bloodline

December 2011
Volume 39 I Number 4

16th Australia & New Zealand Haemophilia Conference

bloodline supplement

December 2011
Volume 39 I Number 4

The

Word
In this, the last Bloodline for 2011, I would like to pay tribute to KiwiFirst. This year marks 15 years of partnership between KiwiFirst and HFNZ. KiwiFirst is responsible for a large proportion of our fundraising from the New Zealand public as well as providing information and updates about the work of HFNZ. Without this partnership, we would certainly not be able to run the many programmes that we do. As a result of the hard work of KiwiFirst and the generosity of the public, we have individuals and families that contribute financially to our organisation every year. We have even received bequests from individuals, families and estates. On behalf of HFNZ, I pass on our heartfelt thanks for the dedication of the team at KiwiFirst. Of course, the success of our fundraising is also closely linked to the generosity of the New Zealand public, so the success of KiwiFirst is also a tribute to the many people from all over the country that have chosen to donate to our cause over many years. With sincere thanks, Deon York HFNZ President

CONTACT DETAILS Website www.haemophilia.org.nz National Office 4 Washington Way, Sydenham, Christchurch PO Box 7647, Sydenham Christchurch Phone: (03) 371 7477 Fax: (03) 371 7479 info@haemophilia.org.nz President Deon York deon@haemophilia.org.nz Chief Executive Officer Belinda Burnett belinda@haemophilia.org.nz Editor Chantal Lauzon chantal@haemophilia.org.nz Manager Outreach Services Colleen McKay colleen@haemophilia.org.nz Linda Dockrill Southern Outreach Worker PO Box 7647, Sydenham Christchurch 8230 Ph: 03 371 7485 Fax: 03 371 7479 linda@haemophilia.org.nz Lynne Campbell Central Outreach Worker PO Box 24014 Manners Street Central Wellington 6142 Ph: 04 382 8442 lynne@haemophilia.org.nz Joy Barrett Midland Outreach Worker PO Box 4357 Hamilton East Hamilton 3247 Ph: 07 856 4442 joy@haemophilia.org.nz Sarah Preston Northern Outreach Worker PO Box 41-062 St Lukes, Mt Albert Auckland 1346 Ph: 09 845 4658 Fax: 09 846 8174 Mobile: 027 512 1114 sarah@haemophilia.org.nz

A first! Australia and New Zealand Inhibitors Workshop

On the evening following the close of the 16th Australia and New Zealand Haemophilia Conference, 25 people gathered for the official opening of the Australia and New Zealand Inhibitors Workshop at the Novotel Sydney Olympic Park.
Participants came from all around Australia and New Zealand and included parents of children with inhibitors, as well as men with inhibitors of all ages and their partners or a support person. Inhibitors are a serious medical problem that can occur when a person with haemophilia has an immune reaction to treatment with clotting factor concentrates. They are considered one of the biggest challenges in haemophilia care today. This educational workshop was the first ever Trans-Tasman event focused specifically on the challenges faced by families with inhibitors. After the workshop was opened with a Mihi Whakatau (a special prayer of welcome and introduction) and Waiata (song), workshop co-ordinator, Colleen McKay, welcomed everyone in anticipation of two days of learning, laughter and support. A little friendly competition with the challenges over dinner ensured that participants had the opportunity to mix, mingle and meet each other in a fun way. and were reminded of the importance of seeking out help if you need it with suggestions of where to turn. Ian DYoung, Auckland-based Haemophilia Physiotherapy Practitioner, then gave tips on Managing Chronic Arthropathy for those with Inhibitors. Participants were invited to Have a go at Tai Chi. While some loved it and others clearly did not, the session was useful in demonstrating non-traditional forms of exercise. Dinner on Sunday evening was short because of a very important engagement the final of the Rugby World Cup complete with the pleasing result of a win for the All Blacks!! People came dressed to represent their favourite sports team everything from the Canberra Raiders to the All Blacks (of course!!).

Contents
A first! Australia and New Zealand Inhibitors Workshop...................................1 My experience: the Australian & New Zealand Inhibitors Workshop.................3 Letters to the Editor.........................................................................................3 Thank you KiwiFirst!.........................................................................................4 The H Project....................................................................................................7 Managing HCV-related Fatigue........................................................................8 WFH Global Forum on Safety and Supply ........................................................10 So long Judy!....................................................................................................14 MRG Reports....................................................................................................14 Council Corner..................................................................................................17 Treatment and Welfare Package for people with hepatitis C: 5 years on...........18 News in Brief....................................................................................................19 Dates to Note...................................................................................................38

Gaining understanding, empathy, knowledge, and forming new relationships with carers, haemophiliacs, health professionals has given me better support
On Monday morning Scott Dunkley, Haematologist from the Royal Prince Alfred Hospital in Sydney hosted an informal session which encouraged participants to ask their individual questions. The Treatment of Inhibitors is challenging and the message that came through was that the treatment of inhibitors is not an exact science; there is not one answer and treatment plans vary between individuals as what works for one person might not work for another. Scotts session also illustrated the importance of monitoring and maintaining communication between all members of the comprehensive care team. The onsite nurses - Mary Brasser (Auckland), Grainne Dunne (Sydney) and Beryl Zeissink (Brisbane) then tested everyone with a Quiz, helping everyone December 2011 BLOODLINE 1

Thanks for the entertainment, laughter and learning!!!

On Sunday morning the Inhibitors Workshop got underway at the same moment as 500 huge honking trucks arrived in the Olympic Park Village as part of the Convoy for Kids Charity Fundraiser and Family Day, a yearly event where truck drivers help raise money for the purchase of equipment for the Childrens Hospital at Westmead. Despite the noise and spectacle, Colleen kept everyone on track. As one participant commented, Even 500 trucks couldnt silence Colleen. In the Caring for ourselves session, Social Workers Sarah Preston and Linda Dockrill introduced the concept of Te Whare Tapa Wha as a model of health and wellness. Together the group discussed each of the four cornerstones of health (physical, mental, social and spiritual)

bloodline supplement
16th Australia and New Zealand Haemophilia Conference.............. 20

Cover photo:

Toby Scott and Corin Hancox showing their support for the All Blacks during the Inhibitors Workshop in Sydney.

Disclaimer: The information contained in this newsletter is not intended to take the place of medical advice from your GP, haematologist or specialists. Opinions expressed are not necessarily those of HFNZ. The purpose of this newsletter is to provide a wide range of accurate and timely information on all aspects of haemophilia and related disorders. Haemophilia is a dynamic specialty and therefore opinion may change or be varied from time to time.

A first! Australia and New Zealand Inhibitors Workshop...

learn in a fun and competitive way. Messages such as using P.R.I.C.E. and tips for treating nose bleeds were reinforced with the Quiz. Katrina Smith from the Independent Living Centre presented participants with a range of gizmos and gadgets designed to assist with getting through the day. She brought along many examples for people to examine. Independent Living Centres do not sell products, but provide an information service which has examples of a wide range of aids for mobility and daily living. There are Independent Living Centres or Enable centres located throughout Australia and New Zealand. The group was then treated to an impromptu and spine tingling haka from T.A. and Holden Stirling, bringing the workshop to a fitting close. All participants left the workshop with more knowledge, having connected with others with inhibitors and with an exchange of email addresses for future support of each other.

My experience: the Australian & New Zealand Inhibitors Workshop

By Rob McIntosh
Overall, I enjoyed the Inhibitors Workshop. I learned a lot, but some particular snippets stick in your mind.
One of best presentations was from physiotherapist Ian dYoung. Although I had heard Ian speak at other HFNZ events, his theory of no gain, with pain really struck a chord compared to the common expression no pain, no gain. The Personal Journeys that some of the participants shared were very good as they really told of the journey their family had gone through with the diagnosis and attempts to overcome inhibitors. They each gave insight into how one really feels when dealing with it, especially listening to the joy and disappointments they felt. Sometimes you have to take risk with new treatments and you just have to trust that you are doing the right thing for you or your child. The Tai Chi session was interesting. The moves are not hard but it proved to be a little difficult to get the coordination quite right. You dont realise how much you are using your muscles and joints without putting any strain on them. Since the workshop I have found some local classes and find that you move your whole body and can work away quite nicely. Dr Scott Dunkley was very easy to understand and it was refreshing that he spoke in plain English and answered everyones questions in a frank way. It was interesting what came up. The quiz time with the nurses just proved how much we dont know.
Rob McIntosh in Sydney

I never knew there were so many gadgets and gizmos out there. There seems to be something to help you do virtually anything or help you adapt. One of the last sessions, Coping with Challenges - Finding Creative Solutions, demonstrated that whatever is wrong in life you just need to adapt to suit it. What hit home for me was how having inhibitors affects not only the person with haemophilia but the whole family. When I was growing up there was no treatment for bleeds except for rest and a cold compress. If I had a bleed it affected the whole family, especially if there was an outing planned. It would swell up and affect everything for a few days. All plans had to be cancelled. These same kinds of disruptions affect young families with children with inhibitors now. No matter the situation if their child has a bleed the parents have to drop things to deal with it. It can put a real strain on the family. There seem to be so many new treatments that will be arriving in the next few years. Each seems like it will be an improvement on the last. Some work by trying to get of the inhibitors or knock them back and others are just to treat bleeds faster. We are so lucky that things are improving and it seems to be happening quicker and quicker. It was over 100 years ago that they performed the first blood transfusion for haemophilia, and then around 50 years ago that Judith Pool discovered cryoprecipitate, then came the first concentrate AHF in the 1970s and things have just sped up ever since. The workshop was a great chance to meet people from Australia and other parts of New Zealand and hear their outlook. Even though we were all different ages, having inhibitors meant we had something very unique in common.

Thank you so much for all the organisation that went into this Workshop. I loved meeting everyone and hearing their stories
The Australia and New Zealand Inhibitors Workshop grew out of an idea that Colleen brought back from consultation with NZ Haemophilia Nurses and a conference and would not have been possible without the will and effort of many people. Thank you to both HFA and HFNZ for making this Inhibitor Workshop a priority for 2011 and all their work in making it happen. Thank you to NovoNordisk for providing funding for the workshop. Thank you to the Nurses and Social Workers who attended, as well as all the speakers. Thank you also to the participants for their sharing and caring of each other. Although the workshop has come to an end, it is just the beginning of being able to support each other.

Well organized and catered to everyones needs. A great way for likewise people to meet and feel involved when they rarely meet people with similar issues
The final session Coping with Challenges Finding Creative Solutions gave groups a chance to discuss their challenges, share their solutions, rate their top three strategies and report back to the whole group. Participants were split into four groups: adults with inhibitors, young adults with inhibitors, parents of children with inhibitors, and carers/partners. While some groups found it difficult to narrow it down to three tips, those common to most groups were: 1. Become informed or educated,. 2. Get connected to others with inhibitors. 3. Maintain a good support network. The Inhibitors Workshop finished with a Karakia from Patience Stirling and the Waiata Tutira Mai Ngi Iwi which carries messages of unity and learning. It is translated as: Stand forth people, Let us unite; Come forth people, Let us be one. Seek for enlightenment, and for love. All people, Work together, Unite together. Let us be one.

Kia Kaha Be Strong!! This Workshop is SO needed for the Inhibitor Community. Please dont wait too long for another one! Thank you! Thank you! Thanks You! Bloody excellent, cant wait for the next one!!!

Bloodline features a Letters to the Editor section. This gives you the members, health care professionals and other interested members of the public a chance to share your opinions, ideas and experiences with the readers of Bloodline. Please send correspondence to info@haemophilia.org.nz or by mail to Editor - Bloodline, HFNZ, PO Box 7647, Sydenham, Christchurch 8240. Bloodline is published in March, June, September and December of each year. Correspondence must be received at least one month prior to publication month to be included in current issue, otherwise letters may not appear until the following issue. Please keep letters relatively brief (250 words or less).

Letters to the Editor

Thank you for the publication of Paul Daggers account of becoming a Blackbelt in Tae Kwon Do in the September issue of Bloodline. His story is a very inspiring one and is one of the aspects of our Foundation I find so interesting and encouraging. We are all involved because we are affected by a bleeding disorder in some way, but as well we are all living our own lives, very individually, and finding our own path as we integrate this more unusual part of ourselves into normal life, Pauls story is one of great success irrespective of his medical condition. When you add the physical and mental difficulties a life of haemophilia has given him to this, it becomes so much more. When haemophilia became part of my life again, this time as an adult, I thought coping with it was all about treatment and seeing haematologists and getting on with it. After becoming involved in the Foundation, especially going to my first New Families Camp, I have learnt that in our country, where we are so well-treated (and so much better now than when Paul and his peers were children), the treatment, though terribly important, sits on a par with the mental attitude of those affected. This truth is all through Pauls account the tenacity, determination and self-belief he had throughout his journey to achieving the Blackbelt wasnt in a bottle of factor. Yours etc. Catriona Gordon

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Thank you KiwiFirst!

2011 marks the 15th anniversary of KiwiFirst raising valuable funds on HFNZs behalf to help us achieve our mission of providing haemophilia care, education, advocacy and support for people with bleeding disorders and their families/whanau in New Zealand.
Although HFNZ receives some funding from the government and our five Sustaining Patrons, the majority of initiatives and day-today costs are covered by public donations, grants, and fundraising activities. In 1996, despite the New Zealand Haemophilia Society (as HFNZ was then known) having developed a real knack for making the most of their H dollars, the demands of trying to ensure people all around New Zealand had equal access to the Societys support meant that a new way of funding programmes needed to be found. At a meeting held for Wellington members in winter 1996, Steve Waring, the manager of KiwiFirst, a company that raised funds for local charities, spoke about how he was confident about being able to fund every interested family in Wellington to Camp Blue Blood in Methven in January 1997. The story goes that although he has severe haemophilia himself, it had never dawned on him to raise funds for the NZHS until his sister, Rosalie, approached him about helping send his nephew to the camp. KiwiFirst fulfilled its pledge, and a very valuable relationship was born. The company initially created a number of booklets that offered coupons for use at local businesses, and taught the purchasers a little bit about the people they were helping at the same time. After the camp, KiwiFirst continued to raise funds for the Society, helping boost the Educational Trust Fund and support regional fun days and activities. The funds from KiwiFirst support over the years strengthened the Foundations financial position and has allowed it to expand its programmes and focus. KiwiFirst, which continues to be HFNZs primary fundraiser, manages to reach over 100,000 people each year to educate them about bleeding disorders and to raise a substantial amount of funds for the Foundation. In 2010, public donations made directly to HFNZ through KiwiFirst totalled just under a million dollars. KiwiFirsts efforts are deeply appreciated and gratefully received. Without their help contacting the public, raising awareness of haemophilia and the challenges it brings, and collecting donations, the Foundations education programmes and member programmes would simply not be possible. Over the years, the Supporters Newsletter distributed by KiwiFirst to educate the public about bleeding disorders has featured a number of our young members, each as cute as the last. Bloodline caught up with a few to see how they are doing now

Jayden Tupara
When Jayden appeared in the Supporters Newsletter we learned how at birth he suffered a near fatal internal bleed which left him with physical and developmental disabilities. Jayden was difficult to treat because he had developed inhibitors and, combined with other health issues, the future looked bleak as following a severe bleed his family were told measures should not be taken to prolong his life. After intense campaigning from his family and HFNZ. Jayden began to receive the standard of care he deserved. Jayden went through immune tolerisation therapy to overcome his inhibitors and thrived, growing into a young boy full of life. Jayden is now 16 years old and attends Kaiapoi High School, where he is a popular pupil. His favorite past times are listening to his iPod and watching rugby and rugby league on TV with his granddad, Tony. Margaret, Jaydens grandmother, reports that there is a lot of noise and colourful language to be heard during these games! Jayden has a particular love of water and goes to Hanmer Springs to enjoy the pools regularly. His enjoyment of the water has helped with maintaining his mobility over the years. The family is involved with CCS and has been attending activities such as mini golf with other families, which they are all enjoying. Jaydens wicked sense of humour makes him a memorable and fun person to be around.

