Letters

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David B Brieger · James Weaver · Leonard Kritharides

In reply

Gabrielle Campbell · Briony Larance · Nicholas Lintzeris

To the Editor: Based on Fremantle Diabetes Study Phase 2 (FDS2) data, we reported in this Journal that the prevalence of maturity‐onset diabetes of the young (MODY) and permanent neonatal diabetes in an urban Australian population was 0.24% and 0.12%, respectively, of people diagnosed with diabetes.1 A further FDS2 participant among those identified as probably having MODY by clinical risk prediction was the only one with a novel heterozygous missense variant (Ala161Thr) in the KCNJ11 gene which encodes the pore‐forming KIR6.2 subunit of the pancreatic β‐cell adenosine triphosphate‐dependent potassium channel.2 This variant was not considered to be a cause of MODY at the time of our publication in 2017,1 but evidence has since emerged that it is a pathogenic activating mutation. It has been identified in two other patients with neonatal diabetes diagnosed before 9 months of age who were responsive to sulfonylurea therapy, and in another diagnosed at 14 years of age who was glutamic acid decarboxylase and islet antigen 2 antibody negative, and had a low (5th percentile) type 1 genetic risk score,3 a body mass index of 21, a stimulated serum C‐peptide concentration of 289 pmol/L (fasting range, 260–1030 pmol/L) 11 years after diagnosis, and a family history of non‐insulin‐requiring diabetes in her brother and mother (both diagnosed at 18 years of age) and maternal uncle and grandfather (unpublished data, Molecular Genetics Laboratory, Royal Devon and Exeter NHS Foundation Trust). Our patient with this novel MODY mutation was also diagnosed with diabetes at 14 years of age. At 19 years of age, she was glutamic acid decarboxylase and islet cell antibody negative, and had a body mass index of 28.7 and a serum C‐peptide concentration of 660 pmol/L with a simultaneous plasma glucose level of 8.2 mmol/L. Her glycated haemoglobin level was 6.8% (51 mmol/mol) on metformin monotherapy. She remained well controlled on metformin at FDS2 assessments at 23 and 25 years of age (glycated haemoglobin ≤ 6.4% or ≤ 46 mmol/mol), but subsequently progressed to requiring insulin. Patients with diabetes due to an activating KCNJ11 gene mutation have a defect in insulin secretion and, in most cases, can be treated successfully with sulfonylurea. This includes those who have been treated with insulin previously (our FDS2 participant has recently been offered this transition).4 Activating variants in the KCNJ11 gene are likely to cause permanent neonatal diabetes, MODY or transient neonatal diabetes that remits and can subsequently relapse during the teenage years.5 Each of our participant's offspring will have a 50% risk of inheriting this variant and thus developing neonatal and/or later onset diabetes. This new variant means that MODY prevalence has increased to 0.29% of people diagnosed with diabetes, or 107 per million of the Australian population, compared with 0.24% or 89 per million in our original publication.1 All MODY and permanent neonatal diabetes cases in the FDS2 cohort were people of European ancestry,1 and the participant newly identified with MODY was of Eurasian background. The present case illustrates the clinical and genetic heterogeneity of monogenic diabetes. The discovery of new variants allows improved understanding of the pathophysiology and treatment of diabetes in young people.

Timothy ME Davis · Ashley E Makepeace · Kirsten Peters · Kevin Colclough · Wendy A Davis

