The new CHS program for women with inherited bleeding disorders

Hem philiaTodayNovember 2011Vol 46 No 3CanadianHemophiliaSocietyServing theBleeding DisorderCommunityThe newCHS programfor womenwithinheritedbleedingdisorderswww.coderougewomen.caHemophilia Today November 2011 | 1

The BeneFACTORs ClubThe Canadian Hemophilia Society (CHS)relies on the generosity of our donorsVISIONARIESto fulfill our mission and vision.We are fortunate to count on a groupof exceptional donors who havecommitted to making an annualinvestment to support the CHSINNOVATORand its core programming needs.To recognize this special groupof donors we have created theBeneFACTORs Club, the CHS’ highestphilanthropic recognition, whichBUILDERSsymbolizes the critical bond betweenour organization, the donor and everyperson we serve with an inheritedbleeding disorder. Corporations thatmake annual gifts of $10,000 or moreto support our organization and its coreBELIEVERSprogramming needs are recognized asmembers of the BeneFACTORs Club.The Canadian Hemophilia Societyacknowledges their tremendous effort.Grand Benefactor – multi-year commitment2 | Hemophilia Today November 2011

INTHISISSUE4 WORD FROM THE EDITOR5 MESSAGE FROM THE PRESIDENT6 COMMUNITY NEWS6 Chapter Spotlight9 2011 CHS James Kreppner MemorialScholarship and Bursary Program10 Meeting with med students at theUniversity of Calgary10 Upcoming Events11 THE SAGE PAGEHemophilia and Aging12 FOCUS ON RESEARCH12 The HRMDC report13 In Gratitude and Commemoration1984-201014 CHS Dream of a Cure Research Program16 Care Until Cure17 Hemostasis Fellowship Program18 InterviewsParticipating in a clinical trial20 FUNDRAISINGCorporate Philanthropy Program21 THE BLOOD FACTOR21 Health Canada approves Wilate forVWD indication22 VOLUNTEER FILECommitted to volunteer23 YOUTH FILEIt is now up to you to advocate for your care!24 A GLOBAL PERSPECTIVETwinning: A tremendousopportunity for sharing25 MEDICAL NEWS25 Hepatitis and HIV Press Review26 New HCV drugs approved in Canada27 Pregnancy planning, HIV testing andpartner notification28 Social Workers Face-to-Face29 The Physio Corner30 THE FEMALE FACTORThe new CHS program for womenwith inherited bleeding disorders31 The 1st Canadian Conference onBleeding Disorders in Women32 OUR STORIESSurvival and triumph by Karen FaheyHemophilia TodayNovember 2011 • VOL 46 • No 3400-1255 University StreetMontreal, Quebec H3B 3B6Phone: 514-848-0503Toll-free: 1-800-668-2686Fax: 514-848-9661chs@hemophilia.cawww.hemophilia.caHemophilia Today is the official publication of the Canadian Hemophilia Society (CHS) and appearsthree times yearly.The Canadian Hemophilia Society is committed to improve the health and quality of life of all peoplewith inherited bleeding disorders and ultimately to find a cure. Its vision is a world free from the painand suffering of inherited bleeding disorders. The purpose of Hemophilia Today is to inform thehemophilia and bleeding disorders community about current news and relevant issues. Publicationsand speakers may freely use the information contained herein, provided a credit line including thevolume number of the issue is given.Opinions expressed are those of the writers and do not necessarily reflect the views of the CHS. TheCHS consults medical professionals before distributing any medical information. However, the CHSdoes not practice medicine and in no circumstances recommends particular treatments for specificindividuals. In all cases, it is recommended that individuals consult a physician before pursuing anycourse of treatment.Brand names of treatment products are provided for information only. They are not an endorsementof a particular product or company by the writers or editors.EDITOR François Laroche | PRESIDENT Craig Upshaw | NATIONAL EXECUTIVE DIRECTOR David PageEDITORIAL COMMITTEE Hélène Bourgaize, Clare Cecchini, Joanna Halliday, François Laroche, David Page, Chantal RaymondPRODUCTION COORDINATOR Chantal RaymondGRAPHIC DESIGNER Paul Rosenbaum | COPY EDITOR Debbie HumTRANSLATORS Debby Dubrofsky, Sylvain Jobin, Sabine Kramer, Normand Latulippe, Marie PréfontaineCONTRIBUTING WRITERS Joyce Argall, Claude Bartholomew, RSW, Cecily Bos, PT, Clare Cecchini, Dr. Anthony K. C. Chan,Norah Clement, Katie Cunningham, Dr. Andrea Doria, Karen Fahey, Joanna Halliday, Dr. Christine Hough, Dr. Paula James,François Laroche, Michel Long, Maurice Marette, Kathy Mulder, Lucas O’Fee, David Page, Ryanne Radford,Chantal Raymond, Liz Racz, Dr. Emily Rimmer, Dr. Jennifer Stinson, Marion A. Stolte, Craig Upshaw, Dr. Elena Vlassikhina,Rick Waines, Kuan-Chieh Wang, Ashley WariasCHS is on facebook Go to the CHS Web site to be directed to our facebook page.Hemophilia Today November 2011 | 3

Word from the editorFrançois LarocheIwas among the roughly 180 participantsat the 7th World Federation of Hemophilia(WFH) Global Forum on the Safetyand Supply of Treatment Products forBleeding Disorders held in Montreal onSeptember 22 and 23, 2011. This forum, asyou will recall, is organized by the WFH everytwo years, in the year no World Congress isheld. It’s really a mini-congress, with selectspeakers from the field of medicine as wellas representatives of product suppliers,regulators and manufacturers and peoplewith bleeding disorders. Two very full daysfocused on four themes: Perspectives on risk,Achieving a safe and affordable supply,Novel technologies and Manufacturers’updates.Several factor VIII or IX therapies areIt was very instructive to learn about recentprogress in current research by coagulationproduct manufacturers. Several factor VIIIor IX therapies are currently in phase I, II oreven III trials for development of longactingproducts, and results are veryencouraging! It will not be long beforethese products will be available on themarket in Canada—some even within thenext two years- whereas for others, it willbe another five years or so.One of the presentations that impressedme most was definitely the conferenceon novel technologies in gene therapypresented by Dr. Amit Nathwani. In aphase I trial, the hemophilia B gene wasdelivered to the liver through peripheral veincurrently in phase I, II or even III trialsfor development of long-acting products,and results are very encouraging!It will not be long before these productswill be available on the market in Canada…administration using an inactivated adenoassociatedvirus (AAV) capsid as a vector. Witha serotype 8 AAV, there is less tendency tostimulate the immune system, a safer optionimmunologically in the long term.Though only six patients with severehemophilia were included in the trial, earlyresults are very promising, because factor IXlevels increased significantly several weeksafter the gene transfer in all patients, risingfrom 1% to as much as 12% of normaldepending on the dose of the vector administered—withlarger doses yielding higherlevels of stabilized factor IX. Two patientswere able to extend the interval between prophylacticdoses and four were able todiscontinue prophylactic therapy completely.This clinical trial, conducted in the UnitedKingdom, is currently recruiting participantsaround the world for phases II and III of thestudy.Thanks to progress in treatment and care,the life expectancy of a person with severehemophilia has increased from 20 years to anage very close to the national average. And itall happened in just over 50 years! This isexcellent news, however, it comes with adownside. The community of people withbleeding disorders is aging… with all the littleaches, pains and aggravations that agingentails. Thus, in the wake of the work of thenational aging advisory group, the focusgroup held during Rendez-vous 2011, and thediscussion groups formed in response to thisnew reality, this issue of Hemophilia Todaypremiers a new column on aging entitledThe Sage Page. I invite you to read theexcellent article by Rick Waines on page 11and to get involved with us at the CHS toensure that appropriate care for an agingpopulation is offered in all bleeding disordertreatment centres across the country.4 | Hemophilia Today November 2011

Message from the presidentCraig UpshawDedicated to researchThe CHS is committed to improve the health and quality of life of all people with inherited bleeding disorders and toultimately find a cure through research. Our mission started in the kitchens and living rooms of parents of children withbleeding disorders almost 60 years ago. Their goal was to improve their children’s health and quality of life, a focus thathas not changed.Despite great advances in treatment and care for those with inherited bleeding disorders, we can still only dream of a daywhen injections are no longer required and painful bleeds have become memories. The CHS’ effort to turn the dream of a cureinto reality started in 1984 when Ken Poyser and colleagues founded the Hemophilia Research Million Dollar Club (HRMDC),funded through personal financial commitments by parents, grandparents, relatives, friends, caregivers and people with ableeding disorder, and through many volunteer hours fundraising in our chapters and regions. The goal was $1 million, achievedin 1989. It has since grown to $1.9 million. Our members have urged us to go further. Our current goal, as defined by ourrecent five-year strategic plan, is to grow this fund to $2.5 million by 2015. This will be achieved through the efforts ofindividuals, chapters and the national organization to contribute and raise $100,000 per year.Our three research programs, whose 2011 projects are described in this issue, are administered by the CHS with contributionsfrom the HRMDC, generous supporters in the general public, families of people with bleeding disorders, chapter fundraisinginitiatives and companies in the pharmaceutical industry. The goals for each of the programs are different, but in the end theyare all focused on finding a cure and enhancing the quality of life for those with an inherited bleeding disorder.The goal was$1 million,achieved in1989. It hassince grownto $1.9 million.Our membershave urged usto go further.Dream of a CureFunders: HRMDC, general public,families, chapters, CHS Nationaland Bayer HealthCareFirst grant: 1991Dollars invested: $3,500,000Projects funded: 37Studentships: 20Chief investigators supported: 25Care Until CureFunder: Wyeth/PfizerFirst grant: 2001Dollars invested: $1,692,981Projects funded (1st and 2nd year): 34Chief investigators supported: 20Hemostasis FellowshipFunders: Aventis (2001-2003) / NovoNordisk (2002-2009) / CSL Behring(2010-present)First grant: 2001Dollars invested: $835,000Projects funded: 12Young researchers and clinicianssupported: 9While the CHS has been successful in developing a rich endowment, the HRMDC, that provides funding to high-calibre researchprojects, current low interest rates are resulting in reduced returns. At the same time, the number of high-quality applicationsfor all of our research programs has tripled in the last few years. There has never been greater hope among researchers forfinding a cure! Sadly, we cannot fund them all.I am hopeful and optimistic that a cure will be found in my lifetime, and it may well come from the bench of an academicscientist funded by the CHS.While contributing individually to research or participating in research fundraising initiatives is crucial, there are other waysto support the research agenda. Providing accurate treatment records adds to the known science about patient outcomes.Participating in clinical studies for new products or new approaches is essential to advancing knowledge. So while the curemay still be years away, current research continues to lead to better clinical care and immediate dividends. Research showingthat clinical approaches such as prophylaxis are worthwhile is critical in the face of tight health care budgets.I hope that you too see funding research as important, that you volunteer in your chapter’s fundraising initiatives, that youconvince your employers to allow you and your colleagues to direct donations to the CHS for research or, if you have a bleedingdisorder, that you seriously consider participating in clinical research studies. Together we can find a cure!Our currentgoal, asdefined by ourrecent five-yearstrategic plan,is to grow thisfund to $2.5million by 2015.Hemophilia Today November 2011 | 5

Community newsC H A P T E R S P O T L I G H THemophiliaSaskatchewanIn July we held our Guys’ Getaway atBlackStrap Youth Camp. This was aweekend which brought togetherfathers and sons to connect, have funand learn together. The staff at theBlackStrap Youth Camp proved again tobe fantastic hosts for the weekend,leading the guys in numerous activitiessuch as swimming, archery, campfires,scavenger hunts and sports. We werefortunate to have Julia Sek, from Baxter,do a presentation on making goodchoices, which got everyone involved ingroup discussion and activities. Overallthis was a very successful weekend andenjoyed by all in attendance.Manitoba ChapterOn September 11, HemophiliaSaskatchewan ran its first water aidstation at the Queen City Marathon inRegina. Thank you to all our volunteerswho came out dressed for our theme ofSuper Heroes and Villains to help runour station and support the runners inthis event.Thank you Nora!The members of the Manitoba Chapter gatheredfor a picnic at Assiniboine Park on September 10,to celebrate the career of Nora Schwetz, RN. Whilewe are sad to lose our long-time nurse coordinatorto retirement, we are happy that she will now havemore time to do the other things in life that bringher joy.Nora is a huge ambassador for the CanadianHemophilia Society and the Manitoba Chapter. Shealways encouraged her patients to join as sheunderstood the value of partnership between thehemophilia care team, the patient and the CHS. Shereminds us that together we achieved a fundedcomprehensive care program in Manitoba, andtogether we must work to maintain it. While wewon’t see Nora at the Health Sciences Centre anylonger, she will continue to volunteer for both theCHS and the Manitoba Chapter.To many of us in thebleeding disordercommunity, Nora was muchmore than a health careprofessional. She is amember of our families.She supported us andempowered us to becomeindependent managers ofour bleeding disorders.She gave us the tools andshowed us the way.6 | Hemophilia Today November 2011