Connor McCone
When Connor was 3 years old his bright smile appeared on the cover of the newsletter. Connor shared how having a port helped with treatment and allowed him to start prophylaxis. Connor now writes: Hi, It is around ten years since I featured with KiwiFirst as a poster boy. I am now thirteen. I attend Dunstan High School here in Alexandra where I am in year 9. My favourite subjects are Music, Art, English, Social studies and Science.

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Thank you KiwiFirst!...

I have played a variety of sports over the years including soccer, cricket, hockey and basketball. For the last few years I have been playing mainly hockey and basketball and have represented Central Otago in both. I also enjoy reading, fishing and music, and I am currently learning to play the guitar. I have received continuing support from the Haemophilia Foundation and have always enjoyed the Family and Teen camps at which I have made some really good friends. I have had the ability and knowledge to manage my own treatment since I was nine years old. This has allowed me to participate in my sport and other activities. Thanks to KiwiFirst for all your fundraising efforts! continues to play soccer and cricket. He is a very determined soccer player and was awarded several times as player of the match. Cricket season has just started and apart from the odd niggle with an ankle Liam is set to have another great season. Liam has learned over the last 6 months that when we have bleeds we must act promptly and the efforts of KiwiFirst to fundraise for HFNZ have meant that Liam has been able to learn the skills he needs to stay fit and well at the many camps that he has attended. It is fantastic to see Liam take responsibility for his health and wellbeing at such a young age. He is still swimming weekly and I am sure that part of the reason he maintains such good health is his fitness. He is now one of these boys who can treat himself and will show younger boys how he does it at these camps. It is through the HFNZ programmes funded through donations raised by KiwiFirst that Andrew has been able to adjust to his lifelong condition and grown and develop into the 11 year old he is today. Thank you to KiwiFirst staff for their efforts and to all those donors/sponsors of these events and other activities.

The H Project
Our team of seven were presented a collaborative project which had to benefit some type of charity. We chose HFNZ because of my connection with the Foundation. Our plan was to paint a large scale collaborative mural on canvas to auction, with all proceeds going to the Foundation. Before formulating a theme for the painting, we worked together to brainstorm ideas and draw concepts. We had numerous meetings which Lauren Nyhan (who has von Willebrand disorder) also attended to help generate ideas. After deciding on a final concept and theme, we were able to move forward with the painting. The idea behind the work was to portray two ways of thinking about Haemophilia. We have the red rose representing the conventional way Haemophilia is thought of, and the white rose representing a brighter way of thinking about Haemophilia. For example, the instant connection Haemophiliacs have with each other, even if we dont know each other. Also, the people we meet through having it. The name of the painting is Whakawhanaungatanga, meaning the process of establishing relationships, or relating well to others. We thought it appropriate, and the idea for the name came from the Hui Roopu Mori I attended in Hamilton. The script writing on the front of the painting has quotes that define attributes, strengths and characteristics of individuals with Haemophilia (Unique, Self Acceptance, Strength, Outstanding, etc). Our painting sold for $1000, which was more than we had hoped for which was amazing. We also had so much community support, with local and distant businesses donating to the charity auction. The total we raised was $1500, making our project a success. The team I worked with were so enthusiastic about the project which helped so much and we learned heaps about each others strengths and are somewhat closer because of it. We would like to thank all our sponsors: Nicolinis restaurant, Gorden Harris, Mind Body Studio, Country Style Furniture,

By Raukura Riwaka

HFNZ member Raukura Riwaka and a team of other students at Weltec created a large scale collaborative mural on canvas to auction in support of HFNZ. You can view a great video montage of their creative process and the auction on their H Project page on Facebook.
Resene for their donation of materials, and also those who donated to our cause. Editors Note: The painting was purchased at the auction by Steve Waring on behalf of his brother Martyn, who has reportedly fallen in love with the painting. Not wanting it to be shut away somewhere where it will not have the chance to be appreciated, Martyn has moved all the KiwiFirst and Tobros signage from the main wall in the foyer of their office building and has mounted Whakawhanaungatanga on that wall so that everyone who comes into the building can enjoyed it!

Andrew Scott
Andrew was featured in the Supporters Newsletter shortly before his third birthday. In the article his mother Lynley takes about the surprise of the diagnosis and how much there was to learn. Little did they know that not long after the newsletter came out that Andrew would also develop inhibitors and the family would be in for some very difficult times ahead. Andrew proved to be an incredibly resilient young boy. Lynley and Andrews dad Richard write: Andrew is now 11 and is an active boy in his school. He still has haemophilia of course. He enjoys swimming and drama classes after school. He has learned to inject his factor himself which he does three times a week. He has recently attended a Haemophilia youth and teen camp by himself (without mum and dad). He also goes to an annual regional haemophilia camp where we learn from other families and medical professionals about the latest treatment options. It is also a valuable time for parents to see how older boys and men have grown up and can allay some of their fears about marriage, job prospects and even reaching older age for their newly diagnosed children. It is through these events that Andrew has seen how older boys manage their haemophilia and how they treat themselves.

Liam Habershon
Liam was an energetic four and half years old when he appeared in the Supporters Newsletter. His diagnosis at 8 months was a surprise as there was no family history, but Liam had been lucky and did not experience many serious bleeds as a small child. His parents David and Lisa took it upon themselves to learn as much as they could about his bleeding disorder. Although they are a busy, active family, even his older sisters Rachel and Grace and twin brother James spring into action if Liam is hurt. Lisa writes: Liam, now 11 years old, is growing at a rapid rate of knots in to a lovely young man. He is an absolute ratbag at heart but tries really hard at school . He really enjoys his sport. With the wonderful support we have from our hemophilia team Liam

Raukura with the H Project team Martyn Waring, proud owner of Whakawhanaungatanga

The creative process

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Managing HCV-related Fatigue

Fatigue is physical and/or mental exhaustion that can be triggered by stress, medication, overwork, or mental and physical illness. Fatigue is the most common symptom reported by people living with hepatitis C (HCV).
study as many as three in four people with chronic, widespread musculoskeletal pain reported having fatigue. Start by talking your doctor or nurse. Report all drug and supplement use to your healthcare team. Include vitamins, herbs, over-the-counter and recreational substances as well as prescribed medications. These may be causing or contributing to your fatigue.

Hydration Dehydration can lead to fatigue, so maintaining

water intake is important. Drink before you feel thirsty and regularly throughout the day.

Laughter and recreation Participating in pleasurable

activities can be revitalising. Pick at least one enjoyable activity and find the time to do it regularly. Having HCV can be painful and burdensome if you let it. Look for opportunities to laugh, its not a cure but it can lighten the load. need it. Pushing yourself unnecessarily may prolong fatigue and make you feel worse.

Remember to report fatigue and other symptoms associated with HCV to your healthcare team. You can increase the quality of your medical care and general wellbeing by providing this valuable information while working in partnership with your medical providers. Although dealing with fatigue can be difficult, there are, fortunately, ways to manage it and stop it from ruining your life. By practicing some simple techniques you can reduce your risk for injuries and conserve your energy for the things in life that are more important to you. The most important goal is to take care of you. Article largely adapted from: Franciscus A, Porter L. (2008) A Guide to Understanding and Managing Fatigue. Hepatitis C Support Project: San Francisco, USA. This booklet and other downloadable resources are available at www.hcvadvocate.org

Self-help for managing fatigue

There are no approved medications to treat HCV-related fatigue, although a variety of drugs have been tried. Many dietary supplements or complementary medicines have been associated with fatigue management but they can have side effects and some should be avoided by people in some circumstances, especially people with cirrhosis or a bleeding disorder. Always talk to a qualified professional and your doctor before using supplements to ensure that is does not put you at an increased risk of liver damage or bleeding. Managing your energy yourself therefore becomes a priority. When we push ourselves beyond our physical capacity, good judgement declines and accidents are more likely to occur. When fatigue sets in, it is easy to become depressed or anxious about the future. There are many strategies for improving energy and managing fatigue. Try some of these simple tips and you may be surprised how much they help.

Listen to your body Allow yourself time to rest when you Nutrition A healthy and nutritious diet includes finding a
balance between the calories you consume with the amount of energy you use. Eat small frequent meals, made up of lowfat, high-fibre foods and a variety of fruit, vegetables and whole grains.

Organise Staying organised is sometimes difficult but it is

the key to maximising limited energy. Clutter can create stress so try to maintain organised work and living spaces so that the thought of cleaning does not get overwhelming. not important and increase your rest. Do not sacrifice needless energy at the expense of more important areas that provide more balance in your life. There are many alternatives to common chores such as to allowing dishes to drip dry, buying clothes that do not need ironing or buying pre-cut vegetables.

Pace and prioritise Decrease certain activities if they are

In a recent survey sent out by HFNZ, fatigue was identified as the symptom that most affects people with haemophilia living with chronic hepatitis in NZ, with over 80 percent of respondents reporting that they have suffered from fatigue in the past year.
Everyone experiences fatigue occasionally. It is the bodys way of signalling its need for rest and sleep. It can become more serious when fatigue becomes a persistent feeling of tiredness or exhaustion that goes beyond normal sleepiness. Physically, fatigue is characterised by a profound lack of energy, feelings of muscle weakness, and slowed movements or central nervous system reactions. Fatigue can also trigger mental exhaustion. Persistent fatigue can cause a lack of mental clarity (or feeling of mental fuzziness), difficulty concentrating, and in some cases, memory loss. Fatigue can range from mild to severe and can affect every area of life. It affects everyone differently and cannot be measured by lab tests, making it difficult to evaluate. Some people with HCV have constant fatigue while others may have fatigue cycles. Sometimes they feel energetic while other times they may feel so tired that they are unable to perform basic daily functions, such as going to work, cleaning the house or engaging in social events.

Personal appearance and grooming Try to maintain

good grooming habits. You will generally feel better if you take care of yourself and are freshly groomed.

Ask for Help Do not be afraid to ask for help from family

or friends. People are usually willing to help but may not want to interfere in your life. If you have available resources, consider using a laundry or house cleaning service. The key is to simplify where possible. powerful ally. Watch for negative self-talk. Fatigue with humour is more tolerable than fatigue with misery. breathing exercises.

Stress reduction and relaxation Many factors

Attitude Attitude cannot cure fatigue, but it can be a

can cause or intensify fatigue. Try simple stress-reduction techniques such as taking a warm bath, stretching, sitting or walking in a place of natural beauty, or try meditation.

Stock on supplies Since the duration and intensity of

Breathe Incorrect breathing can lead to fatigue. Try deep Conserve energy There are numerous strategies that can
help conserve energy such as sitting whenever possible, bathe before going to bed rather than the morning, maintain good posture and comfortable work heights, work in well-lighted environment with good ventilation, wear supportive and comfortable shoes, and avoid stress and rushing. Pace yourself.

fatigue cycles can be unpredictable, prepare for these times by stocking up on supplies. Buy extras of items you regularly use such as toilet paper, soap and non-perishable food. have time. Taking short naps or rest periods may help. Try not to sleep too much during the day because this could affect how well you sleep at night. Getting too much rest may make you even more tired so try to find a balance.

Rest It is important to rest when you get tired or when you

Causes of fatigue
Before you assume your fatigue is associated with HCV, rule out other causes. Fatigue is a symptom of many conditions other than HCV infection. Common examples are thyroid dysfunction, anaemia, depression, sleep apnoea, and vitamin deficiency. Poor diet, dehydration and lack of exercise can also lead to fatigue. Chronic pain and fatigue also often occur together in one

Educate friends and family Talk to your friends and

family about what it means for you to be fatigued. Sometimes just knowing that you can cancel or leave an engagement early relieves the stress of feeling that you are letting people down.

Exercise It may seem counterintuitive, but regular

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light to moderate exercise is one of the best strategies for combating fatigue. Start slowly and do not overdo it. Talk your physiotherapist before starting for tips and to help identifying the best exercises for you.

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WFH Global Forum on Safety and Supply

The 7th WFH Global Forum on the Safety and Supply of Treatment Products for Bleeding Disorders took place 22-23 September in Montreal, Canada. Two representatives from New Zealand, HFNZ Vice-President and Safety & Supply Officer Catriona Gordon and CEO Belinda Burnett, were among those from over 30 countries who took part in the important biennial meeting. Deon York, HFNZ President, in Montreal for the WFH Executive meeting was also able to attend.
precautionary principle, resulting in like situations not being treated alike, and dissimilar situations being treated similarly. The consequence of this has been inconsistency of decision making. The pursuit of freedom from transmissible harmful agents (THAs) has been both spectacularly successful and increasingly expensive and controversial, while impacting on the supply of blood use. Early risk interventions for HIV and HCV testing were unanimously adopted with demonstrable increments in safety. Recent interventions, including vCJD deferral and universal prestorage leukoreduction, have been marked by dissention and disagreement. The following concerns have been expressed: - risk assessments biased toward product quality focused on THAs at the expense of process integrity This Global Forum focused on three themes: Perspectives on risk including donor screening, viral testing and new infectious agents. Achieving a safe and affordable supply including innovative global projects to improve supply, changing trends in national tenders and bio-similar (follow on biologics) policies. Novel technologies including updates on gene transfer studies, longer lasting products and other innovative product developments. Here Catriona Gordon reports on two key issues discussed at the Forum; risk-based decision making for blood safety and an innovative gene transfer study. - regulations are unduly self-protective for governments - risk reduction measures are driven by industry - some actions have created paradoxical reductions in transfusion safety In the absence of an appropriate decision making framework, the pursuit of zero risk creates an atmosphere in which no intervention is deemed too expensive. The socio-political concerns include the scarcity of resources, including health resources, the implications of adopting or not adopting an appropriate decision making framework and whether the same resources could provide better benefit elsewhere (the opportunity cost). The sustainability of the current paradigm is questionable. Zero risk should be neither the mandate nor the goal. The concept of acceptable risk is challenging, but is a more sustainable concept. The issues to be decided are then: - To whom should the risk be acceptable? - Under what conditions will the risk be acceptable? An international consensus conference was convened because blood safety decision making was becoming increasingly complex, inconsistent, difficult to explain and not obviously risk-based. Dissatisfaction in the process was being expressed by blood system operators, reimbursement/funding agents, industry, patient groups, governments, regulators and others. It is important to ensure that the decision-making process addresses as much the science of blood safety, as the ethics, politics, social values, economics, public expectations and historic context in which we exist and operate. There was also increasing recognition that not all systems or jurisdictions need to have the same safety criteria or threat responses. This is because the nature, timing and localisation of the threat will impact the response required, and also because political, economic, public health policy and other

relevant factors may influence the decision to act or not. There needs to be an appropriate basket of safety measures to differ across jurisdictions. Ideally the process of decision making should be standardised, and principles of proportionality, nondiscrimination and consistency should be considered.

