HaemophiliaNationalHaemophilia Foundation Australia www.haemophilia.org.au

No. 155, September 2006

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HaemophiliaAwareness Week8-14 October 2006

Haemophilia Awareness Week 2006 willbe themed Busting the Myths and aimsto dispel many myths about haemophilia.HFA will manage a media campaign andis working with State/TerritoryFoundations to develop activities aroundAustralia.

You can participate in many ways duringthe Haemophilia Awareness Week -

• Host a "MYTHBUSTER" trivia night.

• Set up a stand in your workplace,school, hospital – busting the myths!

• Organise a fundraising event such as acasual clothes day, bike ride, walk, runor swim.

• Organise a theme day at yourworkplace or school – wear red andwhite and donate a gold coin.

• Host a luncheon, sausage sizzle ormorning/afternoon tea.

HFA will provide you with promotionalitems for your event.

For more information please contactNatashia on 1800 807 173 or emailncoco@haemophilia.org.au or visit ourwebsite www.haemophilia.org.au.

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Haemophilia Foundation AustraliaRegistered No.: A0012245MABN: 89 443 537 1891624 High Street Glen Iris, Victoria, Australia 3146Tel: +61 3 9885 7800Freecall: 1800 807 173Fax: +61 3 9885 1800hfaust@haemophilia.org.auwww.haemophilia.org.auEditor: Sharon Caris

Contents

2 Australia/NZ Haemophilia Social Workers’ & Counsellors’ Group

3 Australian & NZ Physiotherapy Haemophilia Group

4 Australian Haemophilia Nurses’ Group

5 Staff Changes Around the Country

6 Speaker’s Kit

7 Global Feast

8 Review of Hemophilia 2006 Medical Sessions

9 New Directions in Diagnosis and Care

10 Musculoskeletal Sessions Explore New Treatments

11 WFH General Assembly Embraces Vision of Treatment for All

13 Recent Advances in the Search for a Haemophilia Cure

14 Gene Therapy

Our Corporate Partners

15 Hepatitis C Awareness Week

Stress and Hepatitis C

17 The Power of Story

19 WFH Twinning Program

20 Natural Evolution of Haemophilia Care

23 Haemophilia and Ageing

24 Calendar

Australia/New ZealandHaemophilia Social Workers’And Counsellors’ Group

The annual meeting of theAustralia/New Zealand HaemophiliaSocial Workers’ and Counsellors’Group was held in Melbourne on 20-21 July 2006. The meeting wasattended by nine social workers andcounsellors from around Australiaand three Haemophilia Foundationof NZ outreach workers. The agendaincluded full and lively discussionabout psychosocial services and carefor the bleeding disorders’community. The meeting, including a professional development sessionfacilitated by Dr Paul Denboroughon development stages foradolescents, chronic illness andtreatment compliance was valuable.

Membership of the Australia/NewZealand Haemophilia Social Workers’and Counsellors’ Group includes:

Sandy Breit, Victoria (Chair)Leonie Mudge, VictoriaKathryn Le Maistre, VictoriaClaire Reeves, ACTRobert Hearn, NewcastlePosition to be filled, NSWMaureen Spilsbury, QueenslandKelly Brady, QueenslandSharon Hawkins, Western AustraliaAnne Till, South AustraliaHelen Spencer, NZColleen McKay, NZDrew Mackenzie, NZ

The following report of activities waswritten by Kathryn Le Maistre. Kathrynis the Haemophilia Social Worker atRoyal Children’s Hospital, Melbourne.

Many different and exciting activitieshave taken place throughout the yearin each of the States/Territories andNew Zealand. This is just a snapshotof the work that members of theAustralia/New Zealand HaemophiliaSocial Workers’ and Counsellors’Group have been doing with peoplewith inherited bleeding disorders andtheir families.

Victoria:

• Annual HFV family camp April2006 attended by Leonie Mudge

• Transition day for older boys withhaemophilia moving across to theadult setting facilitated by LeonieMudge and Sandy Breit

• Living Well with Hep Cinformation session October 2005facilitated by Sandy Breit

• Sandy Breit was interviewed onHep Chat, hepatitis C communityradio program about counsellingissues

• Teachers seminar 2006 -presentations from Kathryn LeMaistre, Director of Henry EkertHaemophilia Treatment Centre,Educational Advisor and NurseCoordinator

• Newly diagnosed haemophiliaparent group facilitated byKathryn Le Maistre

ACT:

• Involvement of Claire Reeves inthe Quality of Life surveyproduced by Dr Dipti Talaulikar

• Claire Reeves and nursing staffdeveloped an education aid forchildren

Western Australia:

Sharon Hawkins facilitated a range ofactivities including:

• Clinic morning teas at FremantleHospital

• Parent/family meetings

• Carer’s meetings

• Young spouse’s/carer’s meetings

• BBQ for families

• Men’s breakfasts

Queensland:

• Transition day for six youngpeople moving across to theadult treatment centre organisedby Maureen Spilsbury

• Genetic information evening

• Partners’ lunch held every monthwith an outreach lunch plannedfor September on the Gold Coast

>>

Activities And Events Around Australia And New Zealand

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• New families’ BBQ picnic held byKelly Brady

• Development of a social supportchecklist and social workpamphlet by Kelly Brady

New South Wales:

• Introduction of fortnightly caseconferences in which LeolaFarnell is involved

• Haemophilia family camp held inNovember 2005 and organisedby Leola Farnell

• The writing of ‘Men’s Stories’ byRobert Hearn

New Zealand:

• Annual Boys Day Out targeting11-16 year old boys organised byHelen Spencer

• Helen Spencer spoke at theeducational evening organised byHFNZ Northern committee inNovember 2005

• First ‘New Families’ campJanuary 2006 co-ordinated byColleen McKay and assisted byHelen Spencer

• Northern Regional CampFebruary 2006 attended by HelenSpencer

• First National Men’s WeekendJune 2006 co-ordinated by HelenSpencer and assisted by DrewMacKenzie

• Central Region Winter EscapeWeekend attended by Drew Mackenzie

• Play group organised by ColleenMcKay in July 2006

It has been a busy year for the socialworkers and counsellors and they areeach looking forward to planningmore activities for 2006-2007.

The Australian and New ZealandPhysiotherapy Haemophilia Group(ANZPHG) is a professional groupassociated with HaemophiliaFoundation Australia. The group ismade up of physiotherapists fromeach state of Australia (except NT) aswell as New Zealand.

The mission of the group is:

‘To facilitate the provision ofcomprehensive care to people withbleeding disorders through qualityphysiotherapy, and to work inpartnership with HFA and HFNZ topromote optimal quality of life forthis population’.

We seek to be a cohesivephysiotherapy special interest groupin order to effectively support thecommunity of people with bleedingdisorders. Some of our goals include:

• To develop and enhance thenational standard ofphysiotherapy care for those withbleeding disorders.

• To advocate for people withbleeding disorders in relation tocondition-specific needs andopportunities at a local andnational level.

• To provide ongoing educationand support to people withbleeding disorders and theirfamilies.

• To provide specialist advice andeducation to otherphysiotherapists andphysiotherapy students regardinghaemophilia care.

• To develop comprehensive andup-to-date educational resourcesregarding physical activity andmusculoskeletal management forpeople with bleeding disorders ofall ages, their families, and thecommunity.

• To promote awareness within thelocal and national community asto the benefits of bothphysiotherapy and an activelifestyle for people with bleedingdisorders.

• To promote and participate inresearch to advance evidence-based practice, improve thequality of physiotherapy care, and enhance the health and wellbeing of people with bleedingdisorders.

As a group we strive to communicateregularly for: service development,information sharing, peer supportand professional development. Oneway we achieve this is throughannual face-to-face meetings. Groupmembers come together each yearto share ideas and work towardsachieving our goals and objectives.This is kindly supported by HFA.

Since last year’s meeting we haveundertaken numerous projectsincluding a group survey. The goal ofthis was to establish currentphysiotherapy work practices in eachtreatment centre and to use theseresults as a basis for lobbying. It is

>>

This article about the Australian and New Zealand Physiotherapy HaemophiliaGroup (ANZPHG) is written by Rebecca Dalzell, Physiotherapist at Royal Brisbane & Women’s Hospital.

Rebecca advised of her intention to step down as one of the Co-Chairs ofANZPHG at the annual meeting of the group held on 18 August 2006 at the HFAoffices in Melbourne as she will be taking maternity leave later this year. Rebeccaand Wendy Poulsen have provided strong leadership over the last three years andhave further encouraged the value of physiotherapy to care and treatment. Weare grateful to both Rebecca and Wendy for their active participation in thebleeding disorders’ community. We are also delighted that Rebecca will continuean international collaboration with her research about the use of Tai Chi and Yogafor people with bleeding disorders.

Australian And New ZealandPhysiotherapyHaemophilia Group

Rebecca Dalzell

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our desire that each state/territory ofAustralia, and numerous areas of NZ,has funding for a designatedphysiotherapist who is then able tohave the time to develop services andenhance haemophilia care in thatregion. Currently there is greatvariation in roles and funding withmany therapists only available for acouple of hours a week for haemophiliacare. This is not ideal and promotesreactive rather than proactive servicedelivery. Achieving greater funding andsupport for physiotherapy services is anongoing endeavour.

Over the past year many groupmembers have also participated inresearch projects, sat on committeesand provided new and improvedservices within their region.Educational articles and materialshave also been produced, andpresentations delivered at campsand conferences.