Nova Scotia ChapterThe Nova Scotia Chapter decided this year to host its annualfamily weekend at Camp Brigadoon. Brigadoon is a camp thatwas built specifically for children with medical conditions. Over$7 million were donated in order to make the Brigadoon dreama reality. We were only the fourth group to hold a gathering sinceit opened only four weeks prior during the summer of 2011. Wehad over 65 members attending. We had guest speakers toprovide the educational part of the weekend and held variousactivities for families to get to know each other more. Everyonehad a great time – Brigadoon was fantastic and we can’t waituntil family camp 2012.C O M M U N I T Y N E W SHemophilia OntarioJust the Guys weekendIt’s a bird! It’s a plane! No wait... It’s Just the Guys 2011 – Superheroes! This pastSeptember 16-18, participants came from all across the province for a fun-fillededucational weekend at YMCA Camp Ki-Wa-Y.Participants enjoyed educational sessions such as My Amazing Blood by Lisa Thibeault,RN, What’s in Your Toolbox? by Georgina Floros, RN, Building a Model Arm and Learningabout Muscles by Karen Strike, PT, Supporting Your Child Through the Pain by GeorginaFloros and Lisa Thibeault, and Transitioning to Adulthood by Linda Waterhouse, MSW.The weekend also packed a punch by getting participants to take part in superherochallenges. The Superhero Fashion Show was by far the best challenge of the weekendas participants got a chance to build their own costumes and walk the catwalk to anepic playlist of songs!We would like to thank our sponsor Bayer for their generous contribution. We’d alsolike to thank our youth volunteers at the weekend, Ryan, Josh, Zachary and Jordan aswell as the Just the Guys Committee. Make sure to mark down next year’s event date ofSeptember 21-23, 2012!Central WestOntario Region(CWOR)Youth Adventures ProgramAhop, skip and a 40 feet high swing! OnJuly 23, youth from Hamilton and Torontoattended a session with McMaster University’sOutdoor Recreation program. The group tookpart in a scavenger hunt that had everyonemoving around campus trying to answer triviaquestions. The group also got a chance toembrace their fears and take on the giantswing!The swing involves teamwork and communicationas one youth is strapped into aharness, while the rest of the group has towork together to raise that youth 40 feet intothe air. Once the youth is ready, on the countof three, the “snake” rope and team rope arereleased to launch the youth on the ropeswing ride. It was an amazing activity enjoyedby all!The Big Sale on the Little StreetOn Saturday September 10, a third partyfundraiser was held for CWOR.The Big Sale on the Little Street is a communityyard sale that is held during the LockeStreet Festival. People were asked to donatetheir unwanted and gently used items to sell(thank you to Rob Dinsdale and his wife andto Julia Sek for their donations) for the eventwith the understanding that all proceedswould be directed to CWOR. The sale had agreat turnout and raised about $500 for theregion. Many thanks to Mary Pedersen forholding the sale.Hemophilia Today November 2011 | 7

C O M M U N I T Y N E W SSouth Western Ontario Region (SWOR)Special thanks to Hillside Daylilies (http://dave.mussar.com)for making this fundraiser possible. Dave Mussar is apharmaceutical representative who also raises daylilies. Heinvited us to glean his acre of daylilies so we went with shovelsand pitchforks to collect the flowers on September 1. We hadparticipation from three regions: SWOR, CWOR and TCOR whopresold the plants and organized pickups at various markets andgarage sales. There are a few left, which can be planted nextspring ($10 per plant, 5 for $25 or 12 for $50). Contact theoffice at 519-432-2365 to get your plants. Dave, on behalf ofSWOR, thanks so much for making this possible!Pinecrest Camp Hooray for HollywoodYet another fantastic year has come and gone for Pinecrest Adventure Camp. This year’s theme, Hurray for Hollywood, was a smash success.Forty-two campers arrived at Camp Menesetung in Goderich, Ontario, ready and willing to give it their all. Campers and staff both cameprepared to tackle crafts, games, swimming, archery, drama, sand castle building, the camp’s new low ropes course and even the ever-popularpolar bear dip. Some of this year’s highlights were the annual talent show, the Shining Star of 2011, and black tie gala. Campers and staff puton their fancy duds and costumes to enjoy each other’s company at a feast fit for Hollywood elite. Congratulations go out to MatthewTravaglini for receiving the Liz Clegg special friend award and to Holly Valenta and Alex Mikler for their presentation of the John Myers award.All recipients were very well deserving of their designations. – by Charlie PangbornPrince Edward Island ChapterWe had a busy summer here on the Island. In August we had a float inthe 50th annual Gold Cup & Saucer Parade, which gave us very wideexposure that money can’t buy, with it being viewed on the route by tensof thousands, and also on air by even more people. We had some of ourchapter children on the float along with our founding father Colin Craig Sr.,and many of our volunteers also on the float and walking along beside it.We also had our first annual Poker Run on September 10, withmotorcycle riders attending from around PEI and Nova Scotia, travelling129 km along scenic PEI collecting playing cards from our chaptervolunteers to try obtain a winning poker hand. The rain held off for themost part and it was deemed a success. Any opportunity that exposes usto different groups of people is a plus, as you never know where a newmember might be hiding! Looking forward to next year already!8 | Hemophilia Today November 2011

C O M M U N I T Y N E W SCHS JAMES KREPPNER MEMORIALSCHOLARSHIP AND BURSARY PROGRAMIn an effort to bring young volunteers into the CHS, and in recognition that a sound education is of utmost importance (particularlyfor those who might not be able to succeed in a vocation requiring strenuous physical labour), the CHS offers the opportunity for thosewho qualify to receive a scholarship or bursary in the amount of $4,000 to attend a post-secondary institution of their choice. The CHSJames Kreppner Memorial Scholarship and Bursary Program is an ongoing tribute to honour James Kreppner, a longtime volunteer andmember of the CHS Board of Directors, for his dedication, intelligence and commitment to the CHS and community service. In 2011,the CHS was pleased to award scholarships/bursaries to three outstanding applicants.The award categories are as follows:— SCHOLARSHIP BASED ON ACADEMIC MERIT— BURSARY BASED ON FINANCIAL NEED— MATURE STUDENT BURSARY FOR STUDENTS OVER 30 YEARS OF AGEThe CHS would like to acknowledge Pfizer for their generous support of the 2011 CHS James Kreppner MemorialScholarship and Bursary Program. – C.C.A C A D E M I CS C H O L A R S H I PKatie CunninghamFredericton, New BrunswickB U R S A R YLucas O’FeeKamloops, British ColumbiaM A T U R E S T U D E N T B U R S A R YNorah ClementSouris, Prince Edward IslandIgraduated fromLeo Hayes HighSchool (2011)with the highestacademic averageand received theGovernor General‘sAcademic Medal.I am attending theUniversity of NewBrunswick in theBachelor of Science program. I am planning aprofession in the medical field and know that,with the help from the CHS James KreppnerMemorial Scholarship, I will be able to followmy dreams... whatever they turn out to be.I am very grateful to be awarded this scholarship.A few years back, I was diagnosed withType 1 von Willebrand disease. It was a shockto find that out so late in my life, but it wasa relief to know why I had such heavy bleedingand bruising. Being aware of vonWillebrand disease has changed my life, butnot in a bad way. It has shown me that withproper knowledge and care, I am still able todo everything I wish and achieve my goals.Ig r a d u a t e dfrom SouthK a m l o o p sS e c o n d a r ySchool in Kamloops,B.C., andhave been attendingthe Universityof BritishColumbia in Vancouversince thefall of 2011. Highlights of my high schoolyears included my performances with manyhonour bands, including the National HonourBand in Montreal and the NorthAmerican Honour Band at Carnegie Hall. Ihave been accepted as a lower brass majorin the Bachelor of Music program with aminor in commerce. From there, I plan toenrol in a Law degree program and eventuallybecome a lawyer. I am very gratefulfor the support I have received from thebleeding disorder community.I encourage younger students like myselfto overcome their condition by developinga passion and using it as a positive focusfor themselves as well as for helping others.Iam the wifeof a hemophiliacandmother of twochildren and I amentering my secondand finalyear of HumanServices at HollandCollege inCharlottetown,P.E.I. I have chosen this field for several differentreasons. I have always been intriguedwith work that assists the individual as wellas their family and friends. In Human Services,we specialize in quality of life for peoplewith intellectual disabilities, autism and otherphysical disabilities. This field takes in theentire life cycle so there are many employmentopportunities. I will be able to have a careerthat not only I truly enjoy, but that willenable me to better support my family. Ihave always been a person who wants tohelp others; however with no formal trainingthe opportunity to do so was very limited.Thank you for assisting me to achieve thisgoal.Additional information including the criteria and application forms for the CHS James Kreppner Memorial Scholarship and Bursary Program isavailable on the Web site at www.hemophilia.ca/en/support-and-education/scholarship-program. The deadline to submit applications fornext year is April 30, 2012.Hemophilia Today November 2011 | 9

C O M M U N I T Y N E W SMeeting with med students at the University of CalgaryRyanne Radford, Calgary, AlbertaIrecently had the opportunity to speak to 200first-year medical students at the Universityof Calgary. Drs. Man-Chiu Poon and DawnGoodyear asked if I would speak to theirstudents about my medical history and share mypersonal story with hemophilia. I think a lot ofboth of them and feel that they have helped meso much – it was the least that I could do.I am a severe factor V hemophiliac and it hasbeen challenging for me over the course of mylife. I estimate that I have received more than2,000 units of fresh frozen plasma and havespent more than five years total in the hospitalso I certainly had a lot to talk about with thestudents.I talked for about ten minutes and then thefloor was given to the students for questions.For about 40 minutes or so they asked abouteverything, from shaving to having babies tothe blood supply. In my opinion it went reallywell. The students seemed genuinely interestedin learning more about hemophilia. After classa few students stayed behind to say thank you.One of them even asked to volunteer with theCanadian Hemophilia Society. It was so nice toget the positive feedback from them because Iam in no way a professional speaker and I stillfind myself getting very nervous no matter howmany times I have had public speakingengagements.During my talk with the students I told themabout my personal blog Hemophilia is for Girlshttp://hemophiliaisforgirls.blogspot.com Someof the students went online after class andwrote some very touching messages. I realizedthen how much the students appreciated havinga patient in the classroom instead of a textbook.Some of them told me how my talk reallytouched them and encouraged them. One of thestudents said that since my talk she isconsidering the field of hematology.Although I did talk a lot about theimportance of staying positive and having agood attitude, I did also tell them a little bitabout what we as patients expect from doctors.I explained to them that at most hemophiliatreatment centres it sometimes takes severaldoctors working together to treat me and it’sreally nice when doctors take the time to buildworking relationships with other doctors inother specialties so things can run smoothly forpatients.I know that at the end of the day doctors arejust regular people and it’s impossible for themto know everything but simply listening to thepatient and having respect for them certainlygoes a long way, and I really think the studentsunderstood my message and appreciated what Iwas trying to say.This has been my second time speaking tomedical students in that setting and if given theopportunity again I would jump at the chance.Upcoming EventsCANADIAN HEMOPHILIA SOCIETY▪ May 25, 2012 – 1 st Canadian Conference on Bleeding Disorders in Women, in Toronto, Ontario.QUEBEC CHAPTER▪ November 12, 2011 – Dance for life – Benefit show at Le Gesù in Montreal. Gala tickets: $100. Regular tickets: $25.Looking forward to seeing you there! www.danserpourlavie.comNOVA SCOTIA CHAPTER▪ December 2011– Curl for Hemophilia.HEMOPHILIA SASKATCHEWAN▪ December 2011 – Family Christmas Party in Saskatoon.▪ January 2012 – North region social event.▪ February 2012 – South region social event.▪ March 2012 – Linger Longer Day – AGM and educational sessions in Saskatoon.MANITOBA CHAPTER▪ November 26, 2011 – Manitoba Chapter Strategic Planning at 944 Portage Ave., Winnipeg, from 9 a.m. to 4 p.m. Light breakfast,lunch and refreshments provided. – All members are welcome.▪ December 4, 2011 – Volunteer Appreciation Event – 944 Portage Ave., Winnipeg, from 2 p.m. to 4 p.m.▪ January 2012 – Women with Bleeding Disorders Brunch and Program – Shooter’s Golf Course, 2731 Main Street, Winnipeg▪ March 9, 2012 – Gala Dinner of Culinary Inspirations, The Spice of Life – Delta Winnipeg Ballroom.Tickets available for purchase in November 2011.SOUTH WESTERN ONTARIO REGION (SWOR)▪ July 21, 2012 – Ride for the Record –Chrome as far as the eye can see as we attempt to set the Guinness World Recordfor the Longest Parade of Motorcycles—18,000 bikes (over 32 km from start to finish).What do you need to know?Who: Anyone with a motorcycle. What: Ride for the Record – Guinness attempt.When: July 21, 2012 Where: Begins and ends at the Western Fair, London, Ont.Cost: $30 per bike and rider / $40 with passenger Non-riders: $30Want to learn more? Check out the Web site www.ridefortherecord.ca. Have a question? E-mail Brendonrecordride@gmail.com or call the London office at 519-432-2365.10 | Hemophilia Today November 2011

