hfact newsletter september 2014 · a few photos of the events are in-cluded here. midwinter dinner...

September 2014

PATRON Dr Richard Pembrey AM, MB BS, MD, FRACP, FRCPA

NEWSLETTER

HAEMOPHILIA FOUNDATION AUSTRALIAN CAPITAL TERRITORY

Haemophilia Treatment

Centre Has New Premises It was good to see the opening of the new Canberra Region Cancer Centre in August. This multi-million dollar facility was jointly funded by Federal and Territory government as part of the ACT Government’s Health Infrastructure Program. What is our interest in this facility? The Haemophilia Treatment Centre is now located on the 4th floor of

this new building. The building is very open and the main foyer has lots of natural light. The 2nd floor includes a Patient and Family Lounge with access to online infor-mation and other resources.

An open day was held on the 9th of August and HFACT was invited to have a stall with some of our bro-chures and posters for people to see.

A few photos of the events are in-

cluded here.

Midwinter dinner The HFACT midwinter dinner, after the CRCC open day, at Ginseng Res-taurant was a success with 18 peo-

(Continued on page 2)

President’s Update

Contents Page

President’s Update ............................. 1

New HTC Premises ........................ 1

Mid-Winter Dinner ........................ 1

2014 World Hepatitis Day ............. 2

Membership Subscriptions 2014/15 ... 3

Women’s Wisdom .............................. 3

Dates for Your Diary ........................... 3

Haemophilia Awareness Week and Red Cake Day ........................ 4

HFNSW Family Camp .......................... 4

Outreach Visit to Boorowa ................. 5

Men’s Business ................................... 5

World Congress Reports ..................... 6

Stronger Together ......................... 6

Exercise & Rehabilitation............... 7

Eliminating the Gap ....................... 7

Low Dose Prophylaxis Protocols in Emerging Nations ................ 8

Growing up with Haemophilia ....... 10

HFACT display at the CRCC open day on 9th August ACT Senator Zed Seselja, ACT Chief Minister Katy Gallagher and Prof Robin Stuart-Harris, Clinical Director, Canberra

Region Cancer Service at the opening of the CRCC

September 2014


President’s Update (cont)

ple attending, including Dr Pati and Nurse James Slade. It was a very

pleasant evening with lots of op-portunities for people to chat about the events of this year and the lat-est developments with the treat-ment service provided at the Can-berra Hospital.

2014 World Hepatitis Day

(28 July) update A few of us attended a lunch-time update on the situation concerning Hepatitis in Australia on 2014 World Hepatitis Day.

The following points are quoted from the presentation and materi-als delivered by John Didlick, Execu-tive Officer, Hepatitis ACT:

“We learned that things are already serious:

Almost half a million Australians are living with hepatitis B & C.

15,000 are newly diagnosed eve-ry year.

Nearly 1,000 Australians die each year from hepatitis B & C.

Due simply to their age and viral hepatitis infection, more than 50% of Australians with hepatitis B & C have reached the ‘liver danger zone’ – an age point at which they devel-op an elevated and ongoing risk of serious liver disease.

In order to avert a liver disease cri-sis, regular liver check-ups must be part of the standard of care for all people living with chronic hepatitis B & C:

Fast, simple and minimally inva-sive tests now exist, such as blood tests, Fibroscan®, and ul-trasound scans, to assess the level of damage to the liver;

Doctors recommend that regular liver check-ups should be carried out every three, six or twelve months depending on the stage of liver disease;

Regular check-ups allow people with viral hepatitis to be in con-trol of their liver health and facil-itate discussion with healthcare professionals about treatment options and how to slow down the progression of liver damage.

We learned that new treatments for hepatitis C, whilst critically im-portant, won’t fix everything.

Affected communities and clinicians alike are understandably excited about the prospect of new treat-ments for hepatitis C. New treat-ments, not yet available in Austral-ia, are more effective, with signifi-cantly shorter treatment duration, and fewer side effects than current-ly available treatments. They are also incredibly expensive and access to them may be restricted. [On Fri-day 22 August 2014, the Pharma-ceutical Benefits Advisory Com-mittee (PBAC) rejected an applica-tion to list the drug sofosbuvir on the basis of cost.]