Can we achieve a common decision making framework for blood safety?

In order to do this we must first understand the domains of risk, which include risk assessment, risk perception, acceptable risk and risk communication. It is necessary that we work towards paradigms other than zero-risk, and recognise both risk and cost as socio-political decisions. The acceptability of risk is, in the final analysis, its acceptance by society, particularly by those who perceive themselves to be the bearers of the risk. For this to occur there must be transparency and public participation in the decision making process. It is important to avoid past temptations, for example to blame the Regulator, and it is essential to speak a common language regarding risk. This international consensus conference was not the first attempt of the blood industry to grapple with this question. The Alliance of Blood Operators has focused on this as an issue of global strategic importance, and one that is critical to sustainability. This Alliance includes the following organisations: American Red Cross Australian Red Cross Blood Service Canadian Blood Services European Blood Alliance Hema-Quebec The additional partners were supportive of the need for a consensus forum, as well as the need to engage expertise outside the traditional blood arena, to ensure the process is iterative, transparent and involves all stakeholders and relevant special interest groups. It was a collaborative effort committed to a process in which the long-term objective is worth the effort. The Consensus development process was modelled on the NIH Consensus Development Process. It was the seventh such Consensus Development Conference hosted by the Canadian Blood Services. The Consensus panel comprised crossrepresentation of international experts in risk, communication, health policy, as well as lay and patient perspectives. There was a programme of presentations by invited experts in various fields. The audience contained delegates, who were able to participate through questions and comments. The statement was drafted at the meeting. An iterative process followed the meeting to finalise the consensus statement. Five questions were posed, from which the consensus statement was developed. 1. What are the key aspects and limitations of current decision making in blood safety? The current blood safety paradigm was described as a drive to minimise risk to the lowest possible levels with secondary attention to cost. There was a focus on infectious disease

transmission and a reliance on the precautionary principle. Efforts were made to engage stakeholder community, specifically donors and recipients. There was emphasis on product quality with a lesser focus on transfusion processes. The rigorous safety regimens are heavily shaped by lessons of 1980s, with a fixation on avoiding repetition of these events. Current blood policy and regulation underpinned an unachievable zero risk orientation which delivers marginal and incremental improvement in blood safety outcomes over time, with an unsustainable trajectory. There is an unclear consideration of costs, either direct or opportunity. Health spending is likely to more severely constrained, and the trend will be accelerated by the global economic crisis and by changing demographics. The consensus panel noted that broader public and private sectors had seen a greater focus on improving risk management practices and risk based decision making. This focus had been in response to major events such as the collapse of large institutions, public policy and regulatory failures, and global financial crisis. It was worthwhile to compare the experiences of the blood industry with other sectors in terms of trends in risk based decision making and risk management. The evidence suggests a need for fundamental change. There are five critical dimensions of a comprehensive approach to blood safety: 1. An integrated risk management framework that encompasses vein to vein, and beyond; 2. Decision making based on transparent principles of risk management 3. A mechanism to balance risk, costs and benefits in a sustainable manner 4. Meaningful engagement with interested and affected parties throughout the process of risk decision making 5. A risk management strategy within the context of wellestablished ethical principles to ensure that the rights of both donors and patients are respected. The assumptions used in making the consensus recommendations were that an integrated risk management framework means all sectors of the blood system, nationally and internationally, are included in the decision making. Vein to vein encompasses issues related to donation on one end and extending all the way through to potential adverse effects of transfusion in recipients on the other. The precautionary principle suggests that, in the interests of public health, risk management action should be taken in the absence of certainty about risk. The goal of proactive risk management is to anticipate, prevent and mitigate risk to the extent that is possible. Risk based decision making implies risk management actions proportionate to the level of potential risk reduction. 2. What are the best practices in decision making to be leveraged and in what manner should they be applied? Decision making about blood safety shares important features in common with decision making in other sectors. Principles and guidelines of risk management need to be developed.

Risk-based decision making for blood safety

Judie Leach Bennett from the Canadian Blood Services presented a paper on the recent International Consensus Conference. The risks of blood, and the concerns about blood safety are of major concern to recipients, the general public, media, public policy makers, regulators and industry. Actual and perceived failures of action in the 1980s and again in the 1990s have dictated subsequent responses, actions and interventions. It is questionable whether these have been appropriate and /or consistent. Political, medico-legal, and governance consequences have caused decision makers to implement numerous safety measures aimed at maximizing safety. There have been few quantitative risk assessments carried out. Nor have there been many cost-benefit and cost-effectiveness analyses, and what few there have been have been limited in their study of the scope and impact. Compounding this problem has been the historical attitude of taking no action until there is sound evidence in some cases; while in other cases there has been the misapplication of the

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WFH Global Forum on Safety and Supply...

Risk management is developed through a framework for decision making that explicitly acknowledges the central role in risk in a given sector. The framework involves risk identification, analysis and evaluation (and where possible, anticipation), including all risk monitoring and communication. Effective risk management will typically incorporate methods of qualitative and quantitative risk assessment. Principles include appreciation of uncertainty, tailored and timely responses, cultural sensitivity, transparency of process, openness, iterative quality improvement, practicality and accountability of decision-makers. Blood services, like other health care services, share three important attributes: Subject to economic constraints; costs are direct, indirect and opportunity, and may vary based on background conditions, for example funding mechanisms, liability climate, cost of testing methods. Responsibility for safe and effective blood rests at many levels within the health care system. Successful risk management framework must work across stakeholder institutions and entities. Blood services are a distinctly social good, with attendant societal obligations of safe and efficient production and fair and accountable distribution. Blood is a special kind of medical resource. The altruistic nature of the gift, unlike most other therapeutic modalities, proscribes a special meaning on blood. The unique attributes of the raw material and gift make tailoring a risk based management framework particularly important and challenging. 3. What benefits can be achieved in the development of a framework? The need for integrated risk management frameworks which address all major policy and operational challenges is clear. It should provide measurable objectives, improve consistency in decision making and improve confidence and trust in the investment decisions. It should allow redirection of resources this improving efficient and effectiveness and increase transparency and communication giving it defensibility and buy-in across the system. Without an integrated risk management framework, the current situation of uncertainty, suspicion and, at times, poor decisions will persist. A common international risk management approach across jurisdictions provides all the benefits of a nationally integrated risk management system and more, such as a scale of assessment of evidence and effectiveness, and avoiding duplication of decision making. It is necessary to take into account differences in government, system governance, resource capacity, history, legal systems, and community attitudes. Flexibility must be built into the parameters of the framework. Regulators work within standards adopted by each respective jurisdiction. Reliance solely in regulatory oversight is important but limiting, and represents a failure to take advantage of enterprise risk improvement ethos. Regulators must seek to collaborate across national boundaries. 4. What are the components to be incorporated in the design of a framework and what does the framework look like? Risks are borne exclusively by recipients. Standard riskbased decision making frameworks are not immediately or directly transferable to the issue of blood. An integrated risk management system requires reliable and valid data on risk profiles. The analysis of costs of treatment, costs of failing to treat and the benefits of treatment are required. Risk assessments should be placed within a broader ethical system of decision making where all members of the system respect each others core values. Experts need to be scrupulous in recognising and acknowledging the limits of the data and use models and modelling as appropriate. Interim assessments need to be examined, validated and opportunities for continuous learning and improvement built in. There must be an avoidance of silo activities in the blood system all players to come together to develop a collaborative process of risk identification, risk assessment, shared metrics, shared evaluation of the reliability of data and shared evaluation of outcomes. No one economic model is inherently preferable to another it is problem and context specific. An appropriate governance structure is essential to ensure all participants act in a coordinated way. A well-defined accountability system is needed to ensure objectives are met and any failure to meet objectives is quickly addressed. All participants have responsibility to ensure that they are using best practices. Multinational collaboration does not mean individual actors should delay developing their own risked based decisions. Since not all risks can be eliminated from the blood system, consideration needs to be given to individuals who have been harmed. 5. What are the necessary next steps to agree upon and implement a risk based decision making framework for blood organisations? To fully implement the five principles that the panel recommends, concrete steps need to be taken. There must be a commitment by all the leaders in the blood system to implement a risk-based decision making framework. There should be agreement on governance structures and a definition of objectives and priorities. There needs to be investment in assessment, evaluation and monitoring. Active prospective biovigilance/haemovigilance systems should be established. There must also be a commitment to investment in research, innovation and education, staff training and infrastructure. In conclusion, the consensus conference has been a unique and timely opportunity to broaden and strengthen approaches to blood safety in an integrated and comprehensive way. The fact that international blood operators convened this consensus forum is laudable and a significant first step. Implementation of an integrated risk management framework must be led by those responsible for blood safety. Doing so will require convening stakeholders over time, to clarify objectives and priorities, gather necessary data, identify knowledge gaps and construct a methodology appropriate to this community. Moving forward in a manner drawing on rigorous risk assessment will determine the success in building and retaining trust that the blood system requires. Following the conference the response from participants showed that a large majority expressed strong support for the outcomes. There was a general feeling that the consensus statement met participant expectations although some had hoped the panel could have gone further in terms of defining the decision making framework per se. Most recognise that is the work of the blood community. The outcome of this meeting is the first step down a new path. International socialisation of the issue is necessary. To effect change, an ongoing concerted effort with other stakeholders internationally will be required, including joint efforts on simulation models to help create and test a risk framework. This was done by using the AAV serotype 8 to circumvent preexisting immunity to AAV2. In the third study the vector was delivered using peripheral veins. The reasoning was to determine whether it made a difference if the vector was delivered by peripheral veins, or directly into the hepatic vein or into the liver itself. In the clinical trial, the primary objective was, naturally, the safety of the patients. The secondary objective was to determine the dose required to raise levels of FIX in patients with severe Haemophilia B. The study consisted of six subjects two requiring low levels of factor replacement, two requiring intermediate levels of factor replacement and two on high levels of factor replacement. Dr Nathwani reported that Subject 1 showed clear FIX expression sufficient to stop prophylaxis The other low and the intermediate dose subjects were able to stop prophylaxis or reduce prophylaxis with no raised liver enzyme levels. The first high dose subject is now off prophylaxis. Initially his liver enzymes were raised. This was dealt with using steroids, and he now has continued raised levels of FIX. The other high dose subject has also shown continued raised levels of FIX. This study is very interesting to our community. As the Chair of this part of the Safety and Supply Conference commented, for many years we have been told that a cure for haemophilia using gene therapy is only five years away. With this study by Dr Nathwani and his associates, there is now evidence that gene therapy may indeed be the path to such a cure. Dr Nathwani commenced his presentation with a tribute to these six very brave men who had consented to be part of this trial. They already were living with a chronic disease and yet were willing to venture down an unknown path for the benefit of our community. These men will continue to be studied for the rest of their lives.

Update on Gene Transfer Studies

Dr Amit Nathwani, from University College London, presented a paper on the gene therapy research he has been conducting. He explained that Haemophilia B is a great model for early proof of the concept of gene therapy. It is a monogenetic disorder, and the therapeutic change goal is modest a reduction in the amount of factor given to the patient. He uses adeno-associated viral (AAV) vectors for the haemophilia gene therapy. This is a non-pathogenic single stranded DNA based parvovirus. It replicates only in the presence of a helper virus and is easy to render gutless which means there is a reduced risk of an immune response to viral proteins. The previous Haemophilia B clinical trials were conducted with AAV serotype 2 vectors. The first study targeted the patients muscles. The vector was delivered by an intra-muscular injection. This was found to have no toxicity but also no efficacy. The second study targeted the liver which is the natural site of FIX synthesis. In this study the lower doses were found to be safe but not efficacious. The higher doses raised FIX levels but an immune response followed which was identified as transient aminitis. This can be an indicator of liver damage. They then adjusted their approach to enhance the potency of AAV vectors to facilitate transduction with lower vector doses.

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So long Judy!
Judy Gilmour has been a haemophilia nurse at Auckland Hospital since April 2008. While we are excited to hear she is pursuing a new career path, she will be missed by her patients.
So long, farewell! Big apologies to those of you that I have not been able to tell in person but I am leaving the Haemophilia service in October 2011 to work with the Community HIV team based at Auckland hospital. Although I am sad to be saying goodbye I will be moving to a position that I have wanted to do for a long time. I have had a fabulous time with the haemophilia community and have particularly enjoyed the young families camp, telephone triaging and teaching opportunities here at the haemophilia treatment centre. I wish you all the very best and have my fingers crossed for longer acting and less invasive forms of bleeding disorder treatments for the future. Best wishes, Judy G sessions just so that we all know everyone else in our haemophilia community. I will keep you informed as dates come to hand. I believe Dr Jim Faed is moving away from treating those affected by Haemophilia and more towards research. For many people down south this means they will have a new haematologist. I hope the transition period is a rewarding one for all. It was sad to learn of the departure of Aly Inder a well loved admired and respected Haematology nurse in Christchurch. Aly was inspirational to most of us parents and to the boys she came into contact with, either in the clinic or at one of the camps or events days. Nothing was ever a problem, she always had plenty of time to discuss issues whether haemophilia related or not. Aly you will be sorely missed by us all! I would like to take this opportunity to wish our red zone counterparts in Christchurch all the very best as they move their precious cargo and memories from their damaged earthquake homes to their new homes. Know we are all thinking of you all. Lastly I want to wish you all a very happy and safe festive season, take care until next year.

MRG Reports
In the afternoon, back at the Ranch we heard from two speakers; a dentist from Wellington Hospital who talked about how to keep your teeth healthy (healthy gums dont bleed!) and HFNZ Manager of Outreach Services Colleen McKay who talked about the Twinning Programme with Cambodia definitely makes you realise how good we have it in New Zealand in terms of haemophilia care. On Sunday morning Carol Reddie ran an interactive poi making session thanks to ready-made packs provided by Colleen. This was fun and a new experience for some; four-strand plaits are harder to do than you think and so is swinging the poi. I have a new appreciation of poi performers. Petone Working Mens Club was the venue for Wellington Caf Evening on Wednesday 16 November. This was an adults-only event with a smorgasbord meal offering a range of food for every taste. Thanks to Willy Te Kira for suggesting the venue. The central region Christmas lunch was held on 27 November at Murrayfield Clydesdales in Levin. The younger ones spent time with the farm animals and imagined how life was in the old days in the heritage museum and miniature village. The children were thrilled to get an early Christmas present (provided by parents) and everyone enjoyed a delicious lunch. Best wishes to everyone for a healthy and happy summer break!

