Comprehensive Care Hemophilia

3Comprehensive Carefor HemophiliaCheck-in… waiting room…meeting members of the team…team members consult withSeng-Kang and his mother…■What is comprehensive care?Hemophilia affects the body in many different ways. Beyondphysical effects such as pain and joint damage, it can also haveemotional and social impacts on the person with hemophilia andhis family. Because of this, medical professionals who treat peoplewith hemophilia take a comprehensive approach to care. Theword “comprehensive” means “complete.” Comprehensive care isabout providing a complete range of healthcare services tochildren and adults with hemophilia. In Canada, comprehensivecare for people with hemophilia began in the 1970s.The comprehensive care team is a group of health professionalsthat works together at a hemophilia treatment centre (HTC). Itoffers a continuum of healthcare services to people withhemophilia throughout their lives. It is also referred to as the careteam or the HTC team.Each member of the comprehensive care team is an expert in adifferent area. The main members of the team are thehematologist, hemophilia nurse coordinator, physiotherapist andsocial worker — together, they make up the core team. This teamwill work with your family to help you understand all the variousaspects of hemophilia and how to prevent and treat bleeds.Together you will develop a plan of care for your child. Otherhealth professionals are available to provide additional support tothe core team if needed.See “Who are the members of the comprehensive careteam?” later in this chapter.All AboutHemophiliaAGuideforFamiliesWe would like to express our appreciation to the comprehensive care team atSte-Justine Hospital, Montreal: Dr. Georges Rivard, Claudine Amesse, Nick Zourikian,Sylvie Lacroix, and to Seng-Kang Lin and his mother who allowed us to document hisvisit to the hemophilia treatment centre. Photos of his visit are used to illustrate thischapter and others throughout the Guide.3-1

3Comprehensive Carefor Hemophilia■Why is comprehensive care important?Comprehensive care means treating the whole person. It embracesall aspects of a person’s health — his body, his feelings and hispsychosocial well-being (how well he fits in at school, work, homeand with friends, etc.). The goal of comprehensive care is to helppeople with hemophilia lead healthy and active lives. Thecomprehensive care team will support you and your family stepby step as your child grows. Everyone can feel secure knowingthere is a care team just for him!The HTC team will teach you, your child and other members ofthe family how to recognize and treat bleeds. If your child hassevere hemophilia, you may need to learn how to give himclotting factor concentrate at home. If your child has moderate ormild hemophilia, you might not need to give him treatments athome. However, if a bleed occurs, you will need to take him to thehospital for treatment with factor concentrates.See Chapter 7, Home Infusion, and Chapter 9, Mild andModerate Hemophilia.The care team will work with your family and your child’spediatrician, dentist, daycare staff and schoolteachers to makesure he gets the best care possible. You should get to know the keymembers of your child’s care team. Do not be afraid to ask fortheir advice if you have a question. If you are not sure who to talkto about a problem, speak to the HTC nurse coordinator. She/hewill tell you what you need to know, or arrange for you to speakto someone who can help you.Check-up with Dr. Rivard3-2All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor Hemophilia■Why do you need to register your child at ahemophilia treatment centre?It is very important that you register your child at a hemophiliatreatment centre, even if he gets most of his routine health careclose to where you live. Going to an HTC at least once a year willallow your family to:“I wasn’t surewhat to do. Therewere too manydecisions to make.The support of ournurse at the clinichelped us calmdown and deal withour son’s health.Our family doctorjust didn’t knowenough about it.We needed theclinic team.”• Get correct information about hemophilia fromthe comprehensive care team.• Get blood tests that can only be done by aspecialized coagulation lab.• Have access to the newest treatments forhemophilia.• Find out about the latest research on hemophilia.• Make sure your child gets a comprehensive“hemophilia check-up” regularly.Most children with hemophilia have a check-up at the HTC every6 to 12 months. How often your child goes will depend on howsevere his hemophilia is, and how often the care team wants tosee him for an assessment. Having check-ups at the HTC is veryimportant to your child’s care. As your child grows, his plan ofcare will change to meet his evolving needs.When he visits the HTC, the care team will review his history ofbleeds and treatment plan. The team will want to know howmany bleeds he has had and how much clotting factorconcentrate he has infused since his last visit. You will need tokeep record in a home infusion diary — this is very importantbecause having accurate records of his bleeds and treatments willhelp the team decide if he is getting the right amount andfrequency of clotting factor. The check-ups are also a good time toAll AboutHemophiliaAGuideforFamilies3-3