Fifteen physiotherapists, includingtwo from New Zealand attend the2006 annual meeting in Melbourne on18 August. We are excited that eachyear we seem to be establishing morecontact with NZ physiotherapists. Atthe meeting this year we hadopportunity to discuss and plan forfuture pilot projects and thedevelopment of new educationalmaterials. We discussed otherresearch projects that are currentlyunderway and looked at how we cancontinue to enhance our servicesthrough new programs and protocols.We had an afternoon of energeticdiscussion regarding treatmentapproaches and managementstrategies, and discussed numerouscase presentations and complexscenarios. It was overall a veryworthwhile day and we now have newplans to move forward within thecoming year. It is our desire that as wecontinue to meet, plan, and achieveour objectives that the community willbenefit through enhanced haemophiliaphysiotherapy services throughoutAustralia and New Zealand.

If you would like further informationon physiotherapy services offered inyour area, or if you have anyphysiotherapy related questions, youare encouraged to contact your localhaemophilia physiotherapist. If youdo not have a name or contactdetails for a physiotherapist, contactHeather at HFA on the freecallnumber 1800 807 173.

The annual meeting of the Australian Haemophilia Nurses’ Group was held inMelbourne 7-8 September. The meeting was attended by 17 haemophilianurses from around the country and 1 from New Zealand. Furtherinformation about the activities of the group will be published in a laternewsletter.

AustralianHaemophiliaNurses’ Group

Australian Haemophilia Nurses’ Group

Australian and New Zealand Physiotherapy Haemophilia Group

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There are some staff changes in thehaemophilia scene around Australia.

Queensland Haemophilia Centre

Rebecca Dalzell, physiotherapist for adults at the QueenslandHaemophilia Centre will leave theposition she has occupied for fiveyears on 13 October to takematernity leave. Carley Ekert willcommence in the position on 16October and will be contactable on the same telephone and contact details.

Kelly Brady commenced as theHaemophilia Social Worker in mid-April and works with MaureenSpilsbury, Senior Haemophilia SocialWorker and the team at theQueensland Haemophilia Centre.Kelly works predominately withpaediatric patients and their familiesat the Royal Children’s Hospital,Brisbane. In addition, she willprovide cover for adult patients andtheir families of the Royal Brisbaneand Women’s Hospital during timeswhen Maureen is away on holidaysand other occasions.

Haemophilia FoundationVictoria

Julia Broadbent has commenced asCommunications Coordinator forHaemophilia Foundation Victoria.Julia’s background is in officemanagement, having had nine years’experience in responsibleadministrative roles. Just before shejoined HFV, Julia was working for apre-school non profit organisationpreparing duty rosters, conductingtraining sessions and designing anumber of business web sites. Priorto that Julia was an office manager inBoston (USA) for a moderntelecommunications company – thisrole included assessing fraudulentactivities and general officeadministration. Julia has alsomanaged a busy dental surgery, andworked as an events organiser inBirmingham, UK.

Staff ChangesAround The CountryHeather Lauder

Women’s and Children’sHospital, Adelaide

Dr Ben Saxon from the Women’s andChildren’s Hospital in Adelaide willbe leaving to work in Toronto for twoyears in mid September. In the firstinstance Dr Tom Revesz will coverclinics, however a clinician isexpected to take on a longer termrole late this year.

Royal Prince Alfred Hospital,Sydney

Leola Farnell, haemophilia counsellorat the Royal Prince Alfred Hospitalrecently left the position after severalyears’ service. A replacementcounsellor has not yet beenappointed.

Kelly Brady

Julia Broadbent

WORLD AIDS DAY

Friday, 1 December 2006

AIDS Awareness Week – Friday, 24 November to Thursday, 30 November 2006

HIV/AIDS - Let's talk about it: many faces, different stories

HFA has completed a Speaker’s Kitwhich will be distributed to allState/Territory Foundations for usewhen they represent theirorganisation or HFA at a speakingengagement. The kit, which iscompiled in a display folder, includesspeaker’s notes, powerpointpresentations to go with the notes,further information about bleedingdisorders and issues affecting thecommunity, school project sheetsand activities to encourage audienceparticipation. The resource isdesigned to assist people in theirpreparation for speakingengagements, and to ensure weportray consistent information andappropriate messages.

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HFASpeaker’sKitPiergiorgio Moro

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Haemophilia Foundation Australiawill join other internationalHaemophilia Foundations includingNew Zealand, Ireland, UnitedKingdom, Portugal, Iran, Argentinaand Netherlands for GLOBAL FEAST2006.

You are invited to be an officialGLOBAL FEAST host. Time yourevent around the month ofSeptember 2006. Invite your family,friends and work colleagues. Askthem to bring a donation instead offlowers, wine or a gift. Explain thatthe proceeds will go to HFAprograms and services and tosupport people worldwide withbleeding disorders. Make sure youadd that every dollar donated willmake a difference to the lives ofpeople struggling with lifelong andoften disabling disorders.

Did you know that 75 % of theglobal population of people withhaemophilia is not diagnosed andthat 25 % do not receive adequate treatment

If a dinner isn’t your "cup of tea", any type of festive event will do — apancake breakfast, pizza party,backyard barbeque, afternoon tea or

Global Feast 2006

picnic lunch. Be creative and have fun!

If you are interested in participatingin GLOBAL FEAST please contact us.We will send you everything youneed to make your event a success;brochures, promotional items,posters and tax deductible receiptsfor your guests.

It’s so easy, but it will make a hugedifference to the lives of others.

What can you do?

• Organise a morning or afternoontea at your workplace.

• Organise a sausage sizzle outsideyour local supermarket (pleaseseek permission).

• Contact your local restaurant andask them to participate. Inviteyour closest friends for a meal.

• Organise a dinner with membersat your Haemophilia Foundation.

• Host a BBQ street party.

• A cooking demonstration party toshare the secrets on how toprepare a favourite dish.

For more information or to registerplease call Natashia Coco on 1800807 173, emailncoco@haemophilia.org.au or visitthe official GLOBAL FEAST websitewww.globalfeast.org.

HAEMOPHILIAFOUNDATIONAUSTRALIA WILL CELEBRATEGLOBAL FEASTAT THE CITYSQUARE INMELBOURNEWITH A SAUSAGESIZZLE TO RAISEFUNDS FORWFH AND HFA.COME DOWN AND BUY ASAUSAGE!

Friday 6 October 2006

Melbourne City Square, CornerSwanston and Collins Street11am-2pm

Did you know that 75 % of the global population of people with

haemophilia is not diagnosed and that 25 % do not receive

adequate treatment

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Particular thought went into the selectionof the topics and speakers for the

plenary sessions in the congress medicalprogram, which aims to provide an insightinto the very latest developments in hemophilia treatment.

OrthopedicsWe heard from Luigi Solimeno (Italy) thatelective orthopedic surgery in patients with inhibitors is now feasible, although the outcome may not be as good as in non-inhibitor subjects, as the incidence of hemorrhagic and infectious complicationsappears to be higher. The very high cost ofblood products needed to cover the surgeryis often a significant obstacle.

Goris Rosendaal (Netherlands) providedinsight into how hemophilia arthropathydevelops in the first place, explaining thatthe liberation of iron from red blood cellsreleased into the joint space associated withjoint bleeds leads to the generation ofdestructive oxygen metabolites, which inturn causes premature death (apoptosis) ofcartilage cells.

Prophylaxis and hemophilia careThe role of prophylaxis in preventing development of joint damage was empha-sized in a session chaired by Victor Blanchette(Canada). Although the efficacy is now clear from several long-term studies, many questions still remain unanswered, such aswhen to start, exactly what dose regimesshould be employed, and whether it is everfeasible to stop in adulthood. In a separatesession devoted to pharmacokinetics,chaired by Martin Lee (U.S.), it was empha-sized that there is considerable inter-patientvariation in the elimination profile of bothfactor VIII and IX. Pharmacokinetic evalua-tions may have an important role in optimizing prophylaxis regimes in individual patients.

Bruce Evatt looked at how hemophiliacare has developed in the last few decades.He emphasized the danger of complacencyin the hemophilia community and the needfor vigilance to maintain what we havealready achieved. Evatt highlighted the difficulties in recruiting medical personnelwith an interest in hemophilia care in manycountries and he recommended broadeningthe scope of work to encompass thrombosisas well.

Hemophilia and the elderlyThis was the first congress to have a sessiondevoted to the problems of elderly patientswith hemophilia. Alison Street (Australia)highlighted that colleagues in disciplinessuch as geriatrics will now have to becomefamiliar with the condition and becomemembers of our multidisciplinary team. Herkey message was that the life expectancy ofpeople with hemophilia in affluent countriesis now very similar to that of the rest of thepopulation. Balance dysfunction in elderlypatients needs to be assessed. The manage-ment of myocardial infarction (heartattacks) can be particularly problematic inolder patients with hemophilia, as conven-tional treatment typically involves the useof potent drugs that inhibit blood coagula-tion. As patients live longer, attention isrightly turning to measurement of quality of life as well as longevity.

Quality of lifeA session chaired by Alessandro Gringeri(Italy) explained the application of newquestionnaires specifically designed to measure quality of life in hemophilia, andshowed how the results can be used toexplore data for clinical research. The datacould be used, for example, to determinecost-benefit analysis as well as to determinepatient treatment preferences and satisfac-tion. Gringeri argued that periodic quality

of life measurement should become part ofthe routine review of patients.