Central Region Report

By Stephanie Coulman

MRG Reports
HFNZ operates six groups that represent our members, four regional branches (Northern, Midland, Central and Southern), the National Youth Committee (NYC) and the Roopu, which represents Mori members.

About 35 central region members got together at our annual Winter Escape camp at El Rancho, Waikanae in September. The weekend kicked off (pun intended) by watching the opening of the World Cup Rugby together. On the Saturday morning we visited Nga Manu Nature Reserve and had a close encounter with a tuatara and attended an educational talk. The children also had the opportunity to feed hungry eels and learn about their precarious existence.

Midland Region Report

By Catriona Gordon The Midland Region has been busy since the last Bloodline was published, as the accompanying photos show. In September, we held a family day at Waitomo and had over 60 people attend, which was absolutely fantastic. We had a perfect spring day for it, which was somewhat ironic seeing as we were going to be spending a lot of time underground! Midland last went to Waitomo in 2006 and since then the Glowworm Caves Centre has built a very impressive new building, incorporating the entrance, caf and, of course the gift shop, through which you exit. Before we got to that stage, we first divided into two groups, with one group going through the original glow worm caves including the boat trip, and the other walking through the Ruakuri Cave which is a few kilometres down the road. The original cave is an amazing trip, with lots of the incredible glow worms. The trip ends with a boat ride, and at this point even the most distracted toddlers managed to put their perceived needs aside to marvel in silence at this natural wonder. The groups joined up for lunch at the caf, which proved to be a real sun trap. After a long winter it was nice to be reminded what summer feels like. We didnt have long before the groups headed off again to do their second cave. Ruakuri is a dry cave, so there are no glowworms. Instead, there are GIANT WETAS! Apparently we did not see any, but by a strange coincidence just as the guide told us about their existence the writer felt a sudden movement in her hair. Either it was a giant weta, or I just delayed the further development of a stalactite by a few weeks.

NYC Report
This year, the first year for the National Youth Committee, has been an exciting one for us and HFNZ, full of opportunity and experience. We have had two face-to-face meetings during the year and one Skype meeting as well. The second meeting of the international youth development programme Step Up Reach Out (SURO) 2010-11 was held in Auckland in March with Hemi Thomas from Auckland being the Kiwi representative. In September, Eathan Huston from Whaiheki was selected for SURO 201112 and travelled to San Francisco. Sam Hawkins and I also took up the challenge of going back to SURO in a mentoring capacity to help up skill and pass on our experiences to others.

As you will see in this issue of Bloodline Raukura Riwaka and his team at Weltech undertook a collaborative mural resulting in a fantastic and positive fundraising opportunity for HFNZ. Thanks for all the support from members and stakeholders this year. We are always on the lookout for more members in the 18-30 demographic so stick your hand up in your local area by calling 0508 FACTOR (322867)! We hope that you all have a great holiday season, a fantastic Christmas and we will see you all in the New Year! Cheers, Karl Archibald and your NYC!

Southern Region Report

By Theresa Stevens
Group eel feeding encounter

Well, another report due and I find it hard to comprehend that 2011 is almost behind us. Southern members in Otago had a great gathering when Linda our Outreach Worker was down a couple of months ago with around 20 folk including Josh, the haemophilia physiotherapist from Dunedin Hospital joining us for a BBQ and chat. Josh gave a 30 minute chat on physiotherapy and the importance of keeping our joints strong and healthy. He was most informative with models for demonstrating that were provided by a pharmaceutical company. Despite the wet weather we all enjoyed the get together. Huge thanks to the Southern Regional Branch for hosting this event. I intend to have gatherings 3 4 times a year, nothing too big coffee and cake

Boys waiting to feed eels; (L-R) Hannes Ramsay, James and Liam Habershon, Javaan de-Gaia, Nicholas Coulman

Group photo at El Rancho Waikanae, Saturday 10 September 2011

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MRG Reports
The younger members enjoyed the water slide multiple times, although it took some chin skin from Joys grandson, Cyrus, and there were a few other bumps suffered by the boys who wanted to find out for themselves why the sign said: Do not stand up. We had a BBQ lunch together, and managed to wind everything up before the rain set in. All in all, it was an excellent way to end 2011 in Midland.

Council Corner
Updates from the HFNZ National Council

The 2012 Budget and governance were the main focus for the last National Council meeting for 2011, held in Wellington on 18 November.
In terms of planned national activities for 2012, there is currently a concentration on education programmes for children and parents in 2012, with both Childrens Education Workshops and the biennial New Families Camp planned, but there is also a push from Council to ensure the needs of ageing men with haemophilia are being met. An ongoing theme for the Council for 2011 has been the style of governance needed by HFNZ. The National Council were unanimous in their view that they are not in a position to change management responsibility at this time. The National Council require alteration to how financial and programme reporting is delivered. Given the nature of our

organisation and the personal connection of all Council members to haemophilia, it is impracticable to achieve a complete governance model at this stage. It was decided that the next meeting in February 2012 would concentrate on setting strategic goals for each demographic of the bleeding disorder community and ensure the budget could accommodate these goals. The national office has already started putting together early plans for next years national activities. It has been recognised that more Outreach hours were needed. All four Outreach Workers will increase their hours by 5 hours a week (Southern and Midland from 20 to 25 and Northern and Central from 30 to 35). The increase in wages will be funded through the Verona McGregor funds for the Southern Outreach Worker and from the funding from the Ministry of Health for support of people with

bleeding disorders and hepatitis C. It is intended that the additional hours will primarily be spent supporting members with HCV. In other news, Mark Uren has resigned from his position as Southern Region Delegate. The National Council extend their thanks to Mark for his service. Rochelle Stott of Ohoka has temporarily taken on the position until the next Southern AGM. National Council also congratulate Midland Region Delegate Vicki Fitzgerald on the birth of her baby (a girl, Cordelia Paige, born on 21 November). If you have suggestions for strategic direction for the organisation or areas of need please communicate these to your local branch delegate, Youth Delegate, Mori Roopu delegate or directly to the President (deon@haemophilia.org.nz).

John Tuck and Lorraine Cross at Waimarino

A very pregnant Vicki Fitzgerald butters buns with Kerry Anne Dean and her mother Marcia

News from the North

By Lynley Scott The last few months have been quite steady for the Northern Region members starting with the Annual Movie Night in September, which also doubles as our Global Feast fundraiser. After a yummy roast, it was into the private movie theatre with lolly bags in hand big thanks to Wayne Hunter for organising this event.

Rex and a friend on the BBQ

Midland kids making a splash

The Australia and New Zealand Haemophilia Conference and Inhibitors Workshop saw a few Northern Members head across the ditch. Lynley and Richard Scott presented with Colleen about the Parents Empowering Parents programme at the Conference, Sarah Preston presented a session at the Inhibitors Workshop, and Holden and T.A Stirling performed a rousing Haka to close the Inhibitors Workshop. It was a great time of learning and meeting other families. The following weekend was the Armageddon Expo which is fast becoming an annual fixture. There were 17 volunteers required and we had enough for each day. T.A. and Holden worked two days - thanks guys. Patience Stirling used her contacts to get some extra volunteers who also worked two of the days. The wages that were generated by the volunteers work filling gift bags and various other jobs were converted into a donation for HFNZ. Richard and Lynley also attended the Masquerade Ball that was part of the weekends events, the over $2000 in proceeds from the ball also all went to HFNZ. Its a fun event to attend even just to see all the people dressed up in their costumes. Thanks to all who volunteered and we look forward to more volunteers next year. Fast approaching is the Christmas Party at Waiwera Hot Pools, a notice will be coming out soon and dont forget the annual camp 16th -18th March, 2012 and AGM. We have chosen a new venue at Mangowhai Heads just for a change and so put this date in your calendar and come join us. This camp is an all ages camp - its great to have lots of different age groups attend this helps our young people meet role models of all ages. Wishing you all a wonderful Christmas season and a bleed-free summer holiday.

Wendy Livingstone-Scott and her daughter Jodie Briggs

Gabriel Larkin Shae Cross

The entrance to Ruakuri is in a small park, so there was a chance to stand around in the sunshine once we had finished that walk and chat a little, before heading home via the maker of the biggest ice creams in the region. Our last event of the year was held on 6 November at Waimarino Adventure Park. This is another gem of the region, set beside the Wairoa River and offering loads of activities for all ages and stages. The weather was very typical of late spring, with four seasons in the space of 15 minutes. We were all pretty busy taking sweatshirts and sunglasses off and on. But that wasnt the only thing we did! We were able to take kayaks and pedallo boats out into the river, which was a great vantage point from which to watch the more adventurous members try THE BLOB. This is a giant floating pillow which sits on the water. The first person has to jump off a high board and land on it. This is Stage One. Sounds fun, doesnt it? Actually it was quite toe-curling. Once Stage One is completed, the first jumper sits on the edge of THE BLOB, facing the river and minding their own business, until Stage Two is completed. Stage Two requires another person to uncurl their toes and leap off the high board onto THE BLOB, catapulting the first jumper high in the air and then down into the water, without warning. It was a lot of fun and hilarious for the observers.

Give a little? Give a lot!

Charitable donations to HFNZ can now be made online at:

www.givealittle.co.nz/org/haemophilia

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Treatment and Welfare Package for people with hepatitis C: 5 years on

Belinda Burnett, HFNZ Chief Executive
In 2006, the Ministry of Health announced the delivery of a Treatment and Welfare package for people infected with hepatitis C from blood and blood products. The package was the result of over 14 years of campaigning for recognition, apology and fair recompense for victims of bad blood. The agreement was finalised between the Ministry of Health, Crown Law and HFNZ following comprehensive submissions and negotiations and was based on improved access to treatment. For the last 5 years, HFNZ has continued to work with and on behalf of people with bleeding disorders who were infected with hepatitis C and ensure they were able to access to the package. Lately, I have heard some criticism of the Treatment &Welfare package. This criticism may result from idealistic expectations of the package. I do not believe it is fair or correct to say that the Treatment and Welfare package isnt working, as it has delivered what was outlined. To clarify, Ive set out the terms of the Treatment and Welfare package and given an update on where we are at: 1. Provision for enhanced treatment The Government made HCV a priority and has provided the following since 2006: Standardised protocols of best clinical practice, this includes assessment of people for treatment, monitoring during treatment and post-treatment follow-up A Working Party (including a representative from HFNZ) to develop best practice guidelines for DHBs to use Update The latest news on this provision is that the Government have launched a new programme where the Hepatitis Foundation of New Zealand have secured a contract to strategise for Improvements in Hepatitis C Services, a large part of which will focus on increasing awareness, diagnosis and treatment. The working parties established over the years since 2006 have consistently included a representative from HFNZ. 2. Establishment of a one stop shop at ACC ACC has established a one stop shop for processing ALL aspects of ACC claims, managing the ongoing claims process and the decisions surrounding treatment and rehabilitation for all people who contracted HCV from blood or blood products. Update This ACC office is situated in Christchurch and HFNZ has a good relationship with the staff employed there. It is up to the individual if they chose to use this service or remain with their local ACC officer where they may already have an established relationship. 3. Acknowledgement of suffering and regret An expression of regret by the Prime Minister on behalf of Government for the suffering caused to people with hepatitis C contracted from contaminated blood was sent to our affected members. As acknowledged at the time, no amount of compensation can fully make up for the personal injury many of our members have suffered. The Treatment and Welfare package was and is broad enough to address the most immediate needs of people with hepatitis, but is bound by the limitations of the current health and ACC systems. HFNZ will continue to work on behalf of our members to ensure they are able to access all the treatment and support that is available. If you or a family member need further support with regard to any aspect of your hepatitis C please contact your Outreach Worker. Update This action has been completed. 4. Government Ex Gratia Payment to Assist Welfare and Treatment The Government made an ex gratia payment to people with haemophilia who contracted HCV. Update This action has been completed for all people who had applied and who had an ACC claim accepted for their HCV infection. There are no HFNZ members living with hepatitis C or who have cleared hepatitis C through treatment who have not had their claim accepted. 5. A reimbursement of actual and reasonable legal costs The Government contributed up to $4,000.00 plus GST as a reimbursement of legal costs to each person with a bleeding disorder who contracted HCV. Update This action has been completed as part of the settlement of the ex gratia payment. 6. A reasonable and justifiable contribution to HFNZs costs of its representation role Update The Government contributed $50,000.00 towards HFNZs legal costs. 7. A contract with HFNZ to provide representation and services for its members The Government agreed a contract for HFNZ to provide agreed representations and services to members with hepatitis C. Update This contract was initially for three years. It has just been renewed for a further three years and allows HFNZ to devote time and resources specifically towards supporting and providing information for members who contracted hepatitis C. 8. Confidentiality Members were asked to respect the confidentiality of the settlement and not discuss the contents with the media.

News in Brief
Bioengineered protein shows preliminary promise as new therapy for inhibitors
A genetically engineered clotting factor that controlled haemophilia in an animal study offers a novel potential treatment for human haemophilia and a broad range of other bleeding problems. The researchers took the naturally occurring coagulation factor Xa (FXa) and engineered it into a novel variant that safely controlled bleeding in mouse models of haemophilia. Our designed variant alters the shape of FXa to make it safer and efficacious compared to the wild-type factor, but much longer-lasting in blood circulation, said study leader Rodney A. Camire, Ph.D., a haematology researcher at The Childrens Hospital of Philadelphia. The shape of the variant FXa changes when it interacts with another clotting factor made available following an injury, added Camire. This increases the functioning of the protein which helps stop bleeding. Roughly 20 to 30 percent of patients with hemophilia A and 5 percent of hemophilia B patients develop inhibitors to haemophilia treatment. For those patients, the conventional treatment, called bypass therapy, is to use drugs such as factor VIIa and activated prothrombin complex concentrates (aPCCs) to restore blood clotting capability. But these agents are costly and not always effective. Camire added that, in the current animal study, they were able to show the variant protein is more effective at a lower dose than FVIIa. The range of options for hemophilia patients could improve if the study results in animals were to be duplicated in humans. The variant we have developed puts FXa back on the table as a possible therapeutic agent, said Camire. Naturally occurring (wild-type) FXa, due to its particular shape, is not useful as a therapy because normal biological processes shut down its functioning very quickly. By custom-designing a different shape for the FXa protein, Camires study team gives it a longer period of activity, while limiting its ability to engage in unwanted biochemical reactions, such as triggering excessive clotting. This potentially could lead to a new class of bypass therapy for haemophilia, by acting further downstream in the clot-forming pathway than existing treatments, said Camire, who has investigated the biochemistry of blood-clotting proteins for more than a decade. Further studies are necessary in large animal models to determine whether this approach can become a clinical treatment for haemophilia patients who have developed inhibitors, or even more broadly as a drug for uncontrolled bleeding in other clinical situations. The study appears in the November issue of Nature Biotechnology.

New Practice Guidelines for HCV Genotype 1 (U.S.A.)