To the Editor: We agree with San Martin Porter and colleagues1 about the importance of mental health screening during pregnancy and acknowledge the role of the Edinburgh Postnatal Depression Scale (EPDS) in screening in Australia and internationally. In the article, the authors stressed that the EPDS has been validated. However, the high heterogeneity demonstrated during these validation studies suggests that it is not equally valid across all populations and settings.2 The authors also suggested that low uptake of screening with Aboriginal women is related to less frequent attendance to antenatal checks. This interpretation fails to consider broader cultural safety issues surrounding antenatal care, and more specifically, the language and cultural appropriateness of the EPDS.3 This tool has not been validated with Aboriginal and Torres Strait Islander women. Many Aboriginal women find the EPDS language complex and confusing, and providers find using it with Aboriginal women challenging.3 Screening processes need to be acceptable to patients and staff, and seen to be easy to use and helpful, or they are unlikely to be well implemented. The need to consider the language and cultural appropriateness of the tool used was acknowledged in the latest Clinical practice guidelines: pregnancy care.4 The Kimberley Mum's Mood Scale (KMMS) is a locally designed approach to screening Aboriginal women.3 Part 1 is an adaption of the EPDS. Part 2 is a “yarn” between health professionals and women about contextual or psychosocial factors that are important to the women. Health professionals work with women to identify how they are coping (strengths focus) without minimising risk factors. Validation of the KMMS demonstrated clinical efficacy and high levels of user acceptability.5 Women identified that “just yarning” was a positive start to understanding and managing their perinatal mental health. An approach such as the KMMS, which values listening (health professional) and talking (woman), is a positive, contemporary and logical next step from the EPDS. We suggest that all women, Aboriginal and non‐Aboriginal, would benefit from this approach. Traditional screening practices are not enough, but the next generation of screening tools provides new opportunities for women and their health professionals.

Julia V Marley · Emma Carlin · Catherine Engelke

In reply

Macarena A San Martin Porter · Steve Kisely · Rosa Alati

To the Editor: We agree with Catalano and colleagues1 that nicotine liquid needs to be regulated. However, we feel that their letter overstates the risk from nicotine poisoning. The authors state that the minimum potentially lethal dose of nicotine in humans is 60 mg, but the reference used for this claim actually estimates a far higher minimum lethal dose of 500–1000 mg.2 While it is correct to say that the “ingestion of even a small volume could cause serious harm or even death,” the reality is less worrying. The bioavailability of ingested nicotine is as little as 20% due to hepatic first pass metabolism.3 Furthermore, most cases of significant ingestion result in prompt vomiting.4 A recent review of all cases of nicotine exposure reported to the Australian Poisons Information Centres between 2009 and 2016 found that most cases resulted in only mild gastrointestinal symptoms.5 We agree with the recommendation to mandate safety labelling and childproof packaging to reduce risk. However, regulation needs to go further; legalising and enforcing quality and safety standards would help to ensure a safer product and minimise unwanted contamination, as found in a recent Australian study.6 Vaping has a potential role in reducing smoking‐related disease in Australia. A recent large randomised controlled trial demonstrated that vaping is nearly twice as effective as nicotine replacement therapy.7 Regulation needs to find a balance between reducing the risk to children and non‐smokers while making high quality reduced‐risk products available to smokers who are otherwise unable to quit. Overly restrictive regulations are likely to have a net negative effect on public health.

Colin P Mendelsohn · Alex D Wodak

In reply

Anthony C Catalano · Noel E Cranswick · Joanne Grindlay · Mick Creati · Margie H Danchin · Jeff Robinson · Nicola Williams · Amanda Gwee

