Summer 2005 ADVANCES in HEMOPHILIA CARE in BRITISH COLUMBIA Erica Purves, RN, MSN Between Moderate and Severe Factor IX Associated with Signiﬁ Cant Morbidity

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www.kidscancer.bc.ca Issue number 5 Summer 2005 ADVANCES IN HEMOPHILIA CARE IN BRITISH COLUMBIA Erica Purves, RN, MSN between moderate and severe factor IX associated with signifi cant morbidity. Advanced Practice Nurse defi ciencies before 6 months of age. Intracranial bleeding occurs in about John Wu, MBBS, MSc, FRCP(C) 3-5% of the patients, usually after Pediatric Hematologist MORBIDITY trauma. More than half of these During the neonatal period, children patients are left with permanent Hemophilia is an inherited bleeding with hemophilia may present neurological sequelae. Oral mucosal disorder, made famous through with persistent bleeding from bleeding after trauma to the tongue, its association with the lineages iatrogenic causes such as heel frenulum or after tooth extraction of Queen Victoria and the Russian pricks, venipuncture or circumcision. can be quite relentless leading to Romanov family. This sex-linked They generally do not develop the signifi cant anemia and iron defi ciency. disorder results from a defi ciency in characteristic hemarthroses or Postoperative bleeding can also cause either the factor VIII (Hemophilia A) intramuscular bleeds until they start signifi cant morbidity and mortality. or factor IX (Hemophilia B) walking and become more protein at severe (<1%), mobile. Children with TREATMENT moderate (1-5 %) or mild and moderate mild (6-30%) levels. Hemarthroses, intramuscular bleeds, hemophilia have few extensive soft tissue and trauma- Due to high rates of spontaneous bleeds spontaneous genetic related bleeding are generally treated and usually bleed with the replacement of the missing mutation in these only with surgery or genes, one-third of clotting factor (either VIII or IX). All trauma. Hence, in the the affected children in the province newly diagnosed factor absence of signifi cant VIII defi cient, and up are given a “Factor First” wallet bleeding challenges card updated annually during their to one-sixth of factor early in childhood, IX defi cient, children clinic visits. It contains the current mild or moderate recommendations for coagulation are newly diagnosed hemophilia may not cases in a family. This factor product type and dosing. present until later on in Families are also sent copies of the can be attributed to de Jaryn with his mom, Brenda, and Erica life. novo germline mutations Purves in the Hemophilia clinic annual consultation letters for their record. They are instructed to seek occurring in the child, his Blood within the joint mother or his maternal grandfather. immediate medical care in their local space incites infl ammation of the communities for serious injuries (head, However, a detailed interrogation can synovium, making it swollen and sometimes unearth an unsuspected eye, neck, chest and abdomen). friable, predisposing it to further They are asked to call our clinic for family history. There are no ethnic joint bleeds. If hemarthroses are not differences in disease incidence. assistance with factor administration, promptly and adequately treated, decision making or evaluation of minor hemophilic arthropathy develops Since factor VIII and factor IX proteins injuries if needed. This is important leading to recurrent bleeding, especially to young families as they are essential components of the pain, joint destruction and severe intrinsic coagulation cascade, their struggle to master decision making and disability. Intramuscular bleeds care. Healthcare professionals taking defi ciencies will lead to an inability can lead to compartment syndrome to form a strong fi brin clot at the care of these children are encouraged with neurovascular impairment to call the Pediatric Hemophilia Clinic site of injury. This, in turn, will lead especially in the forearm and to delayed bleeding or prolonged for any concerns shin. Inadequately treated soft or questions oozing at sites of injury in the affected tissue bleeds can lead to the individual. Clinically, Hemophilia A related to this dreaded pseudotumor formation. bleeding disorder and B are indistinguishable from one Pseudotumors are expansile, another and can only be confi rmed and its overall usually peri-osseous, locally management. by performing specifi c factor assays. invasive lesions that are very Physiologically, Factor VIII is at normal resistant to treatment. They can or elevated levels at birth, whereas Recombinant cause compression and erosion of ® Factor IX is only at about half the factor VIII (Recombinate or Kogenate neighbouring organ tissues. Bleeding ® adult level at birth. This can lead FS ) and recombinant factor IX around strategic areas such as airway ® to diffi culties in trying to distinguish (BeneFix ) concentrates are the and the iliopsoas muscle can be mainstays of treatment and have had Continued on page 2...Hemophilia Care in BC Continued from page 1...Hemophilia Care in BC a remarkable safety record of 15 years appropriate minor treatment decisions and can cause signifi cant morbidity with no infectious concerns. Children independently. Intensive teaching and mortality. There is early evidence with mild Hemophilia A (factor VIII and reinforcement of the decision suggesting that exposure to factor level 6-30%) can alternatively be making process on when and how to concentrate early in life (less than treated with desmopressin (DDAVP) treat, the appropriate handling and one year) or intensive exposure, such for minor surgical