3Comprehensive Carefor Hemophilialet the care team know if your child is having frequent bleeds inone joint. You can then decide together whether to adjust histreatment plan to prevent the development of a target joint.As your child grows and develops, his range of physical activitieswill expand and with this the frequency and dose of factorconcentrate that he needs to treat or prevent bleeds could change.Regular visits to the HTC will allow you and the comprehensivecare team to respond to his changing needs.SudburyComprehensiveCare ClinicHamiltonComprehensiveCare Clinic3-4All AboutHemophiliaAGuideforFamilies

Comprehensive Care3for Hemophilia■Who are the members of the comprehensivecare team?“It is essential tostay connected toyour comprehensivecare team, yourfamily who willsupport and adviseyou, and to otherparents of childrenwith hemophilia.”Many different health professionals and services are involved inproviding comprehensive care for people with hemophilia. Theyfall into three general categories: the core team, extendedmembers and specialized hospital services.Core TeamThe core team is made up ofhealth professionals whohave specific expertise intreating people withhemophilia. Thesemembers are based at anHTC and work together toprovide comprehensive care— they are essential to anyhemophilia program. Parents ofnursecoordinatorphysiotherapistchildren with hemophilia are also part of theteam.doctor(medical director)socialworkerparents/caregiversThe members of the core team are:• medical doctor (usually a hematologist)• nurse coordinator• physiotherapist• social worker• parent/caregiverYour child is at the very centre of this core team. The core teamwill also work closely with your child’s pediatrician, dentist andteachers to help ensure that he gets the best possible care awayfrom the HTC.All AboutHemophiliaAGuideforFamilies3-5

3Comprehensive Carefor HemophiliaExtended MembersOther health professionals are also involved in comprehensivecare but they are not permanent members of the care team. Theyare referred to as extended members of the team. All HTCprograms must have these health professionals available tosupport the work of the core team. Extended members are usuallybased at the same hospital as the HTC — otherwise, the HTC willhave agreements will other healthcare institutions on the referralof patients.“Always haveone really goodquestion for yourdoctor. That's howyou learn.”Extended members of the comprehensive care team include:• dentist• ear/nose/throat specialist• experts in pain management• child life specialist• occupational therapist• psychologist• rheumatologist• orthopedic surgeon• geneticist/genetic counsellor• gynecologist/obstetricianThe specific roles of these health professionals and specializedhospital services are described in the next section.3-6All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor Hemophilia■What are the roles of the members of the coreteam?“I have been soimpressed andencouraged by theknowledge, skillsand researchinterests of ourclinic doctor. I knowhe cares for all theboys very deeply.He doesn’t want tosee the kids havebreakthroughbleeds.”Medical DirectorThe medical director is the doctor whooversees patient health and thecomprehensive care team. In manycentres, the medical director of thecomprehensive care team is ahematologist. A hematologist is a doctorwho understands and treats bloodproblems. If the medical director is not a hematologist, she/he willcall on a hematologist for advice.The medical director’s job is to:• Oversee the care team and the treatment of people withhemophilia.• Help the family deal with any health or medical concernsand answer questions about hemophilia.• Order blood tests and X-rays and review results and thetreatment plan with the family.• Keep up to date on the latest research and tell familiesabout new developments in the management ofhemophilia.You should be aware that the medical director does not take theplace of your family doctor or your child’s pediatrician. The familydoctor or pediatrician can give you advice about general childhealth issues, such as the flu, chicken pox, ear infections andvaccinations. The medical director will communicate informationand updates about your child’s health with his pediatrician anddentist.All AboutHemophiliaAGuideforFamilies3-7

