Volume 185 Issue 10

Moving beyond the uncertainties of risk, and limited, often difficult, preservation options, will require consensus and collaborative research The remarkable cure rates achieved in childhood cancer mean that large numbers of survivors are currently among the young adult population. However, the treatment that has achieved this success may have adverse effects in many organ systems, including the reproductive organs. These adverse effects may result from the impact of cytotoxic chemotherapy (alkylating drugs such as cyclophosphamide, iphosphamide, procarbazine and busulfan, in particular) on gametogenesis, from radiation damage to the gonads, or from radiation delivered to the hypothalamic–pituitary axis. To preserve fertility, it is necessary to determine the risk of fertility impairment before instituting cancer therapy. Predicting the impact of treatment on reproductive function in individual children based on expected exposures is notoriously unreliable. Current tools, both biochemical and biophysical, are unsuitable for assessing actual reproductive impacts in prepubertal and peripubertal children. Even when pubertal onset and progression is apparently normal, the integrity of gametes may have been compromised. A case–control study of 33 male survivors of childhood cancer showed that only a third had normal semen quality, and even in those who were not azoospermic, there were significant differences from normal controls. Seven of 11 azoospermic young adults were prepubertal at treatment, implying that the prepubertal state does not afford protection.1 Similarly, a study of young adult female survivors treated in childhood, all of whom had regular menses or had a history of normal menses if they were using combined oral contraception, revealed partial reduction in ovarian reserve as shown by elevated follicle-stimulating hormone (FSH) levels, lower anti-Mullerian hormone levels and smaller ovary size on ultrasound in those with spontaneous menstruation, and failure to elevate inhibin B levels in response to FSH stimulation in those using combined oral contraception.2 On the other hand, pregnancies have been noted in individuals predicted to be sterilised by their exposures to cancer therapies. The treatment protocols applicable to a child’s particular diagnosis may suggest higher risk. Those exposed to dose-intensive regimes of cyclophosphamide3 and other alkylators, particularly those with Hodgkin’s lymphoma treated with MOPP (mustine hydrochloride [nitrogen mustard], Oncovin [vincristine], procarbazine and prednisone) and other alkylator-intense regimens, including those with metastatic sarcomas, or bone marrow transplantation, are at increased risk. Others at higher risk are those who need pelvic or testicular irradiation, or total body irradiation before marrow transplantation. At the other end of the spectrum, children about to commence therapy on low-intensity protocols, such as those used for low-stage Wilms’ tumour or acute lymphoblastic leukaemia, are at minimal risk of infertility. However, a substantial proportion of children beginning treatment fall into an intermediate risk category for which prediction is fraught with inaccuracy. The cut-off of 7.5 g/m2 of cyclophosphamide recommended by the Children’s Oncology Group as the exposure level to trigger screening for fertility is a reasonable approximation of elevated risk, but not a reliable predictor of outcome at the beginning of therapy. Given the uncertainty of predicting fertility outcomes, what options exist for preserving fertility in children facing cancer therapy, and how should parents and patients be counselled? Cryopreservation of semen and subsequent in-vitro fertilisation is the only standard option for postpubertal males, and spermarche is the watershed around which options for boys are defined. Spermarche typically is an early to mid-pubertal event and occurs before the ability to achieve ejaculation.4 In the mature adolescent, semen is usually obtained by masturbation, with electrostimulation or vibratory stimulation as alternatives (the latter two may be applicable in peripubertal boys). However, the rate at which viable samples are obtained is highly variable. These adolescents are often sick as well as embarrassed and uncomfortable. One study of 62 attempts by adolescents to bank sperm before therapy resulted in totally normal semen in only four.5 Adolescents may be more successful if unaccompanied by parents.6 The advent of intracytoplasmic sperm injection (ICSI) enables in-vitro fertilisation of ova with even single sperm and so, despite the low yield rate, semen cryopreservation should be encouraged — as appears to be the case in the survey of practice in Australia and New Zealand by Heath and Stern in this issue of the Journal.7 Whether ICSI will increase the incidence of abnormalities in the offspring of cancer survivors remains to be seen — the incidence of abnormalities in offspring of cancer survivors conceived by natural means is not elevated.8,9 However, ICSI may bypass the normal protective mechanisms which terminate abnormal embryos. In adult men unable to produce semen for cryopreservation, harvesting of testicular sperm has been undertaken, either from testicular biopsy or percutaneous aspiration. However, in the presence of circulating cancer cells, breaching the blood–testicular barrier may pose the risk of testicular cancer recurrence analogous to the increased rate of central nervous system leukaemia after traumatic lumbar puncture. Of interest, even when sperm is banked, studies in adults suggest that a small proportion of men (10%–30%) retrieve and use the banked specimen.10 For prepubertal males, no current routine option exists. Approaches that may develop include testicular tissue cryo-preservation, and germ cell cryopreservation and autografting. Both are entirely experimental at this time.11 For female patients, the options are more limited. For mature women, oocytes can be harvested and fertilised, and resulting embryos preserved. However, this requires at least 2 weeks of hormonal preparation (daily injections of FSH), and an existing sperm source. Similarly, oocytes can be preserved and subsequently fertilised when a sperm donor is available, although success rates are lower.12 Neither technique is applicable to children or adolescents. As ovarian tissue contains significant numbers of primordial follicles in younger females, the harvesting and storage of ovarian tissue (preferably removed as ovarian cortical strips by laparoscopic techniques) before starting cancer therapy, with subsequent autotransplantation, is an experimental option. There is some evidence of successful restoration of hormone production and two reports of successful pregnancy.13,14 Which patients should be subjected to such invasive techniques needs clarification and consensus, and this procedure should only be undertaken under strict clinical trial conditions in centres with the necessary expertise. The risk of reintroducing cancer cells has not been accurately assessed, but is a real possibility.11 In the face of uncertainty and limited options, many of which are invasive, the paediatric oncology community has been slow to embrace routine and consistent counselling of families about fertility preservation options. Heath and Stern have demonstrated this hesitation among Australian and New Zealand oncologists,7 and similar results have been obtained in North America.15 To be able to provide effective counselling and improve pre-emptive interventions to preserve fertility, paediatric oncologists must inform themselves of the options for their patients, forge links with paediatric endocrinologists and reproductive medicine specialists, define at-risk patients by consensus, and commit to participating in research in this area — as they have already done so well in the context of defining best cancer treatments.

