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Updated Australian consensus statement on management of inherited bleeding disorders in pregnancy

Scott Dunkley, Julie A Curtin, Anthony J Marren, Robert P Heavener, Simon McRae and Jennifer L Curnow
Med J Aust 2019; 210 (7): . || doi: 10.5694/mja2.50123
Published online: 15 April 2019

Abstract

Introduction: There have been significant advances in the understanding of the management of inherited bleeding disorders in pregnancy since the last Australian Haemophilia Centre Directors’ Organisation (AHCDO) consensus statement was published in 2009. This updated consensus statement provides practical information for clinicians managing pregnant women who have, or carry a gene for, inherited bleeding disorders, and their potentially affected infants. It represents the consensus opinion of all AHCDO members; where evidence was lacking, recommendations have been based on clinical experience and consensus opinion.

Main recommendations: During pregnancy and delivery, women with inherited bleeding disorders may be exposed to haemostatic challenges. Women with inherited bleeding disorders, and their potentially affected infants, need specialised care during pregnancy, delivery, and postpartum, and should be managed by a multidisciplinary team that includes at a minimum an obstetrician, anaesthetist, paediatrician or neonatologist, and haematologist. Recommendations on management of pregnancy, labour, delivery, obstetric anaesthesia and postpartum care, including reducing and treating postpartum haemorrhage, are included. The management of infants known to have or be at risk of an inherited bleeding disorder is also covered.

Changes in management as a result of this statement: Key changes in this update include the addition of a summary of the expected physiological changes in coagulation factors and phenotypic severity of bleeding disorders in pregnancy; a flow chart for the recommended clinical management during pregnancy and delivery; guidance for the use of regional anaesthetic; and prophylactic treatment recommendations including concomitant tranexamic acid.


  • 1 Institute of Haematology, Royal Prince Alfred Hospital, Sydney, NSW
  • 2 The Children's Hospital at Westmead, Sydney, NSW
  • 3 Australian Haemophilia Centres Directors’ Organisation, Melbourne, VIC
  • 4 Royal Prince Alfred Hospital, Sydney, NSW
  • 5 Royal Adelaide Hospital, Adelaide, SA
  • 6 Haemophilia Treatment Centre, Westmead Hospital, Sydney, NSW


Correspondence: scottmdunkley@gmail.com

Acknowledgements: 

We are grateful to Steph P'ng, John Rowell, Tim Brighton, Huyen Tran and Ian Douglas for their helpful feedback and comments. We acknowledge Ruth Hadfield for medical writing and editing assistance.

Competing interests:

No relevant disclosures.

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Resilient health systems: preparing for climate disasters and other emergencies

Gerard J FitzGerald, Anthony Capon and Peter Aitken
Med J Aust 2019; 210 (7): . || doi: 10.5694/mja2.50115
Published online: 15 April 2019

A system that integrates all aspects of health care is essential for facing future challenges

After another Australian summer of record‐breaking temperatures, bushfires, floods and widespread drought, it is clear that our health systems should be strengthened to cope with the challenges of climate change. We must also reduce the carbon footprint of health care,1 and continue to advocate that Australia play its part in dealing with the fundamental causes of climate change. In May, the 21st biennial congress of the World Association for Disaster and Emergency Medicine (WADEM) will be hosted by Brisbane. The congress will bring together investigators and practitioners from around the world to discuss disaster health care, future risks, community vulnerabilities, and the strategies required by resilient health systems.


  • 1 Queensland University of Technology, Brisbane, QLD
  • 2 Sydney School of Public Health, University of Sydney, Sydney, NSW
  • 3 Health Disaster Management Unit, Queensland Health, Brisbane, QLD


Correspondence: gj.fitzgerald@qut.edu.au

Competing interests:

No relevant disclosures.