The Sage PageRick WainesVancouver, British ColumbiaHemophilia and AgingI remember sitting in my Grade 9 biology class. It would have been 1980.Mr. Anderson was teaching a module on heredity and, of course, hemophiliacame up thanks to our royal connections. The talk was relatively unremarkableuntil, as an aside, Mr. Anderson mentioned that the average life expectancyfor someone with hemophilia was 11.Now, it could be argued thatteachers should not workwith outdated materials,but this would have beenof no consequence to a mouthy 13-year-old. I raised my hand and lethim know that I had hemophilia andI was not about to die, thank youvery much.In the late 1960s, as the firstadvancements in the treatment ofhemophilia began to make a realdifference in our lives, we couldexpect to live well into our 40s and 50s – but not much further. That wasstill some 20 to 30 years below the national average. Today, a person withsevere hemophilia who has access to advanced care can hope to livealmost as long as someone unaffected. These are great strides.As a hemophiliac who came of age in the 1980s, however, I can tellyou that aging wasn’t on my mind. HIV was. Hepatitis C was. My bleedingdisorder seemed rather tame in comparison. Somehow, 24 years laterI am still here. And if you are reading this, then so are you. Back then, ifI had been asked to imagine myself at 45, I wouldn’t have been able to.I couldn’t have imagined 30 for that matter. But, if I had, I would havedescribed a life wherein every moment was cherished, and every breathwas, well, inspired.It hasn’t turned out that way. The truth is much more complicatedand nuanced. The fact is, surviving has its own costs. Forty plus years ofankle bleeds means that each step costs me something. The painassociated with these target joints creates an underlying anxiety that canwear me down. Knowing that so many of us didn’t make it, despiteadvancements in our treatment, can be a different kind of burden.In Calgary, at Rendez-vous 2011, a group of us aged 40-plus gottogether and shared stories. Stories told by men, and by women, mild,moderate, and severe, too many bleeding disorders to name. Stories that drewus together as only peer support can. We are researchers, we are subjects,and we are attempting to understand this new reality. We are getting old.This is fantastic news. This iscomplicated news. The CanadianHemophilia Society has, to its credit,identified aging as an important issueand is devoting time and energy toaddress the needs of those of us luckyenough to have made it this far.Since Calgary, the National ProgramCommittee has put together a groupdevoted to developing a strategythat will best identify and addressthese needs.It is no small task. This area ofresearch is new and the myriad ways bleeding disorders can be affectedby age, and vice versa, are just now being encountered. While we awaitfurther funding to support our programs and research, we haveundertaken a few modest projects to get a head start. Here is a briefdescription of three of our current initiatives.First appointment wallet cardTo be developed to address the specific needs of older people with ableeding disorder to present to specialists, family physicians and otherrelevant health care providers. This card will encourage medicalprofessionals to contact the hemophilia treatment centre beforeundertaking invasive procedures.A regional workshop on agingTo be organized by Hemophilia Ontario Central Western Ontario Region,with hopes that the workshop can be piloted in the fall of 2012.Clinic initiativesThe B.C. Adult Hemophilia Treatment Centre in Vancouver has undertakenthe task of creating a clinic checklist for use during appointments witholder patients. The B.C. clinic also plans to organize specialized clinics forpatients aged 40-plus – specialists such as gerontologists, dieticians andphysiotherapists would be available.Hemophilia Today November 2011 | 11

Focus on researchFocus on researchMaurice MaretteChair of the Hemophilia ResearchMillion Dollar Club,on behalf of the AdministratorsThe Hemophilia Research GrantsReview Committee, under thechairmanship of Dr. PatriciaMcCusker, met earlier this year andannounced the 2011 grant recipients forthe CHS Dream of a Cure ResearchProgram. Summaries of their projects canbe found on pages 14 to 17. These grantstotal $159,981 and were made possible byfunding provided by the HemophiliaResearch Million Dollar Club (HRMDC) andthe Canadian Hemophilia Society (CHS)respectively, in the amounts of $94,000and $65,981. The HRMDC and the CHShave provided over $3,500,000 in supportof hemophilia research in Canadasince 1991.For those of you who may not beaware, it was with great sadness that welearned that Ken Poyser had passed awayon September 7, 2010. Ken, a person withsevere hemophilia, had a dream. Hedreamed of an endowment of at least$1,000,000 to provide funding forinherited bleeding disorder research inCanada. In 1984, with the help of RichardO’Shaunessey, Ed Kubin and many othercommitted people in the hemophiliacommunity, his dream was brought intoreality with the creation of theHemophilia Research Million Dollar Club.Today the fund has grown to almost$2,000,000!To help us promote and raiseawareness of the Club, we revised ourbrochure describing the Club. It explainshow individuals can make a difference byinvesting in hemophilia and otherbleeding disorder research. We hope this will benefit new families andmembers of our community who don’t know about the Club. Our goal isto reach out to as many people as possible. This new brochure wasdistributed to over 300 participants (hemophilia health care providers,patients and families) who attended Rendez-vous 2011 in Calgary,Alberta. If you would like copies of the brochure, please contact JoyceArgall at 1-800-668-2686 or by e-mail at jargall@hemophilia.ca.We have always depended on our hemophilia community – thechapters and regions, individuals, families and groups – which hasprovided financial support since theClub’s inception in 1984. And each yearthis caring and committed communityunderstands that supporting the MillionDollar Club is the most effective way inwhich they can support bleeding disorderresearch in Canada.Again in 2010, our dedicatedcommunity met the challenge of raisingfunds for the Club: $112,000 was raisedtowards endowment growth and$64,639 towards current research. OnMay 28 of this year, a special appeal washeld during the Rendez-vous 2011banquet to honour the memory of KenPoyser. Eric Stolte and David Pouliot, theemcees for the evening, promoted theClub and pledge cards were found ateach place setting; $2,600 was pledgedthrough individual donations and threeindividuals also purchased Voting ClassCertificates for a total of $15,000.In November 2010, after extensiveinput from the bleeding disordercommunity, the Canadian HemophiliaSociety adopted a five-year strategicplan. One of the five key goals is topromote and fund research to improvetreatment and ultimately to find a cure.One of the desired outcomes of this goalis that the Hemophilia Research MillionDollar Club (HRMDC) will grow by atleast $100,000 annually with a target of$2.5 million by 2015. Another is that thefunds available annually for the CHSDream of a Cure Research Program willhave grown from $150,000 to $225,000.The CHS has challenged the Chapters tocollectively raise $50,000 annually to increase the capital of theendowment, and will match those funds.As is our custom, we are pleased to acknowledge in Hemophilia Todayour members and donors who truly understand that the HemophiliaResearch Fund is “our” fund – a tangible and visible evidence of ourcommitment to research. You can read the complete list of VotingMembers, Non-Voting Members, Honorary Members and Honorees whohave supported the HRMDC since 1984 on the following page. We expressour heartfelt thanks to all of you for your generosity.12 | Hemophilia Today November 2011

R E S E A R C HH E M O P H I L I A R E S E A R C H M I L L I O N D O L L A R C L U BI N G R AT I T U D E A N D C O M M E M O R AT I O N | 1984-2010VOTING MEMBERSHIPSKen, Darlene and Tony PoyserTerry DouglasLynne Kubin and FamilyC. Kang TanMr. and Mrs. Joe LaxdalAudrey Irene SaigeonPoyser, Schultz and GlassHemophilia OntarioManitoba ChapterThe IsaacsNorthern Alberta RegionToronto and Central Ontario RegionRay and Helen PoyserNova Scotia ChapterCentral West Ontario RegionBritish Columbia ChapterDr. and Mrs. Ron GeorgeDesharnais-Pépin FamilyHemophilia SaskatchewanMarcel and Aline LaFranceEstate of Mary Ann OlsonShaun Aaron BernsteinMrs. R.W. RuddMrs. Pat LaxdalAlex, Ken Little and Lisa Sorrenti-LittleDr. Martin InwoodEnid and Douglas PagePoyser, O’Shaughnessy and CHSSusan AndersonIn Memory of Dorothy KiddKen HannaGlass FamilyClam Chops c/o Lois LindnerDWK EnterprisesBlanche SummersIn Memory of Stuart JohnsonQuebec ChapterSouthern Alberta RegionNorth Western Ontario RegionEstate of Janet RuddNew Brunswick ChapterG.W. Cooper FamilyNorth Eastern Ontario RegionAurore Mercure FournierNorthern Alberta RegionIn Memory of Frank SchnabelArt and Leona OlsonO’Shaughnessy–MolinaIan and Gail Austin (Jeff and Tim Austin)Canadian Hemophilia SocietyOttawa and Eastern Ontario RegionSouth Western Ontario RegionClam Chops II Dr. Gerry Growe,Lois Lindner, Diane Rudd, GeorgeStephenson, Cheong K. TanFrank Bott and FamilyIn Memory of Gregory BottJamie HillL. Faye StephensonOttawa and Eastern Ontario RegionIn Memory of Shawn DufordBritish Columbia ChapterIn memory of John CrooksNorthern Alberta RegionIn memory of Ken PoyserNON-VOTING MEMBERSHIPSDr. S.K. AliMontreuil FamilyMr. Normand CampeauRalph MurrayJacques D. FournierDavid PageJohn FultonRobert C. PedersenClaire B. GagnonQuebec ChapterMrs. B. RoseDonat and M-Paule GendronClaire and Éric RoussinGlenys and Ed GurneySavoie FamilyRev. Stephen H. HillElon O. ScottJo-Ann KubinBlanche SummersGhislaine LandrevilleBernice and Henry TrillerFerdinand LabontéIn Memory of Luc LabontéOliver Tulk FamilyJean-Guy LavigneAnthony and Maxime VeilleuxAntoine L’HéraultJoseph WaldnerLouise MainvilleLionel MercierSusan E. AndersonGuy-Henri GodinJamie HillDavid L. HolmesFrançois LarocheElaine ReidIn Memory of Marvin Louis OlsonFriends and Family of Mary MacLeodNova Scotia ChapterIn Memory of Martin BottDr. and Mrs. Ron GeorgeIn Memory of Dr. Barry IsaacIn Memory of Cecil and Pat HarrisFriends and Family of Dr. Barry IsaacIn Memory of Dr. Barry IsaacDr. David LillicrapMargaret CracknellIn Memory of George Forbes-BentleyGodin Family: Fernande, Donald and Guy-HenriNorthern Alberta RegionBritish Columbia ChapterIn Memory of Captain Dick RuddCatherine HordosIn Memory of Andras J. HordosVolunteers from the 1 st AnnualRoad Hockey TournamentIn Honour of Trevor Sauvé and Jaime VilleneuveFriends and Family of Marjorie CalderwoodIn Memory of Marjorie CalderwoodTanya and Flavio AntenucciIn Honour of Andris AntenucciDr. Bruce RitchieKen PoyserIn honour of his “Angel” Darlene PoyserJeff BeckIn honour of his “Angel” Heather MollerTom AllowayIn honour of his “Angel” James BeckwithDavid PageIn honour of his “Angel” Patricia StewartCraig UpshawIn honour of his “Angel” Gayle AchtymichukJames KreppnerIn honour of his “Angel” Antonia SwannTony NiksicIn honour of his “Angel” Loretta NiksicDr. and Mrs. Ron GeorgeIn honour of their “Angels”Dr. Martin Inwood, Dr. Irwin Walker,Dr. J. Cranby, and the Hemophilia NurseCoordinatorsBlanchette-D’Fana FamilyIn honour of their “Angel” Kevin BlanchetteCan-Ital Ladies SocietyOttawa and Eastern Ontario RegionIn Memory of Michel FrankFrank BottIn Memory of Martin and Gregory BottDavid PageIn honour of his “Angel” Julia PageMaureen GriffithIn honour of her “Angel” Amy GriffithCatherine Bartlett and Dave HallidayIn honour of their “Angel” Iris HallidayIn honour of their “Angel” Poppy MarieHallidayJoan and Murray KinniburghIn honour of Benjamin and Nathan GrayOttawa and Eastern Ontario RegionIn memory of John WilsonNewfoundland and Labrador ChapterDimart FoundationIn honour of Alexander Mark ErnstHONORARY MEMBERSHIPSFrank Bott and FamilyCanadian Hemophilia SocietyBritish Columbia ChapterSouthern Alberta RegionNorthern Alberta RegionManitoba ChapterNew Brunswick ChapterNewfoundland and Labrador ChapterNova Scotia ChapterPrince Edward Island ChapterQuebec ChapterHemophilia SaskatchewanHemophilia OntarioHemophilia OntarioOn Behalf of the Maynard FamilyCentral West Ontario RegionNorth Western Ontario RegionOttawa and Eastern Ontario RegionSouth Western Ontario RegionToronto and Central Ontario RegionToronto and Central Ontario RegionOn Behalf of the Estate of Ann Lois BrownTom and Marvin OlsonFrancine O’MearaBert and Joan RebeiroCandace TerpstraHONOREESDr. Agathe BarryGisèle Bélanger and her TeamLorraine Bernier and her TeamHelen and Hunter BishopIn Memory of Martin BottIn Memory of Ann Lois BrownDr. Robert Card, Caryl Bell and Elena KaniganComprehensive Care Team of SouthernAlbertaKathy ConliffeIn Memory of Clifford Roy CrookRay and Pat DanielIn Memory of Ken DanielIn Memory of Ray DanielDr. Barry L. DeVeberBill FeatherstoneIn Memory of Raymond Joseph FontaineFor Persons with Hemophilia who haveDied from AIDS “So We Never Forget”Pierre FournierIn Memory of Robert GibsonMuriel Girard and her TeamDr. Gerry GroweIn Memory of Frank HaslamAnn HarringtonIn Memory of Glen Michael HoferDr. Martin InwoodDr. François JobinIn Memory of Stuart JohnsonFamily of David JoyMarie JutrasDr. Garner King and Dr. John AkubutuDr. Nathan KobrinskyIn Memory of Bradley KoloskiIn Memory of Charles Joseph C.J. KubinIn Memory of Barry Waines KubinIn Memory of Edward KubinNormand Landry FamilyIn Memory of Pierre LatreilleIn Memory of Bill LaxdalDr. Mariette Lepine and her TeamIn Memory of James “Jimmy” Alan LoveIn Memory of Gary MacLeanIn Memory of Dr. Douglas, Mark, Pauland Norine MaynardIn Memory of Art OlsonIn Memory of Ray O’MearaBob O’NeillOttawa and Eastern Ontario RegionDr. Mohan PaiJohn PeachPersons with Hemophilia from SouthWestern Ontario RegionPaulien Peters and Duncan ConradGary N. PetrickIn Memory of John PooleKen PoyserRay PoyserIn Memory of Allan E. QuartermainIn Memory of Brian RebeiroIn Memory of Darryl RebeiroDr. Georges-Étienne RivardJoyce Rosenthal and Lois BedardIn Memory of Howard SayantDr. Brent SchacterIn Memory of Kenneth ShewchukIn Memory of Frank SchnabelMarthe SchnabelIn Memory of Glen SprengerIn Memory of John StrawaDr. Hanna StrawczynskiFrank and Candy TerpstraIn Memory of Frank TerpstraIn Memory of Troy Christian TrépanierDr. Chris TsoukasIn Memory of Neil Kerr Van DusenDr. Irwin WalkerBarbara WebsterGlen WebsterIn Memory of James KreppnerWe would like to thank all those whomade donations:In honour of Marjorie Calderwood,Alexander Mark Ernst, Benjamin Gray,Nathan Gray, Phyllis Gray, David Gray,Darryl Gray, Joan Kinniburgh, MurrayKinniburgh, Victoria Kinniburgh, MaryMacLeod, Alden Mueller.In memory of Ray Abate, Norman Babinec,Martin Bott, George Forbes-Bentley,Marjorie Calderwood, Michael Conliffe,Shawn Duford, John Fulton, JacquelineHébert, Reverend Stephen Hill, Dr. BarryIsaac, William Lindner, Hazel MacDonald,Art Olson, Leah O’Neil, Clayton Petrick, OdasWhite, Neil Van Dusen, James Kreppner, KenPoyser, Brian Lucas.We would also like to thank our numerousadditional donors who each year expresstheir confidence by contributing to ouryearly appeals or supporting activitiesorganized by individuals, chapters andregions.Hemophilia Today November 2011 | 13