Mark L Greenberg MB ChB, FRCPC · Stacey L Urbach MD, MPH, FRCPC

There is now an inexpensive first-line approach for diagnosing pigmented skin lesions Over the past decade, the number of published reports on new technologies for diagnosing primary melanoma and other pigmented lesions of the skin has grown exponentially. However, there is still confusion about the relative merits of the various technologies and in which patient setting they are best used. The technologies discussed here are dermoscopy (also known as surface microscopy, epiluminescence microscopy, or dermatoscopy) and its digital modifications. In dermoscopy, hand-held magnification devices (usually × 10 magnification), together with either application of liquid at the skin–microscope interface or the use of cross-polarising filters that require no liquid, allow the visualisation of morphological features not seen with the naked eye. This increases the accuracy of diagnosis for virtually all pigmented skin lesions, including melanoma.1,2 In a meta-analysis of 13 studies comparing dermoscopy with the naked-eye examination, the mean sensitivity for the diagnosis of melanoma (percentage of melanomas correctly diagnosed) increased by 19%, and the mean specificity (percentage of non-melanomas correctly diagnosed) increased by 6.2%.3 While this analysis suggested that dermoscopy did not markedly increase specificity, other studies have shown it to have a dramatic effect on reducing biopsy rates. In a clinical trial of dermatologists randomly allocated to either naked-eye examination or naked-eye examination plus dermoscopy, there was a 42% reduction in the number of patients referred for biopsy in the dermoscopy arm of the trial.4 This trial followed the observation that, for dermatologists trained in the use of dermoscopy, there was a significant reduction in the benign to malignant ratio of excised melanocytic lesions — from 18 : 1 (pre-dermoscopy era) to 4 : 1 (post-dermoscopy era).5 The impact of dermoscopy has also been assessed in general practice. In a study of the diagnosis of melanoma using dermoscopy by Australian general practitioners, my colleagues and I reported a 39% improvement in sensitivity. No improvement was noted in specificity.6 Our finding has been reproduced in a recent clinical trial of primary care providers in Europe.7 The major endpoint of the trial was the result of re-evaluation by two participating specialists of lesions identified as suspicious. The percentage of suspicious lesions correctly detected (sensitivity) increased by 46% in the dermoscopy group. Again, the specificity remained unchanged. Furthermore, there was a significant improvement in the identification of malignant lesions by the dermoscopy group (although the trial lacked adequate power to detect differences in melanoma diagnosis). Since 2000, there has been an increasing interest in digital dermoscopy sequential imaging.1,2 Digital (computerised) dermoscopy monitoring devices take digital dermoscopy images and allow tiling on the computer screen for comparing change in melanocytic lesions over time. Such devices are used in two clinical settings. First, short-term digital monitoring over a 3-month period is used to monitor suspicious melanocytic lesions lacking features of melanoma on dermoscopy. Patients may present with changing common or mildly atypical naevi, or atypical naevi without an accompanying history of no change in their appearance. In contrast, patients with multiple atypical naevi are monitored for periods of 6–12 months (long-term monitoring). Both these monitoring techniques have been shown to detect dermoscopically featureless melanoma.2 In a large series of 91 melanomas detected by sequential digital monitoring in Australia and Europe, more than half of the lesions were in situ and all were less than 1 mm thick, indicating the safety of monitoring lesions over time.8 Recently, the impact of digital dermoscopy was demonstrated in a cohort of patients at high risk of primary melanoma; 34% of melanomas detected lacked dermoscopic features of melanoma and were exclusively detected by sequential digital dermoscopy monitoring.9 With the realisation that melanoma is a relatively uncommon presentation in general practice and may be suboptimally diagnosed even in a specialist setting, automated diagnostic instruments have been developed that require no diagnostic input by the operator. While many are commercially available, such devices are at various stages of development, so that attempting to compare their impact on diagnosis with that of any other technology in the field is difficult.2 Nevertheless, comparisons can be made of the various instruments by contrasting ideal requirements, as outlined elsewhere.10 While it is difficult to draw robust conclusions about the impact of such instruments in the absence of supporting clinical studies, such studies are being performed and their results are eagerly awaited. In summary, dermoscopy has been shown to improve both the sensitivity and specificity of the diagnosis of melanoma by specialists and to improve the sensitivity of melanoma diagnosis by GPs. As it is an inexpensive technique, it should be recommended to all clinicians as a first-line approach for diagnosing pigmented skin lesions. Sequential digital dermoscopy monitoring devices are readily available and are restricted to diagnosing melanocytic lesions (naevi and melanoma). They have been shown to allow the detection of dermoscopically featureless melanoma in any patient presentation, but, to date, studies have only been performed in a specialist setting. Such devices are more expensive and may currently be beyond the reach of general practice. Nevertheless, a clinical trial on the impact of dermoscopy and sequential digital dermoscopy monitoring on excision rates or patient referrals for biopsy by Australian GPs is due to be completed at the end of this year.

Scott W Menzies MB BS, PhD

New guidelines extend opportunities for GPs to respond In July 2006, new international consensus clinical guidelines — Management of the whole family when intimate partner violence is present: guidelines for primary care physicians — were launched simultaneously in Melbourne by the Victorian Community Council on Crime and Violence and at the General Practice and Primary Health Care Research Conference in Perth (Box).1 Partner violence is prevalent globally, exacerbated by poverty, war and gender inequality.2 In Australia, while 3% of women in the community report partner violence in the previous 12 months, the proportion among primary care patients is 8%.3 Men can also be victimised, but the evidence suggests that women suffer most of the significant harm, especially in the early child-rearing years, affecting the health of the whole family. Partner violence is no less prevalent among gay and lesbian families, and Indigenous families are particularly at risk of harm from partner or family violence, including murder of female partners.4 Abused women are more likely than non-abused women to experience physical and psychological symptoms and seek health care for stress-related and chronic ailments.5 There is also evidence of the damaging effect partner violence can have on children’s emotional, behavioural and cognitive development, as well as on their physical and mental health.6 Perpetrators can exhibit significant comorbidities, especially drug and alcohol misuse, and there is growing concern about early childhood development in this context. Increasing rates of depression and mental illness focus attention on the contribution partner violence makes to adverse social and economic circumstances.7 With a pattern of poor health and increased health care attendances by victims, perpetrators and their children, there are potential opportunities for health care providers to intervene. However, barriers to identification and management include lack of training, time and effective interventions.8 Advice for general practitioners in the medical literature focuses mainly on victims; there is a little on abusive male partners, but children or the wider dilemmas of whole-family management are rarely included.9 As recent systematic reviews concluded that there was inadequate evidence to guide clinical care,10 a Melbourne group of primary care researchers brought together, in a rigorous consensus process, an international collaborative team of clinical experts in partner violence. A systematic review of existing guidelines identified those of best quality, when assessed according to the Appraisal of Guidelines Research and Evaluation (AGREE).11 AGREE helps readers assess, firstly, whether the potential biases of guidelines development are adequately addressed; secondly, whether the recommendations are externally and internally valid; and finally, whether they are feasible for practice. Recommendations endorsed by more than three guidelines were supplemented by those addressing key gaps. These gaps included advice about investigating harm to children and adolescents, and parenting issues (addressing any parenting difficulties that the victim faces as a consequence of the abuse). Further gap recommendations deal with clinic management, including training of all staff in safety protocols when doctors are seeing different members of the family. With each recommendation, further narrative offers clarification and practical advice to strengthen the recommendation’s applicability. The controversial issue of screening had experts in Europe, the United Kingdom, Canada and Australia arguing that the guidelines should recommend case finding only. This was based on the need to obtain evidence that intervention does not harm women and children in the longer term, and the available evidence that practitioners are largely untrained and unsupported. In contrast, participants from the United States believed that, in view of the prevalence of the problem, screening was still vital, and that not screening was bordering on unethical. The majority recommendation resolved that physicians should routinely ask all pregnant women and girls about partner violence, because of their particular vulnerability and the association between partner violence and adverse pregnancy outcomes; they should undertake case finding with all other women and with men. While primary care intervention trials are only just underway, these guidelines offer the best current advice. Clinicians need to be mindful of the range of issues within the family that they may face. Many doctors continue to feel that asking about partner violence is “opening Pandora’s box”.8 There is increasing evidence that partner abuse is an underlying issue in many serious, recurrent symptoms in primary care, and that the damage to the family’s health creates continuing harm. Consequently, federal and state governments should ensure that doctors are provided with sustainable and effective training, support and resources to play their part in society’s efforts to prevent ongoing generations of damaged families. The partner violence whole-family guidelines are endorsed by the Royal Australian College of General Practitioners and available on the College’s website.1 Key selected recommendations from the guidelines on managing partner violence (as numbered in the document)1 Screening 1. Family practitioners should routinely ask all pregnant adult and adolescent women about partner violence 2. In other situations, doctors should ask patients with symptoms of partner violence and those with symptoms of abusive behaviour (case finding only) Men 5. Encourage a patient who has disclosed their abuse of a partner to take responsibility for their behaviour and change Children 14. Discuss any parenting concerns in the partner abuse context 15. Assess the risk to and adult perception of the impact on children 16. Consider the risk to and children's perception of the impact on their lives 17. Consider children's access to significant supportive others Clinic 27. Seek own and staff family violence training for management of all family members experiencing violence 29. Use a clinic protocol for monitoring danger to patient and other family members by any clinician seeing patient

Angela J Taft PhD · Kelsey L Hegarty MB BS, PhD · Gene S Feder MD, FRCGP

Objective: To establish the extent to which sperm, oocyte and gonadal tissue collection and storage is offered to children newly diagnosed with cancer.Design, participants and setting: A cross-sectional survey of all paediatric oncology services in Australia and New Zealand (ANZ) in December 2005.Main outcome measures: Sperm, oocyte and gonadal tissue collection and storage practices at paediatric oncology services; comparisons with recently published North American practices and with current recommendations for best practice.Results: 12 of the 13 centres (92%) completed the survey. All centres offered sperm preservation, but only 10 (83%) offered oocyte/ovarian tissue preservation. Two centres were using gonadotrophin-releasing hormone analogues for fertility protection in postpubertal females. Five (42%) had offered fertility preservation to patients before the completion of their sexual development. All centres were more likely to offer sperm preservation than oocyte preservation for any given disease. The most common diseases for which conservation was offered were lymphomas and sarcomas. The anticipated cumulative dose at which centres elected to offer fertility preservation varied widely, both for the alkylator cyclophosphamide (any to 10 g/m2) and for abdominal/pelvic irradiation (any to 12 Gy) and spinal irradiation (any to 18 Gy). Fertility counselling was offered in a variety of settings by nine (75%) of the centres. Despite 11 centres (92%) agreeing that fertility preservation guidelines would be helpful, only two (17%) had guidelines in place.Conclusions: There are inconsistencies in the indications for and methods of gamete conservation in paediatric oncology centres throughout ANZ. Variations in practice on a background of unresolved medical, legal and ethical issues suggest the development of guidelines would be helpful.