  • 1. Malik A, Lenzen M, McAlister S, McGain F. The carbon footprint of Australian health care. Lancet Planet Health 2018; 2: e27–e35.
  • 2. Hanna EG, McIver LJ. Climate change: a brief overview of the science and health impacts for Australia. Med J Aust 2018; 208: 311–315. https://www.mja.com.au/journal/2018/208/7/climate-change-brief-overview-science-and-health-impacts-australia
  • 3. Kishore N, Marqués D, Mahmud A, et al. Mortality in Puerto Rico after Hurricane Maria. N Engl J Med 2018; 379: 162–170.
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Primary care in disasters: opportunity to address a hidden burden of health care

Penelope L Burns, Kirsty A Douglas and Wendy Hu
Med J Aust 2019; 210 (7): . || doi: 10.5694/mja2.50067
Published online: 15 April 2019

General practitioners provide a flexible response to the changed needs of the disaster‐affected population

In Australia, “a land … of droughts and flooding rains,”1 disasters affect our lives annually, the majority of which are weather‐related.2 They are a part of the landscape, taking the form of cyclones, floods, bushfires, droughts and other phenomena. Cyclone Debbie, which hit northern Queensland in 2017, the Tathra bushfires, which affected the south coast of New South Wales in 2018, and the thunderstorm asthma event in Melbourne in 2016 are just a few recent examples. Such catastrophic events affect rural and urban communities and coastal and inland locations. No community in Australia is exempt, which is reflected in the recent shift in focus by national and international disaster management policy to prioritise improving local community capacity to respond and recover.3,4


  • 1 Australian National University, Canberra, ACT
  • 2 Western Sydney University, Sydney, NSW


Correspondence: Penelope.Burns@anu.edu.au

Acknowledgements: 

We thank the Royal Australian College of General Practitioners Foundation for their support on some early work in this field.

Competing interests:

No relevant disclosures.

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The importance of public health genomics for ensuring health security for Australia

Deborah A Williamson, Martyn D Kirk, Vitali Sintchenko and Benjamin P Howden
Med J Aust 2019; 210 (7): . || doi: 10.5694/mja2.50063
Published online: 15 April 2019

Coordination is required to future‐proof Australia's capacity and leadership in public health genomics

Infectious diseases are an ever‐present risk to society, particularly because of globalisation and the threat of antimicrobial‐resistant organisms. Recently, a World Health Organization (WHO) team conducted a joint external evaluation of Australia's core capacities under the International Health Regulations. The evaluation gave Australia a high scorecard in all areas relevant to protecting health from emerging infectious disease threats.1 However, an area that the evaluation team highlighted for critical improvement was the integration of whole genome sequencing‐based surveillance into existing communicable diseases control systems in the Australian setting.1 While Australia scored highly for laboratory testing of priority diseases, the team recommended “integration of laboratory testing data with epidemiological data particularly in the context of whole genome sequencing”.1


  • 1 University of Melbourne, Melbourne, VIC
  • 2 National Centre for Epidemiology and Population Health, Australian National University, Canberra, ACT
  • 3 University of Sydney, Sydney, NSW



Competing interests:

No relevant disclosures

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The impact of rapid molecular diagnostic testing for respiratory viruses on outcomes for emergency department patients

Nasir Wabe, Ling Li, Robert Lindeman, Ruth Yimsung, Maria R Dahm, Kate Clezy, Susan McLennan, Johanna Westbrook and Andrew Georgiou
Med J Aust 2019; 210 (7): . || doi: 10.5694/mja2.50049
Published online: 8 April 2019

Abstract

Objective: To determine whether rapid polymerase chain reaction (PCR) testing for influenza and respiratory syncytial viruses (RSV) in emergency departments (EDs) is associated with better patient and laboratory outcomes than standard multiplex PCR testing.

Design, setting: A before‐and‐after study in four metropolitan EDs in New South Wales.

Participants: 1491 consecutive patients tested by standard multiplex PCR during July–December 2016, and 2250 tested by rapid PCR during July–December 2017.

Main outcome measures: Hospital admissions; ED length of stay (LOS); test turnaround time; patient receiving test result before leaving the ED; ordering of other laboratory tests.

Results: Compared with those tested by standard PCR, fewer patients tested by rapid PCR were admitted to hospital (73.3% v 77.7%; P < 0.001) and more received their test results before leaving the ED (67.4% v 1.3%; P < 0.001); the median test turnaround time was also shorter (2.4 h [IQR, 1.6–3.9 h] v 26.7 h [IQR, 21.2–37.8 h]). The proportion of patients admitted to hospital was also lower in the rapid PCR group for both children under 18 (50.6% v 66.6%; P < 0.001) and patients over 60 years of age (84.3% v 91.8%; P < 0.001). Significantly fewer blood culture, blood gas, sputum culture, and respiratory bacterial and viral serology tests were ordered for patients tested by rapid PCR. ED LOS was similar for the rapid (7.4 h; IQR, 5.0–12.9 h) and standard PCR groups (6.5 h; IQR, 4.2–11.9 h; P = 0.27).