Health experts are warning against romantic notions that these new cures make redundant evidence-based prevention strategies that have proven effective already. Yes, absolutely, let’s treat people living with hepatitis C. Everybody living with hepatitis C deserves the chance of a cure. Let’s not be confused into believing that curing infection is better than preventing it. We need to do both.

We learned that affected communi-ties are dissatisfied:

Left untreated, one in four peo-ple with hepatitis B will die from it, yet nationally we’re treating only 5% of the affected commu-nity with some 87% not engaged in care

Whilst an estimated 80% of Aus-tralia’s hepatitis C is diagnosed, and antiviral treatments are curative, only 1% of the affected population is treated each year

Viral hepatitis and the people whose lives are affected are di-minished when published litera-ture omits them or relegates them to an after-thought.”

(Continued from page 1)


September 2014


Membership Subscriptions 2014/15

Membership of the Haemophilia Foundation ACT Inc is available to all people who are affected by hae-mophilia or related bleeding disor-ders, their families and carers. An-nual membership of the HFACT is a modest $5 for a single person or $10 for a family.

Membership forms for new mem-bers can be obtained by contacting a committee member or can be downloaded from our website www.hfact.org.au. This year HFACT has posted out invoices to existing

members. If you have not received an invoice, membership may be paid by posting a cheque for the corresponding amount along with your details to: PO Box 331 Mawson ACT 2607

If you prefer to pay via the internet then you can do so. Our bank de-tails are:

Bank: Westpac Bank BSB number: 032 778 Account number: 440919

Account name: Haemophilia Foundation ACT Inc. If you choose to pay using the inter-net option then it would be appreci-ated if you could notify us by post or email to [email protected]

A receipt will be issued once the monthly bank statement has been processed.

The Haemophilia Foundation ACT Inc is a registered charity and dona-tions of $2 or more are tax deducti-ble.

Women’s Wisdom

After a quiet winter it is time for us to get together again. Rebecca Minty has kindly offered her house for our next meeting and Dr Nalini Pati from the Canberra Hospital is our guest speaker.

Dr Pati will talk about the hospital, changes made, future plans and

haemophilia treatment in general, and, give us the opportunity to ask questions. I hope to see you there.

The details are: Date: Sunday 19th October Time: 2:00pm – 4:00pm Address: 75 Hawkesbury Cr, Farrer

Please RSVP to Jenny on 0410 543 086 or [email protected] by Friday 17th October.

Jenny Lees

A copy of the special report pre-pared for 2014 can be found at:

http://www.liverdangerzone.com.au/s/Liver-Checkups-Report.pdf

Comment While the above points are relevant to the Australian situation there are some important lessons for mem-

bers of the haemophilia community who are infected with hepatitis C. The main lesson is that regular liver check-ups should be part of the standard of care. Future drugs also hold out some hope for a better outcome for those who are able to access them.

For more information about viral hepatitis, new treatments, and sup-port options you may contact:

Hepatitis ACT 36 David St, Turner (Opposite the O’Connor Shops)

Phone : (02) 6230 6344

Email : [email protected]

Fred Wensing

President HFACT


Dates For Your Diary

Thursday 25th September 7.30pm HFACT Annual General Meeting

Sunday 12th - Saturday 18th October Haemophilia Awareness Week

Wednesday 15th October Red Cake Day stall at Canberra Hospital

Sunday 19th October, 2.00pm Women’s Wisdom - Guest speaker

Monday 20th October HFNSW Family Camp applications due

Saturday 1st November, 3.30pm Men’s Business - Croquet at Canberra Croquet Club

Friday 14th—Sunday 16th November HFNSW Family Camp

http://www.hfact.org.au/



mailto:[email protected]

September 2014


Haemophilia Awareness Week is an opportunity for Haemophilia Foundations and other organisations, as well as individuals and families, to take part in a cam-paign and activities to raise awareness about haemo-philia, von Willebrand disorder and related inherited bleeding disorders throughout Australia during the week of 12-18 October 2014. During Haemophilia Awareness Week the Canberra Hospital Haemophilia Treatment Centre and HFACT will be holding various activities at the Canberra Hospital.

James Slade is organising education sessions and in-servicing for staff during the week and we hope to launch the medication administration standing orders for the Emergency Department.