John A Heath PhD, FRACP · Catherine J Stern MB BS, FRACOG

Objective: To ascertain whether current practice in teaching hospitals in New South Wales and the Australian Capital Territory delivers adequate dietary protein in the management of malnutrition in adults with cirrhosis, in accordance with European Society for Clinical Nutrition and Metabolism (ESPEN) guidelines for nutrition in liver disease.Study design: Cross-sectional study of dietitians using a self-administered, mail-back survey.Setting: Teaching hospitals in NSW and the ACT treating patients with cirrhosis.Participants: Dietitians seeing patients with cirrhosis in the 12 months prior to completing the survey.Main outcome measures: Current dietary protein prescription practice for patients with cirrhosis (with and without hepatic encephalopathy); use of nutritional supplements and enteral feeding for malnourished patients with cirrhosis.Results: Dietitians following the ESPEN guidelines were in the minority: 36% of the dietitians recommended an adequate protein intake for patients with hepatic encephalopathy. Sixty-four per cent of the dietitians had received referrals from the medical team requesting inappropriate protein-restricted diets for patients without hepatic encephalopathy. Seventy-eight per cent of the dietitians requested clarification of the recommended nutritional management of patients with cirrhosis.Conclusion: Many medical and dietetic staff inappropriately restrict protein intake of patients with cirrhosis.

Joanne K Heyman BSc(Hons) · Carol J Whitfield RN · Kaye E Brock PhD · Geoffrey W McCaughan MB BS, PhD · Anthony J Donaghy MB BS, PhD

Off-label prescribing is the prescription of a registered medicine for a use that is not included in the product information. The practice is common, with rates up to 40% in adults and up to 90% in paediatric patients. Off-label prescribing is not illegal and may sometimes be clinically appropriate, but is associated with a number of clinical, safety and ethical issues. To date, no explicit guidance has been available to help clinicians assess appropriateness in off-label prescribing. We describe the development of a guide for clinicians, policymakers and funders of health care in evaluating the appropriateness of medicines proposed for off-label use. Three broad categories of appropriate off-label use are identified:off-label use justified by high-quality evidence; use within the context of a formal research proposal; and exceptional use, justified by individual clinical circumstances. An appropriate process for informed consent is proposed for each category. If there is no high-quality evidence supporting off-label use, and the medicine is not suitable for exceptional or research indications, its use is generally not recommended. This will reduce inappropriate use, enhance patient safety by reducing exposure to unnecessary risk, and may stimulate more clinically relevant medicines research.

Madlen Gazarian MB BS(Hons I), MSc(ClinEpi), FRACP · Maria Kelly BPharm, DipEd · John R McPhee BCom(Hons)(LegStud) · Linda V Graudins BPharm, DipHospPharm, FSHP · Robyn L Ward MB BS(Hons I), PhD, FRACP · Terence J Campbell MD, PhD, FRACP

Chronic heart failure (CHF) is found in 1.5%–2.0% of Australians. Considered rare in people aged less than 45 years, its prevalence increases to over 10% in people aged ≥ 65 years. CHF is one of the most common reasons for hospital admission and general practitioner consultation in the elderly (≥ 70 years). Common causes of CHF are ischaemic heart disease (present in > 50% of new cases), hypertension (about two-thirds of cases) and idiopathic dilated cardiomyopathy (around 5%–10% of cases). Diagnosis is based on clinical features, chest x-ray and objective measurement of ventricular function (eg, echocardiography). Plasma levels of B-type natriuretic peptide (BNP) may have a role in diagnosis, primarily as a test for exclusion. Diagnosis may be strengthened by a beneficial clinical response to treatment(s) directed towards amelioration of symptoms. Management involves prevention, early detection, amelioration of disease progression, relief of symptoms, minimisation of exacerbations, and prolongation of survival.