3Comprehensive Carefor HemophiliaNurse CoordinatorThe nurse coordinator is your key contact in the comprehensivecare team. She/he coordinates when you and your child see othermembers of the team. In most HTCs, the nurse coordinator is theperson who answers your telephone calls. If your child needs to goto the HTC to treat a bleed, the nurse coordinator is often theperson who makes sure the infusion goes smoothly.An important part of the nurse coordinator’s job is teaching peopleabout hemophilia. She/he can go to your child’s daycare centre orschool to educate teachers and staff. The nurse coordinator canalso give you useful booklets and videos on hemophilia producedby the Canadian Hemophilia Society (CHS). There is a wide rangeof resources for different people involved in providing care forchildren with hemophilia such as family members, teachers,babysitters, and local nurses. The CHS also has colourful activitybooks and guides to help children understand hemophilia, how itis treated and what to do to stay healthy. Ask the HTC for copies ofthese educational materials, or contact the CHS.When your child is old enough, the nurse coordinator will trainyou and your child to do home infusion, if this is needed. At alater date, when your child is older, the nurse coordinator willteach him how to self-infuse. See Chapter 7, Home Infusion.Get to know your nurse coordinator. She/he is there to help you.Do not be afraid to ask questions, even if they seem simple or silly.You can be sure that parents of other children with hemophiliaask similar questions.“She always mademe feel that myquestions mattered.Our nurse hasbecome our friend.She has guided usthrough some toughtimes. My son trustsher and has hisown relationshipwith her.”If you have a problem of any kind, it is important to solve it rightaway. Phoning your nurse coordinator does not always mean thatyou will have to take your child to the hospital. Often you will beable to solve the problem over the phone.The nurse coordinator treats many children with hemophilia. Thismeans she/he knows a lot about the bleeding disorder and isexperienced in treating it.3-8All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor HemophiliaThe physiotherapist teaching exercises…assessing joints…testing muscle strength“Training andsupport from thephysiotherapisthas helped usunderstand theimportance of restand ice when a jointbleed occurs.Physiotherapistsknow their stuffand are good atexplaining the typesof bleeds that mychild gets, and whatis needed to healhis bleeds.”All AboutHemophiliaAGuideforFamiliesPhysiotherapistThe physiotherapist is trained to keep muscles and joints healthy.The physiotherapist will give you advice on how to prevent orlimit bleeding. She/he will also guide your child’s recovery from ajoint or muscle bleed. The physiotherapist will help you:• understand what a bleed is• know whether a bleed is serious or not• learn what to do to help your child get better after a muscleor joint bleedThe physiotherapist’s important role is to help your child recoverquickly and safely from a bleed. Physiotherapists are skilled atdetermining what patients should do, in addition to factorreplacement, to get better from a bleed. This might include:• specific exercises that she/he will teach you and your childhow to do• possible use of assistive devices (braces, crutches, cane,wheelchair, etc.)• the gradual return to activities (walking, running, sports,etc.)The physiotherapist will also give you advice on ways for yourchild to stay active and healthy. Physical activity and exercise areimportant in a child’s development. Exercise keeps muscles strong.Strong muscles protect the joints and may help your child havefewer bleeds.3-9

3Comprehensive Carefor HemophiliaSocial WorkerMost families of children with hemophilia meet the social workerwhen they first learn of the diagnosis. The social worker’s job is tohelp parents, siblings and the child himself deal with thepsychological, social and potentially even financial impacts thathemophilia can have on their lives.One of the early issues that you will face is deciding how much toprotect your child. As your child gets older, other issues may comeup such as how to train a babysitter and whether or not bothparents should work outside the home. Discuss your concernswith the HTC social worker, who can help you find solutions.■3-10Pain Management in HemophiliaIn various ways, all members of the core team are involved inthe management of different aspects of pain. The person withhemophilia, and his parents in the case of a young child, areat the centre of the process.• The nurse coordinator ensures that pain is assessed andtreated by the appropriate team member.• The hematologist develops a management plan for bothacute and chronic pain, which may include medications.• The physiotherapist makes recommendations fortreating acute or chronic pain, with the overall goalbeing to prevent secondary complications such as tightmuscles or reduced joint mobility.• The social worker helps individuals and families livingwith hemophilia manage the complications that occurdue to pain, and deal with related physical orpsychosocial challenges to improve overall functioningand social well-being.“We need thesupport andguidance of doctorswe didn’t expect toneed. Our son hassevere arthritis inhis ankle due torepeat bleeds. He isunder the care ofseveral specialists atthe clinic and he isdoing great, nowthat he takes anarthritis medication.I feel good that he isbeing monitored.”All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor Hemophilia■How do the extended members support thecore team?DentistIt is very important to prevent dentalproblems in children withhemophilia. If your child does nottake good care of his teeth bybrushing/flossing regularly and seeinga dentist for check-ups, he could lose atooth to decay or cavities.He could also need dental surgery to fix a problem that has beenallowed to worsen. For dental surgery, treatment will almostcertainly be needed to prevent bleeding and can include the use offactor concentrates. Although most HTCs offer a dental service forchildren with hemophilia, many families see a local dentist. Thisis common when a family lives far away from the hemophiliacentre.It is also a good idea to discuss your child’s dental care with theHTC nurse coordinator or medical director. No matter where youlive, you and your family dentist can get in touch with the dentalservice at the HTC with your questions or problems.Ear/Nose/Throat Specialist (also called ENT)The ear/nose/throat doctor treats all problems involving the ears,nose and throat. Should your child need to have tubes inserted inhis ears because of ear infection, or have to have his tonsils oradenoids removed, the ENT doctor will perform the operation.All AboutHemophiliaAGuideforFamilies3-11