R E S E A R C HCHS Dream of a Cure Research ProgramSupporting research towards improving the quality of life for people with hemophilia and finding a curehave been goals of the Canadian Hemophilia Society (CHS) since it was founded in 1953. Since 1990 throughfunds provided by the Hemophilia Research Million Dollar Club and the CHS, the CHS provides basic scientificresearch grants and studentships aimed at developing treatments for hemophilia and finding a cure.The following reports describe the projects funded in 2011.DREAM OF A CUREAn evaluation of FVIII expression in phenotypically distinct endothelial cellsSecond year fundingDr. Christine HoughDept. of Pathology and Molecular MedicineQueen’s University – Kingston, OntarioCo-investigator: Dr. David LillicrapFactor VIII (FVIII) is synthesized in some but not all endothelial cells. Our understanding of mechanisms that regulate this FVIII expression is verypoor, in large part because expression of FVIII is rapidly lost when these cells are isolated and cultured. However, culture conditions do not reflect thenatural endothelial environment in blood vessels. Flowing blood exerts shear stress (frictional) forces on endothelial cells, and this causes them toalter the expression of many genes. We want to provide culture conditions that reflect the different endothelial environments throughout thevasculature by exposing the cells to different levels of shear stress. These cells will then be evaluated for the affect that this has on the production of FVIII.Endothelial cells throughout the body are quite heterogeneous and we want to generate a number of phenotypically distinct endothelial cells thatare found in large or small arteries and veins. To do this we will differentiate endothelial progenitor cells under shear stress conditions that are reflectiveof the conditions where these vessels are normally located.This study will provide insights into how shear stress affects FVIII expression in endothelial cells and how inherent phenotypic differences betweenendothelial cells modify FVIII expression. Overall, we expect to advance our understanding of mechanisms that regulate FVIII expression in endothelial cells.DREAM OF A CURENovel imaging techniques for assessment of early cartilage and soft tissue changes in hemophilic anklesFirst year fundingDr. Andrea DoriaDiagnostic and Imaging DepartmentThe Hospital for Sick Children – Toronto, OntarioCo-investigators: Dr. Aaron Fenster, Dr. Marshall Sussman, Dr. Victor BlanchetteHemophilia is an inherited bleeding disorder characterized by the lack of coagulation factors which results in an inability to control bleeding intojoints, leading to long-term joint damage. Prophylaxis reduces the joint symptoms and avoids further degeneration of the joints, however it shouldbe started prior to the development of cartilage lesions. Repeated extravazation of blood into the joint cavity is the factor responsible for cartilagedegeneration in hemophilic arthropathy. Microstructural cartilage changes are thought to precede macroscopic cartilage lesions which are responsiblefor most of the morbidity of hemophilic arthropathy. Conventional imaging techniques are unable to visualize early soft tissue and cartilage changes.Evaluation of soft tissue changes and microstructural cartilage changes with sensitive imaging tools may direct clinical management and prophylaxistowards avoiding further irreversible macroscopic osteochondral damage. New functional MRI techniques and 3DUS anatomic imaging may be ableto diagnose early joint changes at a time when treatment is still effective to avoid further degeneration of the joint. No prior studies have investigatedthe imaging of very early structural and physiologic events in hemophilic joints. We will pioneer the development of novel imaging techniques forassessment of early soft tissue and cartilage changes in hemophiliacs.14 | Hemophilia Today November 2011

R E S E A R C HDREAM OF A CUREValidation of the HEI-Q in adolescents with hemophiliaLab work studentship – Summer 2011 fundingAshley WariasMcMaster UniversityUnder the direct supervision of Dr Vicky Breakey and indirect supervision of Dr V. BlanchetteThe Hospital for Sick Children – Toronto, OntarioTransition is a difficult time for adolescents with hemophilia. We are in the process of completing an Internet-based education program foradolescents with hemophilia. “Teens Taking Charge: Managing Hemophilia Online” will provide teens with eight modules of learning and help themto build self-management skills prior to transition to adult care. As a key part of our evaluation of this program, we will use validated tools that arehemophilia-specific. Our assessment of the website will include determination of quality of life, self-efficacy and stress. We will also see if teens gainknowledge and are satisfied with the program. The Health Education Intervention Questionnaire (HEI-Q, Osborne et al. 2007) is a suitable tool to assessthe impact of the program, but has not yet been validated in adolescents. We aim to validate the HEI-Q in teens with hemophilia. This process willinclude clinical adaption of the language of the survey tool as needed, consensus discussion with investigators, cognitive testing with teens, revisionsas needed, application in the field and psychometric evaluations. I will be involved in the cognitive testing and revisions of the tool. If there is time, Iwill begin the process of application in the field; if not, this will be completed by a clinical research assistant.DREAM OF A CUREQuantitative ultrasound in a rabbit model of blood-induced arthropathyLab work studentship – Summer 2011 fundingKuan-Chieh WangUniversity of TorontoUnder the supervision of Dr Andrea DoriaThe Hospital for Sick Children – Toronto, OntarioBleeding inside the body causes many troubles. Blood might build up in joints and cause extreme pain which prevents the patients to do normalphysical activities. This leads to a decrease in bone quality. For children, it reduces the quality of life and may affect their growth. We will try toestablish the reliability of quantitative ultrasound which is a diagnostic tool that can be easily used to monitor children’s bones.Hemophilia Today November 2011 | 15

R E S E A R C HCare Until CureThe Care until Cure Research Program was established in the year 2000 in collaboration with Wyeth Canada, now Pfizer. Pfizer Canada strivesevery day to help Canadians live healthy, balanced lives. They do that by discovering and developing innovative medicines.This program allows Canadian investigators to conduct research on various medical and psychosocial aspects of bleeding disorders. Grants aregiven for clinical research, including outcome evaluation, in fields relevant to improving the quality of life of people with hemophilia, with vonWillebrand disease or other inherited bleeding disorders, people with related conditions such as HIV or hepatitis C as well as carriers of aninherited bleeding disorder. The following reports describe the projects funded in 2011.CARE UNTIL CUREVon Willebrand Disease plasma and platelets: Functional characterization of quantitativeand qualitative von Willebrand factor mutationsFirst year fundingDr. Paula JamesMedicine and Pathology and Molecular MedicineQueens University - Kingston, OntarioCo-investigators: Dr Walter Kahr, Dr Sue RobinsonIn this study, we propose to perform a detailed analysis of two unique Canadian families; one with individuals with both Type 1 and Type 3 vonWillebrand disease (VWD) and one with Type 2B VWD. VWD is the most common inherited bleeding disorder known in humans and causes excessivebleeding from skin and mucous membranes and in its most severe form, Type 3 VWD, bleeding into muscles and joints as well. We believe that onlythrough detailed study will we improve our understanding of this disease, an understanding that we hope will ultimately lead us to better treatmentsfor individuals affected with VWD. We have identified two interesting VWF mutations in these families and will investigate the significance of thosemutations in our laboratories. Investigators from three Canadian centres will be involved: Dr. Paula James from Queen’s University, Dr. Walter Kahrfrom The Hospital for Sick Children and Dr. Sue Robinson from QEII Health Sciences Centre. We believe the comprehensive set of experiments includedin this proposal will allow for the synergistic interaction of the three Canadian centres, improving our understanding of VWD.CARE UNTIL CUREBone mineral density in Canadian children with severe hemophilia A or B:A multi-centre, cross-sectional, observational study.First year fundingCecily Bos, PTHemophilia DepartmentHamilton Health SciencesHamilton, OntarioDr. Anthony K. C. ChanHemophilia (pediatric)McMaster UniversityHamilton, OntarioCo-investigators: Dr Colin Webber, Dr Kathryn WebertRecent research has shown that bone mineral density (BMD), a measure of bone strength, may be lower among people with hemophilia than amongthose without this disease. The majority of this research has been done in countries where the treatment for hemophilia differs from the standard ofcare in Canada. This study is being done to find out whether Canadian children and youth with severe hemophilia A and B have lower BMD than theirhealthy peers.People with hemophilia do not have sufficient amounts of certain blood components (called coagulation factors) to help the blood clot. Currenttreatment strategies offer a greatly improved quality of life and the expectation of a lifestyle similar to the rest of the population. These strategiescan vary, but the standard of care in Canada for patients with severe hemophilia A and B is to regularly infuse the missing factor to prevent bleeding.In the first study of its kind in Canada, researchers will explore any association between BMD and factor replacement therapy, joint health, bleedinghistory, and physical activity levels by evaluating bone scans of children and youth with severe hemophilia A and B across the country.16 | Hemophilia Today November 2011

R E S E A R C HCARE UNTIL CUREDevelopment and evaluation of an innovative Web-based Educational Program to promoteself-management for teens with hemophiliaFirst year fundingDr. Jennifer StinsonThe Hospital for Sick Children - Toronto, OntarioCo-investigators: Dr. V. Blanchette, Dr. V. Breakey, Danial IgnasIn recent years, the importance of smooth transition of care from pediatric to adult care has received increasing attention. Pediatric hematologistswho care for teens with childhood-onset hematological conditions, such as hemophilia, face challenges in supporting transition to ensure that theseyoung people are educated and able to manage their medical conditions as adults. This program of research aims to develop an innovative web-basedself-management and transition care program to meet the needs of young people with hemophilia. The proposed research includes a usability study totest the user-friendlessness of the website and a pilot study to assess the feasibility of the web-based educational intervention for adolescents withhemophilia. Data from the pilot study will guide the development of a randomized controlled trial that will assess the effectiveness of the interventionon important clinical health outcomes. The knowledge gained through this work will be transferable across chronic conditions affecting adolescents andwill be useful for developing similar programs to improve transition of care. For youth with hemophilia, it will provide ongoing education and supportthrough transition of care and beyond.Hemostasis Fellowship ProgramThe Hemostasis Fellowship Program, a fellowship in congenital and acquired bleeding disorders, was established in the fall of 2001 as a result ofa collaborative effort between Novo Nordisk Canada Inc., the Canadian Hemophilia Society and the Association of Hemophilia Clinic Directors ofCanada (AHCDC). Since 2010, the Fellowship has been made possible thanks to the generous financial support of CSL Behring Canada. As a globalleader in the plasma protein biotherapeutics industry, CSL Behring researches, develops, manufactures and markets biotherapies used to treatserious diseases. Their five core company values underscore their commitment to providing the best possible therapies and services: customerfocus; innovation; integrity; collaboration; and superior performance.The goal of this ongoing annual research program that encompasses a one-year fellowship appointment is to provide fellows in hematology orother relevant fields the opportunity to acquire clinical or research skills necessary to improve the care, treatment and quality of the lives ofpatients with hemophilia and other congenital or acquired bleeding disorders. The following report describes the project funded in 2011.HEMOSTASIS FELLOWSHIP PROGRAMOptimizing hemostasis with DDAVPOne year funding.Dr. Emily RimmerInternal Medicine, Section of Hematology / OncologyUniversity of Manitoba – Winnipeg, ManitobaBleeding disorders are a common clinical problem encountered by hematologists. The most common types of bleedingdisorders are von Willebrand disease (VWD) and hemophilia A and B. These are caused by deficiencies or defects in clottingfactors. DDAVP is a medication that is commonly used to treat VWD and hemophilia A. The way this medication works in these disorders is to raise thelevels of von Willebrand factor (VWF) and factor VIII, the factors that are deficient in VWD and hemophilia A respectively. Using basic principles ofchemistry, we propose that DDAVP could be used to treat other bleeding disorders such as hemophilia B and factor XI deficiency. These are dependentat least in part, on the concentration of factor VIII. This project will look at the laboratory measures of clotting after DDAVP has been given in thesepatients with hemophilia B or factor XI deficiency. The ultimate goal by the end of the project is to develop a clinical trial looking at the treatment andprevention of bleeding in these patients.Hemophilia Today November 2011 | 17

