Every April 17 World Hemophilia Day is recognized worldwide to increase awareness of hemophilia and other inherited bleeding disorders. This is a critical effort since with increased awareness comes better diagnosis and access to care for the millions who remain without treatment.
Hemophilia is a condition in which bleeding is prolonged. Hemophilia is a condition present from birth and is normally inherited; you can’t ‘catch’ hemophilia or pass it onto others.
Now we are going to tell a story of how hemophilia has affected a girl’s life.
Von Willebrand disease has had a big impact on her life. At a fairly young age, she found out that she had a bleeding disorder. It not only resulted in a major lifestyle change, but it also made it necessary for her to learn about the challenges that she would have ahead of her and she asked herself, “How am I going to deal with this?”
She’s 18 years old and a competitive figure skater representing Canada. Her bleeding disorder affects her every single day in life. She trains about 25 hours per week (15 on the ice and 10 off) and one day hope to represent Canada at the Olympic Winter Games. Once her skating career is done, she has aspirations to research better training methods for Canada’s athletes.
Competing in a sport at an elite level requires many hours of training and daily physical stress to the body, resulting in numerous injuries for someone with a bleeding disorder. For a person without a bleeding disorder, injuries such as a sprain or minor impact injury can heal within a week or so and they do not have to be concerned about what is going on inside their body. But for someone like her, a simple sprain can take weeks to months to fully recover and she always has to be concerned with what is happening inside.
Questions like: Could I be bleeding into the joint? Will I need a blood transfusion for blood loss? Do I need to inject myself with DDAVP? Do I need blood products? Should I go to the hospital now or should I wait and see how it goes? These decisions were all previously made by her parents and coaches, but now that she is an adult, she must make those decisions by herself. However, if she has a bleed from a head injury or something of the same severity, She is not in a position to make such decisions, so key people in her life have to be informed as to what to do in those circumstances.
For example, about two years ago she had an injury that could have ended her skating career. It started with a simple sprain to her ankle but within a very short period of time, she had bled severely into her joint. By the time she got to Sick Kids Hospital in Toronto and the decision was made to inject her with DDAVP, infection was setting in. The infection in the blood was very severe and the decision to operate and irrigate the joint had to be made. An operation would get rid of the excess blood and infection, but there was a risk of damage to the tendons. After weeks in the hospital and several IV blood meds, morphine and antibiotics, the infection finally started to respond and they did not need to operate. Months of rehab and medications, along with her great determination, got her back on the ice.
On a day-to-day basis, major problems can arise from the simplest of events. For example, just by bending her arm she can injure blood vessels or tissues and cause major internal bleeds. She has to be aware of any swelling or hot spots around joints and take any bruising very seriously, especially if it is unexplained. Minor cuts and scrapes to people may be minimal, but she almost always ends up in the hospital getting stitches.
On a day-to-day basis, she must make sure that I have supplies of DDAVP at home and at the arena in case she injures herself. Since DDAVP must be stored at a certain temperature and in a fridge, she cannot carry it on her own so she must make sure that she can access it when needed. Wherever she goes, she must have a plan for getting the DDAVP quickly. She has been taught by the Hemophilia Clinic at Sick Kids how to inject herself and she has taught her parents, skating coaches and other key people who will be able to help her in an emergency situation. It can be a tough call as to whether she needs DDAVP or a hospital assessment first.
Along with a bleeding disorder, she has Ehlers Danlos Syndrome. Ehlers Danlos prevents her tissues from connecting properly. This also results in bleeds and severe bruising, as well as tendonitis because it also makes her body so flexible, and that causes rips in the tendons and therefore considerable bleeding.
She has had numerous injuries and daily occurrences that have always led her to the hospital for treatment, but she has learned to cope with and overcome her bleeding disorder.