3Comprehensive Carefor HemophiliaChild Life SpecialistA child life specialist is often available to children and familiesliving with hemophilia. The child life specialist usually works in a“play room” environment. She/he can help children who have afear of needles and anxiety with factor infusions. Using playtherapy, imagery and distraction techniques, the child lifespecialist helps children learn how to relax and learn coping skillsfor treatment and procedures.Occupational TherapistAn occupational therapist develops individual and groupprograms to help people of all ages be as independent andfunctional as possible in different environments (home, daycare,school, workplace, etc.). She/he works closely with people affectedby a medical condition or injury. She helps them maintain, restoreor increase their ability to care for themselves and engage in work,school or leisure. This is done by assessing the individual’senvironment, functional limitations, and specific needs and goals.PsychologistA psychologist in an expert in the human mind and how itfunctions. A psychologist can help someone who is having troubledealing with his hemophilia or having difficulties at school, workor with peers.RheumatologistA rheumatologist treats health problems and diseases that causepain and swelling in and around joints and muscles.“My son feels angryabout receivingmedical care; hedoesn’t want to dealwith hemophilia,doesn’t want to seehemophilia doctors;he’s in denial.”“He got counselingwith the family andby talking about thedisease, he wasreassured that heis ‘normal’.”3-12All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor HemophiliaOrthopedic SurgeonAn orthopedic surgeon is a doctor who treats diseases that affectthe bones and joints, including fractures. If your child needs tohave an operation on a joint, it is the orthopedic surgeon whowill perform it. For more information on joints, see Chapter 8,Complications of Hemophilia.Geneticist/Genetic CounsellorA geneticist studies genes and how people inherit diseases.A geneticist or genetic counsellor has specialized training inproviding genetic counselling for families of children withhemophilia. For more information on inheritance,see Chapter 2, How a Child Gets Hemophilia.Gynecologist/ObstetricianA gynecologist is a doctor who understands and treats women’sgynecological issues. Some carriers can have heavy menstrualbleeding and a gynecologist can find ways to reduce the bleeding.An obstetrician is a doctor who specializes in conception,pregnancy and childbirth. Gynaecologists and/or obstetricianswith expertise in bleeding disorders can work with women whoare carriers of hemophilia. For more information on hemophiliacarriers, see Chapter 2, How a Child Gets Hemophilia, andChapter 14, Symptomatic Carriers of Hemophilia.All AboutHemophiliaAGuideforFamilies3-13

3Comprehensive Carefor Hemophilia■How do specialized hospital services supportthe core team?The hospital where your HTC is based has several laboratories thatperform many specialized laboratory tests and functions. Many ofthe blood tests that your child needs will be done by a specializedcoagulation laboratory. This kind of lab also does specializedblood tests for inhibitors (a possible complication that can developin some patients), factor VIII and factor IX levels (also called factorassays), and any other blood tests that have to do with bloodclotting. The Hemophilia Mutation Laboratory in Kingston,Ontario, provides a national service for the genetic analysis ofinherited bleeding disorders.Transfusion Medicine DepartmentEvery large hospital has a transfusion medicine department, alsoreferred to as the blood bank. The blood bank is where bloodproducts are stored in a hospital. When the HTC doctor or nursecoordinator “orders” factor concentrates, they usually come fromthe blood bank. At some centres, the nurse coordinatororders factor concentrates directly from the local branch ofCanadian Blood Services or Héma-Québec.In some parts of Canada, parents pick up the clotting factorconcentrates from the blood bank in the hospital wheretheir HTC is located. In other regions, clotting factorconcentrates are sent to the local hospital nearest to thefamily’s home. No matter who orders your child’s clottingfactor concentrates or where you pick them up, you need tokeep up-to-date records of his use of factor concentrates inan electronic or paper home infusion diary. These home infusionrecords must be returned to your nurse coordinator as requested.3-14All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor HemophiliaDiagnostic Imaging DepartmentThis department takes pictures (x-rays, ultrasound, MRIs, CTscans) of your child’s joints and bones when needed. These testsand images help the members of the care team decide what kindof treatment is best for your child. They also show whether thefactor replacement therapy is working well.Emergency Care Department (24-hour ER)The emergency department of a hospital isalso called the emergency room (ER). Thisdepartment is responsible for providingmedical care to people arriving at thehospital in need of immediate care, such asin the case of a bleed that requires treatment with factorconcentrate and/or other medical interventions.Additional Healthcare ServicesThe comprehensive care team will also coordinate access toadditional healthcare services as needed. These include:• ongoing education and training in the management ofhemophilia• home infusion program with education and training• other medical and allied health expertiseThe care team can also provide educational services to otherhealthcare services and outside agencies, and outreach andeducation to daycare staff and teachers.All AboutHemophiliaAGuideforFamilies3-15