R E S E A R C HINTERVIEWSParticipating in a clinical trial:a great way to make a differenceIn the previous issue of Hemophilia Today,our editor-in-chief, François Laroche,claimed that while research grants are atremendous help to doctors, they often findit difficult to enrol enough patients toobtain significant results from their researchprojects. In this current issue, CHS President,Craig Upshaw, states that “Participating inclinical studies for new products or newapproaches is essential to advancingknowledge”.To promote and encourage participation inclinical trials, Hemophilia Today spoke withthree families who put their confidence andhope in research by participating in theCanadian Hemophilia Prophylaxis Study(CHPS) study. This study, which began in1997 has two specific objectives: 1) toestimate the incidence of target jointbleeding in patients with severe hemophiliaA treated with escalating dose prophylacticfactor replacement; 2) to obtain accurateestimates of the direct and indirect costsassociated with this protocol for use in acost effectiveness model by comparingescalating dose with standard prophylaxisand with intermittent therapy.Many families participating in this studyhave been doing so for over ten years! Hereis what three of them have to say abouttheir experience.HT: How did you learn about the clinical trial and whendid you start participating?Family A (10 year-old boy from Ontario): We heard about the clinicaltrial when my son was diagnosed back in January 2002. We were informedby the nurse coordinator and immediately decided to participate in anyresearch related to hemophilia since it is valuable and important for us “tolearn and let learn” for a better tomorrow if possible.Family B (14 year-old boy from Ontario): We learned about the clinicaltrial from doctors at the hospital for Sick Kids.Family C (15 year-old boy from Quebec): We were told about the studyback in early 1997 from the treatment centre team where our son wasbeing followed.HT: Why did you agree to participate in a clinical trial?Family A: We participated in the clinical trial because it is important thatour son and other children with hemophilia have a better quality of life.Family B: We understood that it would not just benefit our son andfamily but ultimately other children and their families. We felt honouredto have been given the opportunity. Our son also understood and knewthat he would certainly have the best when it came to treatments.Family C: We knew that our son would not have had access to prophylaxisotherwise. Participating in this study was not only helping researchbut it was certainly helping us protect our son from bleeds.18 | Hemophilia Today November 2011

R E S E A R C HHT: What was involved? Was it time consuming?Family A: The great staff at Sick Kids hospital, work around our schedule.We are seen every 6 months for follow-up appointments that may take4 to 6 hours. Our son is seen by specialists, the physiotherapist for severalmobility evaluations, and then x-rays are taken. This is somewhat timeconsuming since the clinic can be overwhelmed with patients, but thehemophilia staff do a great job in assisting and directing us promptly.Another appointment is scheduled, usually in the summer when my sonis off school, for a complete magnetic resonance imagery (MRI) to evaluatehis joints closely, x-rays are taken and he is seen by thephysiotherapist. This appointment usually runs for a couple of hours andnot much waiting is involved. The clinical team even reimburses us forparking and food, which is very thoughtful of them.Family B: It involves routine treatments on a weekly basis, documentationof all treatments and going for checkups at the hospital with doctorsand a physiotherapist. The time spent at the hospital one day every6 months was and still is time consuming, but, when put into perspectivewith all the benefits, it is definitely worth it.Family C: At first, we had to go to the hospital once every week until welearned to infuse our son. When he turned 5, we went to the hospitalevery six months. Since physiotherapy is an integral part of the study, itisn’t always possible to take the various appointments all at the sametime. We are financially compensated for every trip we have to take tothe hospital with regards to the study.HT: Were there any specific challenges or obstacles youencountered?Family A: We have been in the clinical trial for 9 years now and we havenever encountered any obstacles.Family B: Only juggling family when those appointments came up, sincewe had to ensure that one parent remained behind for our other children.There have been times when it was just easier to take everyone as thisreduced the stress of trying to make it home before the others got homefrom school. They are much older now and can fend for themselves fora few hours.Family C: Paperwork! So much paperwork to fill out. At first and on aweekly basis, we had a good 30 minutes worth of paperwork to fill outevery time we went to the clinic. There were so many questions toanswer... Now, the questionnaire is much shorter. The numerous hospitalvisits were also challenging as our son would sometimes have to missprojects and activities at school. In addition, as part of the treatment, hehas to stop prophylaxis 72 hours prior to going for his follow-up andhence cannot do any physical activities during that period of time.HT: What are the benefits of participating in the trial?Family A: By participating in the trial we see how our son’s growth hasor has not been affected by his condition. We have been given theopportunity to help in any way possible to find new and better ways tofacilitate his and the lifestyle of others.Family B: The benefits for our son were and are fewer bleeds and verylittle damage to joints. For the most part he can and does enjoy being akid. He is still careful and avoids contact sports but thoroughly enjoysswimming, biking and walking. He is now in high school and has joinedthe weight club. He understands that strengthening his muscles is a hugebenefit to him.Family C: Being better protected from bleeds has to be the biggest benefitfrom participating in this trial. Because of his participation in thestudy, our son has always been able to do more than he would have hadotherwise. He has had very few bleeds and no joint damage because ofprophylaxis.HT: Would you recommend it to others? Why?Family A: Yes, we would most definitely advise others to join the clinicaltrial, because the more children are involved the better the research willbe.Family B: Yes, absolutely. Maintaining a routine has reduced bleeds whichin the end means that our child, despite his condition, has known very littlepain due to joint bleeds. It has also put less stress on the whole family.The ultimate benefit is that he leads, for the most part, a fairly “normallife”. Something that was not possible for most just a few decades ago.Family C: Most definitely. It helps research by, among many other things,accumulating much needed data that will show governments that prophylaxisis, without a doubt, less expensive in the long run. On a personalnote, it has, and still does improve, the quality of life of our son to anextent we would not have dreamed of at first.HT: In your opinion, what is the long-term goal of thisspecific trial?Family A: In our opinion the long term goal is to find better care, bettertreatment and a better quality of life for the future. Like our son alwayssays, “If we all participated in clinical trials such as this one maybe wewould find a cure for hemophilia one day”!Family B: Fewer bleeds that reduces the damage they can cause. This isthe long-term goal.Family C: Apart from the important data collected, this study has provento be positive in preventing joint damage, not only through the treatmentitself, but also with the expert physiotherapy follow-up.The CHPS study is one of many. Various clinicaltrials are being held on a regular basis by topresearchers who critically need the participationof patients, both children and adults, to concludetheir study. Ask your treatment centre team aboutany upcoming research you might be eligible for.When information is provided to the CHS,opportunities to participate in research are alsopromoted on the CHS Web site atwww.hemophilia.ca/en/research/opportunitiesto-participate-in-research.It is indeed one greatway to make a difference! – C.R.Hemophilia Today November 2011 | 19

Leave aLastingLegacyso that Benjamin and Nathancan hope for a cure…FundraisingCORPORATEPHILANTHROPYPROGRAMWe would like to thank the followingcompanies, corporate foundations andemployee fund programs for theirgenerous support. Our way of recognizing themfor their generosity is through our CorporatePhilanthropy Program, which acknowledges thecumulative support given to the CHS for coreprogramming needs and program sponsorship.SAPPHIREBayer / PfizerPLATINUMBaxter / CSL BehringGOLDNovo NordiskA legacy or planned gift will ensure that 35,000 Canadianswho suffer from inherited bleeding disorders will benefit fromour programs and research for years to come. When drafting orchanging your will, please remember the Canadian HemophiliaSociety or the Hemophilia Research Million Dollar Club.BRONZEBiogen Idec HemophiliaFondation de Bienfaisance T.A. St-Germain (La)Gilead Sciences Canada, Inc.Héma-QuébecImpression Paragraph Inc.Industrial AllianceLeon’sOctapharmaPower Corporation of CanadaRonald and Arnold Hoffman FoundationYLT ConsultingH E L P U S P R O V I D E H O P E F O R T O M O R R O WFor more information, please call Joyce Argallat 1-800-668-2686 or send an e-mail to jargall@hemophilia.ca.www.hemophilia.ca20 | Hemophilia Today November 2011We would also like to thank our numerousadditional donors – individuals, corporations andfoundations – who each year express theirconfidence in us by making substantial supportingdonations.Working together with individuals and the corporatesector in Canada helps the CHS accomplish itsmission and vision by extending our reach andreinforcing our messages.20 | Hemophilia Today November 2011

The Blood Fact rDavid PageCHS national executive director Increased risk of inhibitors for patients treatedwith factor VIII for surgery at first exposureAMSTERDAM – August 12, 2011 – Patients with hemophilia A treatedwith intensive factor VIII for surgery at first exposure are at an increasedrisk of developing inhibitors to FVIII, as compared to those treated forbleeding or through a prophylaxis protocol. The findings were publishedonline in the Journal of Thrombosis and Haemostasis.Corien L. Eckhardt, M.D., from the Academic Medical Centre, inAmsterdam, Netherlands, and colleagues reviewed available literature toinvestigate the role of surgery on inhibitor risk. A total of four cohortand three case control studies made up of 342 inhibitor patients among957 hemophilia A patients were included in the analysis. The investigatorsfound a four-fold increase in the risk of inhibitor development inthose patients who underwent surgery at their first exposure to FVIII.“This systematic review shows that surgery in combination withintensive FVIII treatment is strongly associated with inhibitor developmentin hemophilia A patients as compared to treatment for bleeding orprophylaxis,” the authors write.The research makes a strong argument for early prophylaxis in children.Should surgery be necessary in their early years, these children wouldalready have been exposed to FVIII and therefore less at risk of developinginhibitors. Ferring announces Octostim Spray roomtemperature stableNORTH YORK – August 8, 2011 – Ferring has announced that new lotsof Octostim Spray (Desmopressin Acetate Nasal Solution), used in thetreatment and prevention of bleeding in mild hemophilia A and vonWillebrand disease, are room temperature stable. Previously, OctostimSpray was stored at 2 to 8 degrees C.The new stock, shipped as of August 22, 2011, with lot numberF12185B and expiry date of May 2014, can be stored at temperaturesbetween 15 and 25 degrees C. The room-temperature-stable batches areidentified by highlighted yellow shading of the new storage conditions.The Drug Identification Number (DIN) remains 02237860.Octostim Spray is stable six months after opening, whether refrigeratedor not. Phase 1/2 trial shows 1.7-fold increase infactor VIII half-lifeKYOTO, Japan – July 26, 2011 – Biogen Idec and Swedish OrphanBiovitrum have announced Phase 1/2a trial data showing that the companies’recombinant factor VIII Fc fusion protein (rFVIIIFc) hasdemonstrated a 1.7-fold increase in half-life compared with traditionalfactor VIII products. The study was conducted with 16 previously treatedpatients with severe hemophilia A. The findings were presented at theXXIII Congress of the International Society of Thrombosis and Haemostasisin Kyoto, Japan.Half-life is defined as the time taken for half the infused clotting factoractivity to disappear from a person’s bloodstream. Longer half-lifewould mean less frequent infusions for those on prophylaxis.“These results are promising and supported the advancement of rFVIIIFcinto a Phase 3 trial last year,” said Peter Edman, Ph.D., chief scientificofficer of Swedish Orphan Biovitrum. “We are excited about the potentialof rFVIIIFc to make a positive impact on the health and quality of life ofhemophilia A patients by providing extended protection from bleeding.”rFVIIIFc was well tolerated in this single-dose study, with no drugrelatedserious adverse events. Mouse receives the first genome therapy,hemophilia B curedPHILADELPHIA – June 28, 2011 – Scientists and researchers have madea breakthrough in gene therapy after they cured mice of hemophilia B.The study, led by Katherine A. High, a hematologist and gene therapyexpert at the Children’s Hospital of Philadelphia, could lead to progressin finding a cure for the disease in humans. The findings were publishedin the journal Nature.The therapy first uses a non-disease-causing virus to deliver a protein,called zinc-finger nucleases (ZNF), a sort of molecular scissor, to theprecise location of the flawed gene for factor IX (FIX). A second virus isthen delivered with the correct genetic recipe for FIX, and inserted atthe point where the cut was made. This precision technique is sometimescalled genome editing.A single injection to five newborn mice with hemophilia B partiallycorrected the animals’ genetic deficiency so that their clotting timeswere not significantly worse than normal. The animals maintained theimprovement in clotting for the eight-month duration of the study, indicatingthat the cells continue to pass on their edited recipes with everydivision.Health Canada approvesWilate for VWD indicationOTTAWA, September 9, 2011 – Health Canada has approved Wilate ® ,manufactured by Octapharma, for the treatment of von Willebranddisease (VWD). This plasma-derived clotting factor concentrate is alsoindicated to treat patients with hemophilia A.Wilate is specifically approved for 1) the treatment of bleeding episodesin patients with all types of VWD, including in children, and 2) preventionand treatment of bleeding in minor surgical procedures.Wilate contains a one-to-one ratio of VWF:RCo (ristocetin cofactor) andfactor VIII (FVIII). It is provided in vials of 500 IUs of von Willebrand factor(or 500 IUs of FVIII) and 1000 IUs of von Willebrand factor (or 1000 IUsof FVIII).Wilate is subjected to two viral inactivation steps: a solvent detergent(S/D) method, and a dry heat treatment in the final container at 100°Cfor 120 minutes.No cases of thromboembolic events (unwanted blood clots) have beenobserved after infusion of 80 million units globally in the last five years.Hemophilia Today November 2011 | 21

