Sue Webzell is Haematology Nurse at the Hollywood Haemophilia Treatment Centre, Hollywood Private Hospital, Perth
Pre-conference the Australian Haemophilia Nurses’ Group met and had time to reflect on changes within the Haemophilia Treatment Centres and in the bleeding disorders community. The nurses who had been to various other international and national conferences shared information and education with the rest of the Group. As a relatively new member of the Nurse’s Group, I saw that the time spent together in this way clarified management of patients and allowed for good relationships between Centres. The Group is always very supportive; discussion throughout the year via email is common especially around best practice.
Chair: Suzanne O'Callaghan
A personal story of hep C treatment ~ Simon
Hepatitis C update – A/Prof Joe Sasadeusz
HIV clinical update – A/Prof Edwina Wright
A personal story about living with HIV – Anthony
Historically hepatitis C and HIV has caused significant problems within the bleeding disorders community due to transmission via blood products prior to the 1990s. This session discussed these health concerns and offered a very encouraging future for patients who have been affected. It used both health professionals and patients to give a greater overview of the problems, the new treatments and a personal experience of living with these viruses.
A/Prof Joe Sasadeusz explained that hepatitis C is now a curable disease: a 95% response rate has been seen with the new treatments. These new treatments have been rolled out across Australia and can be prescribed by general practitioners as well as specialists, side effects are minimal and they can be used to treat a wide range of patients. Over 30,000 prescriptions have been dispensed in Australia in 2017.
A patient gave a personal experience which looked at his feelings of ‘a strange sense of shame, worry and wondering what might happen into the future’ after contracting hepatitis C from blood products taken for a couple of tooth extractions. He discussed previous interferon-based treatments and the significant side effects he suffered and the short lived response. He then talked of his experience with the newer much more tolerable treatments with which he had no side effects and achieved a cure.
A/Prof Edwina Wright spoke about the latest directions in HIV and treatment as prevention. WHO (World Health Organisation) is aiming to end transmission of HIV by 2030; Australia is striving for the same by 2020. This can be achieved by the offer of PrEP (Pre-Exposure Prophylaxis) treatment for all at risk people, education, and the use of HIV anti-retroviral treatments in people living with HIV.
HIV anti-retroviral treatments can reduce the viral load to undetectable and reduce transmission potential to virtually nil; and pre-treatment of non-affected partners or treatment within 72 hours after a potential exposure stops the virus’s ability to attach to the individual so infection does not take place. Using these medications to stop the transmission of the virus to the non-affected partner or the child has enabled people living with HIV to have children safely and given them the opportunity to create a family naturally.
Another patient who had been affected by HIV and hepatitis C gave an inspiring personal account of how he has lived with his diagnosis, the treatments and the support he has had from his family and friends.
Chair: Dr Mandy Davis
VWD – diagnosis, treatment and care – Dr Paula James
Living with VWD – my personal story – Brendan
Canadian haematologist Dr Paula James presented information on the current understanding of von Willebrand disorder (VWD). VWD is the most common bleeding disorder. It affects males and females equally but more women are diagnosed as their symptoms such as menorrhagia and post-partum haemorrhage are more likely to lead to investigation. 1 in 1000 people are thought to have VWD, but many are undiagnosed. VWD can cause excessive bleeding especially of the mucous membranes. Von Willebrand factor (VWF) is normally present in adequate quantities in the bloodstream. VWF is essential for blood clotting; it sticks platelets down in vascular injury and carries factor VIII. In VWD there is either a reduction in the amount of VWF in the bloodstream or it doesn’t function properly. For example, in type 1 there is not enough VWF (70-80% of patients), in type 2 VWF does not work properly (15-30% of patients), and in type 3 VWF is absent (rare).
Levels of VWF can be significantly affected by stress, exercise, pregnancy, hormones, bleeding, infection and age, so repeat testing may be required and timing is important. Day 1 of a period is a good time to test as levels are often lower during menstruation and more indicative of VWD. There are numerous treatment options for intermittent or long term control such as birth control medications, Mirena Coil, DDAVP (desmopressin), tranexamic acid, factor replacement with VWF and FVIII (at present plasma derived but recombinant product should be available in the future) or in some women surgical options such as endometrial ablation or hysterectomy may be used.
Even patients with mild VWD will likely require some treatments if undergoing any surgery or dental work. Trials to assess the effectiveness of treatments such as DDAVP are required pre-operatively; the responses can vary significantly between different people.
Dr Paula James also discussed her website ‘Let’s Talk Period’ (https://letstalkperiod.ca) that has a self-directed bleeding score tool to assist women to determine if they need to get further testing for a bleeding disorder . The intention is that some of the currently undiagnosed women will be able to do a basic self-assessment and then seek further assistance in diagnosis and treatment if appropriate.
Chair: Claude Damiani
My experience with an extended half-life product – Paul
Supply and purchasing, new treatments, cost effectiveness assessment/funding pathways to new medicines – John Cahill
Extended half-life factors and other new therapies – Dr Huyen Tran
Gene therapy – Prof John Rasko
These speakers presented on a range of the new therapies for haemophilia that may be available soon for some patient groups. One type of new treatment is extended half-life products, which decrease the frequency of factor replacement infusions as there is a higher amount of factor present in the body for longer periods. Monoclonal antibodies with easier delivery methods such via a subcutaneous route continue being investigated within trial patients. Genetic therapies for a potential ‘cure’ are also being trialled and showing promising results. These have shown the potential to change the face of haemophilia significantly; however, there are many hurdles to be overcome and questions to be answered before these treatments can be used widely within the bleeding disorder population.
For the health professionals there were also industry-sponsored breakfast, lunch and evening sessions with expert speakers showing new techniques such as ultrasound and recent research results of upcoming treatments, research case studies including videos of participants’ experience of participation, and information on best practice techniques, all making for a very full, very interesting but tiring few days.
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