To the Editor: Patients presenting to an emergency department (ED) may be unable to communicate with treating clinicians. Immediate access to emergency medical information is essential to providing optimal care and avoiding harm. Smartphone medical alert apps, such as those pre‐installed on the two major operating systems (iOS [Apple], Android [Google]), allow patients to store emergency medical information that is accessible to clinicians when a patient is incapacitated and the smartphone is locked. Similar to medical alert bracelets, these apps are designed to store basic emergency medical information. Via a self‐administered app, patients can record as much emergency medical information as they feel comfortable sharing. This information can be rapidly accessed from the smartphone's locked screen, bypassing default security features. We recently asked a convenience sample of 250 adult ED patients, well enough to complete a survey, to complete a questionnaire assessing their smartphone usage, familiarity, attitudes and barriers towards storing emergency medical information on smartphone apps. Ethics approval was obtained through the St Vincent's Hospital Melbourne Human Research Ethics Committee. Two hundred patients completed the survey. The mean age of respondents was 39 years (95% CI, 37–41 years). Most owned a smartphone and had it with them in the ED. Only 15% (31/200) currently used an emergency medical information app, with most using the default pre‐installed app. The commonest barrier to use was a lack of awareness or familiarity with the app. Once informed, most patients (97%; 194/200) were willing to use such an app in the future (Box). Patients who have privacy and security concerns about the government‐controlled My Health Record may view storing emergency medical information on smartphones as a safer option. The depth of information on a smartphone would be considerably less than that accessible via My Health Record, but in an emergency, some information is better than none. Using smartphones to store emergency medical information may lead to better emergency care for incapacitated patients. There is enthusiasm from patients to embrace this technology. General practitioners and other clinicians are well placed to inform patients and facilitate its adoption. ED clinicians should be encouraged to check the phones of incapacitated patients in the initial assessment and triage phase for the presence of potentially lifesaving information. Box – Number of patients currently storing or prepared to store emergency medical information on a smartphone app, by type of information Currently storing (n = 31) Prepared to store (n = 194) Number 95% CI Number 95% CI Name 27 (87%) 74–97% 171 (88%) 84–93% Date of birth 26 (84%) 71–94% 148 (76%) 70–82% Emergency contact 21 (68%) 48–84% 179 (92%) 88–96% Medical conditions 16 (52%) 36–68% 168 (87%) 81–91% Medications 11 (36%) 19–52% 162 (84%) 78–89% Allergies 9 (29%) 14–48% 177 (91%) 87–95% Organ donor status 11 (36%) 19–52% 173 (89%) 85–94% Blood type 13 (42%) 26–58% 184 (95%) 92–98%

Weiyu Fang · Rachel Zordan · Stuart J Dilley

To the Editor: Writing recently in The Lancet, Byng and colleagues1 argue that our clinical guidelines on care of transgender children that appeared in the MJA2 use imprecise language, do not consider long term effects, and lack robust evidence. Several points the authors raise are valid and indeed align with what we and others have written about the state of evidence and need for high quality research in the field.3,4 However, it is important to provide additional clarification. First, the phrase “sex assigned at birth” is not intended to mislead as Byng and colleagues have claimed5 but is standard terminology for clinicians who work in transgender health (the guidelines’ primary intended audience).6 Second, the authors fail to acknowledge not only our direct call for more research on the guidelines’ opening page but also the detailed description of the long term effects of hormonal treatment, which is a key component of the guidelines.2 Moreover, in calling for more research, they ignore existing efforts to improve evidence in this field.7 Third, the authors raise the issue of de‐transition, which refers to the observation that some individuals who identify as transgender may eventually return to living as a member of their birth‐assigned sex. This subject has gained attention in mainstream media, which appears to be the authors’ source of information on this topic.5 While more research is needed, existing studies show true de‐transition is uncommon. For example, only 0.4% of 27 715 transgender people surveyed in the United States de‐transitioned because they felt transition was not right for them; more often, de‐transition occurred due to outside pressures (eg, parents, family, religion) and discrimination.8 In summary, Byng and colleagues’ call for more high quality research is not novel and has long been acknowledged by those of us in the field. Moreover, withholding gender‐affirming treatment via wait‐and‐see strategies mentioned by the authors is not without harm,9 and development and ongoing revision of guidelines such as ours will ensure that transgender individuals receive the best care based on up‐to‐date empirical evidence.