on behalf of the CHF Guidelines Core Writers

A recent High Court decision held that children born with disabilities not caused by medical intervention, but not diagnosed antenatally, could not claim general damages for their pain and suffering, nor special damages for the needs created by their disabilities and their loss of earning capacity. The law has regularly struggled with how to deal justly with disability associated with medical interventions, particularly in relation to competence and consent, as well as causality and compensability. Terminology and concepts10 Wrongful birth — a “wrongful birth” action is one brought by the parent(s) of an initially unwanted or unintended child, born (with or without disability) as a consequence of negligence before birth.11 This was the situation in Cattanach v Melchior, a case of negligent sterilisation.7 Wrongful life — a “wrongful life” action is “. . . one brought by (or on behalf of) a child complaining of negligent conduct before birth which results in its birth when had there been no negligence it would not have been born. In short, the essence of the claim is that the child would have been better off not to be born at all.”11 The defendant medical practitioner does not cause the disability but, rather, fails to avert it. This was the situation in Harriton v Stephens8 and Waller v James.9 In both types of actions, the breach of duty may occur before conception (as was the situation with Waller v James, where the child’s father was not advised that he had a heritable condition), ex utero (eg, in vitro or in relation to fertility treatment), or in utero (eg, in failure to diagnose disability).11 For example, at least since the landmark litigation in the 1970s arising out of the effects of thalidomide,1,2 courts have trod warily in defining what is actionable and what is compensable. In the 1990s, leaving aside the ongoing ramifications of the Rogers v Whitaker decision,3 perhaps the most controversial medicolegal cases dealt with by the High Court of Australia were the decisions in Marion’s Case4 and P v P,5 both of which concerned the rights of children with profound disability, and whether, in their alleged best interests, they should be sterilised. Following judgment in both cases authorising the sterilisation, there was significant and understandable dispute about what enhanced, and what impaired, the human dignity of those with disability.6 Courts have endeavoured to be careful in recognising and awarding damages in novel areas of law, such as “wrongful birth” and “wrongful life”. The High Court of Australia has recently given judgment in both kinds of action: in 2003 allowing a claim for wrongful birth (Cattanach v Melchior),7 but in May 2006 disallowing two separate claims for wrongful life (Harriton v Stephens8 and Waller v James/Waller v Hoolahan9). Here, we briefly summarise the wrongful birth action,7 and then examine in detail the most recent High Court judgment on claims for wrongful life,8,9 which involved the intersection of congenital disability, claims for negligence and the award of damages. The terms “wrongful birth” and “wrongful life” are defined in the Box. The High Court and wrongful birthIn the 2003 claim for wrongful birth (Cattanach v Melchior),7,12 the High Court of Australia by a bare majority of 4 : 3, and contrary to recent authority in the United Kingdom,13 held a consultant obstetrician and gynaecologist, who had negligently performed a sterilisation procedure, liable for the costs of raising the child born subsequent to the failed procedure. The finding of negligence was relatively unproblematic.7,14 However, what did cause concern was the judgment that the medical specialist was liable for the costs of raising the healthy, but initially unwanted, child. Understandably, the decision unleashed a significant tide of comment.15,16 The then Premier of New South Wales, Bob Carr, called on the federal government to introduce legislation “that protects doctors in other categories, in private medicine . . .”.17 One immediate consequence of the Cattanach v Melchior judgment was the decision by three states — NSW, Queensland, and South Australia — to enact legislation that now prevents the bringing of wrongful birth suits in those jurisdictions (Civil Liability Act 2002 (NSW), s.71; Civil Liability Act 2003 (Qld), s.49A; Civil Liability Act 1936 (SA), s.67). The High Court and wrongful lifeThe 2006 decision of the High Court in Harriton v Stephens and Waller v James/Waller v Hoolahan, this time by a majority of 6 : 1, rejecting two separate claims for wrongful life, is certain to be welcomed by medical practitioners and politicians alike. The High Court resoundingly rejected claims for damages by two disabled children. The claims were on the basis that they would have been better off not being born rather than being born with their severe disabilities.8,9 The child appellants argued that their births were the result of negligent action by the treating medical practitioners and they sought compensation for being born with disabilities. Justice Susan Crennan summarised the issue at the end of her leading judgment in Harriton v Stephens: Cattanach v Melchior [in 2003] represents the present boundary drawn in Australia by the common law . . . in respect of claims of wrongful birth and wrongful life. Life with disabilities, like life, is not actionable [par. 277].8 Procedural anomaly and evidenceBefore going to the High Court, these cases were presented to the NSW Supreme Court, and this Court was asked, based only on an agreed set of facts, to determine whether the novel claim for wrongful life could, and should, be recognised. If this was so, the Court was then asked to assess what damages would flow from such a finding. The two children involved, Alexia Harriton and Keeden Waller, were unsuccessful in their claims. Later, in the High Court, reservations were expressed about the lack of a formal trial before the Supreme Court hearing and the difficulties that ensued in properly evaluating the untested evidence presented in the agreed set of facts. As well, the agreed set of facts was somewhat lacking in clinical detail. Indeed, the agreed set of facts posed a range of unanswered questions: for example, why was Dr Paul Stephens advised to concede that he was negligent in reassuring Alexia Harriton’s mother that she did not have rubella when the pathology report was very vague in its detail? Also not explained in any judgment is how or why Alexia’s parents’ claims were out of time and therefore statute-barred. The agreed factsHarriton v StephensAlexia Harriton was conceived naturally. In August 1980, her mother, Olga, contracted a fever and noticed a rash. She also suspected that she was pregnant. She went to her general practitioner, Dr Max Stephens, and told him of her concern about being pregnant and possibly having had rubella. Dr Stephens recommended that Mrs Harriton have a blood test to determine the pregnancy and the likelihood of having contracted rubella. The testing was done by Macquarie Pathology Services. Dr Stephens noted the clinical history of the patient as: “Urgent? Pregn? Recent rubella contact.” Nine days later, Mrs Harriton saw Dr Paul Stephens, the son of Dr Max Stephens. He had the report from Macquarie Pathology Services. It read: Rubella – 30; if no recent contact or rubella-like rash, further contact with this virus is unlikely to produce congenital abnormalities. Preg test – positive. Dr Paul Stephens advised Mrs Harriton that she was pregnant; he also assured her that she had not contracted rubella. In the agreed statement of facts presented to the Court, it was common ground that Dr Paul Stephens had been negligent in advising Mrs Harriton in the way that he did, and that he had, accordingly, breached his duty of care to her (i) in his advice that she did not have rubella, and (ii) in failing to arrange a more detailed blood test. It was also agreed that, in 1980, a reasonable medical practitioner in the position of Dr Stephens would have advised Mrs Harriton of the high risk of her child in utero having been exposed to rubella and, therefore, the risk of it being born profoundly disabled. The parties also agreed that had Mrs Harriton received competent medical advice, she would have terminated the pregnancy. Alexia was born with, and continues to suffer from, what the courts have described as “catastrophic disabilities”, which include blindness, deafness, mental retardation and spasticity. Waller v James/Waller v HoolahanKeeden Waller was conceived via in-vitro fertilisation (IVF) pro-cedures in August 2000. Keeden’s father suffered from “Factor III [antithrombin III; AT3] deficiency” as well as a low sperm count and poor sperm motility. Deficiency of AT3 is generally a hereditary condition, and is characterised by a predisposition to thrombosis. Mr Waller notified the family’s GP about the AT3 condition, and the GP in turn notified Sydney IVF Pty Ltd. Keeden’s father was taking warfarin daily for the AT3 deficiency. Tests were conducted to determine whether there was a genetic reason for the condition of his sperm. Mr and Mrs Waller were advised to proceed with intracytoplasmic sperm injection. This course was accepted. After collection of 19 eggs from Mrs Waller, and sperm from Mr Waller, 17 eggs were successfully fertilised. Two embryos were transferred to Mrs Waller and, on confirmation that she was pregnant, Mrs Waller was referred to Dr Hoolahan, an obstetrician and gynaecologist. One embryo implanted successfully. Subsequently, on the recommendation of Dr Hoolahan, the fetus Mrs Waller was carrying was tested for Down syndrome. This test proved negative. There was no screening of the fetus for Mr Waller’s AT3 condition. Nor were Keeden’s parents advised of the possibility that it was an inheritable condition. Mrs Waller’s labour was protracted. Keeden was diagnosed, 5 days after his birth, with cerebral thrombosis. As a consequence, he suffers from cerebral palsy and has uncontrolled seizures. He also has AT3 deficiency, inherited from his father. It was alleged that the defendants in this case, namely the treating obstetrician, Sydney IVF, the hospital at which Keeden was delivered, and others who attended Mrs Waller in the course of her delivery, should have advised her of the risk of AT3 deficiency being passed on to any offspring. Consequently, it was argued that, had Keeden’s parents been advised of this risk, they would have had the pregnancy terminated. The judgments of the High CourtJustice Crennan wrote the leading judgment in both cases. Gleeson CJ and Gummow, Hayne and Heydon JJ all concurred with her reasons. Callinan J wrote short separate judgments in both appeals which also found against the children in their claims. Kirby J was the sole dissentient in both matters. He accepted that a “compensatory principle” should apply so as to allow recovery. The remainder of this article considers the reasoning of the Court and what it might portend for the future. A crucial issue for the dissentient, Kirby J, was that Denying the existence of wrongful life actions erects an immunity around health care providers whose negligence results in a child who would not otherwise have existed, being born into a life of suffering [par. 153].8 While contentious, especially given that the High Court has never claimed or sought to quarantine health care providers, or anyone else for that matter, from the general operation of the law in relation to negligence, Kirby’s approach is consistent with the majority judgment in Cattanach v Melchior,7 but not with recent decisions in the UK.18 The crucial question for the majority of the Court was whether Alexia and Keeden suffered “damage” which was recognisable at law. The antecedent questions of whether either child was owed a duty of care, and whether there had been a relevant breach of that duty, were effectively subsumed by the focus of most of the majority Justices on whether there was damage recognisable by the law for which the defendants should be held liable. The duty postulated by Alexia was that Dr Paul Stephens should have diagnosed rubella and then advised Mrs Harriton to terminate the pregnancy. This duty also proposed that the measure of damages required that an assessment should be made so as to compensate Alexia to the degree necessary to place her in the position she would otherwise have been in but for the negligence of Dr Paul Stephens. That comparative position was one of “non-existence”. The High Court held that the law does not, and could not, recognise “non-existence” as a relevant comparator and, therefore, there was no compensable damage. Hayne J said emphatically: It is because the appellant [Alexia] cannot ever have and could never have had a life free from the disabilities she has that the particular and individual comparison required by the law’s conception of “damage” cannot be made [par. 172].8 After reviewing judgments from both common and civil law jurisdictions around the world (in which, with very few exceptions, claims for wrongful life have been rejected), and that Alexia had no cause of action against Dr Paul Stephens, Crennan J accepted the remarks of the Chief Justice of NSW, James Spigelman, when he said in his judgment in the NSW Court of Appeal that . . . in cases of this kind, to find damage which gives rise to a right to compensation it must be established that non-existence is preferable to life with disabilities [par. 251].8 Crennan J rejected arguments that (a) life with disability was a devalued life — rather, Alexia’s disabilities “are only one dimension of her humanity”; (b) a “new compensatory principle” required the awarding of damages in this case; and (c) a principle of “corrective justice” in this case would, and should, overcome the difficulties inherent in the claim.19 In relation to the rights of the parents, Alexia’s parents were statute-barred. The wrongful life action of Keeden’s parents has yet to be determined. ConclusionSo what does this case mean for the law and for medical practice? Firstly, it certainly does not diminish the responsibility of medical practitioners in their duty of care owed to patients, especially pregnant women. Secondly, it is clear that a “line in the sand” has been drawn as to what the law will recognise as compensable damage. As Crennan J said: To have a cause of action in negligence [Alexia] needs to show damage suffered by her and a duty of care on Dr P R Stephens to avoid that damage [par. 218].8 The Court pointed out that Dr Stephens did not cause the damage suffered by Alexia; that was caused by her mother’s rubella. Thirdly, it is more than a philosophical or jurisprudential point made by the Court and worthy of further consideration — that non-existence cannot be compared favourably to living a life with disabilities (Disability Discrimination Act 1992 [Cwlth]).20 As Gleeson CJ said in one of the three dissenting judgments in Cattanach v Melchior: The value of human life, which is universal and beyond measurement, is not to be confused with the joys of parenthood, which are distributed unevenly.7 This was undoubtedly the case here. Perhaps the larger question then is what, for a community, is the responsibility owed to people with disability and those who care for them? That is a question that politicians, judges and the rest of us must grapple with.