In October, the American Association for the Study of Liver Diseases (AASLD) approved a new practice guideline for the treatment of genotype 1 chronic hepatitis C virus (HCV) infection. The new guidelines are for use by physicians. They include the addition of one of the two new protease inhibitors--boceprevir and telaprevir--to standard HCV treatment regimens. On May 13, 2011, the FDA approved Mercks boceprevir under the brand name Victrelis; May 23rd it approved Vertex Pharmaceuticals telaprevir under the brand name Incivek. The first new HCV therapies in 10 years, both products represent a new class of drugs called direct-acting antivirals that prevent viral enzymes from replicating. The new drugs help boost the success of HCV treatment, protecting patients from the potentially severe and lifethreatening impact of HCV symptoms, which include liver cancer, cirrhosis, end-stage liver disease and liver failure. The inclusion of a protease inhibitor increases the likelihood that a patient with HCV genotype 1 will reach SVR in up to half the time24 weeks vs. 48 weeks. The guidelines also note that a blood-based genotype test can be used to predict responses to HCV treatment with pegylated interferon, ribavirin and either of the protease inhibitors. Administration of these new drugs is not straight-forward and increases the complexities of patient management. The new AASLD guidelines review how and when to use these agents in the clinic. The recommendations are intended to be flexible, in contrast to standards of care, which are inflexible policies to be followed in every case. That flexibility will be necessary as treatment schedules will vary between patients and side effects will need to be managed carefully. The new drug regimen is not recommended for people with post-transplant HCV or those coinfected with HCV and HIV. Neither boceprevir or telaprevir are yet available in New Zealand. The new guidelines were published in the October 2011 issue of the journal Hepatology. The article is available with open access in the Wiley Online Library (http://onlinelibrary.wiley.com/ doi/10.1002/hep.24641)

coderouge for Women with Bleeding Disorders

The new Canadian Hemophilia Society program for women with inherited bleeding disorders coderouge aims to increase diagnosis and access to care for women and girls with inherited bleeding disorders in Canada. To promote this new program, a Web landing page has been developed, www.coderougewomen.ca, which takes visitors to the newly revamped section of the CHS Web site called Women.

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Contents
Wellness and Physical Wellbeing................................................................ 22 How a happier body can lead to a happier mind............................................................22 Caring for parents and carers............................................................................................22 Keeping the mind and body in tune - children..............................................................23 Keeping the mind and body in tune - adults..................................................................23 Staying on your feet - a session for adults of all ages.....................................................24 Mens business.....................................................................................................................25 Ageing...................................................................................................................................26 Other Bleeding Disorders........................................................................... 27
From 20-22 October, participants gathered in Olympic Park in Sydney for the 16th Australia and New Zealand Haemophilia Conference. The conference brings together lots of different people from the bleeding disorders community people with bleeding disorders and their families, healthcare workers, industry and HFA and HFNZ staff to discuss best practice on many topics related to bleeding disorders. The Conference is truly multidisciplinary with something of interest for all the different types of participants. The two full days of the Conference included sessions on treatment challenges, keeping the body in tune, von Willebrand Disorder, womens health, becoming an adult, ageing, rare bleeding disorders, living with hepatitis and/or HIV and mens business (aka having a healthy sex life). Several New Zealanders gave presentations or chaired sessions. Weve brought you the highlights of the conference as covered by HFNZ staff and members who attended. The slide presentations for most of the sessions are available in the Conference section of the HFA website (www. haemophilia.org.au) if you wish to seek more details. Usually held every other year, there will be no Conference in 2013 as Australia will be preparing to host the WFH World Congress in Melbourne in 2014. This will truly be a fantastic meeting of the global bleeding disorders community and will be an opportunity not to be missed.

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Von Willebrand Disorder...................................................................................................27 The rarer bleeding disorders..............................................................................................28 Womens Health & Reproduction ............................................................... 29 Living with Hepatitis C and HIV................................................................... 30 Communication and Social Media............................................................... 32 Treatment and Challenges.......................................................................... 33 The challenges of prophylaxis............................................................................................33 Transitions: becoming an adult.........................................................................................34 Treatment challenges..........................................................................................................35 Safety of current products used to treat inherited bleeding disorders........................35 Current and emerging challenges, risk factors for inhibitor development ................35 Update from Australian Haemophilia Centre Directors Organisation (AHCDO) Tolerisation advisory committee..................................................................35 Inhibitors in mild haemophilia ........................................................................................36 A personal experience of tolerisation...............................................................................36 Treatment and care - now and the future........................................................................36

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Wellness and Physical Wellbeing

How a happier body can lead to a happier mind
Lynne Campbell
From the moment she began her 1.5 hour session Anna Louise Bouvier, a Physiotherapist and mind-body expert, walked the walk convincingly exemplifying the relationship where body breakdown is a result of physical and mental stress. In her professional life Anna Louise runs her Physiocise program as well as her speaking programs to help people recognise the physical signs of stress and battle fatigue. She highlighted simple strategies that can be used to immediately improve health and wellbeing. Anna Louise acknowledged that in whatever capacity haemophilia affects your life, either as a patient, a parent, a carer or a health professional, the physical, emotional and mental toll is significant. Essentially stress is, the inability to control a given situation. Through fun interaction with the audience, she highlighted how that because of the need to focus on immediate issues there is often a tendency to neglect personal wellbeing. Over time this can lead to a depletion of one of your most important resources.you.

for parents to use that enable them to build a relationship with their child that is supportive and encouraging. As Dr Chong outlined, stress raised by the challenges associated with raising a child with haemophilia can have detrimental effects on all parts of the carers and therefore the familys life. The beauty of this programme is that it is parents teaching and helping other parents. So often we are offered advice from people who really dont understand what we, as carers of children with a bleeding disorder, have to face day-to-day. Having attended this conference and also the inhibitors workshop I can fully vouch for the absolute value in meeting and talking with other parents and sufferers, people who are in the same boat. So often we have the same feelings of guilt and anxiety associated with seeing our children suffer pain and dealing with the disruption in our familys life when bleeds occur, the juxtaposition of allowing our children freedom to enjoy life with the desire to protect them from harm. PEP offers parents a coping mechanism and parenting style to enable them to empower themselves to make sensible choices with relation to safety and wellbeing so that we can nurture honest, self-reliant and independent children. These presentations were so pertinent to my familys life. We have endured a rollercoaster ride of emotions since our son was born and the greatest feelings of support have been from other parents who understand where we are, where we have come from and where we are going. We all want the same things, i.e., happy, confident and successful children, how we get there is a through sharing advice and support and having the opportunity to do so. Thank you HFNZ for enabling us to do just that!

Dr Carolyn Broderick talked about the risk of bleeds associated with physical activity in children with haemophilia. They conducted a thorough study of 104 children with haemophilia and to assess their bleeds, treatment and exercise/sport and how and when they are interrelated. They are creating a calculator to quantify bleeding risk in people with haemophilia and it will help them and their families make informed decisions about sport participation. It will also help them to schedule prophylactic clotting factor administration to reduce the risk. Chris Gordon, a person with severe haemophilia, gave a personal story about his dream to play AFL. As he got older he redirected his dream from playing to becoming an umpire, and now, at the age of 21 he has umpired over 80 matches including the AFL final this year, running a total of 16km per game. He does the best recovery possible and prophylaxis before games and this has enabled him to live out his passion for the sport he has always loved.

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Caring for parents and carers

Vicky and Danny Hancox
This presentation focussed on the issues that carers face surrounding stress, depression and general wellbeing. The first presentation was Taming the Stress Dragon chaired by Dr. Desdemona Chong. This focussed on management of stress and tension for the carer and adopting strategies and coping styles in order to achieve a better work-life balance. A pertinent point made by Dr. Chong was that unpaid carers in Australia save the government an estimated $40 billion a year. They are a vital part the social framework and yet they are frequently featured on the depression scale with the norm being at moderate depression. We all suffer stress and anxiety in some level and when caring for a child with a chronic condition this is often exacerbated. When we suffer ongoing stress the effects on the carer are physical, emotional, behavioural, interpersonal and mental and left unchecked can have detrimental effects on our ability to carry on caring effectively. Dr. Chong pointed out that stress is a matter of perception, what is major stress for one person is a small problem for another, what is more important is how we deal with the effects of stress. Developing coping mechanisms are a vital part of dealing and minimising the effects of stress. Problem-focussed coping is a major strategy, incorporating such things as learning to plan and organise elements in your life where possible, learning to identify problems and prioritise these. Seek support. When you have learned to do these things we can assess what really matters and what really needs to be dealt with and what are just distractions. Some of the major outlets for stress and tension included seeking support dont feel that you have to cope alone or that seeking help is some sort of weakness. Learn to selfcare including taking time out to really relax, exercise and get a good sleep. Develop a sense of humour laughter is the best medicine! The second part of the presentation was focussed on Parents Empowering Parents (PEP) in both Australia and New Zealand. PEP is a programme designed to offer tools

Keeping the mind and body in tune - adults

Chantal Lauzon
This session gave an overview of how important keeping fit is to both the body and mental health, especially if you have a chronic condition such as haemophilia and joint disease. Dr Anne Powell discussed how obesity affects the joints and other related conditions. Aside from haemophilic arthropathy, people with bleeding disorders can also develop osteoarthritis (OA). Both obesity and joint injury are predisposing factors for OA. Obesity has an effect on OA via both metabolic factors and biomechanical factors. For example, for each one point increase in body mass index the risk of hand and knee OA increasing by 9-13%. For each increase of 5 points the risk goes up by 32%. A person who is overweight before the age of 40 years is over 3 times more likely to develop OA. Losing weight lowers the risk of disease and disease progression (i.e., losing weight will make a difference to OA symptoms). Having a tear in the lining in your knees (which would cause a bleed) is much more likely if you are overweight, if you have a high fat mass and if you carry your weight around your mid-section (instead of around your hips or distributed more evenly). It is not just your total weight that increases the risk of tears because having more muscle than fat is protective. When it comes to losing weight there is no magic trick that will work for everyone. Each person needs an individual strategy that works for them. We were then treated to the personal stories of two amazing people with haemophilia who told us of the challenges they had overcome. First there was 21-yearold Andrew Selvaggi, who has severe haemophilia and inhibitors. As a result he has many target joints and by

To Look After Your Body: Move more Sit less Sleep more Look happy Anna Louise explained why to move more, sit less, sleep better and deliberately look happy makes a happy body. Her message for achieving a happy mind was to give more, be grateful and let go. She invoked scientific evidence, discussed the dangers of self-medicating and provided tips to back up how your body can be used to improve mental health and wellbeing and decrease anxiety. As an enthusiastic advocate of physical exercise she hammered home the need to decrease sedentary lifestyle, how to improve sleep and demonstrated that through focussing on changes in posture and body awareness, you can achieve immediately applicable, tangible ways to achieve a happier body and a happier mind. Mind: Give Be grateful Let go

Keeping the mind and body in tune - children

Sarah Preston
Dr Jacqui Dalby-Payne talked about managing diet and dealing with fussy eaters. Psychologists and dieticians with expertise in feeding can assist, but in most cases fussy eating is considered normal toddler behaviour and she provided some helpful tips to try. It may take 10-15 tries before new food is accepted Limit meal times to 30 minutes Provide nutritious food , but the child should chose how much and what to eat or whether to eat at all Never force feed, coax or badger the child Offer realistic, small portions and textures Limit cows milk to less than 600ml per day (and not at night) as it can remove appetite Let them get messy and experiment feeding themselves and clean up after

Happy Bodies

Happy Minds

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age 18 found himself in a wheelchair, overweight, having a couple bleeds a week, and dependent on pain relief. He decided it was time he made a change. By the age of 20 he had lost 35kg and had slowly built up a passion for fitness and become a personal trainer who works out 6 days a week. His haemophilia no longer holds him back and he feels it has made him stronger and more resilient. The second personal story came from Zev Fisherman, a 60-year old father and grandfather. With no treatment available in his early years he incurred a lot of joint damage but grew up to have a productive work life and family. His first wife passed away when his three children were still young and so he changed careers to better suit his family life. He had one knee fused in 1998 and in 1999 had a knee replacement in the other knee. For the next 8 years he suffered from ongoing issues with his replaced knee and finally came to a point where the decision had to be made on whether to amputate it. Zev worked hard to increase his fitness and prove he would be capable of coping with having one fused knee and a prosthetic leg. The operation was successful and for him he feels it was the right option. He can still drive, swim, golf and travel and he has never felt healthier or fitter. He feels that having haemophilia in Australia is not a disadvantage, you can accomplish anything, and that having the tell-tale haemophilia walk is a badge of honour. Finally, Nicola Gates gave some tips on putting positive psychology into practice and making positive health changes, after all health means so much more than simply not having any disease. Some of her key messages included: Having a healthy diet benefits both the brain itself and your mental health It is vital to get at least 7-8 hours of sleep at night for your brain health Exercise can reduce stress and improve life satisfaction. It also reduced the risk of depression and dementia. Reduce risk factors (lose weight, stop smoking, reduce alcohol, etc). Feeling stress is an alarm bell that you have a problem that you need to address. Breath! Taking a breath helps calm you and put things into perspective. Approximately 25 percent of people will experience mental health problems at some stage in their life. Try to reach a good work-life balance and ask for help if you need it. Getting help could be from talking to someone you trust, a doctor, a helpline, a social/outreach worker or priest/rabbi/iman. What we think determines how we feel and how we age. Try to have a caring attitude and accept and appreciate yourself. Know your strengths and do things that make you feel happy as often as you can. Her number one tip for having a happy life is having friends and family nearby.

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take longer to get an erection, they might not be as firm (but they are just as useful), the refractory period (the time between sexual intercourse and achieving another erection) increases, it may take longer to ejaculate, and some men do not ejaculate every time they have sex, ejaculation is less powerful and orgasm less intense (but the sensations are just as pleasurable), and sexual urge came become less frequent. All of these changes are NORMAL with ageing.