procedures, dental documentation of the use of factor as for perioperative coverage, may work or minor injuries if they have concentrate are the cornerstones of increase the incidence of inhibitor shown to be responsive by a previous care. Documentation is essential development. Hence, the exposure to challenge test. The adjunct use of for interim clinical evaluation, blood factor concentrate should be minimized an antifi brinolytic agent, such as product tracking, product accountability during the fi rst year of life, and elective tranexamic acid (Cyklokapron®), for and overall product need forecasting. surgeries, such as circumcision, mouth and nose bleeds has been avoided. shown to decrease the duration of SPORTS ACTIVITIES bleeding and minimizing the need for Extreme sports activities should not The use of coagulation factor products multiple factor concentrate dosing. As be allowed. All patients and their costs British Columbian taxpayers for hemarthroses, basic supportive families are given methodical education over 22 million dollars per year. It is care measures such as resting the on healthy sports choices. They imperative to ensure that their usage is site of injury (e.g. using crutches), are taught to avoid sports with high appropriate and accountable. icing, compression and elevation are velocity (dirt biking), rough contact very important. Post hemarthrosis (football) or unpredictable conditions EXCITING CHANGES IN HEMOPHILIA rehabilitation with joint mobilization (water-skiing). They are encouraged CARE IN BRITISH COLUMBIA and muscle strengthening should also to keep themselves physically active be emphasized. Exciting changes in hemophilia care in order to develop strong muscles, have taken place over the past year, good fl exibility and balance. Physical ushering in a renewed focus on HOMECARE PROGRAM - HOME fi tness has been shown to decrease excellence in comprehensive clinical INFUSION OF FACTOR CONCENTRATE bleeding complications. Activities such care for the approximate 100 children Prompt and adequate treatment with as swimming, walking and cycling and families affected by this disorder factor concentrate is essential for the are encouraged. Any activity causing in British Columbia. In June 2004, the treatment of hemarthroses. This has recurrent bleeding should be avoided Provincial Pediatric Hemophilia Program prompted the development of home and prophylaxis with factor concentrate was relocated from the Mary Pack infusion programs in the late 1970s. should be considered. Arthritis Centre at VGH to BC Children’s “Homecare” has Hospital. The adult clinical component become the mainstay PROPHYLAXIS was relocated to St. Paul’s Hospital. of care for families with Most individuals with Dr. John Wu (Medical Director), Dr. individuals affected severe hemophilia in Jeff Davis (Hematologist), Erica with moderate and British Columbia are Purves (Advanced Practice Nurse), severe hemophilia. on prophylactic factor Anne Rankin (Physiotherapist), Tanya It enables families treatment. This consists Strubin (Social Worker), and Sally to have a shortened of the administration of Hiew (Program Secretary) comprise delay to obtaining factor concentrate on a the “core” pediatric comprehensive treatment, avoiding regular basis (1-4 times care team at BC Children’s Hospital. under-treatment due Alessandro receiving factor from his mother. per week) commencing at We strive to evaluate all patients to inability to get to a hospital, and around the age of one to three years on an annual or semi-annual basis enhancing normalcy and security before the occurrence of permanent and have been working to provide for the child and family. Short and joint damage from recurrent true chronic disease care through long term pain and suffering are also hemarthroses. Poor peripheral venous a multidisciplinary, comprehensive minimized by the early treatment of access at this age can be a real care approach. Additional efforts to hemarthroses, soft tissue or muscle challenge, and central venous line support and liaise with community bleeds. insertion may be required to sustain partners have been successful and will this treatment regime. The dose of continue to be built on in the following Home treatment allows children factor concentrate should always be years along with other current with hemophilia to engage in most given in the morning before activity innovations, such as electronic record reasonable activities safely if factor starts, and on days with the most keeping and increased preventative treatment is given prior to the anticipated activities. Extra doses health education. activity in order to cover for the should be given on special occasions “high risk” period. The ability to such as on sports days. The frequency provide immediate treatment with of prophylaxis should be tailored to the factor concentrates has signifi cantly specifi c needs of the individual. decreased the incidence of hemophilic arthropathies, and has much Signifi cant factor inhibitor formation improved the quality of life in the is a dreaded complication, and occurs affected individuals. Hemophilia in about 15% of Factor VIII and homecare entails that families about 3-5% of Factor IX defi cient learn not only how to prepare and individuals after the fi rst 20-50 infuse factor concentrate, but to factor exposures. High titre inhibitor Hemophilia Clinic Team Dr. Jeff Davis Dr. John Wu Erica Purves also take responsibility for making makes the infusion of factor useless, Sally Hiew Anne Rankin Continued on page 3...Hemophilia Care in BC Page 2 POHN Summer 2005 Continued from page 2...Hemophilia Care in BC