Warwick J Neville BA, LLB, STD · Buddhima Lokuge MB BS, MPH

A veterinarian became infected with Hendra virus (HeV) after managing a terminally ill horse and performing a limited autopsy with inadequate precautions. Although she was initially only mildly ill, serological tests suggested latent HeV infection. Nevertheless, she remains well 2 years after her initial illness. Recently emerged zoonotic viruses, such as HeV, necessitate appropriate working procedures and personal protective equipment in veterinary practice. Hendra virus (HeV) and Nipah virus together comprise the genus Henipavirus within the family Paramyxoviridae (Box 1).1 HeV, formerly called equine morbillivirus, was first described after an outbreak of severe respiratory disease in horses, leading to the deaths of 14 horses and a horse trainer in Brisbane in September 1994.2,3 The trainer had had very close manual contact with frothy nasal and oral secretions, some of which were blood-tinged, from several of the very ill horses, as did a stable-hand; he developed an influenza-like illness but made a full recovery. The horses and both people were infected with HeV.2,3 An earlier outbreak of HeV disease was not recognised until the death, in 1995, of a farmer in Mackay. He had assisted his veterinarian wife with the autopsies of two horses that died suddenly from unknown cause in August 1994. He was hospitalised about 2 weeks later with an aseptic meningitis, from which he apparently made a full recovery.3,4 However, about a year later he became acutely unwell again, and died from a severe encephalitis caused by HeV.3,4 The two horses were (retrospectively) shown to have also been infected with HeV.5 After these two outbreaks, extensive investigations identified fruit bats (Pteropus spp.), commonly known as flying foxes, as the likely natural reservoir of HeV; the infection is probably subclinical in most infected flying foxes.6,7 Although it is not certain how HeV could be transmitted from flying foxes to horses, the isolation of the virus from uterine fluid and aborted fetal tissue of flying foxes suggests that horses could ingest the virus on feed or pasture recently contaminated by birth products (Box 2).6,7 The available evidence indicates that transmission of the virus from horses to people, albeit rare, occurs through physical contact with nasal and oral secretions emanating from very ill, dying or dead horses.8 The third recognised outbreak of HeV disease occurred in January 1999, when a horse in a northern suburb of Cairns died from pneumonia.9 No human infection associated with this equine HeV case was detected. We report here the fourth known outbreak of HeV infection and discuss the implications of this recently emerged virus for veterinary practice. Clinical recordIn early November 2004, a veterinarian notified the Tropical Population Health Unit (TPHU) in Cairns that one of his colleagues had become unwell a week after performing an autopsy on a horse. Two other people who had assisted with the autopsy, both members of the family who owned the horse, had apparently also become unwell, and the notifying veterinarian asked about the possibility of HeV infection in the three individuals. The horse: The horse was a 10-year-old gelding located on a property about 25 km south of Cairns. It had been acutely unwell for 1 day, with restlessness, increased respiratory effort and profuse sweating. On examination, the horse was febrile (41°C per rectum), tachycardic (120 beats/min), markedly dyspnoeic and very weak; it lay in a lateral recumbent position and could not raise its head. It was very dehydrated, and had injected mucous membranes and large amounts of blood-stained frothy secretions issuing from its nose. A decision was made to euthanase the horse, but it had a convulsion and expired before this could be performed. Blood-stained froth emanated from its nose and mouth as it died. Autopsy on the horse: Because the diagnosis was uncertain, the treating veterinarian performed a limited autopsy on the horse in the paddock where it died. She initially wore gloves, but quickly abandoned these as they were not of an appropriate design and soon became contaminated inside. No other personal protective equipment was used. For the procedure she had to reach deep into the carcass to examine some internal organs. The autopsy resulted in the veterinarian becoming heavily contaminated with the horse’s body fluids, especially those from the abdominal cavity. Contamination from the thoracic cavity was less, as this was opened only through a relatively small “window” over the heart. The veterinarian had a thorough shower immediately on returning home after completing the autopsy. The main gross findings at autopsy were massive fluid congestion of the lungs, cardiomegaly with marked thickening of the ventricular walls, and a grossly enlarged liver. The veterinarian concluded that the horse had died from acute heart failure and pulmonary oedema of unknown aetiology; no tissue samples were collected for further laboratory studies. At the completion of the autopsy, the horse was deep-buried using a back hoe. The veterinarian: Seven days after performing the autopsy, the veterinarian developed a dry cough and sore throat, associated with cervical lymphadenopathy and a fever lasting 4 days. She had generalised body aches and was very tired. The illness continued for about 8 days, during which time she was unable to work. She was seen 2 days after the onset of symptoms by a medical practitioner who had been informed (by the TPHU) of the possibility of HeV infection. However, the practitioner considered that her illness was consistent with a tonsillar infection and prescribed an antibiotic; blood was collected for HeV laboratory studies only. Follow-up serum samples were collected for further HeV studies at 14, 30, 50, 363, 470 and 559 days after the onset of symptoms. Laboratory studies: HeV RNA was not detected by a reverse transcriptase polymerase chain reaction (RT-PCR) (TaqMan) assay10 on the initial serum sample. Similarly, HeV IgM and IgG antibodies were not detected by either immunofluorescence assay (IFA) or enzyme-linked immunoassay (EIA).11 However, the subsequent samples demonstrated clear HeV IgM and IgG seroconversions by both IFA and EIA. A serum sample taken nearly a year later was initially reported as having a very high antibody titre on IgG IFA (>1024), but on repeat testing this was revised to a level of 512 (Box 3). Subsequent antibody levels have fallen by one dilution. A plaque reduction neutralisation test11 showed that the serum collected 14 days after the onset of illness neutralised HeV at a dilution of 1 : 5. Autopsy assistants: An adult member of the family who owned the horse held the dying animal’s head; two others assisted with the autopsy. All three were exposed to the frothy nasal secretions to varying degrees. Although the two who assisted were reported as having become unwell after the procedure, further investigation revealed that neither had a febrile illness. Rather, both seemed to have had symptoms of pre-existing conditions. HeV serological studies on samples collected from all three adults 3–4 weeks after the autopsy did not show any evidence of HeV infection. Site visit: A site visit took place on the day of the notification to TPHU, which was about 2 weeks before laboratory confirmation of HeV infection was obtained. The paddock was in a semi-rural area and held seven other horses, all of which appeared in good health. It was surrounded by other paddocks, some of which held horses. There was no obvious flying fox colony nearby; the owner of the property voluntarily quarantined the property (not allowing any horse movement in or out). Once the HeV infection in the veterinarian was confirmed, the other seven horses in the paddock were tested for serological evidence of HeV infection (38 days after the horse died); all were negative. The quarantine was subsequently lifted. DiscussionAll four reported outbreaks of HeV infection have occurred in Queensland, and three have occurred in the northern part of the state. Although no samples were collected from the horse, with hindsight it clearly had an illness consistent with previous clinical reports of HeV disease in horses. The disease is usually fulminant in nature, with fever, tachycardia, respiratory distress and a frothy nasal discharge being the typical reported features in horses.8,12 Facial oedema, physical distress and unease (suggestive of colic), and the close proximity of flying foxes add further support to the clinical suggestion of HeV disease in horses.12 The most obvious gross pathology is marked fluid congestion in the lungs, with a thick, foamy haemorrhagic exudate in the airways.8 Although there was no obvious flying fox colony nearby, large numbers of flying foxes are usually obvious in the evening sky in the latter part of the year in and around Cairns. The timing of the horse’s illness is not only the flying foxes’ birthing season,13 but also the season for many domestic and rainforest fruits in Far North Queensland; flying foxes travel considerable distances on nocturnal forays from their colonies in search of these foods. The owner of the horse reported frequently seeing flying foxes in the vicinity of the property. We assume that the veterinarian’s symptoms were caused by HeV, occurring after an apparent incubation of 7 days. Unfortunately, haematological and biochemical investigations were not requested after her initial medical consultation. The illness was mild, despite her being heavily contaminated with blood and body fluids during the autopsy. This veterinarian is the fourth person known to have been infected with HeV. All four had direct exposure to secretions and tissues from very ill, dying or dead horses; two were directly involved in autopsies of these horses. Two of the four died, whereas the other two had relatively mild illnesses.2-4 The veterinarian has remained clinically well for 2 years since her initial illness. A rise in the antibody titre 1 year after the initial illness was of concern in view of the observed late neurological relapse (13 months after acute illness), associated with increasing antibody levels, in one of the other cases of HeV infection.4 The closely related Nipah virus has also been associated with late neurological relapse in 7.5% of cases, occurring up to 22 months after initial infection.14 In one case, late-onset Nipah virus encephalitis coincided with rising antibody titres.15 The veterinarian undertook a high-risk procedure, taking less than optimal precautions. She was a relatively recent graduate, and her training may not have adequately impressed upon her the need to undertake such procedures with due care. Just as human health workers have had to accept that several recently emerged viruses (eg, blood-borne viruses and the SARS coronavirus) have changed working procedures, those working in animal health also must accept that recently emerged zoonotic viruses (eg, HeV and Australian bat lyssavirus) necessitate appropriate working procedures and personal protective equipment in veterinary practice. After this HeV incident, the Queensland Department of Primary Industries and Fisheries published revised guidelines for veterinarians handling horses suspected of being infected with HeV.12 These guidelines provide clinical case definitions and the recommended response measures, including the personal protective equipment that should be used when managing a suspected case, and the necessary reporting procedures.12 We suggest that these guidelines should be widely disseminated throughout the Australian veterinary community. AddendumIn early December 2004, a horse on a property just south of Townsville died of laboratory-confirmed HeV disease; there were no human infections associated with this fifth recognised outbreak of HeV disease. In mid June 2006, a horse on a property near Peachester on the Sunshine Coast hinterland died of laboratory-confirmed HeV disease; to date there have been no apparent human infections associated with the sixth outbreak of HeV disease. 1 Electron micrograph of the Hendra virus Courtesy, Mr Howard Prior, Senior Technician, Queensland Department of Primary Industries and Fisheries. 2 Possible mode of transmission of Hendra virus (HeV) infection 3 Hendra virus (HeV) laboratory studies in serum collected from the veterinarian at various intervals after the onset of symptoms. The onset occurred 7 days after the autopsy of the horse Laboratory studies Days after symptom onset 2 14 30 50 363 470 559 HeV RNA Not detected HeV IgM (IFA) < 8 8 32 16 < 8 < 8 < 8 HeV IgG (EIA) Non-reactive Reactive Reactive Reactive Reactive Reactive Not performed HeV IgG (IFA) < 8 256 256 128 512 256 256 IFA = immunofluorescence assay. EIA = enzyme-linked immunoassay.