InhibitorsCharles Hay (U.K.) chaired a debate betweenClaude Negrier (France) and Keith Hoots(U.S.) on whether the incidence of inhibitordevelopment is higher among recipients ofrecombinant products as compared to thosewho receive fewer, pure plasma-derivedproducts. Several small studies suggest thatthere is a difference but it is clear that furtherprospective randomized studies will berequired to address this important issue. TheWFH is also keen to encourage sharing ofdata between databases in different countriesin order to provide the statistical power toanswer this important question.

Jan Astermark (Sweden) explored thefundamental question of why patientsdevelop inhibitors. It is not disputed thatthe underlying genetic defect plays a majorrole in determining predisposition.Astermark reviewed other contributing factors including data from the MalmöInternational Brother Study (MIBS), whichsuggests that immune response genes arealso important. He explained that certainpolymorphisms of interlukin-10 and tumournecrosis factor (TNF∂) are associated with a high risk of inhibitor formation.

New treatmentsDavid Lillicrap (Canada), chosen to presentthe prestigious Arosenius Lecture, describedhis work in the field of gene therapy (see page 12). This was complemented by a session on ethical aspects (chaired by Donna DiMichele, U.S.), in which aspectsrelating to gene therapy was one of the topics considered. There was also debate on the place of gene therapy in the contextof the current state of hemophilia care inwhich safe and effective treatments arealready available.

Dr. Paul GiangrandeWFH Vice President Medical

Medical experts share knowledge in state of the art medical program.

Review of Hemophilia 2006 medical sessions

V P M E D I C A L W R I T E S

The following five articles are reprinted from “Hemophilia World”, September 2006, the journal of the World Federation of Hemophilia

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Doctors review the congress program.

Speakers emphasized that safety must bethe first and foremost consideration in clinicaltrials. Steven Pipe (U.S.A.) explained in hisplenary lecture how targeted modificationof the structure of the factor VIII moleculecould yield recombinant products withenhanced properties such as a prolongedhalf-life or reduced immunogenicity.Another potential strategy to prolong theeffective half-life of factor VIII wouldinvolve reducing clearance from the circula-tion through pharmacological blockade ofhepatic receptors, which normally take upfactor VIII from the blood.

Akira Yoshioka (Japan) chaired a separatesession on novel therapies. We heard of anew treatment under development in hisdepartment, consisting of an antibody capa-ble of imitating the activity of factor VIII bymodulating the interaction between facto IXaand factor X. In an in vitro experiment, theaddition of the bispecific antibody to factorVII deficient plasma shortened the clottingtime but without impairing factor VIII activ-ity, which suggests an additive effect.

The session also included a presentationof preliminary data relating to the efficacy,immunogenicity, and pharmacokinetics ofrecombinant porcine recombinant factorVIII, which is under development as a treat-ment for patients with inhibitors. Some dataon the use of recombinant factor VIII formulated in polyethylene glycol-treatedliposomes was also presented. This appearsto prolong the action of the factor VIII andthis study reported considerable prolonga-

tion in bleed-free periods with prophylacticinfusions. The new formulation provided 13 days of protection as compared to sevenwith conventional product.

Other bleeding disordersThe recent WFH strategic review recognizedthe need to broaden the scope of our workto encompass both von Willebrand diseaseas well as the rarer disorders of coagulation.The program amply covered both themeswith a session on rarer disorders chaired byFlora Peyvandi (Italy). There is a need topool experience on the rarer disorders andan international database has been estab-lished (www.rbdd.org).

There is also a need to develop specificcoagulation factor concentrates for many ofthese conditions, as in many cases the onlytreatment available is still only fresh frozenplasma. Much is known about the geneticbasis of these conditions and this could represent an important tool for preventionthrough prenatal diagnosis and counselling:many of these conditions are inherited in anautosomal recessive fashion and arisebecause of parental consanguinity.

Laboratory sessionsLaboratory support is a vital component of hemophilia care. A separate session onplatelet disorders, chaired by CatherineHayward (Canada), focused on new diagnostic tools, such as the use of flowcytometry. Recombinant activated factor VIImay be of value in the treatment of some of

these disorders. Robert Montgomery (U.S.)gave a plenary lecture on von Willebranddisease in which updates on the classifica-tion, laboratory diagnosis, and molecularbasis were presented.

Clinical aspects were explored further ina session chaired by Peter Kouides (U.S.), in which the potential impact on womenwas explained. Many women can sufferfrom menorrhagia and it is important to atleast consider the possible diagnosis ofvWD in women with this problem.Intranasal desmopressin is effective treat-ment in many cases if the response totranexamic acid alone is not sufficient.

Another session devoted to vWD,chaired by Augusto Federici (Italy),explored the role of desmopressin in thevarious subtypes of vWD. No recombinantVWF concentrate exists yet and the sessionalso reviewed the various plasma-derivedproducts available. These all contain a goodVWF multimer profile are subjected to viralinactivation processes, and have provedeffective in surgery. However, it is nowappreciated that there is the potential forvenous thromboembolism in the setting ofsurgery with these concentrates and laboratory monitoring is recommended inorder to ensure that levels of factor VII donot exceed 150 iu/dl.

I would like to thank all members of themedical program committee for their tire-less and valued work in helping to developthe program for the Vancouver congress.

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Ahighlight of the psychosocial programwas Parents Empowering Parents

(PEP), a pre-congress workshop thatfocused on the unique problems faced byparents of children with bleeding disorders.Designed by U.S. social worker DannaMerritt, PEP is presently used only in theU.S., but hopefully will be piloted globallyover the next year.

Miriam Rodriguez from Uruguay lookedat programs to increase the quality of life of children and families by increasing theirself-esteem and involving them in activitiesand workshops.

All speakers agreed that burnout affectsmany families and paid helpers of peoplewith hemophilia. In response to reports ofoverwhelming stress, one congress sessionexamined the causes of burnout as well asstrategies to deal with it.

The effects of HIV and HCVDejan Krstic from Serbia was given a stand-ing ovation for his account of the impactof HIV and hepatitis C (HCV) on his community, where approximately 20% ofhemophilia patients are affected by HIVand 45% by HCV. Delin Kong fromShanghai also described the local implica-tions of viral infection and his attempts toraise public awareness of the situation.

Research by U.S. social worker SusanCutter finds that although HIV and HCVcan have the same clinical effects on individuals no matter where they live, significant differences occur between developing and developed countries in

New directions in diagnosis and care Maureen SpilsburyChair WFH Psychosocial Committee(with other reports)

Participants from bothdeveloping and developedcountries benefited from an extensive range of psychosocial, nursing, andother congress sessions oninherited bleeding disorders.

the incidences of infection, available treatment options, perception of the infec-tions, and impact on quality of life.

Brazilian psychologist Frederica Cassishas found that emotional support and indi-vidual and group psychotherapy for peoplewith HIV and HCV help participants tocope with the reality of constant treatmentand live a productive life.

Robert James presented a moving sessionbased on the U.K. website: “Living Stories:the haemophilia and HIV life history project.(http://www.bl.uk) This resource provides aunique insight into individuals’ experiencesof living with hemophilia and terminal illnesses. “I am always touched by hearingaccounts of family members and worker’sown experiences and can never get enough”,said one social worker. “In a very powerfulway, it connects me to other people who arein similar situations – it’s healing!”

Nurses focus on awareness and earlydiagnosis of rare bleeding disordersBetter awareness and knowledge about rarebleeding disorders, coupled with earlierscreening and diagnosis will improve care,medical outcomes, and patient quality oflife, according to panellists at a pre-congressnursing workshop.

Several case studies demonstrated thatdiagnosis of disorders such as factor XIIdeficiency is often slow. In a U.K. case citedby Mary Edgar, it took 26 days and multiplesevere bleeding incidents before the patientwas referred for appropriate care. Even afterdiagnosis, Edgar said, “We’re often working

in the dark; there’s very little pharmaco-kinetic data, so a lot of the time it’s trial anderror with a bit of science applied.”

“It’s also important to understand thepatient’s particular circumstances and challenges to provide effective treatment,”according to Nadine Alliaume, from France.“Family situation, cultural considerations,distance from treatment, and problems withcompliance all affect care.”

Nathalie Aubin directed participants to www.hemophilia.ca for a series of infor-mation booklets published jointly by theCanadian Hemophilia Society and theCanadian Association of Nurses inHemophilia Care.

Women with bleeding disorders ‘a silent majority’Women with bleeding disorders are a“silent majority” who lack proper diagnosisand care, with those in developing countriesfacing particular challenges due to social,economic, and cultural barriers, according to Roshni Kulkarni of the U.S.

Beyond von Willebrand disease, bleedingdisorders in women also include coagula-tion factor deficiency, immune thrombocy-topenic purpura, platelet function disorders,and acquired hemophilia.

Early diagnosis of women’s bleeding disorders is critical to avoid unnecessaryand invasive procedures such as hysterec-tomies, while early treatment can preventsevere manifestations such as athropathy,Kulkarni said.

Delegates discuss aprevious session

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Musculoskeletal sessions at the 2006world congress included assessment

tools, surgical and non-surgical treatmenttechniques, and guidelines for making decisions about the best treatment. Formalfeedback from participants was highly positive.

Pre-congress workshops were held forphysiotherapy/rehabilitation and orthope-dics.

Physical therapists learned that bonedensity and levels of physical activity areimportant factors in addition to traditionalmeasures such as joint range of motion andmuscle strength.