James is also organising Red Cake Day events on the Wednesday of that week. Red Cake Day is a part of Hae-mophilia Awareness Week nationally in which cake stalls or social events sell cakes decorated red to focus awareness on bleeding disorders and raise funds for the foundation. Further information can be found at www.haemophilia.org.au/red-cake-day or on Facebook at www.facebook.com/RedCakeDay

The Zouki Cafe at the hospital have agreed to sell red iced cakes during the week while hosting donation tins at their various locations for HFACT. As previously men-tioned, on Wednesday 15th October there will be two cake stalls at the hospital - one in the CRCC building foyer and the second in the main hospital foyer. All profits from the stalls will go to the foundation. To make this event a success we need volunteers to both donate the red cakes and assist with running the stalls.

If you can assist in either capacity, please contact James Slade on 0481013323 or email [email protected].

If you can't volunteer you can still contribute by making a donation at www.haemophilia.org.au/redcakeday/donate-now. Alternatively you can organize a Red Cake tea at your workplace and register it online with HFA.

James Slade

Haemophilia Nurse and Bleeding Disorders Co-ordinator

Haemophilia Awareness Week

Haemophilia Foundation NSW 2014 Family Camp

This is a reminder that the Haemophilia Foundation NSW 2014 Family Camp will be held from Friday 14th to Sunday 16th November at the Academy of Sport and Recreation Centre at Narrabeen.

The week-end is a great opportunity to catch up with other families and friends from New South Wales and ACT. It is designed to suit the whole family. The pro-gramme will include educational sessions led by staff from NSW Haemophilia Treatment Centres, as well as indoor and outdoor activities and a special Saturday evening!

The Family Camp is free of charge for our members.

If you or your family members are interested in attend-ing the family camp, you will need to complete an ap-plication form and return it to Haemophilia Foundation NSW by Monday 20th October. Application forms can be obtained by contacting either our counsellor Clare Reeves on 0409 830 472 or [email protected] or our Secretary, Jenny Lees on 0410 543 086 or [email protected]

http://www.haemophilia.org.au/red-cake-day

September 2014


Outreach Visit to Boorowa

The Canberra Hospital Haemophilia Treatment Centre recently received a grant from Novo-Nordisk to un-dertake a rural outreach pro-gramme. It is envisaged that the outreach programme will initially cover two rural areas within the Canberra region. Although other Australian states have rural out-reach programmes, this is the first time for the ACT.

The aim is to facilitate the provision of appropriate care and treatment for people with bleeding disorders in the outreach areas. An important part of the programme is to give information about the services available through the Canberra Hos-pital, as it is a designated haemo-philia treatment centre.

On Wednesday 27th August a dele-gation from Canberra visited Booro-wa in NSW to meet with families, staff of Boorowa District Hospital, pre-school staff, school staff and other interested people. The dele-gation comprised Dr Nalini Pati (Consultant Paediatric Haematolo-gist), Mr James Slade (haemophilia nurse and Bleeding Disorders Co-ordinator), Ms Juliana Dias (physiotherapist), Ms Katie Schu-mack (data manager) from the Can-

berra HTC team, along with our counsellor Ms Clare Reeves and Mrs Maria Wensing, our Vice-President but more importantly, as a mother of two sons with haemophilia.

An introduction and information session was held in the evening at the Boorowa RSL. About 30 people in all attended and the session was well received. Providing infor-mation to the Boorowa community enables both patients and their car-

ers to be more aware of the options available to them when deciding on treatment.

ACT Delegation to Boorowa: L-R: Dr Nalini Pati, Katie Schumack, James Slade, Clare Reeves, Maria Wensing and Juliana Dias

Men’s Business

The next installment of Men’s Busi-ness will see gentlemen trying their hand at croquet! This is an ancient and noble sport (or so I’ve been told) and suitable for all “fitness” levels. For those unfamiliar with croquet, simply consult Google. We have lawns booked at the Can-berra Croquet Club (adjacent to the Hyatt) from 3:30-4:30pm on Satur-day 1st November. Cost is $10 per

person, which includes nibbles and drinks.

As parking at the Canberra Croquet Club is often limited, the fewer cars the better. If you would like a lift to get there, please advise me. Also, if there are people willing to help out with car-pooling, please also advise me.