Ken C Pang · Carmen C Pace · Michelle A Tollit · Michelle M Telfer

To the Editor: Infective endocarditis with Staphylococcus aureus is associated with a high mortality despite optimal antibiotic therapy.1 The synergy between bacteriophages and antibiotics has been shown in vitro and in animal studies,2 and bacteriophages have demonstrated their value in severe bacterial infections.3 AB‐SA01 (AmpliPhi Biosciences) is a bacterial DNA‐free and protein‐free highly purified preparation of three obligately lytic Myoviridae, each at 109 plaque‐forming units per dose.4 This preparation has been recently used successfully for staphylococcal sinusitis by local irrigation.5 A protocol was established for bacteriophage therapy as an adjunct to standard care of severe staphylococcal infections under the auspices of the Therapeutic Goods Administration Special Access Scheme. Here, we report the first intravenous use of AB‐SA01 in a case of severe staphylococcal sepsis with prosthetic valve endocarditis. A 65‐year‐old man with a 30‐year‐old mechanical aortic valve presented with a week of malaise, severe exertional dyspnoea, and central pleuritic chest pain. He had been successfully treated for Haemophilus aphrophilus aortic valve endocarditis 8 years earlier with antibiotics alone. Examination revealed fever, tachypnoea, tachycardia and borderline hypotension (90–100 mmHg systolic), with a praecordial systolic murmur and click. There was no cardiac, renal or hepatic failure or any evident peripheral embolic sequelae of endocarditis (haematuria, splinter haemorrhages) at this stage. Blood cultures repeatedly grew an identical methicillin‐sensitive S. aureus determined by whole genome sequencing, and the patient received high dose intravenous flucloxacillin, ciprofloxacin and rifampicin (Box). Transoesophageal echocardiography confirmed vegetations on prosthetic aortic and native mitral valves, and the aortic root was thickened with possible paravalvular root abscess. Scheduled cardiopulmonary bypass for operative source control was postponed after a haemorrhagic infarction in the distribution of the left anterior cerebral artery on day −7 (ie, a week before starting bacteriophage therapy), despite concerns regarding development of an aortic root abscess, ongoing fevers and hypotension. Intravenous AB‐SA01 was administered twice a day for 14 days in conjunction with the patient's prescribed antibiotics, commencing (Day 1) 9 days after his first positive blood culture. Blood cultures were negative at onset of bacteriophage therapy, and the C‐reactive protein, temperature, and white cell count results showed downward trends within 24 hours (Box). This trajectory was only interrupted by splenic infarction and occlusion of the superior mesenteric artery 48 hours after commencement, which was proven on computed tomography scan (not shown). No fevers, tachycardia, hypotension or rashes were detected after bacteriophage infusions and no adverse sequelae were attributable to the therapy. The patient recovered after 40 days of antibiotic therapy and returned to his home state for follow‐up. A positron emission tomography scan on Day 80 showed no fluorodeoxyglucose‐avid lesions, including intracardiac lesions. Repeat echocardiogram on Day 98 for progressive heart failure showed severely dilated left ventricle with moderate mitral and trivial aortic regurgitation. A possible mechanical aortic valve vegetation and paravalvular phlegmon were again demonstrated. Blood cultures were negative. He declined surgical intervention and died on Day 103. To our knowledge, this was the first case of staphylococcal prosthetic valve endocarditis treated with intravenous bacteriophage (AB‐SA01), which complies with good manufacturing practice standards.4 Bacteriophage infusions were well tolerated. Future controlled trials are needed to evaluate adjunctive bacteriophage therapy, especially when surgical intervention is not feasible. Box – Graphical representation of antimicrobial treatment, bacteriophage therapy and inflammatory markers − = negative blood cultures; + = positive blood cultures; CRP = C‐reactive protein; SMA = superior mesenteric artery; WCC = white cell count. ◆

Timothy Gilbey · Josephine Ho · Louise A Cooley · Aleksandra Petrovic Fabijan · Jonathan R Iredell

To the Editor: I refer to the opinion piece by Brewster and Waxman.1 The authors defined collegiality as “a work environment where responsibility and accountability are shared by colleagues, with mutual respect”. However, in a health care setting, professional competency and patient safety are paramount; medical collegiality is desirable, but should not trump these goals. Collegial courtesy can compromise good patient care, despite concerns of competency in clinical judgement or skills. Poor communication contributes to most sentinel events,2 particularly during clinical handover. SBAR (Situation, Background, Assessment and Recommendation), which was what ISBAR (Introduction, Situation, Background, Assessment and Recommendation) was known as in 2009, was promoted to reduce dangerous transitions in the patient's journey in health care.3 Unfortunately, when first introduced in Australia, unnecessary local adaptations were applied to this simple tool — at least seven versions of SBAR existed across six states between 2009 and 2011.4,5,6,7,8,9,10,11 In 2002, SBAR was introduced by retired United States Navy Captain Doug Bonacum, while working at Kaiser Permanente on patient safety, to address the lack of effective communication in sentinel events similar to nuclear submarine incidents and airplane crashes. A quality and safety expert, he drew on his handoff experience from nuclear submarine crews needing to discuss strategies quickly during shifts changeover, thus improving communication, reducing unnecessary narratives and assisting decision making. SBAR improves communication both within the craft group and in interdisciplinary interaction as it flattens the hierarchy.12 ISBAR can fail when the giver (person providing ISBAR) neglects to prepare or perform ISBAR effectively; this is often due to inadequate training, preparation or consideration of what the receiver requires before initiating the handover. Hence, the success of ISBAR rests more on the giver rather than the receiver of the handover. Kindness should be part of ISBAR, but it starts with the giver initiating the handover. Adequate consideration should be made by ensuring thoughtful preparation and organisation to deliver relevant information professionally and competently.13 Less experienced givers should be ready to furnish any specific details upon request. Such reflections enhance communication, reduce errors and improve confidence and morale. Few can perform ISBAR properly “on the run”; formal re‐accredited requirements involving ISBAR should be mandatory for all health care professionals to ensure this critical clinical handover is done well. Slavish adherence to collegiality should not blur the fundamental issues of professional competency and patient safety.