A diverse panel, including consumers,shared views on “compliance.” Theseincluded the necessity of communicationand negotiation between client and caregiver for more realistic expectations on bothsides. Speakers from different backgroundsdiscussed physiotherapy management ofcontractures, which continue to be a challenge in the developing world. Finally,an open session enabled people to sharetheir current and planned research activities.Many exciting projects are underway and adatabase will be developed to enhanceinformation sharing.

An orthopedic pre-congress day, held forthe first time, reviewed current knowledgeand best practice in the areas of synovior-thesis, joint surgery, bone substitutes, andsurgery for people with inhibitors. It wasstanding room only when more than 130 orthopedic and physiotherapy/rehabilitation participants came together fora session on diagnostic imaging.

Musculoskeletal session participants alsoheard how bone grafting is being used successfully for the treatment of hemophilicpseudo tumours and cysts. Bone substitutes,genetic transduction, and autologous chon-drocyte implantation are seeing success incontrolled experiments, but are still far frombeing practical or readily available as atreatment technique for the tissue damagecaused by hemophilia. Nevertheless, theydo offer hope of some exciting possibilities.

Elsewhere, orthopedic surgery for peoplewith HIV and/or hepatitis C presents someunique challenges. Risk of infection andanesthesia issues must be considered, andsurgery for this group must be conducted in specialized hemophilia centres.

“Synovitis: what is the best time totreat?” is always a difficult question. Thetopic was explored by six speakers on the

second day of the congress. The shortanswer is: prevention is best but not alwayspossible. The next best solution is early andas aggressive treatment by whatever meansavailable in each treatment centre.

Other speakers presented updates onfive important assessment tools:International Joint Health Scores (Clinicaland MRI), Functional Independence Scorefor Hemophilia, and the HemophiliaActivities List. As these tools are refined,they will become the international standardfor assessment of people with hemophilia.

Much of our understanding of manage-ment of hemophilic joints comes from otherdiseases. However, we are now developingevidence specific to hemophilia about someof the treatments we use. Dr. GorisRoosendaal summarized experimental workthat shows that blood causes direct damageto cartilage. The damage increases if the cartilage bears weight in the presence ofblood. This finding emphasizes the need forprevention or very early treatment of allbleeding episodes, and reinforces theimportance of rest.

There is good experimental evidence thatice is an effective pain reliever, but otherexperiments show that ice may slow clotting.The hemophilia team in Pretoria, SouthAfrica, designed a study to measure thedirect effects of ice on bleeding in acutehemarthroses. At present there are somemethodology issues, but the project isimportant to help us understand whether iceshould be a regular part of hemophilia care.

In all, 30 speakers from 19 differentcountries presented original work. Topicsranged from laboratory work with culturedchondrocytes, to gait analysis and physicalactivity levels. While many of these studiesare small and inconclusive, the authors arecommended for trying to enhance the baseof evidence for our practice.

Kathy MulderChair WFH Musculoskeletal Committee

Musculoskeletal sessions explore new treatments

The latest research from 19 countries presented at congress.

Executive officers of the WFH musculoskeletal committee.Left to Right: Tariq Sohail, Rachel Tiktinsky, Pier Luigi Solimeno, Kathy Mulder, Adolfo Llinas.

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Following a highly successful world congress, the 28th WFHGeneral Assembly at the Morris J. Wosk Centre for Dialogue in

Vancouver brought together delegates from national hemophiliaorganizations to review recent accomplishments and set futuredirections.

Great progress has been achieved on many fronts through theWFH since the last general assembly. This could not have beendone without the leadership of WFH president Mark Skinner,invaluable direction from the executive committee, and the hardwork of our staff and volunteers.

The network continued to expand, with Sri Lanka achieving full membership, while Bahrain, Japan, and Lesotho joined the foldto bring the total number of WFH National Member Organizationsto 109.

Treatment for AllThe general assembly heard an overview of the WFH’s new strategic plan focused on making available treatment for all thosewith inherited bleeding disorders, regardless of where they live.

“Treatment for all means ensuring care for everyone in our community — that all people with hemophilia have access to a safeand adequate supply of products and care by a multidisciplinaryteam,” said WFH president Mark Skinner.

“It also means expanding services to von Willebrand disease,rare deficiencies, and inherited bleeding disorders. Another focus ofthe strategic vision is to ensure continued development and sustaintreatment where it is already well established.”

Over the past 18 months, WFH hemophilia care programsreached more than 75 countries. Efforts to help build local capacityexpanded, with 33 ongoing country programs in 2006, while another42 countries were involved in general program activities. The firstthree-year cycle has been successfully completed by the GlobalAlliance for Progress (GAP) project, which aims to close the gap in hemophilia care between developing and developed countries.To date, six agreements have been signed with governments toestablish national hemophilia care programs. The WFH also supports 49 active twinning partnerships worldwide.

Delegates wholeheartedly supported the vision of Treatment forAll, providing the WFH with a plan for the future focused onimproving standards of treatment for all rare bleeding disorders.The plan also involves monitoring, advocacy, research, training andeducation, and resource management.

New executive committee membersThe general assembly saw four executive committee positions up for election this year: Dr. Angelika Batorova (Slovakia) and

Dr. Nigel Key (U.S.) were elected as medical representatives, and re-elected as lay members were Gordon Clarke (U.K.), and CésarGarrido (Venezuela).

Departing executive membersThis year, several executive committee members completed theirterms. Distinguished hematologist Bruce Evatt ended his 10-yearterm of office as VP programs. Bruce has been a tireless WFH volunteer and his outstanding scientific achievements have contributed immensely to the world hemophilia community. The general assembly also acknowledged the service of MohamedAris Hashim as a lay member on the executive committee, andmedical representative Mammen Chandy.

Congress 2010Representatives from the Argentinean and French hemophilia societies gave presentations for hosting the 2010 world congress.The winning vote went to Argentina, and Buenos Aires will be the site of the 29th WFH World Congress.

With a new blueprint for success, the WFH is energized for thework and challenges ahead. “We have made great progress on allfronts,” said Skinner. “We must sustain the many gains we haveachieved thus far. Through focused and deliberate implementationof this plan, we will move closer to making our vision a reality.”

Miklos FulopWFH CEO and Executive Director

WFH General Assembly embracesvision of Treatment for All

With 109 National Member Organizations, the WFH’s reach continues to grow.

Dr. Grace Phiri of Lesothoaccepts WFH membership

on behalf of her national hemophilia organization.

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Professor David LilllicrapQueen’s University, Kingston, Canada

Recent advances in the search fora hemophilia cure

Experts ‘cautiously optimistic’ about theprospects of gene and cell-based therapy for hemophilia.

The goal of a long-lasting treatment for hemophiliausing innovative gene and cell-based strategies

remains a feasible objective, as seen from papers presentedat three independent meetings last spring. Recentadvances were described at the U.S. National HemophiliaFoundation workshop on gene therapy in Philadelphia,the WFH Congress in Vancouver, and the annual meetingof the American Society of Gene Therapy (ASGT).

The best results to date from clinical hemophilia genetransfer have occurred in the Avigen-sponsored trial in theU.S. using a recombinant adeno-associated viral (AAV)vector to deliver factor IX to the liver. (A vector is amethod to transfer DNA sequences from one organism toanother.)

In one patient studied, a plasma factor IX level of 12%was achieved and therapeutic levels were expressed for amonth before returning to baseline levels, accompanied byevidence of liver cell toxicity. Subsequent immunologicanalysis in another patient showed that liver damage fol-lowing vector delivery is most likely due to a host immuneresponse against components of the vector protein coat. Inother words, the patient’s immune system recognized thevector from previous virus exposure and attacked it.

Despite this setback, there is still well-founded enthusi-asm for the AAV delivery system. Recent studies in hemo-philia A dogs show that long-term expression oftherapeutic levels of factor VIII can be achieved with thisvector, while two upcoming human clinical trials will useAAV delivery in hemophilia B patients.

A study coordinated by Dr. Kathy High in the U.S.intends to use a temporary course of immunosuppressionto abolish the host immune response to the vector. A sec-ond study involves centres in the U.K. (Drs. AmitNathwani and Ted Tuddenham) and the U.S. (Dr. AndrewDavidoff). In this second study, researchers are trying tocircumvent the host immune response by switching to avector with a different protein coat (AAV8), which may beless likely to be recognized and attacked. The trials maybegin to enroll patients later this year.

There has also been progress with the preclinical appli-cation of certain types of retroviral vectors. Retroviraldelivery systems have the capacity to insert their clottingfactor gene “cargo” into the patient’s chromosomes, and

are thus well suited for the modification of stem cells thatcan then serve as depot sites for clotting factor expression.(Stem cells are cells that retain the ability to become spe-cialized and take the place of cells that have a specifictask.)

Encouraging preliminary data has shown that thisapproach might be feasible using patient blood stem cells,with subsequent re-introduction of the genetically modi-fied cells into the patient for long-term factor delivery. Theretroviral strategies will still need to demonstrate a lack ofimmune activation and, depending upon where the cellshave been re-implanted, long-term viability.

As several different strategies for hemophilia genetransfer are explored in pre-clinical studies, the challengeof the host immune response continues to limit long-termsuccess. Evidence suggests that the delivery of clotting fac-tor genes to specific parts of the body, such as the liver,may facilitate the development of immunologic toleranceto the newly expressed clotting factor protein.

Furthermore, recent studies presented at the ASGTmeeting indicate that gene transfer approaches using thecell’s own regulatory machinery can limit protein expres-sion to defined cell types, and thus minimize the risk ofadverse immune responses such as clotting factor inhibitordevelopment.

