I have a defect in one of my genes. My blood is missing a tiny protein, called factor 8. Like other boys with hemophilia, my blood doesn’t clot normally. I don’t bleed a lot from cuts, but I do bleed into joints, especially knees, ankles and elbows. That’s why I can’t play hockey like Mario Lemieux, my hero. I go to school and summer camp like other kids my age, and get lots of exercise. Maybe one day, if a cure is found, I’ll even be able to play rough sports. Am I ever happy I was born in 1993 and not in the 1950s! I receive my factor 8 infusion three times a week to protect me from having bleeds. Two years ago, I learned to inject the factor VIII into one of my veins myself.
- A 13-year-old boy with hemophilia A
Von Willebrand Disease is my constant companion. I have worried about it most of my life, although I never even knew the name of the disease or that I had it until a few years ago. I lived under a cloud of fear. When I was three, my six-year-old brother hemorrhaged to death following a tonsillectomy. The family knew how he’d died, but not why. When giving this family history to doctors, there were two responses. “That was so long ago, it doesn’t matter,” or... “Silence”. This cloud of fear stayed with me as I grew up. What about my children, grandchildren and others close to me? Would the same thing happen to one of them? If so, would the doctors be able to stop the bleeding, or would the unthinkable happen? When my children or grandchildren had their tonsils out, the fear was unbearable. Unfortunately, I passed my fear onto my children. Once I was diagnosed and knew I could get treatment, my fear lifted. I knew help was available.
- A grandmother with von Willebrand Disease
Bleeding and arthritis in my joints are not my only health problems. Like many hemophiliacs, I was infected with hepatitis C from blood products. I contemplated treatment for years, but with school and work, there was just never time. Two and a half years into my job in the video game industry, I decided to begin treatment. I knew that there was a risk that I would miss a lot of time from work, but it was something I knew I had to do. I talked it over with my boss, and he said to go ahead and take as much time as I needed. I finished my 48-week hepatitis C treatment in October 2005, and never missed a single day of work because of it. This was no small feat, as jobs like mine are notorious for long hours. It’s not unusual for us to work 12- to 16-hour days for weeks at a time! I won’t know for sure for a few more months if the treatment was successful, but so far, so good. It was not without side effects, but I do not regret the decision.
- A 25-year-old man with hemophilia and hepatitis C
I’m twelve years old and have suffered from factor XIII deficiency since I was born. I had a lot of bruising, and once I had cerebral hemorrhaging. I also have bleeding into my joints. When I was five, the doctors discovered that I had a factor XIII inhibitor. My body was destroying the factor XIII every time it was injected. At first, I was given big doses of chemotherapy in the hope of getting rid of the inhibitor. Later, it was injections of factor XIII concentrate every day. They hoped my body would adapt to it. At the same time, they tried more than twenty treatments with plasmapheresis and immunoabsorption: all the plasma in my body was passed through a machine to remove the factor XIII antibodies. But none of these things worked. Today, I continue to receive six vials of factor XIII every day. My father and mother do the infusions. I’ve been getting shots every day for three years now. The only sports I can do are swimming and walking. The others are too dangerous for me. One day, when I’m cured, I’ll start horseback riding. It’s something I’ve always wanted to do. I love horses. In 10 years, I think I’ll be cured. I plan to study to become a dentist or doctor. What I would tell people is “never give up.”
- A 12-year-old girl with factor XIII deficiency and an inhibitor
Our six-year-old son, who has a bleeding disorder, was treated in several local hospitals and it was crazy. Too many doctors and too many hospitals. A few years ago we were referred to the Hemophilia Treatment Centre in our province. What a relief! It's wonderful. All the services are in one place. Everybody knows us and they know exactly what to do.