Please RSVP to Claude by 24th Oc-tober on 0412 839 135. And if it’s raining or looks particularly threat-ening on the day, contact Claude. Please note: flat-soled shoes are required to be worn on the croquet lawns. Look forward to seeing you there!

Claude

September 2014


World Congress Reports

The remainder of the articles in this edition of the HFACT newsletter are World Hemophilia Foundation 2014 World Congress reports that we did not have room to print in our June Newsletter. Most of these articles

focus on the status of haemophilia care in other countries, highlighting how lucky we are in Australia.

There were many, many sessions that we could not bring to you.

However, more information can be found at www.wfh.org including abstracts and in some instances full transcripts or videos of sessions.

This session left a memorable im-pression on me, as it summarised pretty much the whole congress.

The community around haemophil-ia is unique in the sense that it is made up of a large team of medical professionals, and perhaps a larger one of lay people.

The first presenter, Pamela Wilton from Canada, explained that the close collaboration between medi-cal professionals and non-professionals is a very powerful in-fluence on governments to obtain the right amount of care for people with haemophilia (PWH).

The second presenter was awarded the Advocacy Recognition Award. Bradley Rayner, from the South Af-rican Haemophilia Foundation, in Johannesburg, explained briefly and succinctly how to establish a ‘MASAC’ or Medical and Scientific Advisory Committee. The success Bradley has had in his country was not quick, as it has been established after an enormous amount of work and time from all involved. He ex-plained, the best way to establish a MASAC is to treat it as a project. Being a project manager himself he said this is the best approach to en-sure milestones are achieved. He also advised never turn down any

offer of time, no matter how small. This MASAC faced great cultural challenges and overcame them with tolerance and compassion.

This presentation linked nicely with another one on what the current status of the ‘demand’ for care is at present. In the ‘old’ days (50 years ago) patients were at the doctors’ mercy. Currently, patients are well informed and empowered to take better care of themselves and at the same time, demand a high level of care and work to ensure care is the same for all PWH around the world.

The third presenter, Arafat Awajan, from the Jordan Thalassemia and Hemophilia Society in Amman, gave an insight into the challenges that PWH face in Jordan. In this country there is a Haemophilia Committee already established, made up from volunteers, family members and PWH. With the help of the WFH an Advocacy in Action programme was put in place and that benefited a lot of PWH in regional areas. At the end of the project, the government of Jordan agreed to create a Nation-al Register for PWH and guidelines for care were published.

The last presenter, Mohammad Nu-rul Islam, from the Hemophilia Soci-

ety of Bangladesh, was very inspir-ing because being the dad of a boy with haemophilia, he went the ex-tra mile for his son and his efforts benefited a lot of affected men in Bangladesh. With the support of WFH, an outreach campaign was put in place.

The most challenging issue for Mo-hammad was to raise awareness of haemophilia amongst health profes-sionals and to show them the need for treatment in regional areas. The large number of PWH in country towns was creating a problem for a major hospital in Dhaka.

By travelling on his own means to bring proof to health authorities, Mohammad was heard and two positions were created in Chitta-gong and Jessore. He identified these two places in need, did all the planning, identified the amount of care needed and proposed the cre-ation of the positions. He did all of this by himself. When faced with health authorities, he used an emo-tional approach to convince them of the need for help in these areas. After listening to his inspiring story, I learned that all is fair in love and war…and haemophilia!

Martha Soria

Stronger Together: Medical and Lay Collaboration

http://www.wfh.org

September 2014


I admit, I initially missed the point of the “Crossfire” aspect of the title of this session – I went in wonder-ing what I had missed in regard to the argument for no need for exer-cise post bleed. As it turns out, not much (thank goodness).

The session was intended to pre-sent two extreme points of view but the Canadian physiotherapist, Karen Mulder, who presented the “no need” case acknowledged very ear-ly on in her presentation that there was not much to sustain this argu-ment.

The case for always undertaking exercise and rehabilitation post bleed noted the medical benefits of

undertaking exercise under guid-ance from a physiotherapist. Failure to exercise or extended immobilisa-tion decreases strength which re-sults in weakened joints, puts pres-sure on other joints, adversely im-pacting multiple joints in the body and leading to a cycle of multiple joint bleeds.