Overall, the hemophilia gene transfer communityremains cautiously optimistic about future advancestowards the goal of long-term correction of the bleedingtendency in hemophilia. The next year will undoubtedlyprovide new information about the rate and extent of thisprogress.

Professor David Lillicrap

in Vancouver.

OurCorporatePartnersHFA is delighted to havecommenced a new three yearrelationship with Corporate Partners- Baxter, CSL Bioplasma and Wyeth.HFA recently developed aMemorandum of Understandingwith each of these companies torecognise the common objectives ofimproving care and treatment to thebleeding disorders’ community andthe special relationships,opportunities and responsibilitiesthe relationship creates.

HFA initiated its Sustaining Patrons’Program in 1998 and the programhas been a valuable resource thathas enabled HFA to meet itsobjectives ever since.

In 2006 the Program was renamedthe HFA Corporate Partners’Program and Memorandums ofUnderstanding were established toreflect the framework of cooperationand understanding and recogniseour shared objective to improve thehealth and welfare of individuals andgroups of people in the Australianbleeding disorders’ community.

The partnership also enhancesopportunities to strengthen theknowledge and information needs ofthe bleeding disorders’ communityby education of patients and theirfamilies, and multidisciplinary healthprofessionals through conferences,special projects, and other activities.

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Gene Therapy

Although researchers have alreadyused gene therapy to effectivelytreat mice, dogs, and other animalsthat have haemophilia, transferringthese innovative therapies tohumans has proven more difficult.

In the clinical setting, the humanimmune system eventually rejectsthe gene transfer and, as it attacksthe delivery system (called "vector"),factor levels fall below what isacceptable. Two methods ofovercoming this problem mayprovide exciting results in the nextcouple of years.

People with haemophilia havespecific genetic defects that preventtheir bodies from producing sufficientlevels of clotting factor. So far themost promising method of genetherapy in haemophilia has been theuse of viral vectors – modifying a virusso that it carries genetically modifiedcells for factor delivery.

Viruses are very effective at trickingthe body into replicating theirgenetic contents, and haemophilicdogs treated with this method havemaintained therapeutic levels offactor for years. Although it hasbeen shown that factor levels can becorrected by using viral vectors todeliver corrected genetic material,the human immune system seems torecognse the vector as harmful fromprevious exposure and attacks,eliminating the positive outcome.

Scientists are trying to overcome thisrejection of the viral vector in acouple of ways. One is to usetemporary immunosuppression toprevent the body from rejecting the

vector when it is administered. Theidea is to eliminate the immuneresponse so that the vector willcontinue to work. A second strategyis to try different vectors in the hopethat the human immune system maynot react in the same way to slightlydifferent viral delivery systems. Bothof these methods are now beingattempted in clinical trials in the USand UK.

An alternative strategy to using viralvectors to deliver gene therapy isthe use of retroviral vectors. Thesevectors work to insert the clottingfactor gene into the patient’schromosomes. One version of thistherapy uses the patient’s own stemcells: the retroviral vector is insertedin the lab and the geneticallymodified stem cells are then re-introduced into the patient. (Stemcells are undifferentiated "blank"cells that do not yet have a specificfunction.) This therapy is in muchearlier stages of development but itmay potentially be effective indelivering long-term therapeuticlevels of clotting factor.

For further information and recentpublications on gene therapy see:

Blood, March 2006; Vol 107 No 5

Blood, April 2006; Vol 107 No 7

Nature Medicine, 2006 March12(3):342-7

The following information about two promising strategies for advancing genetherapy in haemophilia is reprinted from information provided by WFH on itsweb site www.wfh.org.

The information is based on material that was presented at the US NationalHemophilia Foundation’s Eighth Workshop on Novel Technologies and GeneTransfer for Hemophilia, in Philadelphia 31 March - 1 April 2006. The NHFworkshop was supported with funding from the Katharine Dormandy Trust, theUS Centers for Disease Control, the Coalition for Hemophilia B, and the WorldFederation of Hemophilia.

The WFH plans to provide ongoing reports on developments in gene therapyincluding news of new clinical trials on its web site and in publications. Readersare also referred to the article by Professor David Lillicrap in this edition of"National Haemophilia".

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National Hepatitis C Awareness Week will be held

1-7 October 2006.

The 2006 Awareness Week aims to promote general

public awareness of hepatitis C, with a special

emphasis on effective self-management practices for

people living with hepatitis, their friends and family,

and those involved in their care.

The Australian Hepatitis Council established a Project

Reference Group to work with State/Territory

Hepatitis Councils, Haemophilia Foundation Australia

(HFA), Australian Injecting and Illicit Drug Users’

League (AIVL), Tasmanian Council on AIDS Hepatitis &

Related Diseases Inc (TASCAHRD), Australasian

Society for HIV Medicine (ASHM), Northern Territory

AIDS and Hepatitis Council (NTACH) to develop

material and events for the week.

The Hepatitis C Awareness web site will have a range

of valuable information about hepatitis C for people

with hepatitis C and their families and there is a

national toll free telephone number for inquiries.

http://www.hepcawareness.net.au/

Call the national toll free hotline number

on 1300 437 222.

The following information aboutstress is abridged from materialcompiled by the NSW Association forMental Health from Strategies forStress (Dr David Lake) and Copingwith Stress (Leichhardt Women HealthCentre) for a Fact Sheet published bythe Hepatitis C Council of NSW inApril 2004.

Stress is an area in our life that we all need to keep undercontrol. If stress is allowed to increase, unchecked, theimpact on one’s physical and mental health can besevere. Some typical signs of stress are insomnia,headache, neck, shoulder and back pain, heartpalpitations, fatigue, irritability, panic attacks, loss ofconcentration and low self esteem. There are a numberof areas in our lives, though, where we can take action tocontrol our levels of stress.

The positive sides of stress

Stress is not necessarily always negative. Without theaction of positive stress, many people would not attemptto reach their full potential. For example, competitivetension usually keeps a footballer or salesperson workingto their best. There is an optimal level of stress at which aperson is likely to be most productive. Knowing one’sparticular stress level is useful.

The negative side of stress

When pressure is continual and when the feeling that youare not in control stays with you over a long period of time,you’re probably suffering the negative effects of stress.When this happens you’ll feel overworked but will actuallybe contributing less and less. Physically, pulse rate willincrease, blood pressure will rise, perspiration increasesand there is an increase in blood adrenalin levels.

Symptoms

You may feel irritable, anxious, nervous or fatigued.There is often a change in appetite and perhaps anincrease in cigarette smoking or drinking alcohol. Othersymptoms may include diarrhea, abdominal cramps,insomnia, trembling, urinary infrequency, increased heartrate and headaches or backaches. As mentionedpreviously, palpitations, fatigue, irritability, panic attacks,loss of concentration and low self esteem may occur aswell. To date, surprisingly little long-term research hasbeen done on the effects of stress - although it has beenlinked to ulcers, infertility, heart disease, mental illnessand cancer.

Stress AndHepatitis C

>>

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Stress and lifestyle

Stress is a normal response tolifestyle and work, and the generalchallenges of living. A certain level ofstress is fine and even necessary inorder to be productive. However, toomuch stress can be a health hazard.People need to learn to be aware ofwhen their stress level is unhealthy.Once stress overload is recognisedyou can assess your current lifestyle,but there are skills you can use toaddress stress - see below.

Nutrition

When we experience stress andnervous tension, our bodies use uplarge amounts of nutrients (vitaminsand minerals.) This can cause dietarydeficiencies which in turn leave ustired, rundown, irritable and lessable to cope with stress-back tosquare one!

Nutrients which are particularlyrelated to stress are Vitamin C, the Bcomplex vitamins, and the mineralscalcium, magnesium, phosphorusand zinc. These nutrients can besupplemented by eating healthyfoods high in vitamin and mineralcontent, or by taking tablets ormedicinal herbs.

Diet considerations

The best diet to cope with stresscontains fresh wholesome foods:

• lots of fruit and vegetables,beans, lentils, bread & cereals

• moderate amounts of dairyfoods, lean meat, skinless poultry,fish & nuts

• small amounts of oils and fats

• salt should be avoided

Counselling

We all live and interact with otherpeople and often cope better withour problems and life stresses bytalking and sharing our feelings withother people. This can be as simpleas talking with a neighbour or friendover a glass of juice or mineral wateror writing a letter or story for yourfavourite newsletter!

Some people may need to phone afriend or relative specifically to talkabout a particular problem.Additionally, many people choose togo to a trained counsellor, socialworker or therapist.

Acupuncture

Acupuncture has been shown to be avery effective treatment for variousconditions as well as a stressmanagement tool. It’s best to see aqualified practitioner who belongs toa professional body.

Massage

Whether done by a trainedprofessional or just a friend, amassage is a simple and inexpensiveway of releasing tension from ourbodies. If done regularly, a massagecan also make for a welcome breakfrom a daily routine.

Meditation

Meditation is a technique that offersa structured way to reduce tensionand stress. Although it may notprovide an immediate release ofstress, over time a meditationprogram will prepare you to handlephysical and mental problems andrecharge your system. Structuredexercise and concentration programssuch Tai Chi and Yoga are also veryeffective in reducing stress.

Choose your attitude

Take some control over the thoughtsthat go through your mind. We allhave such thoughts, some of us moreso than others. Talk yourself out ofrepeating negative or unhelpful selfthoughts. You will be surprised athow much difference this can makeafter a short period of time.

Relaxation

Relaxation can mean spending timeat the beach or local park, doinghobbies or listening to your favouritemusic. The big obstacle to relaxationis physical tension. Relaxation isoften the end result of stressmanagement exercises such as thosementioned above.