-- The mother of a 6-year-old boy with a bleeding disorder
I’m not so lucky. I have an inhibitor. The factor IX treatments don’t work for me. My body rejects them. I’ve had lots of bleeds in my joints. Bleeds hurt. When I'm in pain, I tend to express it by complaining verbally—to tell the truth, by screaming. My family doesn't like to see me suffer and they do their best to distract me. My mother gives me my clotting factor concentrate and also morphine for the pain if necessary. My sister tries to watch TV with me. My father talks to me about hunting and fishing, which I'm crazy about, and we often look at magazines together. When I'm at home, I find that my cat helps me a lot to manage my pain. I see a huge difference since he became part of our lives.
- A 13-year-old boy with hemophilia B and an inhibitor
My physician told me she never realized how much pain people with hemophilia had until she went to a CHS workshop on pain management. She said she couldn’t believe how well her patients hid the pain.
- A 42-year-old man with hemophilia A
I experience pain daily. It can be mild or severe. It can be relentless. It is sometimes unpredictable. I associate my pain with an imaginary companion I like to call the dragon. This dragon travels with me all day, every day. He makes it his point to remind me when I am doing something destructive by breathing his heat. As I like being active, I would hate to see the trouble I might get into if I could silence this dragon completely. My pain has progressed significantly in the last six years. It has an impact on most aspects of my life. My ability to climb stairs, walk distances (especially on uneven ground), type at the computer, and hammer a nail or open a jar, to mention only some examples, have all been affected. On days when the pain is extreme it can have a negative impact on my mood and in turn my mood can and sometimes does affect those around me.
- A 38-year-old man with hemophilia A
When my husband Colin, who has hemophilia, and I decided to start a family, we both breathed a sigh of relief upon the birth of our first daughter and then, when the second girl was born, we figured we were free and clear of any bleeding issues except for dear ol’ Dad. This was not to be. Three months later we were wearily plodding through a day of colic with Olivia, or so we thought. I figured a warm bath would help, only to discover an egg-sized bruise on the back of Olivia’s hand. As soon as Colin saw her hand, he deflated as if sticking a pin in a balloon: “That’s a bleed. She’s got hemophilia.” It was all overwhelming enough to even a battle-hardened veteran like her father, but to have to pass your baby along to waiting surgeons was heart-wrenching. As it turned out, it was the best decision we ever made! Home infusion eased the load for both Olivia and us her parents, with twice weekly infusion, which she is now old enough to assist with.
- A mother of a 4-year-old girl with severe hemophilia A
Unfortunately, just because a woman seeks treatment for a bleeding disorder doesn’t mean that she’ll get it, even if she’s aware of her carrier status. I had had problems with bleeding since my youth, including nosebleeds, bleeding after tooth extractions, bruising and menorrhagia. After my son’s diagnosis with severe factor VIII deficiency, my factor levels were measured at 70-80% of normal. Because of menorrhagia, I sometimes needed a hormone called DDAVP to be able to continue working or attend family gatherings, but eventually had to have a uterine ablation. A few years later, I injured myself in a fall and realized that I was experiencing the exact symptoms in my joint I had told my son to tell me about— tingling inside, hot and boggy. I called to get an appointment with my hemophilia clinic, but was refused. I was told, “By definition, you cannot be bleeding.
The bleeding continued and I consulted an orthopedic surgeon who said it was normal and would settle down in 8 to 12 weeks. I returned to a job requiring me to work on my feet all day. This provoked more bleeding. This continued for a year. The joint continued to be painful and swollen. After one year, a second orthopedic surgeon told me I had stage-4 arthritis, and would need a knee replacement! I told him of my bleeding history before having arthroscopic surgery. Despite bleeding through dressings afterwards, I was told it was ‘normal’ by the orthopedic resident and the ER staff, who refused to consult hematology.
Six weeks after the procedure, my surgeon showed me pictures from the scope, pointing out the blood in the joint, saying I’d bled so much during surgery that I was given DDAVP. I was not told this at the time. When I called the clinic, I was again told it was not related to low factor level. Clearly they weren’t interested. I am now anxious that if I ever need it, I won’t be able to get proper treatment since the local treatment centre refuses to acknowledge my bleeding problem.
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