aged 60-69 years old (39.1%), coming from the state capital (51.4%), and institutional permanence time between 1-5 years (77.5%). The most frequent diseases were related to the cardiovascular system (15.9%) and endocrine, nutritional and metabolic diseases (9.4%). It showed a significant association between self-reported diseases and the age of the elderly (p=0.047). Conclusion: it is expected to raise awareness among health professionals to provide a better assistance to the institutionalized elderly focusing on the real needs of these persons.