My name is Emileigh Van Dusen and I live with mild hemophilia B. I am an addictions and mental health social worker for the Nova Scotia Health Authority. I currently sit on the Board of Directors of the Nova Scotia Chapter of the Canadian Hemophilia Society as the provincial youth representative.

Medical diagnosis: My father, Neil Van Dusen, was a hemophiliac with a factor IX deficiency. Hemophilia was not new to my family but having a daughter who was diagnosed with “low levels” and who was classified as a “symptomatic carrier” was a novelty. I grew up believing the proper way to identify my bleeding disorder was as a “symptomatic carrier” as I was told by medical professionals. I also knew that I had the potential to experience the same types of bleeding symptoms as my father, as well as the added type of bleeding that comes along with menstruation for women. I have a factor IX level of 34 per cent, and I have learned from recent research studies that women living with a factor level of below 50 per cent should be considered to be living with “mild hemophilia.” To understand my relationship with my diagnosis of hemophilia I should explain that this journey has been both very personal and difficult. Growing up I was not only aware of the fact that I lived with a bleeding disorder, like my father, but that my father was unfortunately a tainted blood victim who contracted hepatitis C through receiving treatment for his hemophilia. My relationship with hemophilia as a child was a strained one, to say the least. I would attend clinic visits where I was told to be cautious, be aware of bleeds, and to accept that I was different, and that I needed to live within certain limitations – safe to say the idea of female boxing was off the table – but I played as many other sports as I could. No child, no matter what diagnosis they live with, wants to feel different or limited. With my parents’ support, I learned my own limits and I exercised my right not to tell anyone about my hemophilia unless they absolutely had to know.

I also saw my father’s health begin to deteriorate and worsen due to complications of living with hepatitis C. In my eyes, my hemophilia wasn’t “real” unless I was at the hospital where I required infusions/treatment before procedures and talked about it at check-ups. My dad’s hemophilia was very real to me and it was very scary; the sicker he became, the more bleeding complications he experienced. Before and after my father’s death, I carried around a sense of immense anger and profound grief. My anger was directed at hemophilia in general; to my 13-year-old self, it made sense that hemophilia was to blame for his untimely death. My thought processes went something like this: “If hemophilia didn’t run in my family, then my dad would never have been born with it, meaning he would never have needed treatment for it and he wouldn’t have contracted hepatitis C and ultimately died.” I grieved the loss of my father because he truly was my best friend. He was an amazing family man, an advocate for others, and among many other things he was my mentor who had been guiding me with understanding my own bleeding disorder. After my father passed away I decided I was done with hemophilia, I wasn’t going to talk about it, be involved with it, and if I had children of my own one day I would do everything I could so that they didn’t have hemophilia.

Fast forward to age 16 and I began volunteering at the Nova Scotia Chapter children’s camp, at the very strong encouragement of my older sister, and I have been involved in the chapter ever since. Going to camp and having the opportunity to meet other people with bleeding disorders – who knew about the tainted blood tragedy, had lost loved ones, and who knew what I was going through – made me able to see hemophilia through a completely different lens that I had never thought possible. Slowly but surely I began to accept and, as I like to call it, “take ownership” of my bleeding disorder, an act that has profoundly altered the whole course of my life.

Symptoms/Treatment: Growing up I always had bruises on my arms and legs and I could never really remember where they came from or how I got them. At age 8 I convinced my parents to let me play soccer and I was constantly bruised. I experienced pulled and strained muscles, none of which we could ever be sure was or wasn’t a muscle bleed. My gums always bled from flossing and I would bleed substantially when losing my baby teeth. I first received factor IX concentrate at age 5 when I had my front teeth surgically removed, again around age 7 to have a tumour removed from my ear, and again at age 8 to have the ear surgery repeated. At age 12 I started my period and I was placed on oral contraceptives to combat the heavy and painful bleeding symptoms that would keep me home from school. I also began iron supplements. At age 13 I received factor IX concentrates to have my wisdom teeth removed and I experienced significant bruising of my face. I received factor IX at age 23 when I broke my wrist playing soccer, and again that same year when I had a mole removed.