Shyan Lii Goh

To the Editor: We read with interest the article by Brewster and Waxman1 published in the Medical Journal of Australia. Building kindness and collegiality into regular handover practice is an obligation of increasing urgency in contemporary medical settings. Clinical handover is often led by junior medical staff and can be perceived as a stressful time for them. Junior doctors have expressed fears regarding public approbation as a contributory factor for stress and burnout.2 Obstetric junior doctors within our own tertiary maternity hospital responding to the Australian Medical Association of Western Australia Hospital Health Check survey reported high levels of stress.3 Ultimately, handover is likely to be a precipitating event for stress and anxiety in this group, and addressing psychological wellbeing during this time will have benefits to both staff and patients.4 In 2018, we built kindness into our labour ward handover process, as part of a formal junior doctor wellbeing initiative. The existing handover structure commenced with individual staff introductions for all members of staff in attendance, including name and role for each staff member. We incorporated a specific staff member to handover, whose sole role, stated during introduction, is “I am here to provide emotional support to the team”. This explicit statement places emotional wellbeing of all staff members at the heart of the priorities for the handover session. Induction to the roles and responsibilities of the profession has been identified as part of the purpose of handover.5 By building kindness and support into our organisation on a daily basis, we hope to improve the wellbeing of all staff members involved in this small but vital daily interaction and embed kindness and support in the broader workplace culture. A post‐intervention survey of the response to our emotional wellbeing initiative will be available in July 2019, at which point, we will learn the effectiveness of the added focus on kindness in handover meetings. Preliminary feedback from our junior doctors has been positive.

Alarna Thomas · Katrina L Calvert · Brendan Jansen

In reply

David J Brewster · Bruce P Waxman

To the Editor: We refer to Liaw and colleagues’1 recently published study in the Journal. We acknowledge the positive intentions and rigour of this trial, and empathically concur with Thompson and Thackrah’s2 comment that the results of this research “[do] not mean that efforts to improve cultural competence in health care settings should be abandoned”. To the contrary, this study demonstrates the urgent need for more research to improve cultural competence in the health care setting; in particular, the use of culturally safe research methods that truly benefit Aboriginal and Torres Strait Islander peoples and communities.3 Like Thompson and Thackrah,2 we question the authors’ choice of the cultural quotient questionnaire.4 This generic tool is not designed for assessing cultural competence of health professionals when working with Aboriginal and Torres Strait Islander peoples in Australia. Importantly, it lacks recognition of the unique colonial experiences of Aboriginal and Torres Strait Islander peoples and, therefore, it cannot measure health professionals’ understandings or attitudes about such a key part of any cultural training, where we would hope to see change. We suggest the use of a scale that has been designed and validated by Aboriginal and Torres Strait Islander peoples, such as the Cultural Capability Measurement Tool.5 We fear that, if not carefully interpreted, the study findings have the potential to further complicate and undermine the substantial work — endorsed by the National Aboriginal Community Controlled Health Organisation and the Department of Health — being undertaken to develop the cultural safety of Australia’s health system.6 It is crucial that in all areas of Australia’s health system, including Aboriginal and Torres Strait Islander health, we present a reliable, strategically aligned approach consistent with the vision of an Australian health system free of racism and inequality.6 It is important that we continue to work together to harness the energy and commitment of the workforce towards our shared goals. We look forward to the qualitative findings of the research study.