Separately during the congress, I attended sessions delivering similar messages. An excellent presenta-tion by Andrew Selvaggi (Australia) on how he improved his health and thus general wellbeing, through exercise and healthy eating. A young man from Malaysia who also provided an excellent presentation on how he differentiates between

arthritic and joint bleed pain, and how he uses swimming to manage the pain.

The information contained in these sessions was reinforced upon our return to Canberra when we met for the first time Juliana (the Can-berra hospital physiotherapist) who designed a series of exercises for our son’s ankles post a minor sprain. Having attended the con-gress we were able to reinforce to our son the importance of comply-ing with the exercises by relaying the stories heard and information we received at the World Congress.

Sarah Collins

Crossfire session: Exercise and rehabilitation – always vs no need?

Eliminating the Gap - Report from World Federation Congress

This was one of the most challeng-ing sessions of the Congress be-cause it highlighted the significant differences between developed and developing countries in the man-agement of haemophilia.

At this time around 80% of haemo-philia patients in the world have no access to care. Many developing countries have very low supplies of clotting factor because government funding has other priorities.

The differences can also be geo-graphic (e.g. distance), financial, psychosocial (e.g. what is health), cultural, or socio-historical (e.g. mis-trust of government). There is a significant difference in the level of government support, with devel-oped countries being fully-funded whereas developing countries have

limited or no government funding for haemophilia treatment. To add to this the demands on health sys-tems are increasing due to ageing populations, increased rates of chronic disease, new treatments and rising health care costs.

The World Federation of Hemophil-ia has a number of programs which are aimed at addressing these differences. Two of them are:

Global Alliance for Progress (GAP) Program

The Cornerstone Initiative

Information on these programs can be found on the WFH website (www.wfh.org)

On the other hand, some countries like India are making good progress in increasing awareness and em-

powering patients to lobby for better access to treatments.

It was important for the Congress to highlight the ‘gap’ with a session like this. It certainly makes any Aus-tralian issues look small by compari-son.

The HFACT is pleased to have been able to sponsor attendance at the Congress of a young man from My-anmar (see previous newsletter). He was able to share his concerns about the situation in his country with others who attended. We hope that the situation in his coun-try will improve over time.

Fred

http://www.wfh.org/en/page.aspx?pid=792

September 2014


Introduction There are two main methods of clotting factor concentrate (CFC) replacement therapy: 1) regular replacement therapy, known as prophylaxis, and 2) episodic thera-py, known as on demand treat-ment.

Multiple studies world wide have shown that using prophylaxis re-sults in reduced bleeding rates, better joint outcomes and better quality of life. However, prophylax-is is expensive and has not been practical in emerging nations that have a limited supply of CFC. A re-cent retrospective multi-centre study1 compared prophylaxis in the Netherlands and Sweden since the 1970’s to study the difference in outcomes between intermediate-dose and high-dose regimes. One outcome of the study was the ob-servation that effective prophylaxis is possible with CFC usage of around 2000 IU per kg/yr.

Even 2000 IU per kg/yr is more than many nations have, so the question arises how CFC therapy should be administered when only 1000-2000IU per kg/yr is available.

Three speakers addressed this question by describing to the dele-gates what protocols they use in their respective countries.

Their protocols ranged from prima-ry to tertiary prophylaxis types. A full explanation of the different types of prophylaxis can be found on the WFH web site

http://www.wfh.org/en/abd/prophylaxis/types-of-prophylaxis )

Algeria

Yasmina Berkouk-Redjimi MD, from the University Hospital Beni messous, Algiers described the treatment in Algeria.

Algeria has 1843 identified haemo-philia patients half of whom are severe. Algeria has CFC supply of 2 IU per capita (total population). Pri-mary prophylaxis was started for some young patients in 2008. An evaluation of 54 haemophilia A pa-tients in 2013 showed:

Protocols ranged from high-dose (35-45 IU/kg, 2 or 3 times a week) to low-dose (15 IU/kg, 2 times a week).

The low dose prophylaxis con-sumption was very similar to that of on demand treatment.

Comparing 10 patients on low dose protocol with 10 patients using on demand treatment, showed that the prophylaxis pa-tients had a total 43 bleeding episodes between them over a year, while the on demand pa-tients had a total 216 bleeding episodes over a year.

