Newsletter

Dawn Goodyear, MD
Southern Alberta Rare Blood and Bleeding Disorders Clinic

The experience of having mild hemophilia is very different from that of having a severe form of the disease. Unless there is a family history, persons with mild hemophilia can go undiagnosed until childhood or even adulthood. This delay is often due to the fact that excessive bleeding generally requires a traumatic injury or surgical intervention to be unmasked in mild hemophiliacs. Unlike severe hemophilia, episodes of spontaneous bleeding are rare in mild hemophilia. 

The difference in bleeding symptoms is due to the amount of Factor VIII found in hemophilia of different severities. Persons with severe hemophilia have the lowest amount of Factor VIII, less than 1%, which places them at risk for bleeding with very little provocation. On the other hand, persons with mild and moderate hemophilia have Factor VIII levels between 5%-40% and 1%-5%, respectively. In this case, even small amounts of Factor VIII can be protective except in the instances where the coagulation system has been challenged, such as in the setting of an injury or medical procedure. However, once bleeding develops whether it occurs in persons with mild, moderate or severe hemophilia, it can have serious, and sometimes fatal, outcomes unless treated quickly and appropriately. Bleeding treated late, even if successful, may have long term complications. 

Because persons with mild hemophilia tend to have less bleeding episodes, they often ‘forget’ that they have hemophilia and may be unaware of the symptoms associated with bleeding. It is for these reasons that people with mild hemophilia can run into serious problems. For example, if a person with mild hemophilia suffers an injury that is not adequately and promptly treated, it can be difficult to determine if the symptoms that occur afterward are due to a serious bleeding event. Prompt examination by a physician is necessary to determine whether additional testing should be done to identify the cause of symptoms and the appropriate management of the injury. In the event of a bleed, delay in treatment could be life-threatening. When untreated, or managed late, even minor bleeding can result in long term complications.

Treatment of an injury can further be expedited if the person is regularly followed at the local hemophilia clinic. Routine assessment at the hemophilia clinic allows the medical team to monitor factor levels, test for the presence of inhibitors, develop a management plan to be used in emergency situations and become familiar with any other medical issues or concerns which may impact treatment. Regular collection of this information ensures optimal care for persons with mild hemophilia when the need arises and helps to prevent the complications associated with untreated bleeding.

In order to get a better understanding of what it is like to live with mild hemophilia, a project will be initiated through the Southern Alberta Rare Blood and Bleeding Disorders Clinic in Calgary to explore the medical needs and perspectives of persons with mild hemophilia. This project will allow people to describe their personal experiences with mild hemophilia and provide an opportunity to make suggestions as to how the medical team can improve the current health care services provided through the clinic. Information will also be collected from members of the hemophilia healthcare team as well as the Canadian Hemophilia Society as they may have different perspectives that complement and add to those of persons with mild hemophilia. All information used will be anonymous. The goal of the project is to use the information collected to develop educational materials specific to people with mild hemophilia as well as to maximize the benefits attainable from the hemophilia clinic. Our clinics are meant to work for you, and we appreciate your support for this project!