Daily life: I would say my bleeding disorder has an impact on my daily life in some overt ways as well as discreet ways. The discreet ways are the pain that I experience in my knee, wrist, and during menstruation that only I can feel; as well as recently I have made the decision to switch to a contraceptive method that allows me not to experience a period at all. Similar to my sister, I love to travel and I have become increasingly anxious about travelling abroad and receiving emergency treatment if needed. In the last few years I have started travelling with a supply of tranexamic acid, and last year I took the time to locate and find the nearest HTCs of the countries and places I would be visiting. However, I did experience a very minor head injury while in Indonesia and although I knew where the nearest HTC was located I would not have been able to make it there in sufficient time for treatment if the injury had been serious, a terrifying thought. I try not to let my bleeding disorder affect my daily life in negative ways but I still experience thoughts of anxiety surrounding travel or if I were to be in an accident and be unresponsive, and I often worry about the quality of care I receive. The more overt ways my bleeding disorder impacts my daily life is that I have made a point to share with and educate my co-workers about my hemophilia and inform them that I carry a FactorFirst card in my wallet that would explain my treatment needs in case of an emergency. I am currently awaiting my new medical alert. I have always found a medical alert an interesting point of contact with others and a chance to provide people with information about hemophilia. Additionally, being the youth representative for the Nova Scotia Chapter, I am often thinking about and planning the next youth event or working with other members of the Board of Directors on hemophilia-related projects.

Sports: As mentioned previously, as a youth I had a desire to try all the sports I could. I chalk this up to reverse psychology and being told that I should be careful and limit myself to only certain sports – so naturally out of defiance, I decided to try them all. I played volleyball, basketball, lacrosse, softball, and fell in love with soccer. Soccer has always been my passion and as a youth it was something I dedicated a lot of time to playing competitively. I was always curious about rugby but my parents were firm about me not trying this sport. As I get older I continue to remain physically active and play soccer recreationally, however, something that I have noticed is that when I hurt a muscle or a joint it takes me significantly longer than other women on my team to heal and come back from an injury. I broke my wrist at 23 during a soccer game and my wrist hasn’t felt the same since. I believe this is mostly due to the fact I was delayed in receiving treatment for it because the hematologist on call in the emergency department didn’t deem my bleeding disorder serious enough to require immediate treatment. I live with chronic pain in my right knee and it is easily aggravated from minor exercise. I can’t help but wonder if my slow recovery time and recurring injuries are related to untreated bleeds or my lack of normal factor IX levels.

Accessing care (standard and emergency): I have always been followed and well supported by my HTC at both the children and adult hospitals. Any bleeding issues or needs such as treatment before procedures have been dealt with by my teams professionally and responsibly. However, I have had some undesirable and inequitable treatment experiences in my health care when accessing services outside of my HTC team. An experience that stands out to me is when I arrived at the ER with a broken wrist and I was advised by my HTC nurse, with whom I spoke on the phone, that I should not leave the hospital that evening without an infusion of factor IX. However, the hematologist on call in the ER reviewed my file and deemed my bleeding disorder not significant enough to require immediate treatment and I was told to come back the next day. In that moment my first thought was that if I was a male with hemophilia and a broken wrist, my condition would have been taken more seriously and I would have received the treatment I required. When identifying myself as a woman with hemophilia I am often met with skepticism and my diagnosis is looked upon as irrelevant because of the “carrier status” and the historical belief of men being the only ones to express bleeding symptoms. Additionally, my concerns and the seriousness of my bleeding disorder have been dismissed by other health care professionals and I have been told “you’re just a carrier.” This label of being “just a carrier” has resulted in health professionals delaying my treatment, advising me to forego treatment before procedures, not listening to my concerns when I have voiced them and causing me to experience prolonged bleeding and pain.

Disclosure to friends and partners: As discussed previously, as a youth I purposely chose not to disclose to people that I lived with hemophilia for a magnitude of reasons but the primary one being the fear of being different. My family had always been very open and honest about my father having hemophilia and being a tainted blood victim with us as children, so I became well versed in explaining to my close friends that my dad lived with hemophilia which is a bleeding disorder that runs in our family. In my younger years I would often describe hemophilia as something that affected my father and potentially my future children, rather than acknowledging that it could impact my own life. As I have gotten older I am very open about my hemophilia and I share information and education about bleeding disorders with anyone who asks. I have been involved in a nursing student’s project where I voiced what my experience of living with hemophilia has been like and what I hope future nurses remember about working with people with bleeding disorders. Through my volunteer work with the Nova Scotia Chapter and the CHS, almost all of my family, friends and co-workers are aware that I live with hemophilia and that I am an active volunteer in these communities. Disclosing my diagnosis of hemophilia to my partner was something that came naturally as we have known each other for almost a decade and he was aware that hemophilia was a part of my life. Even though my partner was aware of my hemophilia I still felt some anxiety about explaining how hemophilia could potentially impact our future children if we decide to have kids one day. My fear was around the idea that my partner might want our children to be able to do things that their bleeding disorder might not allow them to do (sports- or career-wise) or that he would decide that he did not want to have children that might have a bleeding disorder. Yet, all of these fears were for nothing as my partner has embraced my hemophilia and the potential of having children with hemophilia with an open mind.