Vancouver is proud to be hosting the USEFUL WEB LINKS biennial congress meeting of the World Canadian Hemophilia Society: http://www.hemophilia.ca/en/index.html Federation of Hemophilia from May 21 Hemophilia Emergency Guidelines: http://www.hemophiliaemergencycare.com/ to 25, 2006. Over 4,000 delegates index.html from all over the world will be World Federation of Hemophilia: http://www.wfh.org/ congregating here to present some of the latest innovations in scientiﬁ c and URTHER EADING clinical research, and will be sharing F R Bolton-Maggs PH, Pasi KJ. Haemophilias A and B. Lancet. 2003;361:1801-1809. state-of-the-art care information Chalmers EA. Haemophilia and the newborn. Blood Rev. 2004; 18:85-92. with health care providers, affected Teitel JM, Barnard D, Israels S, Lillicrap D, Poon MC, Sek J. Home management of individuals and their families. haemophilia. Haemophilia. 2004;10:118-133.

CLINIC CONTACT INFORMATION Pediatric Hemophilia Clinic, BC Children’s Hospital Rm 1B40 - 4480 Oak Street Hemophilia World Congress 2006 Vancouver BC V6H 2P1 Erica Purves – Nurse Practitioner May 21-25, 2006 (604) 875 2345 local 5334 Vancouver Convention and Exhibition Centre Sally Hiew – Program Secretary Vancouver BC (604) 875 2345 local 5335 (www.hemophilia2006.org)

FactorFirst Guidelines for Emergency Management of Hemophilia and Von Willebrand Disease

Major / Life-Threatening Bleeds Minor Bleeds

•Head (intracranial) and neck •Nose (epistaxis) •Chest, abdomen, pelvis, spine •Mouth (including gums) •Iliopsoas muscle and hip •Joints (hemarthroses) •Massive vaginal hemorrhage •Menorrhagia •Extremity muscle compartments •Abrasions and superﬁ cial lacerations •Fractures or dislocations •Any deep laceration Treatment for Minor Bleeds