Exercise

Physical exercise can often help torelease tension and improves youroverall level of health. The mosthelpful thing is to do exercisemoderately and regularly. Anyphysical exercise such as jogging,swimming, gardening and playing

team sports is helpful. It might helpto talk to your doctor about thesuitability of new activities if youhave a bleeding disorder or otherhealth problems.

Some tips to remember

• Be aware of physical and mentalstress and recognise the signs.Stress is only a problem if it isexcessive or if you are nothandling it properly.

• You are not the only one whoexperiences the problems thatlead to stress - such problems area normal part of everyday life.

• Try not to make problems anybigger than they actually are.Identify situations that arestressful for you - deal with themor avoid them.

• Practise relaxation techniques.

• Find interests that really involveyour concentration.

• Plug into support networks -formal or informal.

• Grow a garden and get a pet ifpractical.

• Take regular time out.

• Talk to others about yourfeelings.

• Live in the present and not in thepast.

• Cultivate regular stillness andquietness in your life.

• Set daily routines, giving yourselftime for some of the above ideas.

Take some control over the thoughts that

go through your mind. Talk yourself out of

repeating negative or unhelpful self thoughts.

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As I walked down the corridor in theinfectious disease department ofBeijing You An Hospital I saw thesign to the Mangrove SupportGroup (MSG) which was the firstsupport group for people living withHIV/AIDS in China. We knocked onthe door and we were invited in. Iwas introduced as an AustralianYouth Ambassador for Developmentworking as an HIV educator in thehospital, specifically within theorganisation called Home of LovingCare which provides care andsupport to people with HIV inhospital. I was welcomed in andoffered a seat and a warm glass ofwater. I shook the woman’s hand as Iwanted her to know that I was notafraid of HIV. Soon after, a man cameinto the room on crutches. He had afirm hand which I found refreshing.He sat, and the woman took hiscrutches. I thought nothing of it.

Within a short time he indicated thathe uses the crutches because hisright leg does not have fullmovement. As I should have alreadyguessed, but did not, he hashaemophilia. If I were in Australia Imay have thought of it. I recall manyimages of Andrew Knox, my latepartner, on crutches and we alwayshad a pair in the cupboard. In aforeign land where I had heard somuch about the plasma infectedwith HIV, I had not turned myattention to those with haemophilia.There is little discussion here ofpeople with haemophilia infectedwith HIV, but there is much generaldiscussion about infections via bloodtransfusions.

The exact number of those withhaemophilia who have died ofcomplications associated withHIV/AIDS is unknown due to

inadequately developed surveillancesystems. MSG is currently preparingto conduct a survey on the needs ofpeople with haemophilia and HIV inChina and use it as an advocacy tool.

During my six month stay in China Imet three men with haemophilia andHIV. I also met the mother of one ofthe men and the wife of the other. Ihad not anticipated meeting so manypeople affected by haemophilia inmy work here in HIV yet it was thesemeetings that were the mostpowerful for me. They were powerfulinteractions because they reinforcedfor me the importance of thehaemophilia community both atformal and informal levels in Australiaand abroad. It is within thecommunity of those affected byhaemophilia that we can most easilyshare stories and experiences thatmake living with both haemophiliaand HIV possible. It is what makesthe transition from dying with HIV toliving with HIV possible.

The youngest boy I met withhaemophilia and HIV was a 16 yearold who lived in rural China with hisparents and brother. He had nevermet anyone with either haemophiliaor HIV. He had no friends who knewof his HIV status. HIV was a secretburden. His mother slept in thehospital bed next to him during hisstay in hospital. Each time I saw hershe cried. The mother pulled up herson’s trouser on his left leg to reveal a

The Power Of Story

Angela Kelly’s experience earlier this year in China when she was working with people with HIV and her encounters withpeople with haemophilia reminds us of the importance of having people to talk to and share difficult experiences andfears with others.

Angela Kelly

>>

He had never met anyone with either

haemophilia or HIV. He had no friends who

knew of his HIV status. HIV was a secret

burden. His mother slept in the hospital bed

next to him during his stay in hospital. Each

time I saw her she cried.

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knee badly affected by hishaemophilia bleeds. Unsure of how Icould reach this young man whoseface was permanently solemn, Ishared with him that I had friendswith haemophilia and HIV inAustralia. Showing the first bit ofinterest in my visit, he looked at meand asked if I would tell him thestory of one of them. I told himabout a young man in Australia whoI know. When I finished he said ‘henhao’ which means it was a goodstory. This young man’s story was anexample of what is possible. Afterthat I asked if he would like to meetanother person with haemophiliaand HIV. He was still afraid, but hesaid he wanted to do so. Iintroduced him to one of the menfrom MSG and they talked for twohours. Already proficient in using thecomputer to play games, he wasshown how to send an email so theycould stay in touch. On a practiceemail the young man wrote ‘I hopewe can become good friends’. Thisyoung man’s isolation was no longer.He had a living example of what waspossible in his life – that he couldlive with haemophilia and HIV.

That young boy’s isolation waslessened by that interaction, but itwas the isolation of a mother I metin later weeks whose isolationconcerned me most. She and herfamily lived in a province north ofBeijing where services for peopleliving with HIV/AIDS were poor andwhere there was littleacknowledgement of the virus. Sheshowed me her son’s test results, hisCD4 and viral load tests over thepast few years. He was doing well

medically with a stable CD4 count of350. His hepatitis C was undercontrol and best of all he washappily married. Her son was now 32and had been diagnosed 10 yearsago. The tragedy of this family’sstory did not reveal itself naturally. Itwas only when I asked how his wifeaccepted a boyfriend, then husbandwith HIV that her secret was shared.Grief stricken with the guilt that shecaused her son to have haemophiliashe could not bear to tell her sonthat he was infected with HIValthough he knew about his hepatitisC. With the support of the doctors,they had colluded to keep her son’ssecret from him and now his wife. Itis a secret she thinks spares him painand lets him live, but it is one that iskilling her.

When I shared with her my ownstory of being the partner of a manwith haemophilia and HIV she beganto cry. What was most important wassharing my partner’s love for hismother and his appreciation of herfor giving him life. A life for him withhaemophilia and HIV was betterthan no life at all. I shared that herguilt was not unique and that othermothers shared this in Australia andother countries, China too. She drewstrength from sharing this story and Ican only hope it may enable her to

face her son honestly and tell himthat in spite of all of her love for himthere were things that she could notprotect him from and because ofthat love she could no longer go onprotecting him from it. It is only bysharing with her son that she will beable to transition into a phase a‘living’ with the knowledge of herson’s HIV status. And, in turn, it willonly be then that her son and hiswife can learn to live with HIV andtake the necessary precautions if it isnot already too late.

When I shared with her my own

story of being the partner of a man

with haemophilia and HIV she began

to cry. What was most important was

sharing my partner’s love for his

mother and his appreciation of her

for giving him life.

YOU’VEGOT MAIL

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The WFH Twinning program, a strong example of the WFH visionof Treatment for All, has enjoyedmuch success and growth in recentyears. The WFH Twinning program issponsored by Wyeth and there arenow 48 twins in 51 countriesthroughout the world.

Linking haemophilia organisationsand treatment centres in developingand developed countries is a positivetwo-way experience for the twins,and results in improved diagnosisand treatment and care, staff andvolunteer motivation, and strongrelationships which encourage andimprove care – often in a stepwisemanner, according to the situation.Planned twinning activities includedoutreach, education programs, andtraining in fundraising and advocacy.WFH works with twins to clarifyobjectives and provides support totwinning partners.

In 2005-2006, several of the newtwinning partnerships haveextended the program into newterritories with the inclusion of CapeVerde-Coimbra (Portugal);Guatemala-Colombia; Honduras-Georgia (USA); Tunisia-Quebec(Canada); South Africa-Canada;Shiraz (Iran)-Milan (Italy);Oran/Sidibelabbas (Algeria)-Strasbourg (France); Dharwad(India)-Detroit (USA); Armenia-United Kingdom; San Salvador (El Salvador)-Houston (USA); andVientiane (Laos)-Brest (France).

The Hemophilia Treatment Centre,and Hemophilia Organisationtwinning awards were presented inVancouver at the World HemophiliaCongress in May 2006.Twin of theYear Awards for 2004 werepresented to three sets of twinningpartners:

WFH Twinning Program

• Dr Robert Reid, Cabral Children’sHospital, Dominican Republic andthe National Hemophilia Centre ofVenezuela

• Lebanese Hemophilia Associationand the University Hospital ofGeneva Hemostasis Unit,Switzerland; the Foundation ofSupport to Hemophiliacs

• Dominican Republic and theVenezuelan Association forHemophilia

Twin of the Year Awards for 2005were presented to

• Fairview University MedicalCentre, Minneapolis, USA and theInstitute of Hematology, Yerevan,Armenia

• Pakistan Hemophilia PatientsWelfare Society and theHaemophilia Society of Malaysia

• Centre d’hémophiles, hôpital deCaën, France, and the Moroccancollaboration of the Hopitald’enfants, Casablanca, and theHopital d’enfants

The following report is adapted from World Federation of Hemophilia (WFH) publications.