Jeffrey N Hanna DTCH, MPH, FAFPHM · William J McBride FRACP, FRCPA, PhD · Dianne L Brookes MPH · Jack Shield BVSc · Carmel T Taylor BSc · Ina L Smith PhD · Scott B Craig BSc(Hons) · Greg A Smith PhD

United airway disease is characterised by inflammation of the respiratory tract, in which asthma and rhinitis are the upper and lower respiratory tract manifestations, respectively, of the same disease process. Irrespective of cause, the upper and lower respiratory tract manifestations are characterised by a systemic inflammatory response. Patients with rhinitis or asthma should always be assessed for coexistent disease in the reciprocal area. Treatment of upper airway disease can modify the severity of lower airway disease and vice versa. The potential for early treatment of allergic rhinitis to prevent progression to asthma merits further study.

Janet Rimmer MD, MB BS, FRACP · John W Ruhno MB BS, FRACP

Does allergy predispose to acute infectious sinusitis? Some patients report sinus infections during the hayfever season, and it is commonly supposed that congestion associated with allergic rhinitis can predispose to infectious sinusitis by interfering with the function of the sinus ostia. However, there is no good evidence to support this hypothesis, and no good evidence that specific treatment by allergen avoidance or immunotherapy (or even non-specific treatment with continuous topical corticosteroids) can prevent acute infectious sinusitis in people who do not have underlying chronic sinusitis. Most episodes of acute sinusitis are sequelae to viral upper respiratory tract infections. However, topical corticosteroids are an effective adjunct to antibiotic therapy in cases of acute infectious sinusitis, with or without coexisting allergy (Level II).1 Does allergy cause chronic rhinosinusitis or nasal polyposis? Chronic rhinosinusitis (CRS) (ie, inflammation of the mucosa of the nose and the paranasal sinuses) can be subdivided into two types according to the presence or absence of nasal polyposis (NP).2 The mucosal inflammation in CRS without polyposis is usually neutrophil predominant, but when NP is present (CRS/NP) it is usually eosinophil predominant. The tissue eosinophilia and frequent association of CRS/NP with asthma3 have led to the assumption that CRS/NP is caused by allergy. However, most studies have shown that the presence of IgE to environmental allergens (atopy) is no more common in people with CRS (with or without NP) than in the general population, and the symptoms of CRS are not affected by seasonal changes or allergen exposure. Thus it is now thought that allergy is not the cause of CRS in most cases. Atopy may coexist with CRS, and symptoms of allergic rhinitis can be superimposed on symptoms of CRS. There is no evidence that allergen avoidance or immunotherapy can alleviate CRS, but it may relieve superimposed hayfever symptoms. A subset of CRS/NP associated with chronic fungal infection and systemic fungal allergy has been termed “allergic fungal sinusitis”, but the exact parameters of this entity remain unclear. Antifungal treatments have been disappointing, and the evidence for effectiveness of immunotherapy for fungal allergy in this condition is limited. In most cases, the cause of CRS is not apparent. Bacterial infection frequently coexists, but may not be causative. Current theories of causation include bacterial biofilms, fungal hypersensitivity, sensitivity to bacterial superantigens, and genetic factors. What is the relationship between aspirin sensitivity and sinus disease? About a third of adults with CRS/NP and asthma also have aspirin sensitivity, in which the ingestion of aspirin or another non-steroidal anti-inflammatory drug (NSAID) causes an acute exacerbation of asthma or nasal symptoms or both. This is not an IgE-mediated allergy, but rather a pharmacological intolerance due to dysregulation of prostaglandin and leukotriene metabolism. Patients with CRS may also note symptom exacerbation after intake of alcohol or sulphites, or from aeroirritants such as smoke and fumes. Because these are not true allergies, skin prick testing and allergen desensitisation are not applicable. Aspirin desensitisation by graded oral doses can be effective, but must only be carried out by a specialist under controlled, supervised conditions. Investigation and referral. CRS can cause symptoms similar to perennial allergic rhinitis, and may be indistinguishable on history alone. Anosmia and the presence of nasal polyps seen on anterior rhinoscopy (Figure) are good predictors of chronic sinusitis. However, the complaint of “sinus headache” is not a good predictor of a radiographic finding of sinusitis. Investigation for CRS is appropriate when symptoms of perennial rhinitis occur in the absence of allergy; when allergy is present but not relevant (eg, when there is allergy to seasonal pollens, but symptoms are perennial); or if the symptoms fail to respond adequately to allergy-directed treatment. A computed tomography scan of the sinuses is a useful diagnostic test and is more accurate and informative than a plain sinus x-ray. Evidence-based practice tip Surgery is an effective treatment for chronic rhinosinusitis in patients who have failed medical treatment, with improvement in 75%–85% of patients (Level III).* * NHMRC levels of evidence. Patients with CRS should be reviewed by both an allergist/immunologist and an ear, nose and throat surgeon for consideration of a range of issues including bacterial or fungal infection, coexistent allergy, immunoglobulin deficiency, aspirin/NSAID sensitivity, comorbidities (eg, asthma, Wegener granulomatosis, Churg–Strauss vasculitis, ciliary dyskinesia, cystic fibrosis), or surgery to restore sinus ostial patency. Management. The mainstay of medical therapy is corticosteroids, which act on both allergic and non-allergic inflammation. Topical corticosteroids are effective for treating NP and concomitant allergic symptoms (Level II).4 Brief courses of systemic corticosteroids can significantly reduce inflammation in CRS/NP and alleviate symptoms temporarily (Level III),5 facilitating maintenance therapies or surgical intervention. Although polyp regrowth after surgery occurs quite frequently, surgery remains an important part of overall management.