Treatment for Major / Life-Threatening Bleeds Hemophilia A: (severe / moderate) Recombinant factor Vlll concentrate 15-25 lU/kg Hemophilia A: (severe / moderate / mild) Recombinant factor Vlll concentrate 40-50 lU/kg Hemophilia A: (mild) DDAVP 0.3 ug/kg (max. 20 ug) Hemophilia B: (severe / moderate / mild) Recombinant factor lX concentrate 100-120 lU/kg >15 yrs Hemophilia B: (severe / moderate / mild) Recombinant factor lX concentrate 135-160 lU/kg <15 yrs Recombinant factor lX concentrate 35-50 lU/kg >15 yrs The dosage for recombinant factor lX is substantially Recombinant factor lX concentrate 50-70 lU/kg <15 yrs higher because of its lower recovery, particularly in children The dosage for recombinant factor lX is substantially higher because of its lower recovery, particularly in children Von Willebrand Disease: A VW factor containing factor Vlll concentrate such as Von Willebrand Disease: Humate-P 60-80 Ristocetin cofactor units/kg Type l and Type 2A or 2B known to have used DDAVP safely and effectively - DDAVP 0.3 ug/kg (max. 20 ug) All major bleeding episodes should be considered For patients not responding to DDAVP (such as Type lll) use potentially critical (life- or limb-threatening). Humate-P 40-60 Ristocetin cofactor units/kg The goal is to raise the factor level to 80-100% immediately. For mucosal bleeds in all above add: Cyklokapron 25 mg/kg po tid/qid 1-7 days (contraindicated if hematuria)

Dosages are patient speciﬁ c - these are general guidelines only. Round doses up to the nearest vial. If the products listed are not available, please call the nearest Canadian Blood Services Centre.

Canadian Hemophilia Society - Association of Hemophilia Clinic Directors of Canada (AHCDC)

POHN Summer 2005 Page 3 BBCC CancerCancer AgencyAgency AnnualAnnual ConferenceConference

NOVEMBER 3-5, 2005 - CANCER AND THE FAMILY a Westin Bayshore, Vancouver BC d Every year the Provincial Pediatric Oncology/Hematology Network organizes a day for health care professionals n providing care to pediatric oncology patients to gain new knowledge and to network and share with others across the e province. The Pediatric Oncology sessions will be held on Saturday, November 5, 2005. Please visit the BC Cancer g Agency conference website (www.bccancer.bc.ca/HPI/AnnualConference/default.htm) for other topics offered during A

the rest of the 3-day conference. y Saturday, November 5, 2005 g

o 0830-0915 Radiation Oncology Speaker: Dr. Karen Goddard l 0915-1000 The Management of Hematopoietic Stem Cell Tranplant Patients Post Transplantation in the Community o

c Speaker: Dr. Wasil Jastaniah

n 1000-1030 Coffee O

1030-1130 Neurocognitive Impact of Cancer Treatment Speaker: Dr. Dina McConnell c

i 1130-1200 Reducing Treatment for Children with Cancer - The Challenge for the Future Speaker: Dr. Chris Fryer

r 1200-1300 Lunch t 1300-1400 Challenges in Care in the Community Speakers: Dr. Marie Hay, Denise Murray a i 1400-1500 Psychosocial Support for Families in the Community d

e Speakers: Dr. Corina Brown, Jennie Ireland, Dr. Jocelyne Lessard, Sharon Paulse

Pediatric Oncology Agenda P Pediatric 1500-1530 Teen Mentor Program Speaker: Dan Mornar 1530-1630 Parenting a Child with Cancer Panelists: Parents

Other sessions that may be of interest to pediatric oncology health care providers Friday, November 4, 2005: Clinical/Scientiﬁ c Session 0830-0850 Triumph Over Tragedy Speaker: Dan Mornar

Saturday, November 5, 2005: Family Practice Oncology Network Session 1030-1100 Pediatric Oncology - Follow-Up Adult Survivorship Speaker: Dr. Sheila Pritchard NNetworketwork ActivitiesActivities

Education Events An education day on palliative care was held at Prince George Regional Hospital on May 17, 2005. Dr. Hanna Reysner and Cindy Stutzer focused on topics speciﬁ c to pediatric oncology patients such as “Transitioning from Curative to Palliative Care” and “Pain Management”. There was excellent interactive discussion and case studies.

