Supported by Home care: The road to independence Part 1

PURPOSE H OME CARE : T HE ROAD TO INDEPENDENCE, describes how home care can empower you to take control of a bleeding disorder. Taking such a road requires clear directions and signposts along the way. It is a road that can be safely navigated … with the help of guides on your journey—the members of the comprehensive care team at the hemophilia treatment centre (HTC).

1.BENEFITS and RISKS of home care 2.TYPES of home care 3.STARTING on a home care program 4.ASSESSING BLEEDS 5.TREATING BLEEDS OVERVIEW

BENEFITS OF HOME CARE HOME CARE … BENEFITS …  treats bleeds more quickly;  avoids delays in travelling to the hospital and waiting for treatment;  makes it easier to follow a prophylactic treatment program;  helps the family gain confidence in caring for a child with a bleeding disorder and a greater feeling of control over their lives;  allows the person and his family to be more independent of the health care system;

BENEFITS OF HOME CARE HOME CARE … BENEFITS  reduces the number of in-hospital stays and outpatient visits;  reduces disruption to the family's daily activities;  fosters a strong partnership between the family and the comprehensive care team;  allows the person and his family to participate in normal social, school and work activities;  makes travel easier and safer.

SEVERAL PROBLEMS MAY OCCUR WHEN A PERSON IS ON A HOME CARE PROGRAM : RISKS … RISKS OF HOME CARE  a bleed not being treated properly;  poor response to treatment;  mistaking another health problem for a bleed;  improper storage of clotting factor concentrates and other medications;  infection or damage to veins or the venous access device;  injury to other family members.

RISKS RISKS OF HOME CARE WAYS TO REDUCE THE RISKS OF A HOME CARE PROGRAM :  Communicate immediately with the comprehensive care team when… − the person has an injury to a critical part of the body (head, neck, throat, chest, stomach, eye) − bleeding is heavy − a bleed does not respond to standard treatment − one joint has several bleeds in a short time − the person has an allergic reaction − you are not sure what to do  Be well informed.  Communicate frequently with the comprehensive care team to discuss general concerns around managing bleeds and home care.  Keep accurate home care records of bleeds and treatments.  Regularly review home treatment procedures with the comprehensive care team.

TYPES … TYPES OF HOME CARE THERE ARE TWO APPROACHES TO HOME INFUSION :  On-demand therapy - The infusion is given as soon as the patient or his caregiver is aware of a bleed. The goal is to promptly stop the bleed.  Prophylaxis therapy - The patient receives regular infusions of clotting factor concentrates, usually one, two or three times a week. This is done in order to prevent bleeding episodes from happening. on-demand prophylaxis

THERE ARE THREE TYPES OF PROPHYLAXIS : TYPES OF HOME CARE 1) Long-term prophylaxis – the most common type of prophylaxis. Infusions of factor concentrates are given over a period of many years. 2) Short-term prophylaxis – often used to halt the development of a “target joint”. Infusions are given over a period of 3 to 6 months. Also used after surgery. 3) Occasional prophylaxis – infusions are given prior to participating in an activity that may have a high risk for causing bleeding. TYPES …

HOME TREATMENTS WITH DESMOPRESSIN OR CYKLOKAPRON ® TYPES TYPES OF HOME CARE Some people with bleeding disorders are able to treat their conditions with a drug called desmopressin. These include people with…  some types of von Willebrand disease  some types of platelet function disorders  mild or moderate factor VIII deficiency (hemophilia A). DESMOPRESSIN | A G UIDE FOR P ATIENTS AND THEIR C AREGIVERS CYKLOKAPRON | A G UIDE FOR P ATIENTS AND THEIR C AREGIVERS available online at Desmopressin can be infused intravenously (in a hospital only), injected subcutaneously or inhaled by nasal spray. An antifibrinolytic called Cyklokapron ® can also be administered at home. It is taken by mouth.

STARTING PROGRAM … STARTING ON A HOME CARE PROGRAM  There is no standard age for starting.  Ideally, a home infusion program begins before the child starts school.  Parents are responsible for a child's home care therapy.  Small tasks suitable for the child’s age encourage him to learn about his care.  The child who has been involved with treatment at home learns the steps and becomes more responsible as he gets older.  It is never too late to learn. A person who has chosen not to treat himself or his child may one day decide to do so. THE COMPREHENSIVE CARE TEAM AND THE FAMILY WILL MAKE A JOINT DECISION ABOUT WHEN IT IS BEST FOR THE CHILD TO BEGIN A HOME CARE PROGRAM

STARTING PROGRAM STARTING ON A HOME CARE PROGRAM  describe the bleeding disorder accurately;  recognize and describe the symptoms of different types of bleeds, and the best ways to treat them;  prepare and infuse factor concentrate safely;  store factor concentrate safely;  dispose of used equipment safely;  recognize and describe when to contact the HTC to discuss bleeding episodes;  keep a regular routine of attending clinics at the HTC;  keep accurate treament records and submit them at agreed intervals. BEFORE STARTING A HOME CARE PROGRAM, THE PATIENT OR HIS CAREGIVER MUST SHOW THAT HE CAN …

ASSESSING BLEEDS  Always be in close contact with your comprehensive care team.  When in doubt, treat.  Learn locations of the body where bleeds can be serious or life- threatening … head, neck and throat, chest, abdomen, and eye.  Learn what to look for and what to do.  Learn when to go to the HTC or emergency department for help.  Any bleed associated with a large blood loss is also considered serious.  Any bleed that can result in permanent loss of movement (in the forearm, calf muscle, iliopsoas muscle) is considered serious and limb-threatening. LEARN TO ASSESS. KNOW YOUR LIMITS.

TREATING BLEEDS 1. Treat at the first sign of bleeding, even if the person is on prophylaxis! 2. If unsure, contact your HTC or go to the nearest emergency department. 3. Use rest and elevation along with factor concentrate or desmopressin. 4. Use the right amount of clotting factor concentrate for the type of bleed. 5. Always use the complete vial of factor concentrate unless HTC advises otherwise. 6. Never infuse alone. Make sure someone else is there. 7. If the factor concentrate will not dissolve, if the liquid is not clear, or you have doubts about it for any reason, do not use it. 8. Keep accurate home treatment records. 9. Keep your skills up to date with reviews at the HTC. 10. Remember! When in doubt, infuse. 10 IMPORTANT POINTS TO REMEMBER

ACKNOWLEDGEMENTS The CHS would like to acknowledge those people who contributed to the development of Home care: The road to independence. C LAUDINE A MESSE, RN CHU Sainte-Justine, Montreal, QC D ORINE B ELLIVEAU, RN Horizon Health Network, Moncton, NB H EATHER C ARLSON Member, Hemophilia Ontario, Toronto, ON C LARE C ECCHINI National Program Manager, Canadian Hemophilia Society (retired) C HRISTINE K EILBACK Executive Director, CHS Manitoba Chapter, Winnipeg, MB D AVID P AGE National Executive Director, Canadian Hemophilia Society N ORA S CHWETZ, RN Nurse Coordinator, Health Sciences Centre, Winnipeg, MB (retired) P ETER W ILSON Member, CHS National Program Committee, Halifax, NS

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