Staying on your feet - a session for adults of all ages

Joy Barrett
It was a privilege to attend the Australian & New Zealand Haemophilia Conference. This session was divided into three areas, with each topic covered by exceptional speakers. Each session held so much so feel free to make contact if you want to hear more. I am giving a brief overview here. Dr Sanjeev Gupta spoke on Saving the Ankle Joint. He highlighted the uniqueness of the ankle joint with its ongoing ability to carry the impact of our daily movement. This equates to five times our body weight on our ankle joints with every movement. (So the more overweight we are, the more we significantly increase the load on this joint.) The thinness of the cartilage is as little as 2mm at some places within the joint, the surface areas that rotate over each other are small and the blood supply through this area is also poor. Treatments options once damage is severe follow, in order of difficulty for both patient and surgeon: Arthroscopy Re-alignment Distraction Fusion Replacement Transplant All of these options are considered and evaluated for advantages or disadvantages for the patient. A long term view needs to be considered for each option as huge improvements in designs and techniques are continually being worked on. The purpose of any surgery for the ankle is a Motion Preserving Procedure. What can we do to help our ankles? The international speakers confirmed the messages we are hearing locally in New Zealand. Watch our weight Seek physiotherapy after every bleed. This is crucial. Wear appropriate supportive footwear Exercise regularly at some level

Mens business
Colleen McKay
Sexuality is important. According to the World Health Organization(WHO) sexuality is integral to quality of life. WHO (2011) states that sexuality is a central aspect of being human and encompasses sex, gender identities and roles eroticism, pleasure, intimacy and reproduction. Sexuality is part of ourselves from before we are born until we die. It involves all those aspects of our lives that make us the people that we are. The Jargon? Sexuality? Sexuality includes all those qualities that allow people to feel comfortable with their own bodies and with their ability to form a sexual relationship with someone else. Sexual Orientation? Who the person is attracted to. Sexual Behaviour? Sexual contacts with others. Sexual Identity? How the person self identifies, i.e., heterosexual, straight, homosexual, gay, bi-sexual, asexual. Sexual Performance Issues? - There is greater pressure on males than females to perform sexually. Performance problems can include low sexual desire, premature ejaculation, erectile dysfunction, and retarded ejaculation. Male sexual performance difficulties are more obvious and more likely to make sexual intercourse impossible. There is a greater social expectation of male sexual competence. When intercourse difficulties clearly relate to a mans problems, he may have feelings of inferiority, guilt and inadequacy. The reality is that most men will experience a sexual performance issue once or more in their sexual lives. Problems arise when the man feels traumatised by the event and worries about a recurrence; anxiety and tension can make it less likely that he will get a functional erection. Anxiety and arousal dont mix men need to relax more. Sex and ageing? There are a number of normal changes that happen with male sexual function when ageing - more prolonged and intense stimulation is required, the testicles may become smaller and more flaccid (but this does not affect performance), it may

Erectile Dysfunction is the inability to achieve or maintain an erection that is sufficient to complete intercourse. Erectile Dysfunction is common as men age 40% of men have difficulties at age 40, 50% at age 50, 60% at age 60 and 70% at age 70. Unfortunately many men do not seek treatment or wait too long before seeking treatment. There can be a number of physical and psychological causes of erectile dysfunction. It is important to discuss this with your healthcare profession, in order to investigate and find a solution. Top Tips? Manage your sexual expectations and goals Use condoms if necessary Keep up prophylaxis regimen (iliopsoas bleeds - prevention is better than cure) Try different positions Communicate your sexual desires, needs, frustrations with your partner Practice sensitivity Educate your partner about your desires, needs and frustrations Practice closeness emotional and psychological intimacy Develop trust in your partner to be empathetic, patient and understanding Engage healthcare professionals There can be reluctance to discuss sexual issues with healthcare professionals Make a list of your concerns / questions to discuss with your healthcare professional Healthcare professionals can make suggestions and referrals Access other healthcare professionals - sexologist, urologist, psychologist Discuss physical impairment issues with your physiotherapist REMEMBER: It is not sex that gives the pleasure, but the lover. - Marge Piercy

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Ageing
Chantal Lauzon
Ageing is increasingly popular topic at haemophilia conferences. The life expectancy of the haemophilia population has increased markedly over the last century and is now practically the same as the general population in most developed countries. But with age come many other health complications. Dr Mike Makris from the UK gave an overview of some of the complications increasingly being observed in older people with haemophilia (PWH). Health conditions/ complication Hepatitis C (HCV) Notes for people with haemophilia The problem with HCV is the development of cirrhosis Generally 20-30% of people with chronic HCV develop advanced liver disease. New era of HCV drugs means that rates of clearance should increase. Liver transplantation is also becoming increasingly available. HIV Cardiovascular disease (CVD) There have been dramatic advances in management of HIV since 1997 and there are now very few deaths from HIV due to highly effective HAART treatment. Most studies involving PWH show that they are much less likely to die from heart disease than the general population. Why? PWH have the same risk profiles and levels of artherosclerosis* but do not seem to develop the related clots (have more stable artherosclerotic plaques). So PWH get angina, but fewer heart attacks. The management of heart problems in PWH is troublesome as the use of blood thinners is controversial. There are lots of recommendations but no clear evidence based guidelines. Cancer PWH are at increased risk of some cancers as a result of HIV and HCV infection Outside of these, cancer rates appear to be the same as the general population Diagnosis can be difficult, especially if one of the symptoms is gastro-intestinal bleeding. Clotting factor cover should be used when undergoing diagnostic procedures. Although there are many theories, there are no clear guidelines for treating leukaemia in PWH. Inhibitors PWH aged over 50 years appear to be at risk again of developing inhibitors (the rate goes down in teens/adulthood). Lots of questions remain to be answered (use of prophylaxis, use plasma-derived factor vs. recombinants, FEIBA vs. Novo7). Haematuria (blood in urine) Very common in people with haemophilia. Usual treatment is to increase fluid intake and rest. Tranexamic acid should be avoided and the use of clotting factor is controversial. Should investigate further if aged over 40 years, the problem keeps happening and/ not settling. In the US, there does appear to be higher rates of chronic kidney disease in PWH than in control populations. Orthopaedic/Musculoskeletal The musculoskeletal health of PWH is unsurprisingly much worse than the general population Haemophilia arthropathy worsens with age, reducing mobility and activity. Osteoarthritis can also develop. The biggest issues are pain control and maintaining mobility. There is also a high risk of falls because of difficulties balancing. Increasing numbers of joints replacements are being performed, especially hip and knees. In haemophilia, the indication is to reduce pain rather than increase function. *artherosclerosis: Condition in which an artery wall thickens as a result of the accumulation of fatty materials such as cholesterol.

An Italian study of men with haemophilia aged over 65 years found that, compared to the general Italian population, PWH were at higher risk of hypertension, and much higher risk of hepatitis C and musculoskeletal problems, however, at lower risk of cholesterol problems and cardiovascular disease. Although treatment is highly effective at stopping and preventing bleeds, the idea of ongoing prophylaxis in adults is controversial due to the cost and whether there is a clear need. There are suggestions that more factor will be need as PWH age and other health conditions develop. Although more cases of conditions such as CVD and cancer are being observed they are still rare events in PWH. Treaters will have little experience in managing these types of conditions in this population and because it is very difficult to do prospective studies it can be difficult to know what the best treatments are. Large, multinational studies are needed to study the haemophilia population and track their other health conditions. Stephen Mathews, a clinical nurse consultant from the Royal Prince Alfred Hospital, discussed a case study that highlighted the issue that a lot of PWH rely on their haemophilia treatment centre for all their care. However, when they start to develop conditions associated with ageing they need a general practitioner (GP) to help manage those conditions, especially if they live rurally. A GP can keep track of all the different medical issues being faced by a PWH. When a PWH sees a specialist outside of the haemophilia centre a letter will be sent back to their GP, but not necessarily all their treaters. Another issue is that self-treatment can become difficult as a PWH age due to problems with eyesight, their veins or and/or mobility. It is important to start thinking about this early and either train a family member or make arrangements with a treater nearby. Ian dYoung spoke about the benefits of physiotherapy. Many older PWH had received physiotherapy when they were younger that hurt or were told that they could not be helped. Physiotherapists now know a lot more about haemophilia and how the body works. No matter how bad the disability, there is always something that can be done to help. It can take time to build trust between a PWH and their physiotherapist but education and compliance are key to improvement. Physiotherapy can help with pain management, quality of life, mobility issues, exercise tolerance and co-morbidities. There is no magic want, but supervised, low-resistance, low-impact exercise can be beneficial. Programmes that include both weight and cardio elements should be individually tailored to a persons needs and should be carefully supervised. Little and often is a good way to start and as strength builds the process can be very empowering. Active exercise for men with joint damage is one of the best ways to feel better and reduce pain. It is important, however, to go slow and not overdo it. If it hurts stop and tell your physiotherapist. Exercises need to become part of everyday life, not something extra. Finally Leonie Mudge, a social worker at the Royal Prince Alfred Hospital, spoke about support for older people. Interaction with family, friends and health and community services enables independence, security and choice. It is important for a persons wellbeing to have good supports in place. Funding for aged care

living in Australia is usually only from 65 years of age, however, people with chronic health conditions such as haemophilia may require assisted living earlier. If this is the case funding is available in some circumstances but a referral and assessment is needed. Independent Living Centres (www.ilcaustralia.org) have a number of factsheets available on fall prevention, occupational therapy, gadgets and equipment that might assist with maintaining independence.

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Other Bleeding Disorders

Von Willebrand Disorder
Chantal Lauzon
The session on vWD began with the personal story of Manal Awad, a women with vWD and mother of two children with vWD. She suffered many problems with nosebleeds as a child and eventually with her menstrual cycle. She feels that having vWD herself makes it much easier for her to understand what her children are going through and the challenges they face. Dr Emmanuel Favaloro then shed light on the diagnosis and classification of vWD. The diagnosis of vWD depends on both clinical elements (bleeding history, family history, blood group, physical exam, etc) and laboratory elements. The classification of the vWD and its different subgroups was updated in 2006. In the past there had been more than 25 types of vWD, but now there is type 1, type 2 (2A, 2B, 2M, 2N), and type 3 and the diagnosis not longer insists that vWD is caused by a mutation in von Willebrand factor (vWF). It is important to identify the right type of vWD because it affects how you bleed and which treatments may be effective. For example, DDAVP usually works for type 1, but only sometimes for types 2A, 2M and 2N. It should not be used in people with type 2B and rarely works for type 3. Comparing the results of different laboratory tests can help define the type of vWD, such as levels of vWF antigen, ristocetin cofactor function, collagen binding and FVIII. For example, if the levels of all four are similar but low, then it is probably type 1. For type 3 all tests results levels are nearly zero. For types 2, the different ratios can help determine which type of vWD it is. Dr Favaloro said that there can be problems with definite diagnosis because of the variability in the sensitivity of tests between different laboratories and assays.

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Dr Mandy Davis gave a clinical update on vWD treatment and care. She began by showing video footage of the microscopic platelets rolling along the inside of capillaries and how they are tethered to the side by vWF. She went on to explain how difficult it can be to recognise and diagnose vWD because the condition can be so variable and many people experience mild bleeding symptoms. There can also be other inheritable or environmental factors that increase bleeding risk. Therefore family history does play an important part in the likelihood of a person having true vWD. Future therapies for vWD include recombinant vWF. This product is already in Phase 3 clinical trials in people with type 3 vWD, in at least two centres in Australia. The advantages of having recombinant vWF are that the product is independent of the blood supply and the molecule stays completely intact. The disadvantages could be if the person also has low levels of FVIII that they might then also need treatment with recombinant FVIII. Trials are also looking at the combination of the two recombinant products to see how well they work together.

Patient example: Two brothers, they are the youngest of four children. Treatment is cryoprecipitate, hydrocortisone and phenagen. Their nursing considerations are to teach and guide patients. Penny McCarthy from The Alfred Hospital spoke on Glanzmann thrombasthenia, a platelet disorder or platelet aggregation disorder caused by marriage between blood relatives. Estimates are that only 1 person per million are affected. Patient example: A mum and dad who are first cousins with 3 daughters. The two 2 youngest were affected, both requiring blood transfusions after their first period. The middle daughter died at age of 30. The younger sister yearned to have a baby, but it was not a safe option. She opted instead for surrogacy with the Australian dad, a Russian embryo and Indian surrogate. Twins were born, a girl and boy. A presentation from Dr James Price from the Princess Margaret Hospital for children described Factor X deficiency as a rare autosomal recessive bleeding disorder, with an incidence of 1 per 1million. There are currently four people with FX deficiency in Western Australia. Dr Price describes it as horrendous bleeding. Patient example: A baby girl was diagnosed following continuous bleeding from the heel prick. There was no family history but upon testing the mother was found to carry a Factor X and Factor VII gene deficiency and the father <1% Factor X deficiency. Baby was treated with Prothombinex and then later put onto prophylaxis twice weekly. Factor X concentrate has been available since August. A brother was born shortly after but there were no complications. Finally Dr Tim Brighton from Prince of Wales Hospital in Sydney gave a Haematologists perspective on Platelet Function Disorder (PFD) encompassing a clinical diagnosis, which means its mainly in numbers, evaluations, lab tests and microscopy jargon. The condition is either inherited or acquired and usually results in mild bleeding/bruising but is variable in severity. The condition is rarely spontaneous.

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Womens Health & Reproduction

Belinda Burnett
The speakers for this session were: Dr Kristi Jones from The Childrens Hospital at Westmead who spoke on IVF and embryo diagnosis Dr Claire McLintock from Auckland City Hospital who spoke on Managing menorrhagia Lynne Campbell from HFNZ who spoke on the HFNZ Womens Programme I was privileged to chair this session, which was well attended by both women and men (although the men were outnumbered by 25 to 1). Kristi spoke about the remarkable advances in preimplantation genetic diagnosis (PGD) over the past 20 years. She described how the technology works and gave examples where couples have successfully used the option of PGD. Her session was made even more interesting by the inclusion of a video of actual embryo selection. PGD is an approved method of screening out serious, life threatening conditions and haemophilia is recognised as one of those conditions. Gender selection for social reasons is not permitted in both Australia and New Zealand. Before any procedures can commence the women needs a DNA test to establish the affected gene. This test is 99% accurate. IVF is a significant part of the PGD procedure and success drops dramatically the older
Treatment Mirena IUD Hormonal Treatment Benefits Can control bleeding long term Reduces bleeding

The rarer bleeding disorders

Patience Stirling
This was opportunity to listen to three nurses share their experiences of working with patients with a rarer bleeding disorder. Andrew Atkins, a nurse from Royal Adelaide Hospital spoke about Acquired Haemophilia A. He said there is no previous history when diagnosed, but an inhibitor reduces the FVIII level in the plasma. Each year 1.2 per million cases are diagnosed, 80% of which are severe or life threatening. The mortality rate from acquired haemophilia in South Australia is 25%. Treatment aims to stop bleeding and eradicate the antibody, often by way of steroids. Patient example: Man who was diagnosed at 76 years old who had suffered a heart attack, stroke, prostate problems, depression and then complained of an aching body. Their nursing considerations were awareness, blood tests and patient psychological wellbeing. Olivia Hollingdrake from Royal Brisbane Hospital spoke about Afibrinogenaemia, or Factor I deficiency. This rare absence of fibrinogen only occurs in 5 in 10 million people.

a woman is. New Zealand women who carry the gene for severe haemophilia are able to access funding for two rounds of IVF for PGD, any more have to be self funded. Claire talked about the research into females affected by haemophilia; one reports states that 76% of these women experience heavy menstrual bleeding. Conversely, a high proportion of women in the general population with menorrhagia have undiagnosed bleeding disorders. The definition of heavy bleeding was established in the 1950s and is now considered to be archaic. A new method of recording how many times per day sanitary protection is changed, how many days off work are taken throughout the year and if anaemia is present is considered to be more relevant and useful than the actual weighing of blood loss. There is no known cause in 50% of women who experience heavy bleeding. Whilst management of menorrhagia is difficult when women want to conceive, there are many options available to women presently for the treatment of menorrhagia. She discussed the aspects and benefits of the following:
Disadvantages Side effects: skin disorder, back pain, stomach pain Doesnt stop fertility

Jadelle DDAVP* or tranexamic acid*

An injectable contraceptive that is long lasting Easy to administer Effective at reducing blood loss

Can cause uterine bleeding Side effects: Severe flushing or blood clots

*Can only be used for known carriers of haemophilia or sufferers of vWD

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Factor replacement is only used in females with low levels. If a hysterectomy is called for it is preferable to use a vaginal method of removable rather than abominable surgery. Cost is not a factor in treating women in Australia or New Zealand but is an issue in developing countries. Lynne explained what approaches HFNZ used in response to the increased recognition of the unique issues faced by women affected by a bleeding disorder. She gave the history of the HFNZ Womens Programmes from their start in 2006, to where they stand today. In 2009, HFNZ conducted a survey among its female members to better understand their needs. From the results HFNZ were able to plan a workshop that concentrated on increasing education, empowerment and building a sense of community for these women. Lynne demonstrated the positive effects of the 2009 event where women reported having a greater understanding of issues and options related to their bleeding disorder. Including a wide range of ages and education topics was a successful formula. Overwhelmingly the women reported a reduction in the isolation many of them had felt prior to attending the workshop. Lynne ended by stating that HFNZ recognise the value of education for this important segment of our community and is committed to further initiatives to meet their needs.