A Hematology Education Day was held at Kelowna General Hospital on June 16, 2005. Dr. John Wu and Erica Purves presented on the management of hemophilia and sickle cell anemia as well as thrombosis and anticoagulation therapy in children.

Website The PHSA IM/IT team has been uploading the content of our website www.kidscancer.bc.ca to the new Content Management System. We appreciate your patience and we hope to revise and update the website content once the PHSA team completes the migration.

Palliative Care Working Group Norms of Practice for pediatric oncology palliative care have been established and circulated to key individuals in the regional centres for review and feedback. These will be available on the website soon.

Psychosocial Care Working Group We are compiling a list of available psychosocial services for pediatric oncology patients and their families throughout the province. Please assist us by giving us the names and organizations of professionals or volunteers in your area.

Long Term Follow-Up We were not able to secure funding from PHSA for the Surveillance Program for Adult Survivors of Childhood Cancer. We are pursuing other funding sources and hope to implement a clinic in the summer of 2006.

Page 4 POHN Summer 2005 FFraserraser ValleyValley PediatricPediatric OncologyOncology ProgramProgram

oncology clinic. These nurses greet meetings on-site. These meetings are the children and their families and open to all families in the Fraser Valley deliver the day-to-day care. Many of area. A Sibling Support Group headed our inpatient nurses are chemotherapy by Dr. Corina Brown is also under and central line certifi ed as well and development with hopes of starting this provide relief in the clinic and/or care fall. for our inpatient oncology children. Through donations Our oncology to our hospital program consists of foundation and a multidisciplinary community support, Treatment Room at Surrey Memorial Hospital team which includes we were able to Lani Lardizabal, RN, BN a Psychologist (Dr. have a mural of a Oncology Clinician Corina Brown), wonderful jungle Social Worker theme painted in The Fraser Valley Pediatric Oncology (Amrin Khan-Jamal), our treatment room. Program, which started in November Dietician (Cindy Rae), These donations 2001, is located at Surrey Memorial Occupational Therapist also allowed for the Hospital, within the Child Health Centre (Sandra Fellowes), Physiotherapist purchase of other comfort items such and adjacent to the Pediatric Inpatient (Chiara Singh), Speech-Language as a television, a DVD player, movies, Unit. The program serves patients Pathologist (Colleen Miller), two Child and a video game system which all in the rapidly growing Fraser Valley, Life Specialists (Paula Black and Susie help to distract the children during spanning Burnaby to Boston Bar. Hauff), Audiologist (Karin Rennert), their various treatment interventions. Currently we have 20 children in active and a Pharmacist (rotating position). treatment and 38 children in long term The team meets monthly to discuss Families have expressed that they are follow-up treatment. We are fortunate patients in both active treatment and happy to be able to receive care closer to have the dedicated services of in long term follow-up care. to home and feel secure knowing that two part-time oncologists. Dr. Derek the clinic maintains close ties to BCCH. Prevost, a pediatric oncologist from The Oncology program’s Child Life BC Children’s Hospital (BCCH), directs Specialists, in partnership with the the program . In October 2004, we Starlight Foundation and the City welcomed another pediatric oncologist, of Surrey’s Recreational Services, Dr. Hanna Reysner, to the program. have been able to provide weekly Dr. Reysner brings with her expertise in yoga classes to patients and Palliative Care. families, and these have been well attended. As well, a Parent Our program offers level III care to Group, under the guidance of the children residing in the Fraser Valley. BC Childhood Cancer Parents’ We have central line and chemotherapy Association, was formed in October certifi ed nurses administering inpatient 2004. The group was created and outpatient chemotherapy which to give support to parents, by includes intravenous infusions parents. The group is led by Fraser Valley Pediatric Oncology Team Suzanne Dunbar (an oncology Barb Cyr requiring supportive care greater than Derek Prevost Lani Lardizabal Hanna Reysner Donna Drake Corina Brown 6 hours. We also provide intrathecal parent) and has monthly Amrin Khan-Jamal chemotherapy, supportive care for fever and neutropenia, and other treatment related effects. Our hospital Kelowna Parent Support Group provides diagnostic services such as echocardiograms, glomerular fi ltration On May 27, 2005, two parents, Kelly May and Laureen Kathler, rate analysis, audiograms, bone scans, representing the BC Childhood Cancer Parents’ Association and bone marrow biopsies. Surrey (BCCCPA), and Dan Mornar, representing the Oncology/ Memorial Hospital is located next to Hematology/BMT program at BC Children’s Hospital, held a The Fraser Valley Cancer Centre where “town hall” meeting in Kelowna, BC, with parents of children with cancer and some pediatric oncology patients can blood disorders. Invitations were sent to 60 families from 12 communities in receive radiation therapy. the BC interior. Ten parents attended the meeting. It was a great opportunity for everyone to discuss the opportunities and challenges facing parents, Last spring, Lani Lardizabal, a nurse children and health care professionals in the treatment of childhood cancer. from our inpatient unit who has As part of the Provincial Pediatric Oncology/Hematology Network, BCCCPA experience from the Oncology Program hopes to forge strong ties with many parents, children and families from at BCCH, joined us as our Oncology across BC. A return to Kelowna in the Fall of 2005 is planned with meetings in Clinician. We have a rotating group Kamloops, Prince George and Vancouver Island to follow. of outpatient clinic nurses: Donna Drake, Barb Cyr, Nancy Bell, and If you are interested in participating, please contact Dan Mornar Gwen Faschoway, who all work in the ([email protected], 604-875-2345, ext 6477).