3Comprehensive Carefor Hemophilia■Where are hemophilia treatment centreslocated?Most hemophilia treatment centres are based in large hospitals inbig cities. Some hospitals have separate clinics for children andadults, while others treat both adults and children at the sameHTC.If hemophilia is diagnosed or suspected, your family doctor or aspecialist will refer your family to an HTC close to where you live.If you happen to live in or near a city that has a HTC, then this iswhere your child will receive his ongoing care and assessments.To find a hemophilia treatment centre close to where you live, seeChapter 17, For More Information.■How can you get comprehensive care if you liveoutside a big city?If your family lives in a small town, a rural area or remote part ofCanada, there are three ways to get comprehensive care:• Regular check-ups – It is very important for you and your childto visit a HTC once or twice a year for a full check-up. Duringthese visits, your child will see the doctor, nurse coordinator andphysiotherapist, and have blood tests done. In some cases, thesocial worker may meet with you. The care team will work withyou to develop a plan for your child’s day-to-day care.3-16All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor Hemophilia“Our clinicsgave the guys achance to get toknow other kidswith hemophiliaand gavethe parents aninformal way toshare ideas,concerns andinformation.”• Day-to-day care – Your local health professionals can help youwith day-to-day concerns related to hemophilia. Thecomprehensive care team will gladly work with your familydoctor and local hospital staff to help them understandhemophilia and the correct treatment for your child. You maywant to leave a copy of your child’s care plan at the hospital inyour area. In many cases, treatments such as the infusion ofclotting factor concentrates or another treatment product calleddesmopressin will be given in the ER of your local hospital. TheHTC doctor or nurse coordinator can contact the ER staff tomake sure they know the correct treatment and dose for yourchild’s bleeding episodes.Your care team may be able to set up physiotherapy treatmentclose to your home so that you won’t have to make as manylong trips to the city.• Special travelling clinics – Your child may also be able to attend atravelling hemophilia clinic. These clinics are usually staffed by adoctor, nurse coordinator and physiotherapist. Travelling clinicsare often set up to go to more remote places that do not have anHTC but where there are several people with hemophilia. Thepurpose is to reduce the travel time for people with hemophiliaand their family members. Sometimes the comprehensive careteam holds these travelling clinics at local hospitals, where theycan also teach the ER staff and doctors how to treat hemophilia.All AboutHemophiliaAGuideforFamiliesLondon Comprehensive Care Clinic3-17

3Comprehensive Carefor Hemophilia■What makes comprehensive care a success?Success depends on many things and many people. It is veryimportant that you, your child and the comprehensive care teamhave strong, open lines of communication. Parents should feelcomfortable enough with the comprehensive care team to be ableto pick up the phone and call any time with a question orconcern. As your child gets older, he should feel comfortablecalling the nurse coordinator or physiotherapist when he has aquestion. Growing together as a team will increase everyone’sconfidence and comfort level. Knowing that when you call theHTC team, you won’t have to start at “the very beginning” makesit easier to solve problems as they come up.There may be times when waiting a long time at the ER will makeyou upset with the system. Call your comprehensive care team totalk about what you went through and how you feel. By workingtogether, you may be able to set up new ways to get your child thetreatment that is needed.“We need CHSto be a strongadvocate, workingwith centresregarding standardsof care, that is, withmedical staffing andprograms. We needto work together tocontinue to advocatefor the safety of theblood system.”As you and your child work with the members of thecomprehensive care team, you will gain a lot from theirknowledge and experience. If you wish, your care team can putyou in touch with other families who also have a child withhemophilia. Families can be a great help to each other by sharingwhat they have learned and experienced.3-18All AboutHemophiliaAGuideforFamilies

3Comprehensive Carefor Hemophilia“As you learn moreyou feel moreempowered. Wejoined the Societyand met otherfamilies at socialevents. We neededthem so much, tolearn how tomanage differentbleeds. Our nursegave us so muchperspective. Sheexplained whatmuscle or joint washaving the problem,how common itwas, how long itwould take toresolve and how wecould help our sonat home.”Success also depends on respect and being responsible. To get themost from comprehensive care, a family must try to follow thecare team’s recommendations. This means following your child’streatment plan, and possibly making changes in lifestyle or thesports he plays. It’s very important to keep good records of bleedsand clotting factor infusions. These records show whether or notyour child’s treatment plan is working. Adjustments can be madeif needed.The comprehensive care team is large but works closely together.The same goal is shared by everyone — a child that despitehemophilia is healthy and active. This is the best way to measuresuccess!All AboutHemophiliaAGuideforFamilies3-19