Words describing my bleeding disorder: I choose to identify as a woman living with mild hemophilia B because I feel it is important to highlight that women living with the gene for hemophilia experience bleeding symptoms too and are more than “just carriers” of the disorder. Also, due to the fact that my factor IX levels are 34 per cent, I feel the term “mild hemophilia” better encompasses my treatment needs rather than identifying as a “symptomatic carrier.” My hope for the future is that one day in the medical community we come to a place of understanding where a woman can identify as having hemophilia, whatever the severity, and she receives equitable and proper care for her bleeding disorder. We have lots of work to do but I think it starts with experiences like this, with sharing our stories, getting our voices out there, and demanding better care for women living with bleeding disorders. Thank you for the opportunity for my sister and I to share our stories with you and how hemophilia has affected our lives.

Erin

My name is Erin Van Dusen and I am a carrier of hemophilia B. I am a special education teacher in Nova Scotia. I currently sit on the Board of Directors for the Nova Scotia Chapter of the Canadian Hemophilia Society.

Medical diagnosis: My father was a hemophiliac and therefore I was born an obligate carrier. I grew up with an understanding of my status as a carrier and with the belief that I was clinically unaffected by hemophilia. I believed that my carrier status didn’t affect my personal health but I knew it would be a major factor to consider when deciding whether or not to have children of my own. I was under the belief that the only way my status as a carrier would truly affect my life would be if I gave birth to a male baby. I thought my factor levels were in the high-normal ranges and I didn’t process the idea that I could potentially give birth to a baby girl with bleeding issues. It wasn’t until recent years when I started attending clinic and had my factor IX levels tested that I realized I was below 50 per cent. I then began to think of my medical diagnosis as a little more than “just a carrier.”

Symptoms: As long as I can remember I have bruised very easily. My legs, arms and stomach were often black and blue as a young child. I have two older brothers and I always thought my bruising was linked to roughhousing or road hockey with my siblings. I started playing soccer when I was 12 and I continued to always be bruised. Sometimes I could remember how I got a bruise and other times I had no idea. I also started my period when I was 12 years old. It was awful. I had very heavy and irregular periods with cramping, pain, and blood clots. It wasn’t long after expressing this to my mother that I was placed on oral contraceptives, which helped regulate my periods and reduce the amount of bleeding.

Treatment: I haven’t required much treatment to date. Fortunately, I haven’t had any serious accidents, trauma or surgeries. Oral contraceptives have helped reduce the impact of my heavy periods. My iron is low and it was suggested by my hematologist that I begin taking iron supplements. I started having my bloodwork taken every three months and the supplements weren’t making a significant difference. I requested to start iron infusions. I was recently advised by my family doctor to skip my periods altogether by taking continuous oral contraceptives.

Daily life: I don’t necessarily think about my bleeding disorder on a daily basis. However, I am very aware of my bleeding disorder while I am on my period or if I am participating in an activity that might put me at risk for injury. I am becoming increasingly anxious about travelling and accessing care abroad. I have ordered a medical alert bracelet and I am excited to have this extra precaution to help communicate about my bleeding disorder.

Sports: I play soccer, softball, hike, snowboard, and practice yoga. I haven’t had any serious injuries to date. I have found the worst outcome from sports, particularly soccer, is bruising. I sprained my medial collateral ligament playing soccer and it took about a month and half to heal. I have not felt restricted or limited in regards to sports because of my bleeding disorder.

Accessing care (standard and emergency): I have been visiting my hematology clinic on a yearly basis since I moved back to Nova Scotia. I want to stay connected with the clinic for several reasons. Firstly, I want to get my iron levels increased. I respect and trust the medical team at our hemophilia treatment centre but I worry about a situation arising in which I need to access emergency care. I am confident in the ability of my hematologist and nurse coordinator to properly support me through an emergency but this doesn’t stop me from being anxious about certain situations. For example, my partner and I have been seriously talking about starting a family. I have hesitations and worries about my health and my future babies’ health.

Disclosure to friends and partners: I am very open about the history of hemophilia in my family and how it affects my life. My disclosure to friends began very early in life because my father was a victim of the tainted blood tragedy. He was sick with hepatitis C so I would explain this to my friends by first explaining he was a hemophiliac and then that he contracted a blood-borne virus through no fault of his own. I have progressed to explaining how hemophilia affects me personally, and how it has the potential to affect my future children, either male or female. I believe being involved in my local chapter of the Canadian Hemophilia Society and more recently at a national level has helped me become more communicative about my bleeding disorder.

Words describing my bleeding disorder: I would describe myself as a carrier of hemophilia or a symptomatic carrier of hemophilia. I would explain that I am affected by hemophilia and that just because I am a carrier doesn’t mean I am not clinically affected by this disorder. I do not feel comfortable calling myself a mild hemophiliac because I have not been medically diagnosed as such and through my own inquiries I have found conflicting information regarding factor levels and severity that deem a person a “mild hemophiliac.” I will continue to label myself as a carrier of hemophilia, but this might change in the future.

– August 2018