Adult Bleeding Disorders Program - resources

A comprehensive guide for carriers of hemophilia A or B. Developed and published by the Canadian Hemophilia Society.

What is hemophilia?

What is von Willebrand Disease?

Other Factor Deficiencies

Most procedures can be done safely with adequate planning, treatment and monitoring.

It is important that you notify the Adult Bleeding Disorders Program of any procedures well ahead so that:

• Health professionals looking after you can develop a plan for the procedure to be done as safely as possible.
• Preparations can be made to deal with bleeding problems that may occur during or after the procedure.
• Arrangements can be made for specialized medication administration, if required, after discharge.

Please also note:

• Procedures should be done early in the week and early in the day. Not all facilities are able to conduct specialized blood tests, if required, late in the day or on the weekend. If any complications occur they can be dealt with more easily during a regular workday.
• If sufficient time is not available to arrange safe treatment, your procedure may have to be postponed.
• Most factor replacement products need to be given immediately prior to the procedure (usually within one hour). Blood tests may be needed before and after the procedure to monitor how well the treatment is working.
• Sometimes a test to find out how well the factor replacement or other clotting treatment is working may need to be done before the surgery or procedure is booked.

Surgery and other invasive procedures for people with a bleeding disorder are never without risk, but with careful planning and communication most procedures can be managed safely.

Before you have a procedure:

• Tell your physician, surgeon or dentist that you have a bleeding disorder and give them the contact information for the Adult Bleeding Disorders Program and hematologist provided on your Factor First card.
• Ask your physician, surgeon or dentist what to expect following the procedure:
  • Is there usually much bleeding?
  • How much swelling or bruising is usual, if any?
• Advise the Adult Bleeding Disorders Program and/or hematologist of the date and type of procedure that is being planned. This should be done as early as possible, preferably two weeks or more prior to the procedure.
• Follow the treatment plan set up by the Adult Bleeding Disorders Program and hematologist (for example, pick up and take medication, as prescribed, have blood tests done).

What can you expect from the Adult Bleeding Disorders Program / your Hematologist?

• Communication about your bleeding disorder with other health professionals involved in your care.
• Development of a plan of care specific to your bleeding disorder and the type of procedure you will be having.
• Advice regarding what to do if bleeding problems occur in the days following the procedure.
• If you require home infusions pre and/or post procedure we will set this up for you and a home infusion nurse will contact you to confirm the date and times. If you do not receive confirmation within a week prior to your procedure please notify our office immediately.

• Contact the Adult Bleeding Disorders Program a few days after your procedure or discharge from hospital and let us know how you are doing.
• Follow the advice of the Adult Bleeding Disorders Program and your hematologist regarding what to do if you experience more pain, bleeding, bruising or swelling than you were told was usual, or if any of these symptoms continue to get worse instead of better. If you are concerned and unsure of what to do, go to your nearest emergency department for assistance.
• Use RICE (rest, ice, compression and elevation) as appropriate, until pain and swelling have gone away.
• Contact the Adult Bleeding Disorders Program or your hematologist if you have any concerns.

In case of an emergency or urgent procedure:

• Notify the Adult Bleeding Disorders Program of any procedures as soon as possible
• Let relatives and friends know that they should contact the Adult Bleeding Disorders Program directly, if you are not able to
• Once notified, the Adult Bleeding Disorders Program may provide assistance to the local health care providers as possible in any emergencies.

Guidelines for Emergency Management of Patients with Adult Hemophilia and von Willebrand Disease

Dental procedures can result in significant bleeding episodes if a bleeding disorder is not managed appropriately. By notifying the Adult Bleeding Disorders Program of any dental appointments ahead of time, recommendations can be prepared to minimize bleeding and result in the best outcome. The following booklet provides useful information that you and your dentist might like to review.

You might need a Home Infusion Program referral before or after your procedure. If this is required, a nurse from the Home Infusion Program will come into your home to give your infusion. Or, the nurse will take you to a dental office to give you your infusion. Our office will give you a referral for the Home Infusion Program. A nurse will contact you at least 1-2 weeks before your procedure to confirm the dates and times. Please contact our office if you do not hear from a nurse a week before the procedure.

Home Infusion Program patient care agreement

DDAVP is an effective medication to treat or prevent bleeding in some Mild Hemophilia A (Factor 8) and von Willebrand disease patients and who are responsive. You may read more about DDAVP in this pamphlet.