POHN Summer 2005 Page 5 Return Undeliverable Canadian Addresses to Publications Mail Agreement No. 41074013 The Provincial Pediatric BC Children’s Hospital Oncology/Hematology Network Provincial Pediatric Oncology/Hematology Network Attn: Grace Chan, Network Coordinator The Network is an interdisciplinary Room A119, 4480 Oak Street organization whose goal is to ensure Vancouver, BC V6H 3V4 appropriate diagnosis, management, follow-up, and end-of-life care for pediatric patients with malignancies and blood disorders. The Network supports community hospitals and practitioners, and develops partnerships with other health care facilities to enable CCongratulationsongratulations seamless and integrated care for patients and families on treatment and Congratulations to our inaugural off treatment. dragon boat team “The Strength It will further develop and enhance the research programs of basic, Within”. The majority of paddlers on translational, and clinical research to this team are young cancer survivors, better childhood cancer control and many being first-time paddlers. improve outcomes for these patients and their families. In their first appearance at the For More Information 2005 Alcan Dragon To learn more about the Provincial Boat Festival, they Pediatric Oncology/Hematology participated in four Network, or to submit articles or th stories to this newsletter, please races, placing 8 , contact: 8th, 4th and a very thrilling 2nd. Grace Chan Congratulations to all the teens, coaches, families and supporters! Network Coordinator 604-875-2345 ext 7435 [email protected] Dr. Chris Fryer TTeeneen AdventuresAdventures - SpiritSpirit QuestQuest 20052005 Network Clinical Consultant 604-875-2345 ext 6884 Teen Adventures - Spirit Quest was first started in the Summer of 2000. Since then, [email protected] teens with blood disorders and teen cancer survivors have participated in 22 expeditions. These have included kayaking, river rafting, horseback riding, Steering Committee Chairs Dr. Paul Rogers tallships sailing, dog sledding, skiddooing, and surfboarding. 604-875-2345 ext 7839 [email protected] Barbara Poole 604-877-6000 ext 2403 [email protected]

All activities are sponsored by the Oncology/ Hematology/BMT department through Balding for Dollars. For more information about Teen Adventures - Spirit Quest, contact Dan Mornar at [email protected] or (604) 875-2345 ext 6477.

Page 6 POHN Summer 2005