hV lunteer FileMarion A. StolteChair, National Chapter Relations CommitteeCommitted to volunteerMy parents believed in volunteering and passed that same commitment on to me.What prompts someone tovolunteer? I asked thatquestion to Ashley Tolton,a volunteer on the CHS ChapterRelations Committee. Her reply: “Myparents believed in volunteering andpassed that same commitment on tome. In fact, I began volunteering at ahome for seniors while I was still inhigh school. Then, as I prepared to goto university, I found out that I couldapply for a scholarship from theManitoba Chapter of the CanadianHemophilia Society due to vonWillebrand disease in my family.”Once she finished university, Ashley and herhusband Lawrence realized they wanted to giveback, especially to the hemophilia society. So,she and Lawrence volunteered at the PumaRoad Running for Hemophilia event for acouple of hours and had a great time. Peoplemade an effort to get to know them and theyexperienced tremendous positive energy andencouragement.As a result of this, Ashley took the initiativeto participate in more events, be on planningcommittees, and is now a Board member of theManitoba Chapter, a coordinator of one of the committees, andon our national Chapter Relations Committee. The VolunteerCommittee she coordinates won an award for excellence in 2010from the Manitoba Chapter. Hard work, collaboration, enthusiasmand treating others with kindness and respect have all contributedto Ashley’s ability to work well with others. Lawrence realized that,for him, his investment as a volunteer needed to be on an episodicbasis – and so he is available to serve according to his scheduleand the needs of the chapter.“My continuedinvolvement hasblossomed out ofreally positiveexperiences;I feel (and know)that I’m a part ofsomething veryimportant;it has given megreater personalsatisfaction as Icontribute tosomething beyondwork and home.”hAs Ashley said: “My continued involvement hasblossomed out of really positive experiences; I feel (andknow) that I’m a part of something very important; ithas given me greater personal satisfaction as Icontribute to something beyond work and home.”So what contributes to someone wanting to volunteer?1) values being passed on from others;2) desire to give back to society;3) being valued (positive experience) when volunteering;4) experiencing a sense of community.What prohibits people from volunteering?1) previous negative experiences;2) lack of care/appreciation for the volunteer;3) values of giving to others not promoted by others.Do you have to be on a committee, on a Board, serving nationally?No. You can be an episodic volunteer or someone who can give timeon a regular basis. People contribute as they can. Our job (other volunteersand staff) is to help them feel a part of something important,experience a sense of community, and know that they are valued forwhat they bring as we work together to improve the lives of people inour bleeding disorder community. What a joy – what a privilege – towork together!22 | Hemophilia Today November 2011

Youth FileRyanne RadfordCo-chair, National Youth CommitteeIt is now up to youto advocate for your care!For years our parents were advocating for our care but now it’s our turn.My name is Ryanne Radford and I am the co-chair of theNational Youth Committee. I am a severe factor Vhemophiliac who has had many bleeds over the years andwho has spent many, many hours in medical clinics, hospitalbeds and emergency rooms.In the past, we all have heard about the importance of advocatingfor our own care, but now this is more important than ever. Recently, Ihad a particularly bad experience with the medical system. I went to theemergency room with acute abdominal pain. As recommended, I gavethe triage nurse my FactorFirst card. Being a patient of this hospital theyalready had on hand my medical file. It took seven hours before I receivedmy treatment, four units of fresh frozen plasma. A CT scan revealed thatthe source of the pain was a ruptured ovarian cyst.The next morning I was admitted to the Foothills Hospital only toencounter more problems, including receiving 50 mg of Gravol insteadof Benadryl. This was of concern because the Benadryl is a pre med I takeprior to receiving my fresh frozen plasma. Unfortunately, I have a historyof life-threatening reactions to blood products and need Benadryl beforereceiving any plasma. Not receiving my Benadryl put me at risk of havinga reaction. I spoke to the nurse about the mix up and she didn’t seem tofeel it was that big of a deal. I asked to speak to the nurse in charge andto see my doctor. The nurse in charge didn’t feel the need to come andsee me, but a doctor did come and did fill out a prescription for Benadryl.However, by that time it was too late as I had already received two unitsof fresh frozen plasma.Since being released from the hospital, I have contacted the AlbertaHealth Services patient concern representative. She has informed me thatshe plans on speaking to the people involved and intends to seek out asolution as to how this can be avoided in the future. At least some goodthings will come out of this episode.This is my own experience but I’m sure that several of you have hadsimilar experiences in the past. For years our parents were advocating forour care but now it’s our turn. The National Youth Committee of theCanadian Hemophilia Society has decided to address this issue and host aworkshop on how young people with bleeding disorders can become goodadvocates. Our objective is to provide all young people with bleedingdisorders with the tools they need to learn how to advocate for themselveswhether it be in the workforce or within the medical system.Young people with bleeding disorders will greatly benefit from thisworkshop by learning the steps to better communicate with peopleresponsible for their care. If you are a young person with a bleedingdisorder and are between 18 and 25 years of age, this workshop is foryou. Information regarding the workshop will be posted on our Web siteand circulated through your local chapter. Please visit our Web site on aregular basis at www.hemophilia.ca/en/youth-web.In addition to this workshop the National Youth Committee will beworking on several other initiatives. The committee is made up ofrepresentatives from across the country who are anxious to hear fromyou. We would like to know how you envision the organization’s future.What are your main concerns? Are there specific issues that affect youngpeople and are not being met? To send us an e-mail, visit our Web pageat www.hemophilia.ca/en/youth-web/to-contact-us.Hemophilia Today November 2011 | 23

A Global PerspectiveTWINNING:A tremendous opportunity for sharingMichel Long, CHS national program managerThe World Federation of Hemophilia (WFH) Twinning Program wasestablished more than 15 years ago and since then many nationalmember organizations (NMOs) have experienced the importanceand significance that twinning partnerships can have for peoplewith hemophilia. Many successes have resulted from these relationshipsestablished between two communities which often have contrastingrealities. Canada has had many successful twinnings, with threepartnerships recognized with the Twins of the Year Award in the lastthree years alone. Yet, in a survey conducted recently by the CHSInternational Committee, it became apparent that the twinning programis not well known and, in some instances, misunderstood. This articleattempts to shed some light on some of the key elements of the twinningprogram.What is twinning?The twinning program pairs hemophilia organizations or medicalcentres in emerging and established countries to encourage the transferof skills, resources and information. The program has been shown toimprove treatment and care for people with bleeding disorders aroundthe world. Twinning is a positive two-way experience that motivates staffand volunteers, attracts youth involvement, and enables both sides tolearn from one another. Twinning is a formal, two-way, short-termcollaboration or partnership of three to four years with a focus onsustainability and capacity building.Two types of twinning programsThe Hemophilia Treatment Centre Twinning (HTC) program pairsemerging HTCs with well-established, knowledgeable and experiencedones to help increase the levels of diagnosis and medical attention forpeople with hemophilia through coaching, training and transfer ofexpertise.The Hemophilia Organization Twinning (HOT) program links emergingwith established hemophilia societies to share knowledge in areas suchas patient education, outreach, governance, fundraising, and all otheraspects of operating a successful hemophilia patient society thus helpingthem become a driving force for change and progress. They work togetherto promote access to hemophilia care and to maintain or improve thequality of care.The WFH helps match interested and “partnership-ready” hemophiliatreatment centres or organizations with counterparts elsewhere in the world.What the twinning program is not…While the need is great for factor concentrates and other products inmost parts of the world, the twinning program is not designed to providetreatment products through the partnership. Rather, support is given andlessons learned are shared to help build capacity so emerging twins canget a regular supply of essential products, thereby providing a moresustainable solution.Many may think that twinning equals lots of exotic travelopportunities: this is not the case. While there can be some travelingopportunities, most of the work gets done in many other ways and noteveryone involved in a partnership gets to travel.The benefits of twinningTwinning helps build capacity, allows the sharing of best practices,encourages collaboration, builds relationships, offers new challenges,promotes solidarity, broadens horizons and builds a global movement.For the established twins some of the benefits are an increasedappreciation of our own quality of care, recognition of the role of chapteradvocacy work, new perspective on working with fewer resources, backto basics (care, support, volunteer involvement…), strengthened tiesbetween organization and HTC personnel. For individuals involved thereis personal and cultural enrichment, new volunteer skills learned anddeveloped, an inspiration to continue volunteer work, and the feeling ofhaving made a difference.Requirements for twinningTwinning involves a few requirements such as: chapter support forthe project; several people involved; four-year commitment; skills andexperience in advocacy, fundraising, administration and/or governance,program development, volunteer development, communications/computer skills; a common language…How are twins supported?The WFH Twinning Program provides an unrestricted educationalgrant that supports twinning activities and administrative costs. Theprogram provides an annual payment of $1,500 US to all twins, andadditional project grants ($1,500 to $8,000 US) are available through acompetitive process. The WFH also provides publications and othermaterials to help with twinning activities; regional program officers keepin touch with partners and provide support as needed.The CHS International Committee members and the CHS programmanager are also available to provide guidance and support in additionto the project grants available thanks to the CHS International Fund andthe support of Pfizer. These funds can augment funds received from theWFH and raised by chapters through fundraising activities as they see fit.Thinking of getting involved?Contact Michel Long at the national office at 1-800-668-2686 ormlong@hemophilia.ca to find out how you can become involved andconsider taking part in a International Partnership Training workshop tooccur in February 2012. More information atwww.hemophilia.ca/en/international-development.24 | Hemophilia Today November 2011

Medical newsH E P A T I T I S & H I VP R E S S R E V I EWMichel Long, CHS national program managerand Dr. Elena Vlassikhina, volunteer collaborator First liver transplant patients receive experimental drug to preventhepatitis C infectionA Phase 2 clinical trial has started to test if the novel monoclonalantibody, designated MBL-HCV1, prevents re-infection of patientschronically infected with HCV who are undergoing liver transplantation.The idea here is to clear the virus from the bloodstream before it has anopportunity to re-infect the new liver.www.eurekalert.org/pub_releases/2011-01/uomm-flt011811.php Ultrasound guided liver surgery makes tumour removal saferUltrasound has one enormous advantage over traditional techniques,such as magnetic resonance imaging (MRI) and computer tomography(CT), since it can be used intra-operatively. Mortality rates are lower byup to 5 times using ultrasound guided techniques than with traditionaltechniques. Making the need for major hepatectomies around 10 timesless likely is a massive and positive step forward in the care of hepaticcancer patients says Prof. Guido Torzilli, world renowned liver surgeon.www.medilexicon.com/medicalnews.php?newsid=230363 Maraviroc improves liver fibrosis in HIV/HCV co-infected patientsTreatment with maraviroc is associated with regression of liver fibrosisin HIV-positive patients co-infected with HCV, a small randomized studyshows. Researchers believe that treatment with maraviroc could providean important option for patients who are unresponsive to or ineligiblefor HCV treatment.www.aidsmap.com/page/1882378 Statins greatly reduce risk of death in HIV cohort studyTaking a cholesterol-lowering statin, combined with antiretroviraltherapy, further reduced the risk of death by 67 per cent in anobservational cohort study of people living with HIV, according to a newreport. The results add to a growing body of evidence supportingtherapeutic strategies aimed at minimizing inflammation in people livingwith HIV.www.poz.com/articles/hiv_statins_inflammation_761_20953.shtml Russians developing cirrhosis cureRussian pharmacologists have developed a medicine with good prospectsof curing cirrhosis, which has been considered incurable. It is said to benot only capable of recovering the diseased organ but also of preventingthe pathology from developing further. This medicine mobilizes the stemcells not only in the liver but also in the marrow, which is the depot ofstem cells. After taking the medicine, marrow stem cells enter intoperipheral blood and then settle in the affected organ. The invention bySiberian scientists is undergoing clinical tests. Specialists believe that thelong-awaited medicine, which has been named “life tablets”, will beavailable on the market in five years.http://english.ruvr.ru/2011/09/01/55511779.html Treatment of HIV/HCV co-infected people with compensated livercirrhosisHIV-positive people with compensated liver cirrhosis related to chronicHCV responded as well to treatment with pegylated interferon andribavirin as co-infected patients without cirrhosis, but they should receivecloser monitoring and may need more intensive management of sideeffects.www.eatg.org/eatg/Global-HIV-News/Hepatitis/Treatment-of-HIV-HCV-coinfected-people-with-compensated-liver-cirrhosis Saffron shows promise in preventing liver cancerFindings from a new research study suggest that saffron provides an anticancerprotective effect by promoting cell death (apoptosis), inhibitingproliferation of cancerous cells, and blocking inflammation. Furtherinvestigation of saffron extract and its mechanism of action inhepatocellular carcinoma is currently underway.www.medicalnewstoday.com/articles/233190.php Pharmasset drug works against hepatitis C in mid-stage trialIn a mid-stage study, Pharmasset’s experimental drug PSI-7977 eliciteda sustained virologic response in 98 per cent of HCV patients when takenonce daily with standard therapy of peginterferon alfa 2a and ribavirinfor three months. Researchers will monitor the virus level in patients sixmonths after treatment with Pharmasset’s compound.www.reuters.com/article/2011/09/06/us-pharmassetidUSTRE7855ZX20110906 New hepatitis C vaccine offers hopeFrench scientists have developed a novel hepatitis C vaccine that mayoffer the first effective way to prevent HCV infection which can causechronic liver disease and cancer. The latest experimental shot has beentested successfully on mice and monkeys, but not humans, and has beenshown to activate a broad response from immune system proteins calledneutralizing antibodies. The antibodies fought off multiple variants ofHCV in tests, suggesting the new vaccine should be effective even afterthe virus mutates, the researchers reported.http://tvnz.co.nz/world-news/new-hepatitis-c-vaccine-offers-hope-4338903Hemophilia Today November 2011 | 25