Garcia also spoke about fertility and HIV. With effective HAART treatment, there are now many reproductive options for people with HIV. While untreated HIV may reduce fertility by reducing sperm count and fitness, treatment for HIV does not adversely affect sperm quality. During the 1990s and 2000s, IVF was increasingly used for couple where the male was HIV positive and now natural conception is considered by some to be a possible alternative for couples where one of partners has HIV, as long as complete suppression of the virus with HAART is achieved in the positive partner. Simone Strasser provided an update on hepatitis C. The main reason for treatment of HCV is to prevent the development of cirrhosis and the burden of disease associated with it. If the cause of the cirrhosis can be eliminated, cirrhosis can be partially reversible. If HCV virus can be eradicated you can also reduce the many conditions and complications associated with liver disease: fibrosis; progression to liver failure; portal hypertension; incidence of liver cancer; insulin resistance and survival. Risk factors for liver disease progression in HCV include: Duration of HCV infection Older age at infection History of alcohol abuse Obesity, Type 2 diabetes HCV genotype 1 Co-infection with HIV Liver damage can be assessed by three methods. Liver biopsies are not routinely performed in people with haemophilia in Australia and New Zealand due to the high cost of factor needed and high risk of complication. Serum markers, including commercial tests such Fibrotest and Hepascore, are not often available or used in Australia although the ALT/AST ratio is considered. Fibroscans are instead increasingly being used to measure liver stiffness and therefore fibrosis. Fibroscans are a great tool for distinguishing between a healthy liver and cirrhotic liver but are not great at distinguishing between moderate and severe fibrosis. Fibroscans are now available to most hepatitis clinics throughout Australia (only 3 available in New Zealand: Auckland, Hamilton and Christchurch). The advantages of Fibroscans are that it is a quick, noninvasive test that can easily be repeated. A study at the Royal Adelaide Hospital found cirrhosis in 32% of people with haemophilia and chronic hepatitis C tested with a Fibroscan, although most had had no previous clinical indication. Why treat for hepatitis C? The vast majority of people experience a big improvement in their quality of life once they clear the virus. For many, the impact of infection on their health and mood was not apparent until after they were clear of the virus. Treatment is now fairly tailored to the individual to maximise response rates, which is successful in 65% of patients. The new treatment options with an added DAA have response rates of 70-80%. A test for the IL28B gene is now available in Australia. This test can indicate whether a person has a predisposition for treatment working and perhaps for clearing the virus (C/C genotype) instead of a less favourable predisposition (T/T or T/C). How quickly the viral load responds to

treatment is the most important predictor of successful treatment. It is hoped that boceprevir and telaprevir will be registered for use in people with HCV genotype 1 in Australia in 2012. The new triple therapy that combines these agents with standard therapy (pegylated interferon + ribavirin) will shorten therapy but, unfortunately, will not reduce the side effects. The regimen for telaprevir will be 12 weeks of triple therapy (telaprevir + pegylated interferon + ribavirin) followed by 12 weeks of standard therapy. Itch, skin rash and anaemia appear to be the biggest problems. With boceprevir, standard treatment is started for 4 weeks and then the new agent is added in and all three are taken for at least 24 weeks. Boceprevir has shown similar response rates (>70%) to telaprevir but anaemia can be a significant problem. Both agents have been shown to work better than standard therapy even in people with cirrhosis. One of the challenges with the new agents is that the dosing regimen gets complicated. Even though they are pills, they have to administered several times a day, one with meals and the other on an empty stomach. Adherence may be therefore become a problem for some people. There are also many other DAAs and combinations of treatment in clinical trials. Strassers recommendations were for people with genotypes 2 and 3 to start treatment as soon as possible as although clinical trials are underway, pegylated interferon plus ribavirin will remain standard therapy for the foreseeable future. For people with genotype 1, she is

not starting any new patients on standard therapy unless they test positive for the C/C type of the IL28B gene. Otherwise she would wait until the new triple therapy becomes available or recommends enrolling in a clinical trial of new products if possible. Now people with haemophilia can access some clinical trials for HCV therapy as they are beginning to take away the requirement for a liver biopsy as Fibroscan technology becomes more widely available. The hope for HCV treatment is to find a combination of DAAs that will eliminate the need for interferon completely and be able to clear HCV with only a short regimen of pills. So again, why treat hepatitis C? It is an opportunity to clear a chronic condition, improve your liver function and structure and improve your quality of life. The symptoms of HCV infection (fatigue, nausea, discomfort, depression, malaise) are common feelings so many people do not realise that they are related to their hepatitis C. When you are ready to consider treatment it is important to: choose the right time for you (not before a major event or a period of stress) remember the work up and start of treatment may take time so you may not be able to start right away get educated. Become familiar with all the options, possible side effects and sources of support available to you ahead of time.

Commit to treatment. Adherence to the treatment protocol is vital for the treatment to work. If you begin to skip days or weeks the virus can gain a new foothold or become resistant to treatment. In reality a multi-disciplinary team is needed to treat HCV and the side effects. Most mild side effects can be helped by using panadol and maintaining a healthy diet. More complicated side effects are treated as they arise as they can be different for each person. Insomnia, though common, tends not be treated unless very severe. Mental health issues in some form are common but taken seriously. Counselling and antidepressants are frequently prescribed if needed. A number of blood disorders (low levels of red blood cells, white blood cells, etc) can also occur as can thyroid dysfunction and should be closely monitored for. Tips for living with chronic hepatitis C: Avoid alcohol Try to reduce or give up smoking Eat a healthy diet and avoid saturated fat Exercise within your capabilities Get immunised for hepatitis A and B Minimise stress Rest when you need to Be kind to yourself

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Living with Hepatitis C and HIV

Chantal Lauzon
To start the session, Luke Ahem gave his personal story of his recent experience with treatment with pegylated-interferon and ribavirin for hepatitis C. He suffered many side-effects and still tires very easily but feels healthier, mentally and physically, than he has for many years. Although there is no guarantee of success and it can be difficult, he still recommends treatment as he is now clear of the virus.. Dr Roger Garcia of the Royal Prince Alfred Hospital gave a medical update on treatment for HIV. In his practice few people with haemophilia and HIV passed away from HIV once HAART was introduced. Treatment with HAART for HIV aims for lifelong suppression of HIV and for no progression to AIDS. There can be side effects, including issues with metabolism and bone marrow. Diseases of ageing and cancer are becoming increasingly important when treating people with HIV and pose a clinical challenge. People with HIV and HCV co-infection have increased rates of mood disorders and seizure. Treatment to clear HCV follows the same algorithm as in people without HIV but it is best to commence treatment when the person is as healthy as possible as pegylated interferon depresses the CD4+ T-cell count. HCV treatment in people with HIV/HCV co-infection has a lower success rates, can require longer treatment and there are more drug interactions to consider.
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Communication and Social Media

T.A. and Holden Stirling
The first speaker was Suzanne OCallaghan, the policy officer for Haemophilia Foundation Australia (HFA). She spoke on the benefits of using websites social networks, e.g., Facebook, Twitter, Bebo, emails, etc. for the Haemophilia community. An example she gave was if you live 3 hours from the hospital and had a bleed, that meant 3 hours travelling, 30mins waiting for a doctor only to tell you it is minor. This could be easier if you could Skype your local treatment centre and within 5mins you could have your answer. Suzanne gave some interesting facts that shows how networking is beneficial in the Foundation: 82% of Australians are using social networks, mostly Facebook HFA have a Facebook and Twitter page HFA Facebook page was created In April 2009, currently holds 850 fans today Better ways to engage 35% of users are between 18-25 years of age HUK use Twitter (Haemophilia United Kingdom) Natasha Samy and Lyn Biviano are hospital staff from the Prince Alfred in Sydney who also spoke on social networking. Along with Suzanne they are all in the same boat because they are learning as they go, because the truth is they are always in new territory as this is a new way of communicating and will always be part of our future. As this is new we must tread lightly, Suzanne makes a point that new policies will need to be in place or modify old policies to protect not only the individual (patient) but also the doctors and nurses and Foundation members. So here are some risks:

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Protecting privacy of community members Potential of inaccurate or unsafe advice Cyber bullying, offensive or inappropriate behaviour Advertising commercial gain Risk to HFA/HFNZ community reputation Boundaries should not be crossed (asking your nurse on Facebook what she likes to do). There are a lot of risks with anything being so new, thats why we must debate and prepare ourselves before moving forward, so here are some benefits: Connections are made easier to community and supporters Engaging in supportive roles,i.e., parents or caregivers asking others about haemophilia Dialogue notes sent via email if in a small town Promote events, activities and ask for resources

Treatment and Challenges

The challenges of prophylaxis
Chantal Lauzon
This session began with a clinical update on prophylaxis by Dr Alessandro Gringeri from the University of Milan. The aims of prophylaxis are to: Prevent life threathening bleeds Reduce bleed frequency Prevent re-bleeding in target joints Prevent arthropathies Types of Prophylaxis
Primary: Starts before bleeding occurs or after first bleeds Starts later, after several bleeds Started in adults to reduce worsening joint damage and reduce bleeding in target joints

as early as possible. There is not yet enough data on lowdose prophylaxis regimens for them to be recommended. Tertiary prophylaxis has also been shown to work well to at least maintain health if not improve it. In conclusion, the dilemma is not whether to use prophylaxis over episodic treatment but what prophylaxis regimen works best and is most cost-effective. Robyn Shoemark and Dr Susan Russell of the Childrens Hospital at Westmead then spoke about the challenges of venous access for prophylaxis in children. Although prophylaxis gives a family more control and avoid trips to the hospital, the big question is often when to start. The are many different approaches but in their centre most children with severe haemophilia start prophylaxis between 18 and 36 months of age depending on whether they have had any bleeds. The options for accessing veins for prophylaxis include venupuncture or ports/central venous access devices (CVADs). Venipuncture is the best option as it is quick and clean, but it is not always possible as people may have trouble with their veins or parents or the child may be scared or apprehensive. With kids the biggest hurdle to overcome is usually the fear of pain. Numbing creams can help with this. It is also quite difficult to explain to a 2 year old why they have to sit still and have a needle put into their arm. Some parents feel guilt and very anxious about missing a vein. Their advice was to accept that missing a vein will happen and that it is ok, you just need to start over again. There is a lot of equipment to help parents learn how to access veins. Using distraction techniques to help children keep still can be very helpful (iPhone, toys) or having a factor box or bag with little surprises that the child can draw from at each hospital visit. With ports or CVADs there is also a lot of equipment available to help parents learn how to use them to access veins. In Sydney they often introduce the family to another with a port so they can see how it works before it is put in. New users will practice first at the hospital and then move to home infusion. Reasons to insert a CVAD include the patient/parent being unable to access veins, the child is starting on immune tolerisation therapy, or they are in a situation where learning to access veins would be very difficult. The insertion requires surgery under general anaesthetic and factor cover. The device is inserted into a large vein, preferably near the shoulder as this has shown to less problems then devices inserted around the waist in toddlers. Complications of CVADs can include bleeding around the site, infection, thrombosis, blockages or the child can sometimes outgrow the line.

Secondary: Tertiary:

There are two approaches to primary prophylaxis. Either the goal of prophylaxis is to maintain factor levels of at least 1% or the goal is to prevent bleeding. If preventing bleeding if the goal then using a dose escalation regimen can be easier to implement. It is easier on the family, allowing them to learn to infuse and can be adapted to the individual and family. While this approach might not totally prevent bleeds it is a less demanding and less costly way to start. The earlier prophylaxis is started the better joints can be preserved. Prophylaxis was recommended as the treatment of choice for PWH by the WFH/WHO in 1995. In 2008, the European Haemophilia Consortium also made a statement that prophylaxis should be available. However, until the results of the Joint Outcome Study were published in 2008 there was little published information showing a clear advantage of prophylaxis over on-demand therapy. Since then several studies have backed up the findings or explored other prophylaxis options, including dose escalation. It is now clear that primary prophylaxis is very effective, especially if started

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Over 40% of ports become infected and these infections can be life threatening. Sometimes antibiotics work and the port can be saved but other times they must be removed. Signs of infection include a fever over 38.5C and maybe redness or swelling around port (although not always). A child with a port infection will be on antibiotics for at least 48 hours to a week minimum. Parents should contact their treatment centre for further instruction if their child with a port has a fever - any fever even if only a cold is suspected - or there are skin changes around the port. They should also contact their treatment centre if the port is accessed but they cannot infuse (line may be blocked) or their child is having a bleed and theyve missed when trying to infuse. It is also very important to keep a treatment record. It doesnt need to be formal, even just a notebook will do, but it is very helpful to see patterns and know if the dose needs to be adjusted. Ian dYoung then spoke about how prophylaxis is not the only factor when caring for your joints. Most long term damage to joints is the result of bleeding in childhood. There is really no such thing as a spontaneous bleed because there is always a cause, even if it unknown. The joint may have simply twisted or hinged, tearing the synovium and causing a bleed. The cycle of joint destruction can begin with less than four bleeds or even one bad bleed. The damage to the joint is proportional to the number of bleeds and the speed of rehabilitation. Problems with one joint quickly leads to problems with others as they take on more load or how you move changes. Delaying treatment increases the risk of more bleeds and long term damage. Changes in the muscles and joints happen rapidly following a bleed; for example, muscles deactivate or shorten making the joint vulnerable to further bleeding. Every bleed should be reported to the haemophilia treatment centre and assessed by a physiotherapist because every bleed must be properly rehabilitated to prevent further bleeding. Ian suggested that when you have a bleed to infuse factor and apply first aid, including P.R.I.C.E. You should then call your treatment centre physiotherapist or nurse and then go in for a rapid assessment to ensure correct diagnosis and differentiate the bleed from other sources of pain. People with haemophilia also break bones or develop arthritis and the pain can be mistaken for a bleed. Having a correct diagnosis ensures the correct approach to management. Historically, PWH were discouraged from exercise and sent to bed after a bleed for weeks. This approach led to many problems with muscles and flexion. PWH were only reviewed by a physiotherapist if there was a problem or on request. Unfortunately the weaker, stiffer and less coordinated a PWH is, the higher the risk of bleeding and joint damage. Now the approach is more about using prophylactic exercise to match prophylactic treatment and providing a rapid response to all bleeds. Treatment is not just factor. Every bleeds needs to be properly rehabilitated with appropriate rest and exercises under the supervision of a physiotherapist. To minimise the risk of having bleeds, Ian suggested: Using prophylaxis correctly this means taking it at the right time of day for you (i.e. NOT before bed) and/ or before activity.
2011 16th Australian & New Zealand Haemophilia Conference

Keep fit and keep your weight down Find the right types of exercise for you and avoid high contact sports. The heavier you are the more likely you are to do damage if you do have a bleed. You need to make exercise and activity part of your lifestyle. Wear sensible shoes! It is important to have good support for your feet and ankles. Avoid jandals and high heel shoes. Most trainers provide good support, but not skate shoes (with flat soles). Adapt your footwear to the terrain and tie your laces! Be sensible and listen to your body If an exercise is hurting, stop! Rest when you need to. Use swelling as an indicator for how long you should rest a joint after a bleed instead of pain.

the set time frame, only 27 of them had ever attended an appointment at the adult hospital. The study found that young people were often very attached to the staff they had worked with most and that there was an identified lack of preparation by staff for the move to adult services. The recommendations that came out of the report included a need to look at the timing of clinics, ensuring the mild and moderate sufferers were engaged in adult services adequately and the promotion of a Health care checklist to ensure youth were getting the support that mattered to them. New Zealand is fortunate in that having a small population means that transitions do not occur from hospital to hospital. However, we do need to pay attention to how our youth are supported in the move from paediatric services to the adult services. We need to engage in a team work model that encourages youth to take responsibility while supporting them in ways that will work for each individual. A one size fits all approach to transitioning is unlikely to work with youth.