DDAVP Appointment process would be as follows:

• Come to our office (Room 491) and pick up your lab requisition
• Lab work as baseline
• Return to clinic for subcutaneous injection of DDAVP
• Lab work 1 hour later
• Lab work 4 hours later
• Lab work 24 hours later (next day)

All lab work needs to be done at St. Paul's Hospital (Main Floor, Providence Building)

Between 4 and 4 hour labs and between 4 and 24 hour labs you do not need to wait at St. Paul's Hospital

DDAVP testing

In order to evaluate responsiveness to DDAVP, lab testing can be done before and after a test dose of DDAVP in the clinic. Here is a sample schedule of what happens during a DDAVP test/evaluation (please note all lab work needs to be done at the St. Paul’s Hospital lab):

• Meet with nurse and receive instructions (in clinic or Hemophilia office)
• Go to lab for first blood sample
• Go back to nurse for injection of DDAVP (this is done in the arm or abdomen, with a very small needle, similar to an insulin injection)
• Go back to lab for blood sample 1 hour after DDAVP injection
• Go back to lab for blood sample 4 hours after DDAVP injection (3 hours after previous blood sample)
• In some cases, you are asked to go back to lab for final blood sample the next day, 24 hours after DDAVP injection

Reactions and Side Effects

You will stay with the nurse for about 10-20 minutes to assure you do not have a reaction. Serious reactions are uncommon. Some patients notice a warm flushing feeling in the face and upper body. This is not serious, and passes in minutes or a couple of hours. You may read more about DDAVP in this pamphlet.

Important note about fluid intake during DDAVP testing period:

DDAVP can cause the body to retain fluid. From one hour before your DDAVP injection, and for 24 hours after the injection, make sure you do not drink more than 1.5 liters of all fluids (6 cups). This includes all beverages, and foods that are high liquid content (soup).

Your DDAVP Evaluation Results

Your lab tests are usually ready in about a week. We will create a report that will go to your Family Physician. We will also book a phone or Skype follow-up appointment with you to review the results. You will receive a Factor First card to carry in your wallet that will include treatment recommendations for bleeding, which will include DDAVP information if you are responsive.

Tranexamic Acid is a very useful medication, especially for preventing bleeds in the mouth or gastrointestinal tract. It is also helpful for some women who have very heavy menstrual periods. Do not use this medication if you have blood in your urine. Your Bleeding Disorders Team can talk with you about this medication and if it is appropriate for you. You may read more about tranexamic acid in this pamplet: Tranexamic Acid Pamphlet

See the Hemlibra website.

Travel to Clinic Appointments

We recognize that coming to clinic takes time, and costs money. You may be eligible for some support to offset these costs. 

We recognize that coming to clinic takes time, and costs money.  You may be eligible for some support to offset these costs.  The following information will help you determine what support may be available to you.

The Travel Assistance Program of BC (TAP) may be a source of reimbursement for ferry or flight costs.  If eligible, request a TAP form from the Hemophilia Team.  You may find out more about the TAP program here.

If your round-trip travel to clinic is more than 200 kilometers in total, the BC Chapter of the Hemophilia Society may be a source of reimbursement for some travel costs.  You will require the following to be considered for funding:

• Membership with the BC Chapter of the Hemophilia Society.  Complete and submit the membership form found here. 
• A letter confirming you are registered with the Adult Bleeding Disorders Program of BC.  You may request this letter from the Adult Bleeding DisordersTeam. 
• A funding request form is to be completed and submitted after your appointment.  You may request this form from the Hemophilia Team.

If you have further questions about support to attend your clinic appointment, please contact our office directly 604-806-8855, toll free 866-806-8855 or email.

Travel Within Canada

Find a Canadian Bleeding Disorders Clinic

Travel Outside of Canada

Find an international Bleeding Disorders Clinic

If you are a visitor to Canada, there is a cost associated with receiving factor concentrates or other treatments. Individuals with a bleeding disorder who plan to study, work, or vacation in Canada are advised to carefully check what health care insurance they require and what health care services they are eligible for while in the country. Immigration Canada is the best source of information regarding questions about who can come to Canada to visit, work or study. The following information is general in nature and in no way a formal statement on Canadian Immigration policy.

• If a foreign student has a study permit from Immigration Canada, valid for longer than 6 months, they may be considered “equivalent” to a resident of BC and therefore able to apply for Medical Services Plan (MSP) coverage and benefits.
• If an individual has a work permit, or permit for a working holiday program issued under the Federal Immigration and Refugee Protection Act of Canada, valid for longer than 6 months, they may be considered “equivalent” to a resident of BC and therefore able to apply for MSP coverage and benefits.
• Tourists or visitors to B.C. do not qualify to apply for MSP coverage and benefits

Here are some websites you will want to consult before travelling to Canada:

• Visit Canada
• MSP
• Are you eligible for MSP coverage?