M E D I C A L N E W SNew HCV drugs approvedin CanadaOn August 3, Merck’s protease inhibitor Victrelis (boceprevir) was approved inCanada for treatment of HCV genotype 1 patients over 18 years old, combinedwith pegylated interferon and ribavirin. It is now available in Canada at theprice of $1,050 a week. That approval was followed later in the month byCanadian approval of Vertex’s protease inhibitor, telaprevir, now called Incivek.Michel Long, CHS national program managerGenotype 1 patients make up the majority of those infected withHCV, and have been more difficult to treat. Victrelis has beenshown to triple the response rate in previous non-responders,and double response rates in treatment-naïve patients, and mayallow patients to finish treatment sooner with response-guided therapy.Approval by Health Canada is just the first step as the federal agencyonly assesses the safety and therapeutic effect of a drug and grantspermission to market the drug in Canada. Once Health Canada gave itsgreen light, both Merck and Vertex were able to put their drugs on themarket in Canada. Governments in each province must now decidewhether to include the drugs in their provincial drug insurance programs.For this to happen, the drugs need to go through two more reviews.First, they need a positive recommendation from the Common DrugReview (CDR) – a national review (except for Quebec which has its ownprocess) managed by the Canadian Agency for Drugs and Technologiesin Health (CADTH). Both Incivek and Victrelis are presently being assessedby CADTH and results should be known early in 2012.As a second step, once a positive recommendation has been given,provincial Ministries of Health make their own pharmaceutical coveragedecisions by considering health care policies, programs and resources;and evidence-informed recommendations from their provincialassessment bodies, which normally take into consideration review ofavailable clinical and pharmaco-economic evidence, clinical practice andethical considerations, patient input and the recommendation of the CDR.In Quebec, once the release of a product has been approved by HealthCanada, the manufacturer can submit an application to the Institutnational d’excellence en santé et en services sociaux (INESSS) so that itsproduct can be evaluated with a view to have it registered on the Quebecmedication lists. The INESSS was created on January 19, 2011. Itsucceeded the Conseil du médicament and the Agence d’évaluation destechnologies et des modes d’intervention en santé (AETMIS). The INESSSprocess takes about six months to complete after which INESSS submitsits recommendations to the Minister of Health and Social Services, whocan approve or reject the recommendations. Once they are made public,the List of Medications for the basic prescription drug insurance planand the List of Medications — Institutions are published in hard copyand electronic format by the Régie de l’assurance maladie du Québec.The entire process, whether in Quebec or the rest of Canada, isexpected to take about 12 months so the earliest Incivek and Victreliscan be expected to be available through provincial drug plans is midyear2012. Concern has been raised that this may be too late as access tothe drug could be critical for some people who may not be able to waitso long for treatment. It is also hoped that private insurance plans willcover the therapies now that they have been approved by HealthCanada. 1Immediate access for 1986-90 compensationclaimantsIndividuals admitted under the Hepatitis C (HCV) January 1, 1986–July 1, 1990 Class Actions Settlement needing treatment do not need towait for Incivek and Victrelis to be listed on the provincial formularies tobegin their therapy. The cost of treatment would be reimbursed after theprescription is made by a physician or preferably a specialist (hepatologist,gastroenterologist). Once the compensation program administratorreceives a claimant’s request for reimbursement of the treatment costs,the approval criteria as per Section 4.06 of Schedule B (Hemophilia Plan)will be implemented. If a provincial plan (formulary) lists a drug, or aperson has private drug coverage, then the compensation program canrequest that the province or private insurance plan cover the treatment.It should be noted that those admitted under the Pre-1986/Post-1990Hepatitis C Settlement Agreement, given that approved claimantsreceived lump sum compensation based upon their age, current diseaselevel and the probability of disease progression in the future, are notcovered by a treatment reimbursement mechanism.26 | Hemophilia Today November 2011

M E D I C A L N E W SProtease inhibitors not easyWhen the new drugs were approved in the U.S. in May 2011,patients rushed to their doctors asking, “When can I start?” It’snot that easy. You can’t just buy them, go home, take them,and report back. The American Association for the Study of theLiver Diseases (AASLD) has recently published new 2011 practiceguidelines indicating that the drugs must not be used withoutpegylated interferon and ribavirin. Our sources indicate thatthe Canadian Association for the Study of the Liver (CASL) willbe developing new consensus guidelines for hepatitis Ctreatment this fall. There is now a longer list of side effects:there is more anemia with Victrelis, and there are more rasheswith Incivek. Patients must be educated and monitored. Mostspecialists will be able to start only a limited number ofpatients each week. There is a treatment algorithm (flowchart)according to response, and stopping information, but it is notavailable quite yet. The new treatments are however beingdiscussed in various international meetings. Both of the newdrugs come with a long list of drugs that cannot be used incombination due to possible serious consequences (oralmidazolam and some cholesterol-lowering drugs, for example).Herbs like St. John’s wort can be a problem as well.For now, these protease inhibitors are approved only forHCV genotype 1. They are not approved for children, posttransplantpatients or those co-infected with HIV; however,phase II trials for people who are HIV and HCV co-infected arecurrently underway with both drugs and show promise.Individuals who are co-infected may want to discuss with theirphysicians whether they are eligible to enter these trials orwhether the clinician will prescribe the therapies “off-label.”It is important that the therapies be stopped if the patient doesnot respond as recommended. There have been no head-toheadtrials involving both drugs. Patients and their doctorsshould choose according to preference and risk factors.“Officially” Victrelis declares a 63 per cent sustained viralresponse (SVR), while Incivek has recorded a 79 per cent SVR,but it’s hard to compare them because the design of the trials,for example, lead-in times, were different. Good managementof rash and anemia are important for high SVR rates. 2Are there more therapies in the works?Research is now focusing on non-responders to the tripletherapy, and looking at quadruple therapies, therapies withoutIFN or RBV, and other drugs such as polymerase ornonstructural protein 5A inhibitors. Early results are verypromising, pushing the cure rates to almost 90 per cent. Newtherapies without interferon are very exciting as they havefewer side effects. The progress in the last five years in this fieldhas been very impressive. Future trials will focus on acombination of oral medications to treat hepatitis C in a shortperiod. Already, trials are in progress to reduce the treatmentduration to three months. Trials are also being done withmedication with fewer side effects. Another area that has seenless progress is the development of effective vaccines toprevent HCV infection. Unlike hepatitis A and B, there is novaccine to prevent HCV infection. 31. Source: natap.org/Canada NewsWire.2. Source: http://publish.aasld.org/aboutus/publicpolicy/Pages/newhepctreatments.aspx; Hepc.bull: Sept. 2011.3. Source: www.baltimoresun.com/health/bs-hs-ask-the-expert-0908-20110908,0,6134959.story.Pregnancyplanning,HIV testingand partnernotificationLiz Racz, National HIV-Hepatitis Committee memberIncreased life expectancy and improved quality of life due to advances intreatment for HIV has resulted in more serodiscordant couples consideringfamily planning. As couples access fertility services, gaps in access andprivacy have been experienced; specifically, the topic of partner trace backon notification of a positive HIV antibody test.The standard of care for women who are pregnant or considering pregnancyis to offer HIV antibody testing by the health care provider. Althoughthis is not part of routine pregnancy testing, it is recommended for prenatalcare. However, fertilityclinics that were contactedin a recent survey reportedtesting for HIV for bothpartners as part of theirroutine blood work. Aspolicies differ amongprovinces, this is a point onwhich we are seeking furtherclarification.An informal survey ofcommunity members, aswell as clinicians workingwith HIV discordant couplesplanning pregnancy,yielded a fairly uniformresponse. Good communicationremains the key toall health care interactions.Couples seeking theservices of fertility clinicsshould act proactively,ensuring they are informedand also that the clinic isaware of their concerns of privacy, partner trace back and notification. Therefore,if a couple is aware of one member’s positive status, they should informthe physician so that he or she can report back to Public Health appropriately.If newly positive cases tested at the fertility clinic must be reported, the physiciancan take responsibility for any notifications and counselling; an indicationon an epidemiology form can be made that notification by Public Health isnot necessary.Hemophilia Today November 2011 | 27

M E D I C A L N E W SSocial Workers Face-to-FaceHemophilia and rare bleeding disorders:A continued opportunity to learnCongress of the International Societyon Thrombosis and Haemostasis (ISTH)July 23-28, 2011, Kyoto, JapanClaude Bartholomew, RSWHemophilia Program - Adult DivisionSt. Paul’s Hospital, VancouverEarly in our exploration of bleeding disorders and aging, our patientsemphasized the importance of the research by committing toparticipate and see it through. The year that followed seemed morelike weeks. Dr. Shannon Jackson, Neale Smith, and I discussed thepossibility of a poster session at the International Society on Thrombosis andHaemostasis (ISTH) in Japan. We were pleased to have our submissionaccepted: Understanding the patients’ perspective: Issues in the agingindividual with hemophilia and other inherited bleeding disorders.As a well-travelled North American, Japan has always been somewhat ofa mystery to me. Acknowledging this fact, I embraced and engaged in all thatwould be offered. I must note each morning consisted of a United Nationstype discussion with individuals from all over the world (Europe, USA, Korea,Australia, China, etc.). My Japanese breakfasts were mysteries at times, and Iwas not prepared for the 30-40°C weather that we experienced most days.The rest of the world would benefit in experiencing the efficiency ofJapan’s public transportation system. Trains ranged from high speed(approximately 300 km) to milk runs and every variation in between; theyare safe, comfortable, extremely clean, and devoid of inappropriatebehaviours, as well as noise pollution. I experienced this pleasure eachmorning for approximately 20 minutes as I travelled from myaccommodations to the Kyoto Conference Centre.My introduction to ISTH came with a few hiccups, but what large-scaleevent does not have a few issues to address as it is opening? The landscapereminded me of Central Park in New York, Stanley Park in Vancouver, cottagecountry in Ontario, and the like. Unique to the Kyoto International ConferenceCentre is its Japanese garden; large numbers of turtles and fish populatedthe ponds which offered an inviting backdrop to congregate. The structureitself (some would describe it as a fortress) was meshed concrete and stone,with a hint of Salvador Dali’s multi-dimensional spacing. I considered thegrounds and structure a marvel to experience.(Left to right) Congress President, Dr. Yasuo Ikeda, and Kyoto City Mayor,Daisaku Kadokawa.The conference was teeming with physicians, researchers, industrypartners, nurses, and just a few social workers. My first two days involvedattending the nursing forums, along with sessions on factor VIII, factor IX,and von Willebrand. I continued with the von Willebrand andhemophilia themes throughout the conference, attending, in all, a dozendiscussions covering a variety of topics. There were countless sessionsbut the ones I found particularly interesting included: Gene Therapy forHemophilia: A Long and Winding Road; Challenges and Progress in theManagement of Joint Health in Haemophilia Patients; and one simplyentitled Von Willebrand Disease.ISTH comprised primarily quantitative research, with some relatingto the possibility of future clinical trials, to serve as mapping, and finally,to address the “what is next?” questions. The poster sessions seemed toreflect similar undertakings. Our poster, which was qualitative in nature,received numerous queries, mostly targeting the unknown in relationto aging with a bleeding disorder. There was an overarching focus onassociated costs as individuals with bleeding disorders age and theirrelationship with the various health care providers increases in areas ofcardiac care, scopes, dental procedures and finally, possibly complex carefacilities. I found representatives from the United Kingdom and theUnited States most concerned with the noted examples.The experience of attending ISTH allowed the British Columbia AdultHemophilia Program an opportunity to share its experiences with a largecommunity, while contributing to the body of research, and a forum tonetwork in terms of service delivery. As noted earlier, I attended the nursingforums and truly believe there is a place for allied health care providers tocontribute. I would encourage the allied health care disciplines to initiateresearch relating to ISTH and attend national and regional meetings as ameans of sharing their undertakings. My experience of attending ISTH haslent itself to exponential career and personal growth.28 | Hemophilia Today November 2011