Current and emerging challenges, risk factors for inhibitor development

- Dr Julie Curtin. The Childrens hospital at Westmead Now that we have improved factor concentrates and viral inactivation, the risk of viral infection from factor concentrates is greatly diminished. Inhibitors are the most serious complication of the treatment of haemophilia. Inhibitors occur in 20-30% of patients with Haemophilia A and 5% of patients with Haemophilia B. The patient related risks are genetic mutation, ethnicity, and family history of inhibitor development. Genetic risk is highest in those with multi- domain deletions (88%), large deletions (41%), intron 21/2 Inversions (21%). Age of first treatment is another risk with studies showing those treated under the age of 6 months had a 45% incidence of inhibitors, those treated at 7-12 months 29%, and over 12 months 12%. Mutation type is more important than exposure date. Those with large deletions are also more likely to need treatment earlier in life. Peak treatment moments such as large doses required for surgery or a large bleed may link the danger signal to receiving factor VIII. Unfortunately the need to treat in these circumstances reduces the options. In conclusion, genetic risk factors, particularly gene mutation, are important. Environmental/treatment related factors play a role but the evidence is questionable.

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Transitions: becoming an adult

Linda Dockrill
Haemophilia care is separated into Paediatric and Adult services with the move between these negotiated with each individual, taking account of their age, needs and family situation. It is recognised that this is an active process that requires planning and team work, as it is not just a medical process. There are key differences between the Paediatric and Adult services. Paediatric services are family-oriented; decisions are made by parents with some input from the child or young person and the multi-disciplinary team. The adult services are more individual focussed, relying on the patient to develop their own self care and advocacy. The adolescent is now seen without input from his parents and the appointments may be held in a different physical environment. In Queensland and in Western Australia the paediatric and adult services are in completely different hospitals, so the transition is a complete one from one venue to another, including a change of staff. Transitions are often problematic due to a lack of planning and preparation by staff. Home treatment has meant there is a general lack of organised systems of education and support around transitions and it needs to be acknowledged that it is a common time of change and upheaval in a young persons life. Some of the potential challenges are around self-management of treatment, non-attendance at appointments, risk-taking, moving house frequently, and all of this often coincides with a significant bleed. In Queensland they have tried to address the challenges of transitioning by using planning and preparation. The multi-disciplinary team engages in a partnership model with each young person and the aim is to address issues and encourage participation by the youth in their treatment process. Assuring young people that they will be given complete confidentiality, patience, text reminders and lots of chances has become part of the approach to engaging them in adult services. This transition process starts being discussed with the family and young person from around the age of 12 years. In Perth, a study was conducted of 16 people with haemophilia who had transitioned from the paediatric to adult services. Staff were shocked to discover that out of the 78 people who had transferred to the Adult services in

Treatment challenges
Richard and Lynley Scott

Safety of current products used to treat inherited bleeding disorders

- Dr Mike Makris Sheffield Haemophilia and thrombosis centre, UK Current products are the safest they have ever been but we should not be complacent. Manufacturers are improving products by making them purer and safer. Each country has systems for reporting adverse events. The European Haemophilia Safety Surveillance System (EUHASS) captures the events from 64 haemophilia centres from 27 European countries (www.euhass.org). For rare events in a rare disease you need an international cooperative approach. The events reported are allergic or acute reactions, transfusion transmitted infections, inhibitors, thromboses, malignancies, and deaths. Findings from event reporting show that there have been no transfusion related infections, that it is very rare for adults to develop inhibitors, and that there are few but have been some allergic reactions, usually within 10 minutes of administering treatment. New products are tested in 4 phases: Phase 1 Safety test few patients on a low dose Phase 2 Test if the drug works Phase 3 Randomised trials followed by Market Authorisation Phase 4 Post market surveillance There are five new products on the market and all need to be monitored. Doctors and patients shouldnt assume that an adverse reaction is a common side effect and need to report all adverse events so that rare or common adverse effects are detected.

Update from Australian Haemophilia Centre Directors Organisation (AHCDO) Tolerisation advisory committee
- Dr Chris Barnes. Royal Childrens Hospital, Melbourne Development of inhibitors is the most serious complication of haemophilia therapy. Bypassing agents are available but haemostatic efficacy cannot match factor replacement in patients without inhibitors. Immune tolerance therapy (ITT) is the preferred approach for patients with inhibitors. There has now been three decades of experience with different protocols. All protocols involve ongoing, uninterrupted exposure to FVIII / FIX (+/- immune modulation) over a period of weeks to months with the goal of producing antigen specific tolerance. UKHCDO, International consensus panel (ICP) and European consensus panel (ECP) have all concluded: Deferring ITT until the inhibitor titre < 10BU is preferable (level IIB or III) Avoid FVIII prior to ITT (IIB) Insufficient evidence to make strong recommendations regarding dose. No evidence to support superiority of individual product for ITT VWF containing products should be considered in those patients who fail ITT (IIB) Immune modulation (Retuximab) is not recommended for first line therapy but should be considered for patients who have failed ITT

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ITT is Important, Expensive, and Difficult. There are few reliable predictors of success available. Because there are only a few patients available in each centre the tolerisation advisory committee provides a platform for discussion of difficult cases in Australia against background of limited scientific evidence. Risk stratification of patients for ITT is now well established, in keeping with international recommendations. They have developed a collegial network for peer review of cases. Timely collection of data in uniform fashion continues to be a challenge.

Treatment and care - now and the future

Chantal Lauzon
In the closing session of the conference, several perspective where offered on the issues facing the bleeding disorders community. Geoff Simon demonstrated the complex web of issues that are involved when considering treatment for haemophilia and how most have been touched on over the two days of the conference. HFNZ President Deon York then discussed the expectations of the community. The needs of the bleeding disorder community sit alongside many other healthcare issues the ageing population, increased needs with ever shrinking resources, concerns about quality and safety and care disparities. Understanding all these things are important, the bleeding disorder community nevertheless expects: A stable supply of safe and effective treatment products Comprehensive care Ease of access to care The ability to involve family in their treatment Representation at a government level or on committees whose function impacts on their health There has been an evolution of the doctor-patient relationship towards a partnership model, especially in the bleeding disorder community. The community expects to be a partner with decision makers of all levels involving their treatment and health. Stephanie Gunn of the Australian National Blood Authority (NBA) then spoke about optimising the supply and demand for clotting factors. Essentially the NBA is concerned with patients, products, planning and procurement. Although, they are tasked with managing the supply chain and advising on policy development they do not make decisions on policy or funding themselves. They have worked with various parties on establishing the Australian Blood Disorder Registry (ABDR) and believe it will bring real benefits. Having a national database will help clarify some of the complexities around products usage, severities and inhibitors and allow for comparison with other countries. Clotting factor products consume 22% of the 1 billion AUD budget for blood products. Internationally there has been general growth of factor use for FVIII at the rate of 5% per year. In Australia, there has been a general increase in demand for factor VIII and IX over recent years, whereas demand for bypassing agents (rFVIIA and FEIBA) has been more variable. This variability makes it difficult to predict trends and set a budget for blood products. Part of the NBAs challenge is to work out what level of FVIII use is optimal or cost-effective. Usage (units) per patient varies across each state and although they try to map treatment demand by age, there appear to be bubbles of patients who receive more treatment. This variability raises questions over the future stability of the sector, especially with projections showing that the blood budget will need to double in the next 10 years.

Inhibitors in mild haemophilia

- Dr Simon McRae, Royal Adelaide Hospital Mild Haemophilia is normally defined as having factor VIII levels of 540 IU/dL. Around 5% of people with mild haemophilia develop inhibitors. The consequence is the development of a severe bleeding pattern. Two-thirds of patients exhibit a bleeding pattern similar that that seen in acquired haemophilia; i.e. mucocutaneous bleeds, severe GI bleeding, and urogenital bleeding. Inhibitors in mild haemophilia are often diagnosed after a failure to respond to standard therapy, often following surgery. The type of genetic mutations appears to be the risk main factor for development of inhibitors in mild haemophilia and the other is exposure to an intensive treatment episode. Hay and colleagues noted that 16 of 26 cases were associated with recent intensive treatment. Canadian paediatric data showed that all patients developing inhibitors did so after a peak treatment period. A Dutch paediatric study showed a high risk if the first intensive treatment for was surgery, and a difference if the first intensive exposure to FVIII was over 30 years of age (OR 13.54; 2.757) or before 30 years of age (OR 1.55;0.610)

The outcome of the 2011 recombinant FVIII tender in Australia means that clinicians/patients now have the choice of two suppliers Bayer (Kogenate) and Pfizer (Xyntha). Baxters FVIII product Advate will no longer be available in Australia after the 1 year transition period. Gunn noted that the tender process has lead to improvements in supply contracts that have resulted in significant savings and security of supply. A number of issues have been raised with the NBA about the outcome of the tender and the consequence of having patients switch products. One concern raised was that switching products may increase the risk of inhibitor development. Gunn responded that the NBA does not believe that there is sufficient evidence to suggest that there is an increased risk of inhibitor development from switching. They use a very long process to determine which products are the best choice and their priorities are to get safe products and deliver them within budget. Gunn noted that, as a sector, we cannot move forward if different parties have such different views of what constitutes risk. There are no medical interventions that do not carry some measure of risk. Ideally having a realistic shared understanding of risk would be productive. She also cautioned that in the future it will be increasingly difficult for the government to continue to provide so many products for free and that taking measures to contain costs is one way to ensure product continues to be subsidised. Finally, Dr Mike Makris from the UK gave a personal view of the future of haemophilia care. To begin with there are new products constantly in development. At the moment there are four new fibrinogen concentrates, new rFVIIa, rVIII, rFIX, rFV and generic recombinant products all in development. There are also longer acting concentrates and new transgenic products. In Russia they have even developed their own version of rFVIIa, but they are using it without having any clinical trials or reporting. Highlights of future care include: Longer-acting concentrates. The aim of having products with longer half lives (the time period they are active for) is to be able to decrease prophylaxis to once weekly dosing. Once they are available researchers will need to work out how often to use them, how to use them in situations such as surgery and how to monitor them. He believes that a longer-acting FIX product may be able to reduce prophylaxis to once weekly but people using FVIII products will still probably require twiceweekly injections. Individualised dosing. Each person metabolises factor products at a different rate. Easier to use and more widely available tests mean that determining the best dose for a persons individual half-life is possible. Makris feels that standard dosing will become a thing of the past as dosage will be tailored to each person, their metabolism and their activities levels. For example, a professional cyclist with haemophilia in the UK has daily prophylaxis but only with a half-dose of product to maintain a steady level of factor cover, even if it is lower.

Gene Therapy. Gene therapy to cure haemophilia has been shown to work in dogs and monkeys but until recently the process did not translate into success in humans. A recent study means that for the first time a cure via gene therapy for humans with haemophilia B may become a reality. The trial had initially been intended as a safety trial but has also shown that the product is effective - the patients were producing their own factor IX even 150 days after the treatment. Factor levels were around 8%, high enough to no longer require prophylaxis. The study has been accepted for publication by the New England Journal of Medicine but Makris warned that clinical usage of the technology may still be 5-10 years away (see the report of the WFH Global Forum for further information). Financial Pressures. The bleeding disorders community, researchers and industry will have to provide better data, especially cost-effectiveness data, to ensure standards of care are maintained or improved. The UK has experienced a 7% increase in total factor usage per year. There is a lot of variability in dosages between centres and this will have to be standardised to make a strong case with the government. Funders will increasingly ask for outcome data. The data that need to be collected include reporting of all bleeds (location, severity, amount of product used), joint scores, quality of life data, and days of school/work lost to bleeds. Role of haemophilia nurse consultants. The reality is that most haematologists specialise in leukaemia, not haemophilia, and most junior doctors have minimal experience in treating bleeding disorders. Specialised haemophilia nurses are the key to the future of care delivery as they provide an experienced, knowledgeable workforce. Nurse consultants will also be able to prescribe medication as needed. Haemophilia nurse consultants will deliver the majority of care to people with haemophilia in the future and are already in places in some centres in the UK. In closing, Makris strongly urged people with haemophilia to consider taking part in clinical trials as numbers are needed. With rare disorders you need as many people as possible taking part to show any difference between options and to try to establish the best outcomes.

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A personal experience of tolerisation

- Hamish Robinson, NSW Hamish Robinson shared his story of having haemophilia with inhibitors, and some great bleed photos! Diagnosed at 11 months, he underwent tolerisation when he was 9 and successfully tolerised. Following this, he has had no major problems.

2011 16th Australian & New Zealand Haemophilia Conference

16th Australian & New Zealand Haemophilia Conference 2011

Dates to Note
24-26 February 2012
Parents Empowering Parents (PEP) Programme Auckland

16 -18 March 2012

Northern Region 2012 Camp Campbell Park Mangawhai Heads

24 March 2012

HFNZ National Annual General Meeting TBC

17 April 2012 April 2012

World Haemophilia Day HFNZ Childrens Workshops Dates and Locations TBA

8-12 July 2012

WFH 2012 World Congress Paris, France

6-9 October 2012

HFNZ New Families Camp Forest Lakes, Otaki More details on all events are available from your local Outreach Worker.
Jeff Oliver Print - Whangarei

Visit www.haemophilia.org.nz for more information on bleeding disorders, HFNZ news and past issues of Bloodline

38 BLOODLINE - December 2011