The bleeding episodes com-prised 23 haematoma and 15 haemarthrosis in the prophylaxis group versus 39 haematoma and 148 haemarthrosis in the on de-mand group.

Overall the comparison showed the prophylaxis group had an average number of bleeding epi-sodes of 4.3 p.a. vs 21.4 p.a. for the on demand treatment group.

The prophylaxis group had sig-nificantly better joint scores us-

ing the Gilbert and Pettersson metrics.

Only 20% of the prophylaxis group had a target joint, while all the on demand treatment pa-tients had a least one target joint.

The conclusion was that prophylaxis with a low dose led to significantly better outcomes than on demand treatment with a similar annual us-age of expensive CFC.

Tunisia Emna Gouider from the Hemophilia Treatment Center at the Aziza Oth-mana Hospital in Tunis described the situation in Tunisia.

Tunisia is a country of 11 million people and they have identified 360 haemophilia patients through the Tunisian Hemophilia Association.

In Tunisia they started using low dose prophylaxis in 2007. At that time supply and use of CFC was only 0.27 IU per capita. They now have 75 haemophilia patients on low dose prophylaxis, including 45 chil-dren under 14 years old.

Prophylaxis in the children ranges from 20 to 40 IU/kg/week, adminis-tered as one, two or three infusions weekly.

The children on prophylaxis had an obvious reduction in bleeding epi-sodes with a total of 460 bleeds per annum before prophylaxis versus 42 after prophylaxis.

In Tunisia they also measured He-mophilia Joint Heath Scores (HJHS) and Functional Independence Score in Hemophilia (FISH), both of which showed good to excellent results for the prophylaxis group.


Low Dose Prophylaxis Protocols in Emerging Nations

1 Intermediate-dose versus high-dose

prophylaxis for severe haemophilia:

comparing outcome and cost since the

1970’s, K. Fischer, K. Steen Carlsson, P.

Petrini, M. Holmström, R. Ljung, H. Ma-

rijke van den Berg, E. Berntorp Blood 2013 122:1129-1136

http://www.wfh.org/en/abd/prophylaxis/types-of-prophylaxis

http://www.wfh.org/en/abd/prophylaxis/types-of-prophylaxis

http://www.bloodjournal.org/content/122/7/1129

http://www.bloodjournal.org/content/122/7/1129

September 2014


Low Dose Prophylaxis Protocols in Emerging Nations (cont.)

Following the positive results in children, prophylaxis was then ex-tended to adults who have frequent bleeding episodes. Doses varied from 10 to 30 IU/kg/week, adminis-tered one or two infusions weekly. Bleeding episodes reduced from a total of 317 annual bleeding epi-sodes to 104 after prophylaxis.

The conclusion was that low dose prophylaxis is essential for improve-ment of treatment, quality of life and reducing arthropathy. The in-troduction of low dose prophylaxis in Tunisia resulted in CFC use of 0.99IU per capita.

Morocco Mohamed El Khorassani of the he-mophilia treatment centre at the Children’s Hospital, University Hos-pital in Rabat described the situa-tion in Morocco.

Morocco has a population of 30 million, with about 1,100 diagnosed haemophilia patients. About 60% of the teen and adult patients have haemophilic arthropathy.

Prophylaxis has become the stand-ard treatment for children with hae-mophilia in Morocco. However, in 2006 their centre decided to intro-duce tertiary prophylaxis to patients with target joints to clarify the place of long term prophylaxis.

Morocco gradually introduced prophylaxis with dose escalation with the objective to reduce spon-taneous bleeds, reduce and stabilise joint damage and improve quality of life.

In the trial the maximum target dose was 50 IU/kg once a week. The doses were adjusted according to

results, usually reduced. They evalu-ated the number of haemarthrosis and the quality of life, using the HJHS and FISH metrics for joints and independence. They also measured the total CFC consumption per pa-tient and from that calculated the total CFC cost and compared it to the theoretical cost of equivalent on demand treatment.

Before prophylaxis almost all pa-tients had advanced arthropathy and at least 2-5 bleeds per month. After prophylaxis bleeding has de-creased to 1-2 incidents every two months.

However, while successful, some patients have stopped prophylaxis due to lack of CFC or lack of access to a treatment centre.

Overall the results were:

Average CFC dose was 30 IU/kg, with a range of 15 to 50 IU/kg/week.