Conclusion: Rapid PCR testing of ED patients for influenza virus and RSV was associated with better outcomes on a range of indicators, suggesting benefits for patients and the health care system. A formal cost–benefit analysis should be undertaken.

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  • 1 Centre for Health Systems and Safety Research, Australian Institute of Health Innovation, Macquarie University, Sydney, NSW
  • 2 NSW Health Pathology, Sydney, NSW
  • 3 Prince of Wales Hospital, Sydney, NSW
  • 4 Sydney Medical School, University of Sydney, Sydney, NSW


Correspondence: nasir.wabe@mq.edu.au

Acknowledgements: 

The project was part of a partnership project funded by a National Health and Medical Research Council of Australia Partnership Project Grant (APP1111925), in partnership with NSW Health Pathology and the Australian Commission on Safety and Quality in Healthcare.23

Competing interests:

No relevant disclosures.

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Diagnosis of Mycobacterium ulcerans disease: be alert to the possibility of negative initial PCR results

Daniel P O'Brien, Maria Globan, Janet M Fyfe, Caroline J Lavender, Adrian Murrie, Damian Flanagan, Peter Meggyesy, Jonathan T Priestley and David Leslie
Med J Aust 2019; 210 (9): . || doi: 10.5694/mja2.50046
Published online: 8 April 2019

Mycobacterium ulcerans causes necrotising infections of the skin and soft tissue (Buruli ulcer), a disease that is endemic in the coastal regions of Victoria and northern Queensland. Most lesions (> 85%) are painless ulcers, but some are non‐ulcerative.1 As the incidence of Buruli ulcer rises in Victoria,2 Australian health practitioners are increasingly required to recognise this disease in people who reside in or have travelled to endemic areas, with early diagnosis vital for good outcomes.3

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  • 1 University Hospital Geelong, Geelong, VIC
  • 2 Mycobacterium Reference Laboratory, Victorian Infectious Diseases Reference Laboratory, Melbourne, VIC
  • 3 Sorrento Medical Centre, Sorrento, VIC
  • 4 South Coast Medical, Blairgowrie, VIC


Correspondence: DANIELO@BarwonHealth.org.au

Competing interests:

No relevant disclosures.

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Family planning, antenatal and post partum care in multiple sclerosis: a review and update

Anneke Van Der Walt, Ai‐Lan Nguyen and Vilija Jokubaitis
Med J Aust 2019; 211 (5): . || doi: 10.5694/mja2.50113
Published online: 1 April 2019

Summary

 

  • Multiple sclerosis is more prevalent in women of childbearing age than in any other group. As a result, the impact of multiple sclerosis and its treatment on fertility, planned and unplanned pregnancies, post partum care and breastfeeding presents unique challenges that need to be addressed in everyday clinical practice.
  • Given the increasing number of disease‐modifying agents now available in Australia for the treatment of multiple sclerosis, there is a growing need for clinicians to provide their patients with appropriate counselling on family planning.
  • Providing better evidence regarding the relative risks and benefits of continuing therapy before, during and after pregnancy is an important research priority. International pregnancy registries are essential in developing better evidence‐based practice guidelines, and neurologists should be encouraged to contribute to these when possible.
  • The management of women with multiple sclerosis, especially when they are taking disease‐modifying agents, requires careful assessment of fertility and disease characteristics as well as a multidisciplinary approach to ensure positive outcomes in both mothers and their children.

 


  • 1 Monash University, Melbourne, VIC
  • 2 University of Melbourne, Melbourne, VIC
  • 3 Alfred Health, Melbourne, VIC
  • 4 Royal Melbourne Hospital, Melbourne, VIC



Competing interests:

Anneke Van der Walt has received travel support, speaking honoraria and served on advisory boards for Biogen Australia, Novartis, Merck, Sanofi, Roche and Teva. Ai‐Lan Nguyen has received grant support and travel support from Biogen Australia. Vilija Jokubaitis has received travel support and speaking honoraria from Biogen Australia.