Sophie Hickey · Roianne West

In reply

Siaw‐Teng Liaw · Vicki Wade

To the Editor: We strongly agree with St Clair and colleagues1 that telehealth is a “game changer” for the provision of health services to Aboriginal and Torres Strait Islander communities in Australia. For more than 15 years, we have been engaging with Indigenous communities throughout Queensland, to plan and establish telehealth services for a range of clinical disciplines, including diabetes, ear, nose and throat, and aged care. Telehealth is contributing to positive changes in our health system and this is evidenced in our recent studies highlighting the value of telehealth for Aboriginal and Torres Strait Islander people. A systematic review of the outcomes of using telehealth for the provision of care to Aboriginal and Torres Strait Islander people reported improved social and emotional wellbeing, clinical outcomes and access to health services.2 Other benefits included improved screening rates and reduced need for travel.3 This review reinforced the importance of partnerships between Aboriginal community controlled health services (ACCHSs) and public hospitals. Telehealth also helps with the delivery of culturally appropriate care. In a qualitative investigative study,4 we found that telehealth allowed specialist care to be delivered from Aboriginal medical services which were familiar to the patient and local care providers. According to this study, a telehealth consultation with a specialist held in the ACCHS resulted in less stress and greater convenience; and also meant that an Indigenous health worker could be present during the telehealth appointment to provide advocacy and support for the patient.4 Telehealth also reduced out‐of‐pocket expense for the patient. These factors contributed to the interpretation of culturally appropriate services. Telehealth is already benefiting Indigenous people throughout Australia. All studies recognise the pivotal role of ACCHSs as advocates for telehealth. While telehealth applications are predominantly reported in rural and remote areas, we believe that telehealth is just as important for the delivery of specialist health care services to metropolitan ACCHSs, in lieu of community members attending mainstream health services. Telehealth is one mechanism to help close the gap. Telehealth empowers community health services and can improve equity of access to health services in rural and remote — and urban — settings.

Anthony C Smith · Nigel R Armfield · Liam J Caffery

To the Editor: In the recently published article by Morgan and colleagues1 and associated podcast, the authors raised the issue of future research into the outcomes of identification of Aboriginal and Torres Strait Islander peoples in hospital. The implementation of a visual identification methodology has previously been described2 and was put in place in partnership with the hospital department of Aboriginal health. The purpose of this identification initiative was to encourage all medical professionals who care for Aboriginal and Torres Strait Islander patients to question what they can do to assist them while they are in hospital. We describe here the positive outcomes of this process. In addition to hospital pharmacists,2 the visual alert system is also used by other departments and professionals within the hospital, such as cardiothoracic care, the emergency department (ED), Aboriginal health, and speech pathology, as well as ward‐based nursing and medical staff. The Aboriginal and Torres Strait Islander patients’ identification system is used by health care providers within the hospital to easily identify the patients they need to visit, to provide culturally appropriate resources and services and links to other services, and to enhance the care patients receive and enable them to remain in a caring hospital environment. At ward level, nursing and medical staff use the identification system to highlight the referral process needed to link with Aboriginal health workers — connecting the right resources to the right people. Because of the visual alert,2 staff feel more aware of the cultural needs of the patient before they meet them. Specifically in the ED, there is a system‐wide approach that firstly identifies Aboriginal and Torres Strait Islander patients presenting to the ED using the visual alert at triage, and then implementing initiatives that aim to reduce the level of “did not wait for treatment” episodes. The Heart and Lung Stream at St Vincent's Hospital uses the system to identify Aboriginal and Torres Strait Islander patients early during their admission in order to provide culturally specific education, resources and follow‐up after hospital discharge. We encourage others to explore this initiative in their hospital, together with cultural responsiveness training, to enhance the care of Aboriginal and Torres Strait Islander peoples during and after their hospital visit.

Susan A Welch · Sonia Robinson · Tamra Langley · Pauline Deweerd

In reply

David JR Morgan · Martin Whitely · Tania Harris