The average annual CFC use per patient is 1,757 IU/kg with a range 1,300 IU to 2,700 IU.

The annual cost of CFC is be-tween $35,000 and $150,000.

The theoretical cost of on de-mand treatment for those pa-tients was $70,000 to $300,000.

Prophylaxis in these patients represents a 50% reduction on the theoretical cost of similar on demand treatment.

Their conclusion was that tertiary prophylaxis decreases bleeding, improves symptoms and protects the joints. It’s indicated in patients with arthropathy and frequent hae-marthrosis. The dose can be adapted per person and averages 30 IU/kg/week.

Summary I took away two major points from the session, relevant to the Australi-an experience:

I have always been told that prophylaxis, especially tertiary prophylaxis, consumes high amounts of CFC and is therefore very expensive compared to on demand treatment. The three emerging nations described here are not necessarily using the same doses used in Sweden or the Netherlands. Nevertheless, it is interesting to see that chang-ing the treatment protocol from on demand to prophylaxis with-out using extra CFC has pro-duced such beneficial results.

Having formulated a treatment plan involving prophylaxis, it’s important to measure the re-sults of the treatment plan and, in consultation with your HTC, adjust it accordingly. This under-lines the importance of data re-cording with tools like MyABDR and also measurement of joint health, functional independence and QOL using standard interna-tional haemophilia metrics.

Ron Lees


September 2014


HAEMOPHILIA FOUNDATION AUSTRALIAN CAPITAL

TERRITORY

PO Box 331 MAWSON ACT 2607

This newsletter is printed on 100% recycled paper, carbon neutral if possible. Contact us if you would prefer to save paper and postage by receiving the

electronic (full colour!) version via email.

Growing Up with Haemophilia

The session about growing up with haemophilia discussed the impact on people with bleeding disorders, their families and carers. Here is the perspective of one of our members who grew up in the days prior to factor replacement - Ed.

Like many other sessions at the 2014 World Congress, it confirmed many of the thoughts and practices that I have had and followed throughout my life living with hae-mophilia. The impact and effect that the occurrence of the condition had on parents who previously were unaware of it was enormous, especially so in the days before fac-tor replacement was a reality. Even the effects on parents, who were aware of it being in their lineage,

were substantial in those days (prior to the availability of factor replacement).

The emotional and psychological impact on siblings was considerable as very often the focus of parental attention was on the child with hae-mophilia. A two, three, four year old etc sibling did and does not have the maturity to understand and comprehend what's going on: why he or she is not receiving as much "love" and attention as their sibling with haemophilia. This was brought home to me only quite re-cently in conversation with one of my siblings. For over 60 years I was unaware that she was resentful of the attention I received which she did not get.

As for the people with haemophilia, there was little else that he could do in those days but hopefully grin and bear the pain and discomfort of a bleed: there was no factor re-placement and no pain relief as there is today. And there was the guilt and frustration of the parents who had "caused' this situation to occur.

Today, the situation is not fraught with those problems but there is still the challenge to parents who have a son with haemophilia and one without haemophilia: striking a balance in dealing with the traumas that inevitably will arise.

Haemophilia Contact Numbers

Canberra Hospital Switchboard: 6244 2222 www.canberrahospital.act.gov.au

Haemophilia Treatment Centre: 0481 013 323 (Mon to Fri 9am - 5pm) Via email: [email protected]

Physiotherapy: 6244 2154

Paediatric Day Stay Centre 6174 7372

Haemophilia Foundation ACT President: 6291 7920 or 0409 831 844 [email protected] Counsellor: 0409 830 472 [email protected] Secretary: [email protected] Web site: www.hfact.org.au

Haemophilia Foundation Australia Office: (03) 9885 7800 or 1800 807 173 Web site: www.haemophilia.org.au

Our Mission

“To improve the wellbeing of the haemophilia community

through mutual support, networking, advocacy and striving for

optimal health care.”

Acknowledgement

Haemophilia Foundation ACT would like to acknowledge that our news-letter has been produced through

funds provided by ACT Health.

http://www.canberrahospital.act.gov.au





http://www.hfact.org.au/

http://www.haemophilia.org.au

hfact newsletter september 2014 · a few photos of the events are in-cluded here. midwinter dinner...

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