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Pre‐exposure prophylaxis for HIV prevention during pregnancy and lactation: forget not the women and children

Lisa Horgan, Christopher C Blyth, Asha C Bowen, David A Nolan and Andrew P McLean‐Tooke
Med J Aust 2019; 210 (6): . || doi: 10.5694/mja2.50052
Published online: 1 April 2019

Summary

 

  • Pregnancy is known to be a time of increased susceptibility to acquiring to human immunodeficiency virus (HIV) infection and this increased maternal risk places the unborn child at risk of vertical transmission.
  • Pre‐exposure prophylaxis (PrEP) involves the provision of antiretroviral therapy to an HIV‐negative individual with ongoing risk of HIV exposure to limit the likelihood of HIV transmission.
  • The inclusion of PrEP as part of a comprehensive strategy is recognised as an effective and safe means of reducing HIV infection in serodiscordant couples, thereby reducing the risk of vertical transmission of HIV.
  • Current data suggest that PrEP is safe to continue during pregnancy and breastfeeding in HIV‐negative women who remain vulnerable to acquiring HIV.
  • The recent Pharmaceutical Benefits Scheme subsidisation of PrEP has reduced the financial and practical obstacles of PrEP provision, and a subsequent increase in patient awareness and acceptance of PrEP is expected.
  • The framework for appropriately identifying and managing at‐risk pregnant and lactating women requiring PrEP is poorly defined and warrants further clarification to better support clinicians and this patient group.
  • This review discusses the current recommendations highlighting the gaps in the guidelines and makes some recommendations for future guideline development.

 


  • 1 Perth Children's Hospital, Perth, WA
  • 2 University of Western Australia, Perth, WA
  • 3 Princess Margaret Hospital for Children, Perth, WA
  • 4 Wesfarmers Centre of Vaccines and Infectious Diseases, Telethon Kids Institute, Perth, WA
  • 5 Royal Perth Hospital, Perth, WA
  • 6 PathWest Laboratory Medicine WA, Perth, WA



Competing interests:

No relevant disclosures.

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The Guttmacher–Lancet Commission on sexual and reproductive health and rights: how does Australia measure up?

Deborah J Bateson, Kirsten I Black and Shailendra Sawleshwarkar
Med J Aust 2019; 210 (6): . || doi: 10.5694/mja2.50058
Published online: 1 April 2019

There have been many advances in sexual and reproductive health and rights in Australia but we must also recognise the gaps that affect our most vulnerable populations

Australia is a signatory to the United Nations Sustainable Development Goals, which see sexual and reproductive health and rights as central to achieving progress in health and gender equity by 2030.1 Historically, sexual health and reproductive health have been separate entities but the necessity of integrating them is now recognised. In May 2018, the Guttmacher Institute partnered with The Lancet to create a commission on sexual and reproductive health and rights,2 which proposes a bold vision in which everyone is able to exercise their rights and responsibilities regarding sexual behaviour and reproduction freely and with dignity. And while sexual and reproductive health and rights are universal, some populations have distinct needs (Box 1).

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  • 1 Family Planning NSW, Sydney, NSW
  • 2 University of Sydney, Sydney, NSW
  • 3 Royal Prince Alfred Hospital, Sydney, NSW
  • 4 Westmead Clinical School, University of Sydney, Sydney, NSW
  • 5 Western Sydney Sexual Health Centre, Westmead Hospital, Sydney, NSW


Correspondence: deborahb@fpnsw.org.au

Competing interests:

No relevant disclosures.

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Medical abortion: it is time to lift restrictions

Caroline M de Costa, Kirsten I Black and Darren B Russell
Med J Aust 2019; 210 (6): . || doi: 10.5694/mja2.50060
Published online: 1 April 2019

Lifting the special drug status applied to medical abortion medications will enable equitable access

In 2016, the Committee on Economic, Social and Cultural Rights (a collection of human rights experts tasked with interpreting these rights), in its groundbreaking interpretation of the right to sexual and reproductive health, asserted that abortion services are an integral part of the right to health.1


  • 1 James Cook University, Cairns, QLD
  • 2 University of Sydney, Sydney, NSW
  • 3 Royal Prince Alfred Hospital, Sydney, NSW
  • 4 Cairns Sexual Health Service, Cairns, QLD


Correspondence: Caroline.DeCosta@jcu.edu.au

Acknowledgements: 

We thank Philip Goldstone for his assistance in the preparation of this article.

Competing interests:

No relevant disclosures.

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