Executive Director, CHS Manitoba Chapter, Winnipeg, Manitoba
Learning to ride a bike is a rite of passage for most kids. But when you grow up with hemophilia and an inhibitor, you can miss out on some of the things that regular kids get to do; even some of the things that regular kids with severe hemophilia get to do. Sometimes a child with an inhibitor gets to try something, but circumstances can present obstacles. This past summer our 16-year-old son, Eric, learned to ride a bike.
Eric wasn’t diagnosed with severe hemophilia A until he was 14 months old. I am a spontaneous carrier and the diagnosis came as a surprise. Within the first few treatments he developed a high titre inhibitor. Just as we were adjusting to life with hemophilia we had to make another adjustment involving admissions to hospital, surgeries for central lines and port-acaths, and the demands of immune tolerance induction therapy. Bleeding episodes were challenging to control.
Families who are managing inhibitors will tell you that it can be pretty overwhelming! Our family felt so lucky to have the support of the Manitoba Bleeding Disorders Program and our new family, the members of the Canadian Hemophilia Society's Manitoba Chapter. The support we received expanded exponentially when we attended out first CHS Inhibitor Family Weekend in Montreal in 2000. We were pretty isolated in Manitoba as we were the only family at the time dealing with an inhibitor. Having the opportunity to meet other families was such a blessing. The educational opportunity and the chance to have a consult with Dr. Georges-Étienne Rivard was such a boost to our resolve to fight this.
As time went on and we were unable to convince Eric's immune system to accept factor VIII concentrate, we did experience some deep disappointment. At one point during the first round of immune tolerance therapy, we asked our hemophilia treatment centre (HTC) to reduce the frequency of inhibitor screens because of the emotional turmoil it caused us.
It was better for our family to focus on meeting the daily challenges the inhibitor presented us. We decided to focus our energy on acceptance and adaptation. We wanted to teach Eric that it was important to live his best life and not dwell on what we couldn’t control.
We still had hope though! My husband Shane and I read everything and spoke to everyone we could about hemophilia and inhibitors. We heard of a novel way to approach inhibitors at the 2006 World Congress of Hemophilia and our HTC supported our request to try immune tolerance induction using a von Willebrand medication. Unfortunately, Eric experienced a difficult knee joint bleed and we had trouble meeting the demands of controlling the bleeding using two plasma products and factor VIIa at the same time. We regretfully discontinued this round of immune tolerance therapy.
Just as Eric was about to enter high school, our HTC proposed to us a new immune tolerance option. We could try again with a different von Willebrand medication. The decision to take on immune tolerance again was completely up to Eric. We made it clear to Eric that it was at least an 18-month commitment to have a treatment once a day. He had been doing venous access for quite some time so we wouldn’t have the convenience of a port-a-cath.
Eric decided that as long as we would help him get his treatments done, he would do it! Having been to the most recent Inhibitor Family Weekend, Eric had the chance to meet two wonderful young adults who had overcome their inhibitors as teens. This time, within a couple of months of starting immune tolerance, we noticed some significant and positive changes in his bleeding patterns and even in how his body behaved when we removed the butterfly needle after treatments. Now we were clamoring for an inhibitor screen! Eric was inhibitor-free for just over a year. We did experience a recurrence of his inhibitor in the late fall of 2014. The titer was very low and we were able to overwhelm the inhibitor again, in just a few months.
Back to the bike! Eric did have a tricycle as a small child. He did ride a two-wheel bike for a short period of time. He might not have graduated from training wheels. I don’t recall. The frequent bleeding episodes made his experience riding a bike pretty short. Every spring, Shane and I would consider buying Eric a bike but then, sure enough, he’d have a bleed and getting a bike would fall by the wayside. To be honest, the idea of Eric riding a bike when he had the inhibitor did cause me a lot of anxiety.
This past summer a very good friend of the family offered Eric a bike. He was so excited! Imagine me holding the bike while my six feet tall, adult-size son is about to try to ride it! “Stop looking at your feet! Hold the handlebars steady! Watch for cars!” It was comical as I ran down the street with him and then … let him go.
The joy and freedom of riding a bike is not a new story in our community. I have heard stories from other young men with hemophilia (with and without inhibitors) who have enjoyed the new experience of riding a bike and what it meant to them. For our family, it represents Eric’s new independence and freedom. Now, he joyfully explores the boundaries of Winnipeg, his growing independence from his parents, and the new possibilities for his life.
The Keilback family in 2010.
- March 2015