Linking haemophilia organisations and

treatment centres in developing and

developed countries is a positive two-way

experience for the twins, and results in

improved diagnosis and treatment and

care, staff and volunteer motivation

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The Natural Evolution ofHaemophilia Care: DevelopingAnd Sustaining ComprehensiveCare Globally

Comprehensive care is vital forpatients with haemophilia to preventearly death and free patients fromthe complications that inhibit livingnormal lives. Experience has shownthat once introduced in a country,there is a progressive restoration ofnormal healthy lives to thehaemophilia community.Accompanying this progress is agradual decreased dependency onthe haemophilia comprehensivecentre – except during brief periodswhen expertise contained within thecomprehensive centre is mandatoryfor life-saving clinical managementor to prevent severe morbidity.During each stage of the naturalevolution of comprehensivehaemophilia care in a country,challenges to the existence of thecentre occur, which threaten thecomprehensive treatment concept.The haemophilia community mustunderstand this natural evolutionand be prepared to workcollaboratively with governments,physicians and other patients toensure that centres retain theexpertise to meet the emergentneeds when they arise.

Prior to the 1960s, when nocomprehensive care was available,individuals with haemophilia suffereda similar fate worldwide. Severe jointdisabilities appeared in early teens,and most patients died before theage of 20. Haemophilia was treatedprimarily with fresh blood

transfusions, and as a resulthaemophilia associations wereestablished for the purpose ofrecruiting the donors.Cryoprecipitate was discovered in1964 and subsequent developmentof clotting factor concentratesdramatically increased clinicalmanagement options. Asconcentrates could be easily stored,administered at home and carriedwith patients during travel, hometherapy was adopted. In developedcountries, early treatment ofbleeding episodes and hometherapy quickly evolved as theprimary management option.Training and education of patientsabout disease management becamenecessary with the increasingpopularity of home therapy.Specialised centres soon deliveredservices to meet these needs. Theseapproaches on patient careproduced significant effects onpatient general health and survival,and as a result, the haemophiliacommunity requested and receivedsupport for networks of haemophiliatreatment centres fromgovernments.

The comprehensive care centremodel provided comprehensiveservices based on an integratedpublic health approach and has beenone of the most successful publichealth programs in many developedcountries, resulting in significantlyimproved health for patients withhaemophilia and reduced use of

health care services. These programshave been so successful at reducingthe complications of haemophilia thatthe dynamics of haemophilia carehave changed significantly. However,these changes are now producingother challenges for sustaining thecurrent model.

Comprehensive care for haemophiliadiffers from routine patient care forseveral reasons. First, it is veryspecialised and affects many otherareas of the patient's physical andmental health and is best metthrough a multidisciplinary teamapproach. Appropriately trained andexperienced medical staff are neededto avoid poor therapeutic decisionsthat can lead to severe disability andmortality. The tasks of thecomprehensive care team, which arecritical to preventive care includediagnosis and assessment, educationof the patient and his/her family,management of acute bleedingepisodes, initiating and supervisinghome therapy, routine follow-up,preoperative assessment andpostoperative management of thepatient when surgery is necessary. Inaddition, the treatment centreprovides support to the patient andfamily to prevent social orpsychological distractions frominterfering with health-care outcomes.

A primary treatment team isorganised in each centre to maximisethe effect of the resources available.In most centres, this consists of a

Dr Bruce L Evatt

The following is abridged from an article which appeared in the State of the Art Book, XXVII International Congress ofthe WFH, "Haemophilia" journal, July 2006, Vol 12, Supplement 3, pages 13-21. For the full text and references pleaserefer to the journal or request a copy from HFA.

Dr Bruce Evatt ended his ten year term of office on the WFH Executive Committee as Vice President Developing World,in May 2006. A standing ovation for his scientific achievements and outstanding contributions to the global haemophiliacommunity at the Awards Ceremony at the Vancouver Congress in May 2006 was a special way to acknowledge histireless work and dedication.

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nurse coordinator, a medical director(usually a haematologist), aphysiotherapist and a social worker.A referral team is organised to meetthe specialised needs of individualpatients and may include specialistsin dentistry, orthopaedic surgery,clinical genetics, rheumatology,infectious diseases or other areas ofclinical medicine.

Second, maintaining trained andexperienced health-care providerscan often be achieved only byconcentrating patient care intospecialised centres. Thecomprehensive care team workscollaboratively with other centres,and these networks enable continualevaluation of treatment trends andmodify treatment standards. Tomaximise the efficiency of thenetwork, it is often divided into twoto four layers or levels of treatmentcentres depending on the number ofpatients in a geographic area andthe resources available. For example,a country may form a basic cliniclevel, a haemophilia treatmentcentre level and a comprehensivetreatment centre level.

Third, priority is placed onpreventive medicine, becausecomplications of haemophilia, suchas infection with hepatitis viruses,HIV and progressive joint disease,are severe and extremely difficultand expensive to treat. Successfulmodern haemophilia care dependson the principle of anticipating andpreventing the complications ofhaemophilia rather than treatingthose complications following theiroccurrence. This principle ofmanagement of haemophilia hascome to be termed comprehensivecare and requires more resourcesand organisation of care deliverythan the standard physician–patientrelationship used for themanagement of many diseases.However, it yields dramatic results byproducing productive individualsliving normal healthy lives withoutthe crippling effects and early death

produced by the complications ofhaemophilia.

Fourth, maintaining a coordinatednetwork of speciality centres enablespatient access to clinical researchand evaluation needed to make well-informed decisions. Goodprevention requires that patients andtheir families make well-informeddecisions regarding their physical,psychological and social health,which is consistent with the most up-to-date medical knowledge. Underthe comprehensive care model, theteam has responsibility for assistingpatients and their families with thesedecisions fall into the comprehensivecare team.

Careful structure of the specialitycentres enables optimal delivery ofcare based on the allocation oflimited resources.

The Economics Of Haemophilia– The ‘Nine Hundred PoundGorilla'

Economics affects how countries ofdifferent economic developmentapproach comprehensive care withtheir governments. Over 20 years,the cost of care has risenexponentially as improved safety oftreatment products has produced a5–10 times increase in cost.Optimum care is beyond the reachof individuals with haemophilia, soothers governments or insurancemust bear the cost. As a result, thehaemophilia community mustcontinually convince others to acceptthis financing responsibility. Bynature, the payer’s focus is on thebottom-line economics, ie 'the mostserved with the least cost'. Thisattitude complicates the efforts ofthe haemophilia community. Incountries with emerging economies,economic issues will be the primaryforce determining how far patientsand physicians can push their goals.In the developed countries, onlyconstant pressure from patientgroups prevents reductions inservices for haemophilia patients,when these countries attempt toreduce rising health-care costs

In fact, if cost alone were thedeciding factor in haemophilia care,it might be very difficult to convincegovernments of the developedcountries to fully finance haemophiliacare. For countries with emergingeconomies, the prospect is evenworse. Cogent arguments against

considering the cost factor as thedecisive one must include aconsideration of the government'sview of the cost factor on thenation's total health expenditures.

Many countries with low GNP maybelieve haemophilia care is notaffordable in their country becausethey would need to spend significant(and often unrealistic) amounts oftheir total health-care budgets toprovide adequate care for theirrelatively few patients withhaemophilia.

To counter this, proposed programsmust be compatible with eachcountry's economy and it must beargued that the comprehensive caremodel can produce significantimprovement in health status andquality of life without theexpenditures used in the developedcountries and relatively smallexpenditures used for theorganisation of the care delivery,education of medical personnel andpatients and modification of bloodbank practises yields large benefitsto patients. Addition of modestamounts of concentrates greatlyamplifies this trend. As moreresources become available,additional services such as

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orthopaedic surgery, prophylaxis andimmune tolerance can be added, butthese will increase the cost andbenefit for fewer patients.

Rather than being discouraged bysuch economic realities, thehaemophilia community must usethis information to clearly argue forthe benefits of comprehensive care,based on scientific data, advocatingfor a health-care plan in line withtheir country's economic capacity.Stepwise improvements in thedelivery system should subsequentlybe requested, as the economyimproves and resources becomeavailable. In the developedcountries, where cost cutting is aconstant threat, the haemophiliacommunity (patients and physicians)must understand the changes thatthese benefits have produced inhealth-care utilisation, speak withone voice of the benefits ofmaintaining high-quality care andadvocate for changes in thehaemophilia treatment centre toensure its continued existence.

The Argument ForComprehensive Care

In developed countriescomprehensive care leads tosubstantial socioeconomic benefits,increased employment, decreasedhealth-care resource utilisation andlower cost of care. Patients anddecision makers might ask whethercomprehensive care is necessarywhen they see happy and wellpeople with haemophilia, butoutside the comprehensive careenvironment costs increasesignificantly.

In countries with emergingeconomies with limited resourcesand restricted access to clottingfactor concentrates, even modestexpenditures to modify the structureand organisation of the care delivery(with emphasis on prevention),patient and doctor education andmodified blood bank practises toimprove the safety and supply oftherapeutic products yield hugeeconomic and quality of life benefits.

Impact Of The Success On Clinic Utilisation In TheDeveloped Countries

The success of comprehensive carehas reduced the need for frequent or

day-to-day management of patientsand a shortage of trained healthprofessionals with experience andthis may lead to funding challenges.

Impact On Trained PhysicianAvailability

Another problem resulting from thedecreased dependency on treatmentcentres is a growing shortage ofdoctors with training to managebleeding disorders. Many doctorsneed to treat patients other thanbleeding patients (eg oncology or haematological malignancies) to make a living, haemophilia andblood coagulation becomessecondary and young doctors arediscouraged or unable to seek trainingin the area. Many young doctors willbe attracted to the growing area ofoncology than haemophilia. Thisthreatens the existence ofhaemophilia treatment centres.