William B Smith PhD · Peter-John Wormald MD

To the Editor: We recently read with concern the article by Scott and Ward on public reporting of hospital outcomes.1 While we do not want to enter into the debate about whether the public release of hospital performance reports is beneficial or harmful, we would like to address some issues relating to the accuracy of administrative data. The authors stated that “data are often [our italics] inaccurate, incomplete, or provide insufficient clinical detail” and that the “accuracy of diagnosis coding is vari-able”. They also mention the potential for “gaming” or “up-coding” by hospitals to make their institutions look better in public reports. We believe the authors’ argument regarding coding inaccuracies is flawed. One of the articles they cited was not about coding accuracy but about mortality differentials between metropolitan and non-metropolitan regions.2 Another cited article quoted an example of a 100% miss rate for coding of dementia as a comorbidity that was based on only three cases.3 As the authors later stress in their article, it is important that sample size be considered when interpreting data, to ensure that the effects of random error are minimised. We agree with the authors’ final point that distinguishing complications from presenting diagnoses (or comorbidities) is currently difficult using hospital coded data. However, the Victorian and Queensland hospital data collections now include an “alpha flag”, which is a letter attached to each coded diagnosis to indicate whether the diagnosis was present on admission to hospital or whether it arose during the episode of care. Moves are underway to introduce a minimum national requirement to use the alpha flag as one method of identifying complications arising from medical or surgical care. There are a number of national and state initiatives that aim to ensure the national morbidity data collection is as accurate as possible and that it provides data directly related to its purpose (and therefore not necessarily useful for other purposes). There is currently a national debate about the purpose of this collection. It is clear that there are issues surrounding the capture and coding of hospital data that are not well understood by data users. Because of that misunderstanding, reports such as the one by Scott and Ward paint an unjustifiably bleak picture of the quality of the data.

Kerry Innes · Kirsten McKenzie · Sue Walker

In reply: Innes and colleagues accuse us of overstating the potential inaccuracy of coded administrative data. They refer to state and national initiatives underway to ensure such accuracy, but offer no hard statistics that would reassure us that such data, in their current form, are as accurate as they need to be for purposes of quality monitoring and public disclosure. Until they do, we feel we have good reason to recommend caution in light of the few published Australian reports that are available (which we cited1,2), together with other research3 and feedback from clinical directors, about significant error rates when coded diagnoses are audited by clinicians or compared with independent datasets maintained by clinicians (Professor David Johnson, Director of Nephrology, and Dr Paul Garrahy, Director of Cardiology, Princess Alexandra Hospital, personal communication). In Queensland, formal regular audits on coding accuracy were initiated only in October 2005. They involve small numbers of randomly selected charts from each hospital and focus on specific coding issues identified for each hospital (Professor Stephen Duckett, Executive Director of Reform and Development, Queensland Health, personal communication). While we welcome (and were aware of) the introduction of “alpha flags” to distinguish in-hospital complications from pre-existing conditions, these remain a recent development (especially in Queensland), and others with considerable experience in their use express caution in interpreting results in the absence of rigorous validation.4,5

Ian A Scott · Michael Ward

To the Editor: Your 3 July issue featured task substitution and task transfer. It is a principle of commercial organisation that if a task can be standardised, it can be delegated, automated or computerised, provided there is good central management, supervision and communication. However, I suspect that this is only a part of the new face of general practice, as we are being expected to undertake tasks for which my age cohort (I am 54) was neither trained nor prepared. Practice administration is far more complex than ever before, and most of the clinical caseload has shifted from episodic care of infections and surgical conditions to the long-term systematic management of chronic cardiovascular, respiratory, musculoskeletal, endocrine, and other illnesses. Diabetes is a good example. Managing patients with diabetes requires high-level skills in practice management and protocol-driven chronic disease care. I suggest that a basic and fundamental difference between doctors and allied health personnel is that allied health personnel are extremely comfortable with protocol-driven chronic management while doctors, especially of my age cohort, are more focused on detecting and dealing with difference, variation and abnormality in our patients’ health. Rather than force all groups to do the same tasks, is it not better to build on their skills and interests in a logical and structured manner? General practices are no longer only places where general practitioners work; they are evolving into teams of GPs, other doctors, administrators, allied health personnel and office and information techno-logy staff, who work together in an integrated and coordinated way for the benefit of patients. This provides mutual support, flexibility of work hours and increased job satisfaction for all. Our practice has been steadily working towards this for the past 20 years.

Christopher D Hogan

To the Editor: A young man, a minor when sentenced in Sydney, was diagnosed with lymphoma soon after incarceration. Appropriate treatment was initiated, including pretreatment collection and storage of his semen. A local newspaper report that his sperm was collected and stored at taxpayers’ expense prompted outrage in some sections of the community. In response, the New South Wales Government drafted the Corrective Services Legislation Amendment Bill 2006, which would make it a crime for an individual imprisoned or awaiting sentencing for a “serious indictable offence”, such as homicide, rape or terrorism to store “reproductive material” (semen or ova).1 It is routine (many would say mandatory) for men of reproductive age who are about to undergo therapy for cancer to be offered the option to store semen. Without this option, male cancer survivors might be unable to father their own offspring. There is no current routine technology for storing unfertilised ova. In current practice for male prisoners, semen is stored before commencing treatment for cancers or similar conditions that may induce temporary or permanent infertility. This is the accepted standard of care, offered before such treatment to men who may not have completed their families. It is not current practice in NSW to store prisoners’ semen in any other circumstances. The proposed Bill will discriminate against prisoners in the quality and costs of their health care. Members of our community who require chemotherapy for cancer are offered collection and storage of their semen, provided free of charge by several public services in NSW. Under the proposed Bill, prisoners are required to pay storage fees during their imprisonment, even if their sperm were placed in storage before their incarceration. Discriminating against certain prisoners by demanding payment for otherwise free services could be seen as a “cruel and unusual punishment”. The NSW Legislative Assembly passed the Bill on 25 May 2006. Medical, legal and human rights organisations, and individuals expressed concern to parliamentarians. In the Legislative Council on 7 June 2006, a majority vote referred the Bill to the General Purposes Standing Committee No. 3. This Committee has received submissions and will provide recommendations as to how the Bill should proceed. If passed into law, the Bill would breach the principle of equivalence of health care for prisoners. The Australian Medical Association position statement on the Health care of prisoners and detainees states: “The duty of medical practitioners to treat all patients professionally with respect for their human dignity and privacy applies equally to the care of those detained in prison, whether convicted or on remand, irrespective of the reason for their incarceration.”2 I argue that the Bill implies an intention to rid society of “criminal seed” and begins a move towards eugenics. If our society really accepts the idea that inmates of correctional facilities may one day return to a full and productive life, then it is unreasonable to deny them the possibility of having their own children because they developed a serious cancer. If this legislation is passed, a discriminatory practice of medicine according to convict status will be enshrined in NSW law.

John E J Rasko

Re: “Doctors behaving badly?”, by Martin H N Tattersall and Ian H Kerridge, in the 18 September issue of the Journal (Med J Aust 2006; 185: 299-300). The first reference was attributed to the wrong newspaper. The reference should read: “1 Stop the gravy train [editorial]. The Sydney Morning Herald 2006; 7 Aug: 8.” The html and pdf versions of this article were corrected on 20 Nov 2006.