M E D I C A L N E W SThe Physio CornerThe Mild Hemophilia Project:questionnaire results from CalgaryKathy Mulder, PTHealth Sciences Centre, WinnipegDuring Rendez-vous 2011 in Calgary, the Canadian Physiotherapistsin Hemophilia Care (CPHC) proposed the use of a self-assessmenttool for men with mild hemophilia to help them determine whena bleed may require more than first aid measures. After weintroduced the tool, we surveyed the audience by questionnaire. This articlesummarizes the results.Nine different “occupational” groups responded: physicians, nurses,social workers, physiotherapists, people with mild hemophilia (whichincluded carriers), severe hemophilia or other bleeding disorders,pharmaceutical reps, and CHS staff. Responses were collated for each group.Two-thirds of all respondents felt that people with mild hemophilia arenot able to differentiate the symptoms of bleeds that require treatment.Over 80 per cent agreed that people with mild hemophilia delaycontacting the hemophilia treatment centre (HTC) until they havesignificant disability. Half of those with severe hemophilia disagreed withthis statement, but some of the physicians commented that “it’s not justthose with mild” who delay.There was considerable variation in the amount of time thatparticipants thought should elapse before a person with mild hemophiliacalls the HTC about a musculoskeletal bleed, from minutes to days. CPHCis proposing a “48 hour rule”, hypothesizing that if a bleed is not improvingwithin that period of time it will require more than first aid measures toresolve. This will need to be explored in more detail.While most treatment team members felt that some injuries do notrequire treatment with desmopressin (DDAVP) or factor, half of the peoplewith hemophilia were not sure.The majority agreed that a self-assessment tool could be helpful,especially if it is accompanied by education.Half of the physicians and half of the people with mild hemophilia didnot agree that the term “mild hemophilia” gives the wrong impression ofthe condition. These two groups were also equally divided as to whetherthe “48-hour rule” is too long or reasonable.While 75 per cent of respondents thought that the physiotherapistshould see the people with mild hemophilia at each clinic, only 30 per centstated that this actually happens.We hope that these early stages of our research will encouragehemophilia care teams to examine and discuss their practices, and lookforward to presenting the results from the next phase of our study atRendez-vous 2013.WE ARE ALSO STILL LOOKING FOR VOLUNTEERSto give their opinion about the self-assessment tool and help us evaluateits utility. If you have mild hemophilia and would like to participate in ourproject, please contact JoAnn Nilson, PT in Saskatoon, via e-mail atjoann.nilson@saskatoonhealthregion.ca or by phone at 306-655-6628or 306-655-2431.Kathy Mulder, PT,during the mild hemophilia sessionat Rendez-vous 2011Hemophilia Today November 2011 | 29

The Female FactorThe new CHS program for womenwith inherited bleeding disordersJoanna HallidaySpruce Grove, AlbertaThe column The Female Factor haschanged guard: after many years,Patricia Stewart is passing the torchto Joanna Halliday.It is important to acknowledgePatricia’s contribution as founder ofThe Female Factor and her effortscoordinating the column for the pasttwelve years. In addition, herdedication to the organization’svarious programs for women at boththe national and provincial levels, hasbeen outstanding. For all you havedone, Patricia, we thank you!Joanna, who is personally affectedby a bleeding disorder along with hertwo children, has been involved withthe National Program Committee forseveral years, and has been a memberof the CHS coderouge advisory groupfor the past year. She is also theeditor-in-chief of the Alberta Chapternewsletter, Alberta News.Hemophilia Today is very pleasedto welcome Joanna as the newcoordinator of the column. – F.L.As a woman with an inherited bleeding disorder, I find networking with other affected womento be extremely valuable. It gives me the necessary tools to cope when there is mutualunderstanding, compassion, to strive for answers when there is doubt, and to feel moreconfident when there are decisions to be made. I have the blessing of never having to feelalone in this journey. It is not so for thousands of undiagnosed women who are struggling alone.And, from such a need has come CODErouge.CODErouge is the name of the new women’s program spearheaded by Dr. Rochelle Winikoff,director of the Women’s Hemostasis Program at CHU Sainte-Justine in Montreal, and the CanadianHemophilia Society and with the financial support of CSL Behring. A multidisciplinary advisory grouphas been working for almost a year to assist with the development of the CHS program. Their onegoal: to create a program that would increase diagnosis and access to care for women and girls withinherited bleeding disorders. They felt strongly that to achieve this goal several objectives would needto be accomplished:▪ To raise awareness, through outreach efforts, among undiagnosed women who have symptomsof inherited bleeding disorders;▪ To increase knowledge and understanding about inherited bleeding disorders among health careproviders who may come into contact with women with inherited bleeding disorders, and tooptimize diagnosis and access to comprehensive care;▪ To increase the number of comprehensive care programs for women with inherited bleedingdisorders;▪ To make CHS programs and services better known to women with inherited bleeding disorders.Throughout the process one thing was very clear. This program would have to have a strong name,strong character, and a strong visual to stand out amongst the sea of other women’s programs. Incoordination with Tam-Tam/TBWA, a worldwide communication agency, the logo and branding forCODErouge were developed.The CODErouge team is excited to announce that the official launch of the program will takeplace at the very first Canadian Conference on Bleeding Disorders in Women to take place onMay 25, 2012, in Toronto. This conference, hosted by the CHS, will take place in conjunction with theannual meetings of the Association of Hemophilia Clinic Directors of Canada (AHCDC), the CanadianAssociation of Nurses in Hemophilia Care (CANHC), the Canadian Physiotherapists in Hemophilia Care(CPHC) and the Canadian Social Workers in Hemophilia Care (CSWHC).CODErouge ambassadors, who will have been identified in each province, will be sponsored toattend the conference and to receive training to help with the implementation of CODErougeat the chapter level.30 | Hemophilia Today November 2011

The CODErougeadvisory group includesboth health care providersand affected womenas follows:HONORARY CHAIRDr. Rochelle WinikoffDirectorWomen’s Hemostasis ProgramCHU Sainte-JustineMontreal, QuebecHEALTH CARE PROVIDERSClaudine Amesse, RNCentre d’hémostaseCHU Sainte-JustineMontreal, QuebecDr. Christine DemersClinic directorCentre de l’hémophilie del’est du Québec, CHA Hôpitalde l’Enfant-JésusQuebec City, QuebecDr. Diane FrancoeurObstetrician/gynecologistWomen’s Hemostasis ProgramCHU Sainte-JustineMontreal, QuebecSherry Purcell, RNSouth Eastern OntarioRegional Inherited BleedingDisorders ProgramKingston General HospitalKingston, OntarioCatherine Thibault, RNWomen’s Hemostasis ProgramCHU Sainte-JustineMontreal, QuebecWOMEN AFFECTED BYAN INHERITED BLEEDINGDISORDERKatie CunninghamFredericton, New BrunswickKaren FaheyMontreal, QuebecJoanna HallidaySpruce Grove, AlbertaAnia SzadoToronto, OntarioMay 25, 2012 – TorontoFor the very first time in Canada, health care providers and womenaffected by an inherited bleeding disorder will meet for a one-dayconference exclusively dedicated to bleeding disorders in women.This unique event will bring together health care professionalsfrom the fields of:HEMATOLOGY | NURSING | OBSTETRICS | GYNECOLOGYFAMILY MEDICINE | PHYSIOTHERAPY | SOCIAL WORKCODErouge 2012 will feature the following topics: Overview of bleeding disorders in women Quality of life: affected women share their stories Management of bleeding disorders in women State of the art research relating to bleeding disorders in women Multidisciplinary programs for women with bleeding disordersThe conference will be held in conjunction with the annual meetings of theAssociation of Hemophilia Clinic Directors of Canada (AHCDC),the Canadian Association of Nurses in Hemophilia Care (CANHC),the Canadian Physiotherapists in Hemophilia Care (CPHC)and the Canadian Social Workers in Hemophilia Care (CSWHC).Be part of history by attending thispresented by CSL BEHRINGCHS STAFFClare CecchiniCHS national programmanagerDeborah Franz CurrieCHS national director ofresource developmentChantal RaymondCHS nationalcommunications managerFurther details regarding CODErouge and the 1st Canadian Conferenceon Bleeding Disorders in Women will be posted on the CHS Web site.In the meantime, people interested in attending the conference areencouraged to reserve the May 25, 2012 date in their agendas.Hemophilia Today November 2011 | 31

Our StoriesOur StoriesSurvival and triumphKaren FaheyLaval, QuebecMy name is Karen Fahey and I have an inherited disorderknown as factor VII deficiency.At the age of 14, life as I knew it changed forever.I began to menstruate… and that’s when my nightmarebegan as well.The main cause of my nightmare was heavy and prolonged periods,also called menorrhagia. I want to tell my story and to share withyou some of my experiences with menorrhagia and the impact it hashad on my life.At first, I wasn’t sure if what was happening to me was normal,because I always bled very heavily when I menstruated and oftenexperienced embarrassing little incidents.Others thought I was making a mountain out of a molehill, thatI just had to be more careful to prevent such accidents! For example,when I was 16, a classmate touched me gently on the shoulder asI was getting out of the school bus to let me know I had stained mywhite pants. I think “traumatized” is the right word to describe howI felt when I had to go to the infirmary with my bloody stain in plainview for all to see.At age 18, as I was getting out of the car of the young man I wasdating, I noticed a bloody stain on the beige vinyl seat... I cried withshame for a long time.Over the years, the bleeding increased, and fatigue and anemiabecame a part of my life. I began to have difficulty at work and athome. Yet still people felt I was overreacting.Because of my insecurity, I would often work late to make up forthe time I spent going back and forth between my office and thewashroom. Not to mention my fatigue and my lack of energy. I wasafraid of losing my job.As I neared my 35th birthday, I just couldn’t take it any more.I was not happy with myself. I was always tired, nervous, difficult toapproach. By that time, my husband and my two daughters weredirectly affected by my mood.I had consulted many different doctors over the years whoprescribed iron, saying I was too young for a hysterectomy, thatI should wait a little longer before making such a momentous decision.I continued, as a result, to live as I had in the past.One day, my family doctor thought she had found a solution forme. She prescribed Depo-Provera, a hormone injection administeredonce every three months. I went faithfully to my appointments everythree months for four years, and my life was transformed. My joy wasshort-lived, however. In 2004, a report was issued about the risks ofthis drug, and my doctor stopped prescribing it for me.Instead, she referred me for a consultation in hematology at Sainte-Justine Hospital, and I met Dr. Rochelle Winikoff. Dr. Winikoff understoodme immediately, and she sent me to a gynecologist, Dr. DianeFrancoeur, who also completely understood what I was going through.Karen Fahey (L) and Dr. Winikoff at Rendez-vous 2011 in Calgary.Dr. Francoeur and Dr. Winikoff worked as a team, putting their headstogether to find a solution for me. We talked about the possibility of ahysterectomy. Considering my bleeding disorder, surgery of this sort wasrisky. We opted instead for Mirena (an intrauterine device [IUD] thatcontains a low dose of Depo-Provera), to buy time for advances to bemade in medicine and technology. A hysterectomy could be reconsideredin a couple of years.Unfortunately, for some unknown reason, the IUD pierced my uterus.There was no longer any choice. I had to have a hysterectomy.Dr. Winikoff was directly involved, and once again her close collaborationwith Dr. Francoeur was remarkable. Together they restored myconfidence, assured me that they would take every precaution to ensurethe success of the surgery. Still... I was very frightened.The operation took place in May 2006. The bleeding was kept undercontrol throughout the entire surgical procedure, and I returned homebarely a week later.Not long after, I noticed drops of blood on my towel after a shower.Worried, I went to the hospital. Unfortunately, neither Dr. Winikoff norDr. Francoeur was present. I saw the doctor on duty, who had no knowledgeof my case. He did the best he could and sent me home.Exhausted, I went to bed at 6 in the evening, only to awakensuddenly at 11 p.m. covered in blood. The entire family took off in apanic for the emergency room. Once again, neither Dr. Winikoff norDr. Francoeur was there. I tried to explain my situation but found myselfsurrounded by nurses and doctors who knew nothing of my case andcould not give me optimal care.By the following morning, I had lost a great deal of blood and emergencysurgery was required. I spent several days in intensive care andreceived 13 transfusions of blood products. A three-week hospital stayturned into three months. I worked very hard to get back on my feet. Iconfronted my fears, and little by little, my energy returned.After all this pain, all these fears, anxieties and frustration, here Iam at age 45 finally wearing white pants without any fear. The roadto this place was marked by obstacles and disappointments, but I knowthat, with the support of a multidisciplinary team of medical expertsand a network like that offered by the Canadian Hemophilia Society,solutions are possible.I was ecstatic to learn of the creation of a national CHS programdevoted exclusively to bleeding disorders in women. And I am delighted tobe part of the coderouge advisory group that will champion the resourcesrequired to ensure no woman will ever have to go through what I did.32 | Hemophilia Today November 2011