Options For Maintaining Care

It is necessary to attract youngphysicians by providing trainingopportunities and a young physiciantraining in haemostasis must feel thathe/she will practise his/her specialitymore than 20–25% of the time. Weneed to stimulate the researchenvironment for the research of

blood coagulation disorders. Clinicsneed to be expanded to includeother people with bleedingdisorders and those with clottingdefects that produce thrombosis.

Role Of Patient Advocacy

Patient advocacy has been critical inthe past to improve safety andsupply of products and needs to bere-energised. To ensure the gainsmade in the past are not lost.

Future Role Of PatientOrganisations

Patient organisations must recogniseand adapt to the changingenvironment and define a newpurpose for their organisations. Theymust understand political processesand health economics processes intheir country. Patient organisationswill have more credibility and powerincreases when workingcollaboratively with clinicians.Objectives must be set to meetneeds and national plans must bebased on sustainable goals.

The comprehensive care centre model

provided comprehensive services based on

an integrated public health approach and

has been one of the most successful public

health programs in many developed

countries, resulting in significantly

improved health for patients with

haemophilia and reduced use of health

care services.

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The paper addresses falls in the olderman with haemophilia, their causesand consequences and cardiovascularproblems in particular. Very littleliterature has been published aboutthese common problems. We need tobe aware of the ageing issues inhaemophilia and develop 'wellness'programs which are directed to theearly identification of disease as wellas preventative strategies to reducethe physical and psychologicalimpacts of ageing.

Men with haemophilia have not onlythe challenges of living with HIVand/or HCV infection and prematurearthritis as complications of theirdisorder, but they also confront theother ails of ageing. These includegenitourinary problems such asprostatic hypertrophy, prostatic cancerand renal stone disease, and arterialdisease for which haemophilia is notprotective. Progressive arthritis anddeclining fitness may lead to loss of independence which causes great concern.

Associated with the physical aspectsof ageing, many patients also sufferfrom psychological symptoms whichmay be precipitated by changes inwork such as early retirement andaltered family dynamics.

Many older men with haemophiliamay never have consulted primarycare physicians because of the rarityand complexity of their disorder.Haemophilia centre staff oftenassume responsibility for theidentification and management of allhealth problems of their patients.Even when other clinicians areinvolved, patients require theircentre's involvement in theinvestigation and support of manyprocedures such as coronary arterysurgery and urological surgery.

Advances in haemophilia care;beginning in the 1950s led to aworldwide movement to concentratehaemophilia care in designatedtreatment centres. Initially,haemophilia treatment focusedprincipally on the musculoskeletalmanifestations of the disease, whichrequired expertise in Physiotherapy,Rheumatology and OrthopaedicSurgery available throughcomprehensive treatment centres.New biological therapies to managehaemophilia promised huge successand a normal life expectancy formany patients until the enormoustragedy of contamination of theblood supply with HIV and hepatitisC in the 1980s and 1990s. Duringthat period, haemophilia care oftenconcentrated on the treatment ofthese dreaded complications. Withthe advent of recombinantcoagulation concentrates, the issuesof inhibitor formation, optimaldosage and duration of factorsupport and treatment of an ageingcohort of patients with haemophiliahave gained importance.

The new challenge is how to provideexcellent care to an ageingpopulation, including women, withhereditary bleeding disorders. Withage, people with haemophiliadevelop medical and surgical diseasepreviously not seen in this group. Thecare of older people withhaemophilia may require input fromservices inexperienced in themanagement of patients withcongenital coagulation disorders,such as Cardiology, CardiovascularSurgery, Urology and Oncology.

Management requires early planningwith all members of an extendedhaemophilia team with particularemphasis on preparinginexperienced hospital units for thecare of these unique patients.

Diseases such as osteoporosis,hypertension and diabetes are seenwith increasing frequency as thepatient cohort ages. Lifestyle issuesand common screening programsneed to be included in themanagement of many older patientsattending haemophilia treatmentcentres with referrals and guidanceto appropriate specialties.

Balance dysfunction and fallsbecomes increasingly common withage in the non-haemophiliapopulation. Many falls also cause aloss of confidence in mobility, whichoften results in activity curtailment,and subsequent deconditioning andfurther risk of falls. Recent researchhas shown that identifying andmanaging risk factors for falls amongolder people can be effective inreducing falls rates. Exerciseinterventions, particularly those witha balance-training component, havebeen shown to be effective inreducing falls in this high-risk group.

Haemophilia is associated withbleeding episodes, which occuroften internally into the joints ormuscles. Clinical staff at the RonaldSawers Haemophilia Centre at TheAlfred Hospital, Melbourne, recentlyidentified that falls appear to be acommon problem for their clientswith haemophilia and other bleedingdisorders. Falls in this client groupwere considered particularlyimportant because of the high risk ofserious bleeding associated withinjuries, potential impact on themusculoskeletal system and function,and costs associated with treatmentfor these fall-related injuries andconsequences. However, there hasbeen virtually no research to dateevaluating falls and balancedysfunction in people withhaemophilia and other bleeding

Haemophilia and Ageing

The bleeding disorders’ community is becoming increasingly concerned about ageing. Several sessions at the recentWFH Congress addressed ageing and related topics. Clearly, in Australia we are faced with an ageing bleedingdisorders’ community and our approach to services and care needs to adapt to reflect need. HFA will explore how wemight address the needs of our members and will work with State/Territory Foundations and special interest healthprofessional groups to ensure we respond appropriately. Haemophilia Foundation Australia has funded the authorsbelow to undertake research to evaluate balance dysfunction and falls in people with haemophilia (aged >30 years), andto determine the feasibility of a home exercise program targeting balance training for this group.

The following piece has been extracted from an article by A Street, K Hill, B Sussex, M Warner and MF Scully publishedin the State of the Art book, XXVII International Congress of the WFH, Haemophilia journal, July 2006, Vol 12,Supplement 3, pages 8-12. The full text with references is available from HFA.

NATIONAL HAEMOPHILIA is a publication of Haemophilia Foundation Australia. Every effort is taken to ensure accurate and relevant content,

however opinions expressed in NATIONAL HAEMOPHILIA do not necessarily reflect those of the Foundation or the editor, nor is any information

intended to take the place of advice from a qualified medical practitioner or health professional.

We welcome reproduction of articles or quotations from NATIONAL HAEMOPHILIA on the understanding that acknowledgement is made of

NATIONAL HAEMOPHILIA as the source.

Haemophilia Foundation Australia acknowledges the funding and assistance received from the Commonwealth Department of Health and Ageing

which makes this publication possible.

Corporate

PartnersHaemophilia FoundationAustralia (HFA) values theindividuals, Trusts andCorporations who donatefunds to support ourobjectives.

Among our valued donorsare our current CorporatePartners who provideunrestricted grants to HFAto support our programs:

CalendarHepatitis C Awareness WeekAustralia 1-7 October 2006ph 03 9385 9107email heather@hepcvic.org.au

Haemophilia Awareness WeekAustralia 8-14 October 2006ph 03 9885 7800fax 03 9885 1800email hfaust@haemophilia.org.au

Australasian Sexual HealthConferenceMelbourne 9-11 October 2006ph 02 8204 0770fax 02 9212 4670conferenceinfo@ashm.org.auwww.sexualhealth2006.com.au

18th Annual ASHM ConferenceMelbourne 11-14 October 2006ph 02 8204 0770 fax 02 9212 4670conferenceinfo@ashm.org.auwww.ashm.org.au/conference

Youth Leadership and Mentoring ProgramLaunceston, 12-15 October 2006ph 03 9885 7800fax 03 98851800hfaust@haemophilia.org.au

14th National Symposium onHepatitis B and CMelbourne 18 November 2006eleanor.belot@svhm.org.au

14th Australian and New ZealandHaemophilia ConferenceCanberra 4-7 October 2007ph 03 9885 7800fax 03 98851800hfaust@haemophilia.org.au

World AIDS Day1 December 2006www.worldaidsday.org.au

Hemophilia 2008Istanbul, Turkey, 1-5 June 2008www.wfh.org

disorders other than investigationsaround bone strength in children withhaemophilia, and potential benefitsand harms associated with exercise(primarily strength and fitnessexercise, not on balance training).

In the Melbourne study results from acomprehensive assessment batteryhave been used as a basis for a homeexercise program prescribed by aphysiotherapist, based on thesuccessful Otago program (targetingfunctional balance and strengthtraining to address problems identifiedon the assessment). This has beenshown to be successful in improvingbalance, strength, and significantlyreducing falls among older people.Results may indicate the need for agreater focus on preventative balance-training exercise programs for personswith haemophilia.

Hereditary bleeding disorders arerare lifelong chronic illnesses, whichrequire episodic treatment, makingit very difficult to develop anevidence-based clinical trialsapproach to therapy. Developing anevidence-based approach to themanagement of patients withhereditary bleeding disorders asthey age will be a challenge.

The publication goes on to considerhaemophilia and atherosclerosis,increasing age and the risk factors forcardiovascular disease in people withhaemophilia. Management ofcardiovascular interventions in thosewith haemophilia remains challengingand requires a team approach. Anumber of successful approacheshave been described for angiograms,angioplasty, bypass surgery andvalvular surgery. Haemophilia isassociated with an increased risk ofhypertension. This is, in part, relatedto the increased risk of renal disease.

As haemostasis is central to theevolution and treatment ofcardiovascular disease, it is to beexpected that disorders ofhaemostasis, such as haemophilia, willhave a major impact on morbidity andmortality among other risks.