Martin H N Tattersall FRCP, FRACP, MB BChir · Ian H Kerridge FRACP, FRCPA, MPhil

Medicine in quotations. Views of health and disease through the ages. 2nd ed. Edward J Huth, T Jock Murray, editors. Philadelphia: American College of Physicians, 2006 (xvi + 581 pp). ISBN 1 930513 67 4. Why, in these times of rapid electronic access to information, do we need books that compile the wit and wisdom of the famous and not so famous? Could it be that these tomes conveniently satisfy an abiding human curiosity to know what was said about what, who said it and where? And doctors are no exception. Medicine is a treasure trove of quotations made by its practitioners from antiquity to modern times, and this rich tapestry is on display in Medicine in quotations. Edited by EJ Huth, Emeritus Editor of the Annals of Internal Medicine, and TJ Murray, a past Chairman of the board of trustees of the publisher, the American College of Physicians, the book details more than 3500 quotations distilled from submissions by 106 contributors. It presents the quotations under broad headings arranged in alphabetical order. The currency of the quotations is reflected by topics such as AIDS, Gulf War syndrome, chronic fatigue syndrome, health policy and so on. The quotations are identified by their authors and source and are arranged in chronological order. Finding relevant quotes is a breeze using the book’s comprehensive subject index. For the more curious, there is also a listing of the publication source of each quote. It should not be surprising that the most frequent quotations are by Hippocrates and Sir William Osler. The only modern person with a substantive entry is biologist–author Lewis Thomas. Medicine in quotations, despite its expense, will supersede its ageing competitor, Familiar medical quotations (published in 1968). The book should be an obligatory addition to medical libraries and be on the shelves of medical writers, editors, lecturers and after-dinner speakers. After all, to quote Dorothy Sayers, the British author and scholar: “I always have a quotation for everything — it saves original thinking”. Martin B Van Der Weyden Editor, Medical Journal of Australia

Martin B Van Der Weyden

Caesarean section. A manual for doctors. Caroline M De Costa and Paul Howat. Sydney: MJA Books, 2006 (v + 104 pp). ISBN 085557 045 8. Caesarean section is a must for any doctor considering obstetrics. It is well written, easy to read, exceptionally informative, and practical. It was written by two rural obstetricians with a wealth of experience, in collaboration with two anaesthetists. I know from personal experience that both authors spoke with many colleagues to find out how they cope with some of the difficult caesarean complications. My only criticism of the book is that there is some repetition of the topics, particularly anaesthetics, but that happens because each chapter could be considered to be a small monologue that stands alone. The organisation of the book is very good. It begins with the decision-making process that is necessary before performing caesarean section, goes through informed consent, and gives the pros and cons of carrying out the procedure and its anaesthetic. For readers who have not performed a caesarean section, or may have only performed a few, there is a good description of how to proceed. There is also a description of the basic surgical instruments required. After dealing with the basic procedure, the book describes some complications of caesarean section (eg, difficulties delivering the head). There is a chapter devoted to intra- and post-operative bleeding and practical hints for coping with the various causes of bleeding. Further chapters deal with caesarean section for placenta praevia and the indications for a classical caesarean section and how to perform it. The management of caesarean section in women who are HIV positive, and sterilisation at the time of caesarean section are discussed. The last two chapters deal with anaesthesia for caesarean section and are very good reading for procedural general practitioners who may need to administer anaesthetics, particularly in rural areas. One deals with regional anaesthesia (epidural, spinal, and combined anaesthesia); the other deals with general anaesthesia, outlining the potential problems of giving a general anaesthetic to a pregnant woman and how to cope when unable to intubate a pregnant woman. There is also a good appendix detailing references for further reading. In summary, this is an excellent practical book which should be read by all doctors who are about to embark on performing caesarean section and anaesthesia. It is also of value for experienced practitioners as it describes how other people have coped with some of the difficulties of caesarean section. Ian PettigrewAssociate Professor, Department of Rural Obstetrics and Gynaecology, Monash University, Mildura, VIC Order this book

Ian Pettigrew

Weigh by day Daily self-weighing has been linked with a decreased risk of regaining lost weight in a US trial. In a group of 314 participants who had recently lost at least 10% (and about 20 kg) of their body weight, the Study to Prevent Regain (STOP Regain) trial compared the weight regained during an 18-month face-to-face intervention or an Internet-based intervention with that regained by a control group. Both intervention groups received a similar program that stressed daily self-weighing and self-regulation. Although the face-to-face group regained the least weight overall, fewer participants in both intervention groups who weighed themselves daily regained 2.3 kg or more in weight, compared with those who weighed themselves less often. N Engl J Med 2006; 355: 1563-1571 Getting older? If you or your patients are seeking an anti-ageing hormone supplement, be advised that the adrenal sex steroid dehydro-epiandrosterone (DHEA) is unlikely to be effective. Mayo Clinic researchers led a 2-year study in which 56 elderly men and women with low baseline levels of sulfated DHEA were randomised to receive DHEA supplementation. Although plasma DHEA levels increased to values in the high-normal range for young adults, there was no detectable effect on physical performance, insulin sensitivity or quality of life. There was a small effect on bone mineral density. N Engl J Med 2006; 355: 1647-1659 From on high Researching high-altitude pulmonary oedema may help us to better understand and treat some common lowland pulmonary oedemas, suggests a US expert.1 Swenson was commenting on a small European study conducted at high altitude which has unexpectedly found that prophylactic dexamethasone prevented not only acute mountain sickness but also high-altitude pulmonary oedema (HAPE) in HAPE-susceptible individuals.2 Taken as 8 mg twice daily, dexamethasone was commenced the day before a rapid ascent from about 1100 m to 4559 m and continued throughout the duration of the study. This prolonged intake seemed critical to its effectiveness in reducing pulmonary arterial pressure, which had not been notable previously. Swenson said it was intriguing to speculate that the pulmonary vascular hyperreactivity of HAPE-susceptible persons may account for the severe secondary hypertensions sometimes seen with sleep apnoea, heart failure or lung disease. 1. Ann Intern Med 2006; 145: 550-552 2. Ann Intern Med 2006; 145: 497-506 Psoriasis and the heart Patients with psoriasis have an increased risk of myocardial infarction, according to UK researchers. In a prospective study using data collected over an average of 5 years by general practitioners, they compared outcomes in 127 139 patients with mild psoriasis, 3 837 patients with severe psoriasis and 556 995 control patients. The risk of myocardial infarction was greater for patients with psoriasis; it was greatest in young patients with severe psoriasis. The findings add to the growing evidence linking T-helper cell type 1 immunological diseases, like psoriasis and rheumatoid arthritis, to atherosclerosis and coronary artery disease. JAMA 2006; 296: 1735-1741 Making no bones about it Tasmanian researchers have determined that calcium supplementation in childhood is unlikely to reduce the risk of fracture to a degree which would be of major public health importance. They conducted a meta-analysis of 19 randomised controlled trials, involving 2859 children aged 3 to 18 years, that compared calcium supplementation, taken for at least 3 months, with placebo. After at least 6 months of follow-up, calcium supplementation was found to have no effect on bone mineral density (BMD) at the femoral neck or lumbar spine. However, there was a small effect on total body bone mineral content and upper limb BMD. Although the upper limb effect persisted after supplementation ended, the size of effect was thought to be clinically insignificant. The researchers suggested that it may be appropriate to explore possible alternative nutritional interventions, such as increasing vitamin D concentrations and the intake of fruit and vegetables. BMJ 2006; 333: 775-780 I apologise . . . Apology is an emerging clinical skill in medical practice, according to a US expert. Lazare believes that an effective apology is one of the most profound healing processes between individuals and groups. Effective apologies are characterised by up to four parts — acknowledgement of an offence; an explanation for committing the offence; an expression of remorse; and reparation. As well as restoring damaged relationships, effective apologies can strengthen existing good relationships, for example, between doctors and their patients and colleagues. As with other activities that have the power to heal, Lazare says that it is essential for physicians to develop skills and ethical principles to allow them to use apologies effectively and honestly in practice. JAMA 2006; 296: 1401-1404

Ann Gregory

Med J Aust 2006; 185 (10 Suppl).